Lung Adenocarcinoma With Solitary Metast

Monday, October 31, 2005
Airway I
4:15 PM - 5:45 PM
DISSEMINATED RECURRENT RESPIRATORY PAPILLOMA- common sites, in descending order of frequency. Symptoms of RRP
TOSIS IN AN ADULT MALE WITH SARCOIDOSIS include hoarseness, wheezing, cough, and stridor. Children are often
Dominic J. Valentino III DO* Anne E. O’Donnell MD Georgetown misdiagnosed as having asthma, croup, allergies, or bronchitis because of
University Hospital, Washington, DC the symptomatology. Prompt laryngoscopy or bronchoscopy is the key to
making a diagnosis. Surgical removal is the mainstay of treatment, with the
INTRODUCTION: Recurrent respiratory papillomatosis (RRP) is the goal of maintaining a patent airway. Adjuvant medical therapy includes
most benign laryngeal neoplasm in children, however it is less common in direct intralesional injections of cidofovir subcutaneous interferon, reti-
adults. The causative agent is human papilloma virus (HPV), typically noic acids, photodynamic therapy, and ribavirin. Recurrence is common
subtypes 6 and 11. The incidence of distal tracheal or pulmonary spread and leads to a high morbidity.
is less than 5%-16%, but morbidity can be high. We present a case of CONCLUSION: RRP follows an unpredictable course and can result
disseminated RRP in an adult male with newly diagnosed sarcoidosis. in severe airway compromise. The management is primarily surgical, but
CASE PRESENTATION: A 54-year old diabetic male presented from an direct and systemic medical therapies are adjunctive. To our knowledge,
outside hospital, after unsuccessful treatment for a diffuse painful lower this is the first reported case of simultaneous RRP and sarcoidosis in an
extremity rash. Routine chest radiograph (FIGURE 1) showed bilateral hilar adult.
adenopathy. Computed tomography scan of the chest demonstrated diffuse REFERENCES:
fine nodule parenchymal infiltrates bilaterally with marked hilar and subcari- 1 CS Derkay. Recurrent respiratory papillomatosis. The Laryngo-
nal lymphadenopathy. The patient had no fevers, dyspnea, or weight loss. He scope 2001:111:57-69.
reported an occasional dry cough. He has tongue “polyps” that have to be 2 KJ Syrjänen. HPV infections in benign and malignant sinonasal
removed every few years. He was diagnosed with obstructive sleep apnea lesions. J Clin Path 2003:56:174-181.
about 10 years ago, but has refused to wear the prescribed CPAP at night. He 3 DA Silverman, MJ Pitman. Current diagnostic and management
had been on a 2 week course of intravenous antibiotics with piperacillin/ trends for recurrent respiratory papillomatosis. Current Opinions in
tazobactam for presumed cellulitis. A workup for vasculitis was negative. Two Otolaryngology and Head and Neck Surgery 2004:121:532-537.
days after arriving at our hospital, he developed the same diffuse erythema- DISCLOSURE: Dominic Valentino III, None.
tous painful rash over his elbows. On physical examination, in addition to the
rashes, we noted papillomatous lesions on the tongue and verrucae on his
hands. No other masses or lymphadenopathy were present. Lungs sounds ENDOBRONCHIAL METASTATIC DISEASE MASQUERADING
were unremarkable. Skin biopsy and bronchoscopy for transbronchial needle AS BRONCHIAL ASTHMA
aspiration (TBNA) of hilar lymph nodes were performed. During bronchos- Karthikeyan Kanagarajan MD* K. Perumalsamy MD V. Rupanagudi MD
copy, a large grape-like lesion was encountered 5cm distal to the vocal cords G. Gandev MD P. Krishnan MD S. Dhar MD Coney Island Hospital,
(FIGURE 2). Additionally, we visualized similar, but smaller mucosal lesions Brooklyn, NY
distally (FIGURE 3). Endobronchial biopsies revealed squamous metaplasia
and inflammation, consistent with RRP. There was no histological evidence of INTRODUCTION: Endobronchial metastasis (EBM) from non
sarcoidosis in the endobronchial tissue. TBNA was also non-diagnostic, so pulmonary tumors is uncommon. EBM mimicking bronchial asthma is
mediastinoscopy was performed and confirmed sarcoidosis with the presence rare. We report a patient with prior colon cancer presenting with
of diffuse mediastinal non-caseating epithelioid granulomas. Skin biopsy also symptoms of bronchial asthma unresponsive to treatment, who was
revealed granulomatous inflammation, consistent with cutaneous sarcoidosis. found to have EBM. Her symptoms resolved when EBM responded to
He was started on naproxen for the cutaneous symptoms and had a good radio and chemotherapy.
response to treatment within four weeks. CASE PRESENTATION: 76 year old non smoking woman without
atopy or asthma presented with cough, dyspnea and wheezing of 3
months duration unresponsive to bronchodilators and systemic ste-
roids. Examination revealed wheezing over right chest. Chest radio-
graph was normal. Her FEV1 was 67% predicted(p) and FEV1/FVC
ratio of 69%p without any bronchodilator response and a normal flow
volume loop. 5 years ago she underwent right hemicolectomy followed
by adjuvant chemotherapy for adenocarcinoma of colon Duke Stage 3.
Chest CT and bronchoscopy revealed an endobronchial mass in right
bronchus intermedius (figure 1) and a 3mm submucosal nodule on the
carina. Biopsy of both lesions showed metastatic colonic adenocarci-
noma. Bronchodilators and steroid were discontinued and with exter-
nal beam radiotherapy and adjuvant chemotherapy her symptoms
resolved and no endobronchial tumor was seen on repeat bronchos-
copy 2 months later (figure 2). Repeat spirometry was normal. She
remains asymptomatic 1 year after her presentation.
DISCUSSIONS: Symptoms and signs of airway obstruction may be
due to causes other than bronchial asthma. Keys to accurate diagnosis
include a consistent history and physical findings of airway obstruction,
demonstration of reversible airway disease by spirometry and a favorable
response to bronchodilator and steroid treatment. Absence of these key
features should prompt further evaluation for other disorders that mimic
asthma. Our patient’s past history of colon cancer, localized wheezing, lack
of response to asthma treatment and a normal chest radiograph prompted
further evaluation for endobronchial disease with chest CT and bronchos-
copy. EBM was defined in a recent study as bronchoscopically visible non
pulmonary tumors, metastatic to the sub segmental or more proximal
central bronchus and with lesions histologically identical to primary
tumors previously documented. The incidence of EBM from non pulmo-
nary tumors is estimated to be approximately 2%, but is probably
underestimated as endobronchial lesions are seen on bronchoscopy in
patient’s with metastatic lung disease in as high as 28-42%. The tumors
causing EBM includes breast, colorectal, renal, ovarian, thyroid, uterine,
DISCUSSIONS: While HPV has been defined as the etiologic agent, testicular, nasopharynx, prostate and adrenal carcinomas; sarcomas; mel-
the true epidemiology of RRP is poorly understood, and management of anomas and plasmacytomas. The predominant primaries are breast,
the lesions is problematic. Rarely, RRP can transform into squamous cell colorectal and renal carcinomas. The presenting manifestations of EBM
carcinoma. In adults, the typical age at diagnosis is 20 to 40 and the adult are similar to that of centrally located primary bronchogenic carcinoma
form generally is more indolent. Extralaryngeal spread is reported in 16% with cough, hemoptysis, atelectasis and post obstructive pneumonia
of adult cases, with oral cavity, trachea, and bronchi being the most though in 50-60% the patients are asymptomatic. Dyspnea and wheezing
408S CHEST 2005—Case Reports

Airway I, continued
are far less common. Chest radiographic findings may be normal or show CONCLUSION: PDT followed by mitomycin is a reasonable alterna-
atelectasis, nodules, hilar mass and mediastinal adenopathy. Bronchos- tive to restore and maintain airway patency in this clinical setting of
copy is diagnostic in these centrally located lesions while chest CT is nonmalignant hypervascularized endobronchial overgrowth.
sensitive in detecting and localizing the lesions. The mean time for the DISCLOSURE: Gabor Varju, None.
appearance of the EBM can be as long as 5 years (as in our patient) after
the diagnosis of primary tumor. Therefore EBM should still be considered
in a patient with unexplained respiratory symptoms even though the BRONCHOMEDIASTINAL FISTULA CAUSED BY IMPLANT-
primary tumor is not recent.Therapeutic approach to EBM is generally ABLE CARDIOVERTER DEFIBRILLATOR EPICARDIAL
radiation and chemotherapy as most patients have extrapulmonary metas- PATCH ELECTRODE
tases. In some individuals with good performance status, where an EBM James A. Driscoll MD* Kaharu C. Sumino MD Washington University
represents the sole metastatic site, surgical resection is a viable option. School of Medicine, St Louis, MO
Palliative endobronchial therapies include cryotherapy, brachytherapy,
Nd-YAG laser or mechanical resection and stent placement. Mean and INTRODUCTION: The implantable cardioverter defibrillator (ICD)
median survival of patients with EBM reported is 9 and 15.5 months was approved for general use in the United States in 1985 1. Although
respectively. most current devices employ transvenously placed endocardial leads,
CONCLUSION: Though uncommon EBM can masquerade as bron- earlier devices utilized epicardial electrodes placed by thoracotomy. We
chial asthma. In patients with prior history of malignancy, presenting with describe a case of a bronchomediastinal fistula caused by an epicardial
asthmatic symptoms unresponsive to asthma therapy, EBM should be ICD patch electrode.
considered even if the chest radiograph is normal. CASE PRESENTATION: In March 2005, a 63 year old male pre-
sented with hemoptysis of approximately 100 mL of fresh blood while
taking warfarin and aspirin. His past history included coronary artery
disease requiring coronary artery bypass grafting, congestive heart failure
and ventricular arrhythmias. An ICD with epicardial leads was placed in
1987 and revised several times subsequently. In 1999 he received a new
ICD with transvenous endocardial leads. The epicardial electrodes from
the earlier device were not removed. Physical examination revealed a
temperature of 38.2 C°, an irregularly irregular heart rhythm and
diminished breath sounds at the left lung base. His International Normal-
ized Ratio was 2.34. A chest radiograph showed numerous mediastinal
wires and a lingular infiltrate that was not seen on a prior radiograph from
October 2003. Anticoagulation was discontinued, and antibiotic therapy
was initiated. A CT scan of the chest showed an air collection in the left
mediastinum and multiple mediastinal wires (figure 1). Fiberoptic bron-
choscopy revealed a pair of wires eroding into the medial wall of the
lingular bronchus with an associated bronchial defect that opened and
closed dynamically with breathing (figure 2). The patient was referred for
surgery. A thoracotomy revealed an epicardial patch electrode eroding
into the lingular bronchus. The left upper lobe was resected but the
offending electrode could not be removed from the epicardium because
of extensive fibrosis. He tolerated the surgery well but suffered a
cardiopulmonary arrest of unclear etiology on post-operative day 7. His
neurological status did not recover, and ventilatory support was withdrawn
DISCLOSURE: Karthikeyan Kanagarajan, None. 8 days later.
PHOTODYNAMIC THERAPY (PDT) FOR GRANULATION TIS-

SUE DESTRUCTION 10 YEARS AFTER STENT PLACEMENT
Gabor T. Varju MD* Cynthia D. Brown MD Ron Allison MD Rosa
Cuenca MD Claudio Sibata PhD Jon Moran MD Gordon Downie MD
East Carolina University, Pitt County Memorial Hospital, Greenville, NC
CASE REPORTS
INTRODUCTION: Airway stent placement for palliation of malignant
airway obstruction symptoms is an accepted intervention in the care of
lung cancer patients. A common complication of stent placement is
granulation tissue reaction that can occlude stents. Removal of stents for
this indication can require rigid bronchoscopy and hours of manipulation
with ablative technologies. PDT is currently used as part of multimodality
therapy for airway management in lung cancer.
CASE PRESENTATION: A 68 year old African-American female
limited stage small cell lung cancer survivor received a left main stem
wall-stent followed by chemotherapy and radiation in 1995. She presented
nine years later with hemoptysis and 3 years of progressive dyspnea on
exertion. Bronchoscopy revealed 90% stenosis over 4 cm in the left main DISCUSSIONS: Epicardial placement of ICD electrodes via thora-
bronchus with granulation tissue overgrowth. A chest CT scan demon- cotomy has been largely supplanted by transvenous placement of endo-
strated volume loss in the left lung. Biopsy showed no recurrence of cardial leads, which is associated with shorter recovery times and lower
malignancy. The stenosis was treated with PDT with a complete tissue perioperative mortality rates.1 However, in some patients transvenous
response; follow-up CT scan demonstrated increased volume. Mitomycin lead placement is not possible, and many patients received devices prior
C at the stent site was applied to prevent recurrence of granulation tissue. to the development of transvenous methods. Therefore, physicians should
DISCUSSIONS: Nonsurgical lung cancer patients appear to be sur- remain aware of the potential complications of epicardial ICD electrodes.
viving longer in selected cases. Some are experiencing local complications We report a patient who developed a fistula between the lingular
of their aggressive therapies. Symptoms from granulation tissue airway bronchus and the mediastinum many years after an epicardial patch
obstruction can limit the quality of life of these patients. In our case PDT electrode was placed. Two similar cases have been reported. Lick and
was used to evoke destruction with excellent clinical response and no Conti 2 described a patient who developed an infected cavitary lesion from
complications. PDT provided an extensive rapid elimination of granula- an extrapericardial patch electrode that had eroded into the lingular
tion tissue as an outpatient using a flexible bronchoscope. Mitomycin C bronchus. He was treated successfully with surgical removal of the
prevented recurrent granulation tissue formation at 6 months follow up. electrode, closure of the bronchial defect, and antibiotics. Dasgupta et al3
CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 409S

Airway I, continued
described a patient in whom an epicardial patch electrode had migrated incomplete excision. Our patient was not a surgical candidate because of
into the left lower lobe of the lung. The patient was treated successfully tumor size and extent; thus, she was treated with combined chemoradia-
with lobectomy, patch electrode removal, and antibiotics. In cases such as tion. During her ICU stay, several critical issues were encountered:
these, surgical intervention is necessary to prevent chronic infection. unstable airway, intubation through the stent, timing of tracheostomy,
CONCLUSION: Epicardial ICD electrodes may occasionally migrate intrahospital transport, stent migration, excessive secretions, lung col-
and erode into adjacent structures, even many years after initial place- lapse, and administration of chemotherapy in the ICU.
ment. Erosion into a bronchus can cause hemoptysis and chronic infec- CONCLUSION: Although rare, FDC tumors involving the paratra-
tion. Surgical removal of the electrode and closure of the bronchial defect cheal region may cause life-threatening airway obstruction and unique
is indicated when possible. management issues. Intensive care support combined with primary
REFERENCES: chemoradiation can result in a favorable outcome.
1 Gollob MH, Seger JJ. Current Status of the Implantable Cardio- REFERENCE:
verter-Defibrillator. Chest 2001; 119: 1210-21. 1 Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic
2 Lick SD, Conti VR. Automatic Internal Cardioverter-Defibrillator cell sarcoma: clinicopathologic analysis of 17 cases suggesting a
Patch Erosion Into the Upper Airway Presenting as a Cavitary malignant potential higher than currently recognized. Cancer 1997;
Lesion. Chest 1997; 112: 1144-46. 79:294-313.
3 Dasgupta A, Mehta AC, Rice TW et al. Erosion of Implantable
Cardioverter Defibrillator Patch Electrode Into Airways: An Un-
usual Cause of Recurrent Hemoptysis. Chest 1998; 113: 252-54.
DISCLOSURE: James Driscoll, None.
UPPER AIRWAY OBSTRUCTION AND RESPIRATORY FAILURE

CAUSED BY A PARATRACHEAL FOLLICULAR DENDRITIC
CELL TUMOR
Ali Hmidi MD* Louis Voigt MD Stephen M. Pastores MD Diane L.
Carlson MD Neil A. Halpern MD Memorial Sloan-Kettering Cancer
Center, New York, NY
DISCLOSURE: Ali Hmidi, None.
INTRODUCTION: Follicular dendritic cell (FDC) tumor is a rare
neoplasm displaying immunophenotypic and morphological features of
follicular dendritic cells (1). Approximately 70 cases of FDC tumors have
been reported usually in young adults with a 16% mortality rate. FDC
tumors occur mainly in lymph nodes but also in extranodal locations such
as the oral cavity and tonsils, soft palate, hypopharynx, and in the
gastrointestinal tract and liver. FDC tumors are often mistaken for other Cancer Cases I
neoplasms. To our knowledge, we report the first case of FDC tumor
involving the paratracheal region causing airway obstruction and manage-
4:15 PM - 5:45 PM
ment dilemmas in the ICU.
CASE PRESENTATION: A previously healthy 29-year-old woman PRIMARY MEDIASTINAL YOLK SAC TUMOR IN A 45-YEAR-
presented to an outside hospital with a month-long progression of neck OLD MAN
swelling and dyspnea. The dyspnea rapidly progressed to stridor necessi- Salim S. Harianawala MD* Vinay K. Sharma MBBS Alan D. Haber MD
tating orotracheal intubation. CT confirmed a large, lobulated subglottic The Graduate Hospital, Philadelphia, PA
mass (5.1 x 6.0 cm), replacing the right posterolateral wall of the trachea.
There was intraluminal airway involvement and encasement of the right INTRODUCTION: Extragonadal germ cell tumors (EGCT) account
proximal common carotid artery by tumor. Biopsy of the mass revealed a for 10% to 15% of all primary mediastinal tumors. Approximately 12% of
FDC tumor. She was transferred on mechanical ventilation (MV) to our mediastinal EGCT are yolk sac tumors (YST). We report a case of
ICU. On ICU day 6, three expandable covered metal stents (in tandem) mediastinal YST that was initially misdiagnosed as non-small cell lung
were placed in the trachea extending 1 cm from the subglottic region to carcinoma (NSCLC), with detrimental consequences.
2 cm above the carina (Figure 1, arrow 1 shows mass, arrow 2, stent). CASE PRESENTATION: 45-year-old male smoker was diagnosed, by
Postoperatively, she was jet ventilated through a 14-French catheter CT-guided fine needle aspiration (FNA), to have NSCLC invading the
inserted into the proximal tracheal stent. Because of worsening respiratory mediastinum. He was transferred to our institution for further manage-
failure and retained secretions, she was intubated with a 7.5 mm ment two weeks later. Within 24 hours of admission, he developed septic
endotracheal tube on ICU day 8. Subsequently, cancer therapy was shock requiring mechanical ventilation, vasopressors and broad-spectrum
implemented with 5 weekly cycles of Gemcitabine and external beam antibiotics. CT scan of chest revealed a mass in the right upper lobe
radiotherapy. Follow-up CT on ICU day 20 showed significant decrease in invading the mediastinum and encasing the superior vena cava (SVC). The
the size of the tumor. Improved access to the trachea allowed for patient developed SVC syndrome and an SVC stent was placed. Due to
tracheostomy on ICU day 22. Post-tracheostomy, the stent intermittently the rapid progression of the tumor, a repeat CT-guided FNA was
migrated into the right mainstem bronchus with resultant collapse of the performed on day 9 of admission. Repeat scrotal examination was normal,
left lung. Frequent bronchoscopies (14) were necessary for stent reposi- and alpha-fetoprotein and beta-HCG levels were sent. The patient went
tioning and pulmonary toilet. The patient was subsequently liberated from into cardiac arrest the next day and ACLS protocols were unsuccessful.
MV. On ICU day 42, the stents were extracted uneventfully. On ICU day The initial pathology report of the repeat FNA was undifferentiated
45, she was discharged to the ward for physical therapy and ongoing NSCLC. However, additional immunohistochemical stains were per-
chemoradiation. On hospital day 49, she was decannulated. Follow-up formed postmortem when his alpha-fetoprotein level was reported as
neck CT revealed near-complete resolution of the tumor. The patient was 24,305ng/ml. The tumor cells were found to express cytokeratin, alpha-
discharged home and is completing chemotherapy. fetoprotein and placental alkaline phosphatase, but were negative for
DISCUSSIONS: This case demonstrates several unique challenges in CK7, CK20, TTF-1, CD30, EMA and beta-HCG. This immunohisto-
the diagnosis and management of a rare paratracheal FDC tumor. Biopsy chemical profile is consistent with yolk sac tumor.
of the tumor demonstrates a pleomorphic population of large oval to DISCUSSIONS: Probably, the first report of a mediastinal YST was by
spindle-shaped tumor cells against a background of small mature lympho- Teilmann in 1967. With a few exceptions, mediastinal YST appears to be
cytes and plasma cells (Figure 2a). Diagnosis is confirmed by immuno- restricted to males and usually presents in the second or third decades of
histochemistry staining for CD21 and/or CD35 antibodies (Figure 2b) and life. At the time of diagnosis, almost 90% of the patients are symptomatic.
for clusterin. Poor prognostic factors include tumor size ⬎ 6 cm, nuclear The common symptoms include chest pain, shortness of breath, weight
pleomorphism, necrosis, high mitotic rate, intra-abdominal location, and loss, chills and fever, and SVC syndrome. Over 85% of patients have
lack of efficacious adjuvant therapy. Complete surgical resection is the extension of the tumor outside the mediastinum at diagnosis. Conditions
treatment of choice. Neoadjuvant therapy with radiation and chemother- associated with YST include Klinefelter’s syndrome and hematologic
apy is recommended for tumors with poor prognostic factors and for malignancies. The presence of the latter generally indicates a worse

Cancer Cases I, continued
prognosis. The diagnosis of EGCT should be considered in young males who response to surgery alone. Over six years later the patient presented with
present with a mediastinal mass and have elevated levels of alpha-fetoprotein endobronchial metastasis from an intra-abdominal adrenocortical carci-
or beta-HCG. The histology of mediastinal YST displays the same wide noma. There were no abnormalities of the serial hormone assays. Though
spectrum of patterns as seen in the gonads, with the reticular pattern being there has been a case report of endobronchial pheochromocytoma, A
the most common. Immunohistochemical staining helps differentiate YST MEDLINE search in the English language revealed no cases of endo-
from other germ cell tumors. YST usually stains positive for cytokeratin and bronchial involvement from a primary adrenocortical carcinoma.3 The
alpha-fetoprotein, negative for Ki-1 and beta-HCG, and may be positive or patient is currently undergoing chemotherapy.
negative for placental alkaline phosphatase. Mistaking mediastinal EGCT for REFERENCES:
undifferentiated NSCLC has been reported previously1. In this patient, the 1 Kiryu T, Hoshi H, Matsui E, Iwata H, Kokubo M, Shimokawa K,
initial erroneous diagnosis was, in part, due to the small yield from the initial Kawaguchi S. Endotracheal/Endobronchial Metastases. Chest 2001;
FNA resulting in inability to run the usual immunohistochemical markers for 119: 768-775.
NSCLC. In the past, long-term survival was rare in patients with nonsemi- 2 Khan JH, McElhinney DB, Rahman SB, George TI, Clark OH,
nomatous EGCT. Incorporation of cisplatin-based chemotherapy has im- Merrick SH. Pulmonary Metastases of Endocrine Origin. Chest
proved survival in these patients, with reported remission rates of 40% to 50% 1998; 114: 526-534.
in most series, usually irrespective of histologic subtypes. Patients with 3 Sorensen JB. Endobronchial Metastases from Extrapulmonary Solid
residual mediastinal mass but normal serum tumor markers require complete Tumors. Acta Oncologica 2004; 43: 73-79.
surgical resection. Recurrent disease does not respond well to salvage
chemotherapy.
CONCLUSION: When faced with a rapidly progressing mediastinal
mass, a high degree of suspicion is necessary so as not to miss the diagnosis
of EGCT. These tumors carry a better prognosis with chemotherapy than
advanced NSCLC, emphasizing the importance of making this distinction.
Our case highlights the difficulties that may be encountered in establish-
ing the correct diagnosis.
REFERENCE:
1 Richardson RL, Schoumacher RA, Fer MF, et al. The unrecognized
extra gonadal germ cell cancer syndrome. Ann Intern Med 1981;
94:181-6.
DISCLOSURE: Salim Harianawala, None.
PRIMARY ADRENOCORTICAL CARCINOMA WITH ENDO-

BRONCHIAL METASTASIS
Himanshu Desai MD* Neelima Chintapalli MD Jonathan Glass MD
Shawn Milligan MD Louisiana State University Health Sciences Center,
Shreveport, LA
INTRODUCTION: Pulmonary metastases to the adrenal gland are

common. Malignant endocrine tumors infrequently metastasize to the
lungs and treatment options are limited. Primary adrenal tumors with
metastases to the lung are even more uncommon. We present a rare case
of endobronchial metastasis from a primary adrenocortical cancer.
CASE PRESENTATION: A 61 year-old white female with known
adrenocortical carcinoma diagnosed in August 1998 presented to our
pulmonary clinic for abnormal radiographs. The patient initially presented
in August 1998 with signs and symptoms of virilization. She was subse-
quently diagnosed with stage IV adrenocortical carcinoma. She underwent
a surgical resection of the right adrenal gland and right kidney that same
year. She received no further therapy and has been followed with
DHEA-S levels and serial thoraco-abdominal CT scans every 3-5 months.
Serial thoracic CT scans were normal until January 2005 (Figure 1). The
CASE REPORTS
patient was asymptomatic. Physical examination was normal and did not
reveal lymphadenopathy. Patient has a 20 pack year history of smoking
which stopped 6 years ago. Pertinent laboratory include a pre-operative
DHEA-S level of 797 micrograms/deciliter (normal: 60-255␮g/dl), post-
operative levels of 12␮g/dl (8/98), 57␮g/dl (6/01) and 151␮g/dl (1/05)
respectively. Patient underwent a fiberoptic bronchoscopy which revealed DISCLOSURE: Himanshu Desai, None.
a smooth, well rounded endobronchial lesion in the right lower lobe
(Figure 2). This area was washed, brushed and biopsied. The biopsy
results revealed metastatic adrenocortical carcinoma. CAVITATING PULMONARY HODGKIN’S DISEASE
DISCUSSIONS: Adrenal tumors are usually suspected when the Shubhra Ray MD* Stephen R. Karbowitz MD New York Hospital
patient presents with signs and symptoms of excessive cortisol secretion. Queens, Flushing, NY
Often, tumors are found incidentally on abdominal CT scans. When
symptoms are present they include oligomenorrhea, hirsutism, acne, INTRODUCTION: An 18-year-old female presents with 5 months of
purple striae, osteoporosis, and muscle wasting. Though no clear epide- pleuritic pain, cough, hemoptysis, 12 lb weight loss, reduced appetite and
miological data exists, adrenal tumors with metastases to the lung are fever.
uncommon. Furthermore, endobronchial metastases from nonpulmonary CASE PRESENTATION: The cough,initially dry, later was blood
tumors are uncommon.1 CT scans are effective in localizing metastatic streaked. She was of average size. Vitals signs were stable and she was afebrile.
disease to the lung. The diagnosis can be confirmed by serum assays for She reported no exposure to sick contacts or tuberculosis. Physical examina-
excess hormone secretion.Survival in patients with adrenocortical con- tion: rhonchi in right upper lung zone, no lymphadenopathy. Mild finger
fined to the adrenal gland at resection is 30% at 5 years, but the median clubbing noted. She was PPD negative and anergic. Laboratory findings:
survival of patients with metastatic disease is six months.2 Mitomycin is Hemoglobin 11.6 gm%, Hct 35.1, Platelets 402K, WBC 24.1K(Neutrophils
used when metastatic disease is present with mixed results. 85%, Bands 2%), ESR 110 mm/hr, CRP 8.5 mg/dL and ACE level 25 U/L.
CONCLUSION: Thoracic manifestations of adrenocortical carcinoma Chest radiograph demonstrated a large right upper lobe cavitary opacity and
are infrequent and not well documented. We cared for a patient with mediastinal widening. Chest CT showed the consolidation measuring 8.5 cm
metastatic disease at diagnosis in 1998 that initially had a favorable and extensive mediastinal lymphadenopathy. Bronchoscopy and bronchoal-

veolar lavage(BAL) without biopsy were negative for AFB on smear. She was
treated with antibiotics and discharged on isoniazid, rifampin, ethambutol and
pyrazinamide, pending cultures. Four weeks later the symptoms worsened.
Cultures were negative to date. Repeat chest CT showed new patchy
consolidations in the right upper and lower lobes with cavitation. The
mediastinal nodes had enlarged. Repeat bronchoscopy with transbronchial
biopsy revealed Hodgkin’s Lymphoma(HD), confirmed by immunohisto-
chemical markers (CD45, CD15 and CD30). Staging revealed splenomegaly
and positive bone marrow. She underwent chemotherapy and went into
remission.
DISCUSSIONS: The differential diagnosis of multiple cavitating
pulmonary densities includes: developmental, traumatic, thromboem-
bolic,vasculitic and rheumatic diseases; sarcoidosis, silicosis, coal workers
pneumoconiosis, infections(bacterial,tuberculosis, nontuberculous myco-
bacteria, fungal), cystic fibrosis, immunodeficiency, primary neoplasms
and metastatic tumor. The lung in HD is a commonly involved extranodal
site (1), and particularly the nodular sclerosing subtype (2). Secondary
spread via lymphatic or hematogenous routes results in interstitial or mass
lesions. Cavitation occurs in less than 1% of adults, developing initially or
after treatment (3). Explanations for cavitation include central necrosis,
secondary infection with liquefaction, and abscess formation. TNF-␣ may
play a role in cavitating lung lesions in HD(1). Clubbing in a child with
DISCLOSURE: Shubhra Ray, None.
HD is unusual (3). Intrathoracic neoplasms are a major cause of clubbing
and hypertrophic osteoarthropathy in adults but rare in adolescents. This
patient had clubbing, but no clinical or radiographic evidence of osteoar-
thropathy. HD presenting as lung masses in the pediatric population is
uncommon. Cavitation of those masses is even rarer. In a 10 yr retrospec- MASSIVE INTRATHORACIC MALIGNANT PERIPHERAL
tive analysis of 161 pediatric lymphoma patients by Blane et al (4) only NERVE SHEATH TUMOR: WITH TRACHEOBRONCHIAL OB-
12% had HD involving the lung and, among all forms of pulmonary STRUCTION
lymphoma (HD, Non-Hodgkins Lymphoma and post-transplant lympho- Bryan Barnosky DO* Lawrence D. Shulman DO Arunabh Talwar MD
proliferative disorder) only 9% had cavitation. In personal discussion and North Shore University Hospital, Manhasset, NY
review of the series with Dr. Blane, she did not find even a single case of
cavitating HD of the lung. INTRODUCTION: Intrathoracic neurogenic tumors are relatively
rare. We present the case of a patient presenting with progressive dyspnea
CONCLUSION: The rarity of cavitary HD relative to cavitary tuber-
with tracheobronchial obstruction secondary to a massive intrathoracic
culosis in our adolescent population led the initial bronchoscopist to malignant peripheral nerve sheath tumor.
perform BAL only. Despite the negative BAL empirical antibiotic and CASE PRESENTATION: A 23 year old male with no medical history
anti-tubercular therapy was given for four weeks, allowing her condition to presented to the emergency room with progressive dyspnea and worsen-
worsen. We are reporting this rare case of cavitating HD of the lung in an ing cough over the previous four weeks. Initial symptoms included low
adolescent to demonstrate the diagnostic dilemma in our patient popula- grade fever and non-productive cough. The patient also complained of
tion. The delay in diagnosis with empirical treatment in spite of negative intermittent, right-sided chest pain and on further questioning admitted
smears in the context of a known high yield of a BAL in cavitary to recent night sweats and unintentional weight loss. The patient’s vital
tuberculosis emphasises the need for early lung biopsy. signs on presentation were: T 36.3 C, HR 100, RR 26, BP 132/67, and
REFERENCES: SaO2 96% while breathing ambient air. Examination was significant for
1 Hudson MM, Donaldson SS. Hodgkin’s disease. In: Pizzo PA, markedly decreased breath sounds over the right hemithorax and a small
Poplack DG, editors. Principles and practice of pediatric oncology. skin lesion in the region of the right eighth intercostal nerve. Chest x-ray
New York: Lippincott-Raven Publishers; 1997. p 523–543 revealed opacification of the right hemithorax with mediastinal shift to the
2 MacDonald JB. Lung involvement in Hodgkin’s disease. Thorax left. Laboratory evaluation was unremarkable. CT of the thorax revealed
1977; 32:664 – 667. a complex mass, 20 cm in diameter completely occupying the right
3 Multiple cavitating pulmonary nodules and clubbing in a 12-year-old hemithorax with chest wall invasion in the right eighth intercostal region
girl.Pediatr Pulmonol. 2002 Aug; 34(2): 147-9. along with right hemidiaphragm invasion and mediastinal deviation. The
4 Pulmonary involvement in pediatric lymphoma. Maturen KE, Blane patient underwent biopsy of the chest wall lesion. Post-procedure the
CE, Strouse PJ, Fitzgerald JT Pediatr Radiol. 2004 Feb;34(2):120-4 patient remained intubated and required ventilatory support with failure
to wean. Bronchoscopy revealed extrinsic compression of the right main
stem bronchus with significant [90%] extrinsic compression of the proxi-
mal left main stem bronchus. The left mainstem bronchus was opened
with balloon dilation followed by Nintinol stent placement, which then
allowed the patient to be successfully weaned off the ventilator and
extubated. The histopathology was reported as malignant peripheral nerve
sheath tumor. The patient was considered for chemotherapy as resection
of the tumor was deemed not possible.
DISCUSSIONS: Neurogenic tumors arise from embryonic neural
crest cells, which normally constitute ganglia, paraganglionic, and para-
sympathetic systems. Thoracic neurogenic tumors are most commonly
found in either the costovertebral sulcus arising from the sympathetic
chain or one of the rami of an intercostal nerve. These tumors are most
often asymptomatic although infrequently dyspnea, cough, or other
respiratory symptoms may be noted. (1) In adults, the malignancy rate of
neurogenic tumors is less than 10% (and probably only 1 to 2%). (2)
Malignant peripheral nerve sheath tumors (MPNST), sometimes referred
to as malignant schwannomas, neurogenic sarcomas, and neurofibrosar-
comas, are thought to arise de novo or from the transformation of a
plexiform neurofibroma. Accordingly, although MPNST can occur in
individuals in the general population, individuals with neurofibromatosis 1
have a significantly increased risk. (4) Clinically, these tumors are

aggressive, locally invasive, and highly metastatic. (5) A review of the fibrosis and destruction of small vessels or granulomatous inflammation of
literature reveals that such tumors hardly ever reach such large size as in the vessels. The response of PAH to treatment for sarcoidosis is uncertain,
our case. Resection followed by radiotherapy and/or chemotherapy is the in case series, the hemadynamic response to steroid therapy lagged behind
usual mode of treatment. We believe the tumor in this case arose from the the radiographic and PFT improvement, and was not universal. In a small
eighth intercostal nerve. study, patients with severe PAH secondary to sarcoidosis were responsive
CONCLUSION: Although relatively rare, malignant peripheral to vasodilator therapy. PAH in association with sarcoid-like reactions is not
nerve sheath tumors must be included in the differential diagnosis of described and management is unproven.
massive intrathoracic mass. CONCLUSION: This case underscores the association of lymphoma
REFERENCES: and sarcoid-like reactions and the possibility that PAH in these patients
1 Reynolds, M. and Shields T.W. (2005) Benign and Malignant may be underappreciated. It also highlights the importance of a systematic
Neurogenic Tumors of the Mediastinum in Children and Adults. In: evaluation for lymphoproliferative disease in patients with lymphadenop-
Shields, T.W. ed. General Thoracic Surgery. USA: Lippincott athy presumed to be sarcoidosis.
Williams and Wilkins. REFERENCES:
2 Roberts, J.R. and Kaiser, L.R. (1998) Acquired Lesions of the 1 Brincker H. Sarcoid reactions and sarcoidosis in Hodgkin’s disease
Mediastinum: Benign and Malignant. In: Fishman, A.P. ed. Fish- and other malignant lymphomata. Br J Cancer 1972; 26(2):120-123.
man’s Pulmonary Diseases and Disorders; USA: The McGraw-Hill 2 Preston IR, et al. Vasoresponsiveness of sarcoidosis-associated pul-
Companies, Inc. monary hypertension. Chest 2001; 120(3):866-872.
3 Ferner, R. and Gutmann, D. International consensus statement of 3 Gluskowski J, et al. Effects of corticosteroid treatment on pulmo-
malignant peripheral nerve sheath tumors in neurofibromatosis 1. nary haemodynamics in patients with sarcoidosis. Eur Respir J 1990;
Cancer Research 62, 1573-1577, 2002. 3(4):403-407.
4 Cameron, R.B., Loehrer, P.J., and Thomas, C.R. (2005) In Devita
ed. Cancer: Principles and Practice of Oncology. 7th ed. USA:
Lippincott Williams and Wilkins.
DISCLOSURE: Bryan Barnosky, None.
PULMONARY ARTERIAL HYPERTENSION: AN UNUSUAL PRE-

SENTATION OF HODGKIN’S DISEASE
Matthew C. Exline MD* Namita Sood MD The Ohio State University,
Columbus, OH
INTRODUCTION: Pulmonary hypertension is a known complication

of sarcoidosis; however pulmonary hypertension in patients with sarcoid-
like reactions has not been described. We report a case of a patient with
pulmonary arterial hypertension associated with a sarcoid-like reaction
and Hodgkin’s disease.
CASE PRESENTATION: 48 year-old Caucasian female presented
with progressive dyspnea on exertion for three years, markedly worse over
last six months. A CT scan one year prior revealed diffuse mediastinal
lymphadenopathy. A mediastinoscopy with lymph node biopsy showed
granulomas and diagnosis of sarcoid was made at that time. Symptoms
continued to worsen despite treatment with prednisone. Past medical
history was also significant for hypothyroidism and fibromyalgia. She
smoked 40 pack-years. Review of symptoms revealed arthralgias,
Raynaud’s phenomena, night sweats, and a 25-pound weight loss over six
months. Cardiovascular exam revealed a normal S1, S2 with loud pul-
monic component, S3 present. A II/VI systolic murmur appreciated over
left sternal boarder. Pulmonary function testing showed mild restrictive
disease TLC 3.52 (72% predicted) with markedly reduced diffusion
capacity DLCO 6.2 (27%). During six-minute walk she traveled 1033 feet
desaturating to 82% on six L/min oxygen. Computerized tomography
showed enlarged pulmonary vasculature and extensive lymphadenopathy.
CASE REPORTS
Right heart catheterization revealed: pulmonary artery pressure 79/38
mmHG (mean 46 mmHG) cardiac output 6.7 L/min, cardiac index 3.7
L/min/m2. There was no significant response to inhaled nitric oxide at
20ppm. Review of previous lymph node biopsy showed a sarcoid-like–
granulomatous lymphadenitis with foci of Reed Sternberg cells, a diag-
nosis of stage IIB mixed cellularity Hodgkin’s lymphoma was made. She
was initiated on epoprostenol therapy by continuous infusion and under- DISCLOSURE: Matthew Exline, None.
went 6 cycles of vinblastine, chlorambucil, procarbazine, and prednisone.
She is in remission 20 months post chemotherapy without reoccurrence of
lymphadenopathy off steroids. She failed attempts to wean epoprostenol EXTRASKELETAL MYXOID CHONDROSARCOMA PRESENT-
therapy, but displays improved exercise tolerance on epoprostenol, 1355 ING WITH HEMOPTYSIS
feet on six minute walk desaturating to 88% on room air. Repeat right Carlos R. Vassaux MD* Katherine Hendra MPH St. Elizabeth’s Medical
heart catheterization showed: pulmonary artery pressure 48/21 (mean 30 Center, Boston, MA
mmHG), cardiac output 7.8 L/min, cardiac index 4.6 L/min/m2.
DISCUSSIONS: Sarcoid-like reactions are defined as areas of non- INTRODUCTION: Extraskeletal myxoid chondrosarcoma (EMC) is a
caseating granulomas seen on biopsy in patients without symptoms of rare malignant soft tissue tumor derived from mesenchymal chondrocytic
systemic sarcoidosis. Radiographically, patients with sarcoid-like reactions cells. We recently encountered a patient with hemoptysis, who was
may present with hilar or mediastinal adenopathy, ground-glass infiltrates, subsequently diagnosed with primary EMC of lung.
or perivascular nodularity. Sarcoid-like reactions in malignancy may occur CASE PRESENTATION: A 24-year old man was admitted to our
at the primary tumor site, in lymph nodes draining the region, or in distant facility complaining of intermittent hemoptysis for one year, with progres-
organs such as the spleen, liver, or bone marrow in up to 4.4% of patients sion over the previous two weeks. His past medical history and review of
with carcinoma, 7.3% patients with non-Hodgkin’s lymphoma, and 13.8% systems were unremarkable. He was employed in construction, and
patients with Hodgkin’s disease. Pulmonary arterial hypertension (PAH) recently traveled to Cancun. On presentation his physical exam was
develops in up to 28% of patients with sarcoidosis. The etiology of PAH normal. Routine laboratories included a hemoglobin of 9.3 g/dl. Chest CT
in sarcoidosis is generally presumed to be secondary to parenchymal scanning showed a hazy, right lung infiltrate, and mediastinal adenopathy.

Cancer Cases I, continued Cardiovascular Disease
4:15 PM - 5:45 PM
Fiberoptic bronchoscopy was performed revealing thick bluish, non- IATROGENIC AIR EMBOLISM DURING CONTRAST CT OF
purulent mucous plugs in the posterior segment of the right upper lobe. THE THORAX
Microbiology studies, including stains for acid-fast bacillus were negative. Sameh G. Aziz MD* Frank W. Ewald, Jr. MD VA Medical Center
Transbronchial biopsies of the right upper lobe were non-diagnostic.The Augusta and Medical College of Georgia, Augusta, GA
patient improved on antibiotics and was discharged home. However, 11
days later he returned with recurrent bleeding, and a hemoglobin of 6.7 INTRODUCTION: Venous air embolism is a well-known complica-
g/dl. Because of ongoing hemoptysis, a right upper lobe lobectomy was tion during head and neck surgery. Also it commonly occurs during IV
performed. Cytogenetic analysis of the tissue was consistent with an EMC. injection of contrast for radiographic studies and may occur during central
Additional radiographic evaluation, including CT, PET and bone scans venous catheterization. Large-volume venous air embolism may lead to
were negative for a primary location of the malignancy. cardiovascular collapse or to paradoxical arterial emboli with resulting
DISCUSSIONS: Extraskeletal myxoid chondrosarcoma, first described as central nervous system injury. The newer generation computerized
a distinct clinicopathologic entity in 1972, is a rare, intermediate grade, tomography (CT) scanners with multi-slice, rapid acquisition capability
malignant soft tissue tumor. It is more frequent in males than females (2:1) are identifying small-volume air embolism more commonly. Thus, the
and usually presents in middle aged and elderly patients. A series of 117 cases pulmonlogist will be consulted more fequently to evaluate patients for the
found a predilection for the proximal extremities or limb girdles in 80% of significance of this compication.
cases, where it often presents as a palpable mass (1). However, EMC has also CASE PRESENTATION: A 54 year-old man with myasthenia gravis,
been described to present in other areas of the body including the nasophar- had a CT of the thorax with contrast, as an outpatient, for the evaluation
ynx, orbits and chest wall. To our knowledge, this tumor has never been of thymoma. Interpretation of the CT scan, after he had left the radiology
reported to present initially in the lung. The diagnosis of an EMC can usually department, showed evidence of air in the brachiocephalic vein, superior
be made by fine needle aspirate and subsequent cytogenetic analysis, vena cava, right atrium, and right ventricle. The amount of air was
demonstrating the tumor specific t(9;22)(q22;q12) translocation (2). In our estimated to be 4 to 6 milliliters(ml). The patient was contacted at home,
case the cytogenetic analysis revealed this characteristic finding. Treatment of had no symptoms, was asked to return, and on examination had normal
EMC includes excision with wide surgical margins and occasionally adjunct vital signs and a normal neurological and cardiac examination. Oxygen
radiotherapy (1). Chemotherapy does not appear to be of benefit, although saturation via pulse oximeter was 100 % on room air.
there is an isolated report of metastatic pulmonary EMC responding to
interferon alfa-2b. Despite tumor resection there is a high incidence of local
recurrence (48%) and metastatic disease (46%) including to the lung.
Reported survival at 5, 10 and 15 years is 90 %, 70% and 60% respectively (1).
CONCLUSION: We believe this is the first reported case of EMC
presenting as a lung primary. Despite an extensive search for an alternate
primary location, none was identified. Other unusual features include this
patient’s young age and history of hemoptysis. Although rare, EMC should
be considered in those with similar presentations.
REFERENCES:
1 Meis-Kindblom JM, et al. Extraskeletal myxoid chondrosarcoma: a reap-
praisal of its Morphologic spectrum and prognostic factors based on 117
cases. American Journal of Surgical Pathology. 1999 Jun; 23(6): 636-502.
2 Bjerkehagen B, et al. Extraskeletal myxoid chondrosarcoma: multimodal
diagnosis and identification of a new cytogenetic subgroup characterized by
t(9;17)(q22;q11). Virchows Arch. 1999 Nov;435(5):524–30.
DISCUSSIONS: Iatrogenic air embolism requires that two conditions

be present. These are a direct communication between the air and the
vasculature, and a pressure difference favoring air entry rather than
bleeding. Recent studies show the incidence of small and moderate size
air embolism (diameter ⬍20 mm) in patients undergoing CT scan of the
thorax with contrast is as high as 24%, using early generation scanners.
Large volume venous embolism is less common, is usually reported in case
reports, and has not been systematically studied. Symptoms of air
embolism depend on the amount of air injected and the rate of injection-
.The fatal dose in humans is unknown, but in animals injection of 300 to
500 ml of air at rate of 100 ml/sec can cause cardiac arrest. Mild air
embolism may be asymptomatic, but major air embolism can lead to loss
of consciousness, chest pain, shortness of breath, or cerebrovascular
accident. Examination may reveal cyanosis, tachypnea, hypotension, or the
classic mill-wheel heart murmur, which is a splashing sound due to the
DISCLOSURE: Carlos Vassaux, None. presence of gas in the cardiac chambers and great vessels. Lung exam may

Cardiovascular Disease, continued
reveal wheezing or crackles. Exhaled end-tidal carbon dioxide may be MID-CAVITY OBSTRUCTION WITH APICAL LEFT VENTRIC-
reduced. Arterial blood gases may show hypoxemia and the electrocar- ULAR ANEURYSM: AN UNCOMMON FORM OF HYPERTRO-
diogram may show sinus tachycardia, sinus bradycardia, or conduction PHIC CARDIOMYOPATHY
abnormalities. Paradoxical embolization of air may occur through a patent Mazen S. Abu-Fadel, MD Beau Hawkins MD* Pedro Lozano MD
foramen ovale or through the pulmonary microcirculation. Doppler Chittur A. Sivaram MD The University of Oklahoma Health Sciences
ultrasonography and transesophageal echocardiography are both sensitive Center, Oklahoma City, OK
in detecting intra-cardiac air.The potential for developing venous air
embolism is reduced by preventive methods, such as proper positioning of INTRODUCTION: Hypertrophic Cardiomyopathy (HCM) has sev-
the patient during insertion of central lines, and insuring that the IV eral distinct morphological patterns. Asymmetric left ventricular hyper-
tubing is free from air bubbles. Once venous air embolism occurs, the trophy with outflow obstruction, mid-cavity obstruction and apical hyper-
patient should immediately be placed in the left lateral decubitus position trophy are types of morphological variants in HCM. Apical aneurysm
and in Trendelenburg (Durant’s position), which helps move the air formation is an uncommon feature associated with HCM. Apical aneu-
bubble to the right atrium or into the right ventricle away from outflow rysms have been described in the asymmetric septal, apical, and mid-
tract, and administer 100% Oxygen to enhance the diffusion of nitrogen cavity obstruction types of HCM. We describe a patient with HCM
into the blood. Comatose or somnolent patients should be intubated. presenting with mid-cavity obstruction and apical aneurysm, and show
Immediate aspiration through a central venous catheter, already in place, unusual flow patterns within the apical aneurysm detected on Doppler
may be attempted. echocardiography.
CASE PRESENTATION: A 62-year-old male investigated for an

episode of syncope showed non-sustained ventricular tachycardia and
required hospitalization. Past medical history included cerebrovascular
CONCLUSION: Although our patient’s experience with venous air accident and hypertension. Echocardiography revealed severe left ven-
embolus was inconsequential, it served to point out that air embolism is a tricular hypertrophy, hyperdynamic left ventricular (LV) function, mid-
frequent and usually mild event, that the newer generation of computered cavity obliteration of the LV in systole, and a small localized apical
tomography equipment is likely to recognized incidental air embolism aneurysm. Doppler echocardiography showed a mid-cavity gradient of 36
associated with contrast injection more frequent than before, and that mm Hg without any LV outflow tract gradient. In addition, Doppler
early recognition and intervention is necessary when air embolism is echocardiography revealed abnormal flow originating from the apical
severe and life threatening. aneurysm towards mid cavity during the isovolumic relaxation period of
CASE REPORTS
diastole. This resulted as the apex was excluded from the remaining LV
chamber during systole. A pressure gradient then developed between the
aneurysm and LV cavity. Coronary arteriography showed an occluded
right coronary artery and diffuse disease in the left anterior descending
coronary artery. The patient was managed conservatively with beta-
blockade due to his dementia.
DISCUSSIONS: The interesting features in this patient are the
presence of mid-cavity obstruction and an associated apical aneurysm
exhibiting paradoxic diastolic flow. Apical aneurysms occur in HCM
rarely. Pathophysiologic mechanisms remain speculative, but abnormal
myocardial vasculature, septal perforator artery compression, oxygen
supply-demand mismatch, and pressure overload are thought to be
important contributing factors. Patients with apical aneurysms and HCM
have an increased frequency of embolic phenomena and as such should be
considered for anticoagulation.
CONCLUSION: Hypertrophic cardiomyopathy with mid-cavity ob-
struction, apical aneurysm, and paradoxic diastolic flow is a rare entity. It
is best diagnosed with echocardiography. Patients with this condition
should be treated with negative inotropic agents and ICD placement to
prevent SCD. In addition, anticoagulation should be considered to reduce
the risk of embolic events even in the absence of atrial fibrillation.
REFERENCES:
1 Elliott P and McKenna WJ. Lancet 2004;363:1881-91.
2 Eriksson, MJ, Sonnenberg, B, Woo, A, et al. JACC 2002; 39:638.
DISCLOSURE: Sameh Aziz, None. 3 Tengyong J, Zhihong H, Wang J et al. Chinese Medical Journal

2002;115(5):782-4.
4 Nakamura T, Matsubara M, Furukawa K et al. JACC 1992;19(3):51
DISCLOSURE: Beau Hawkins, None.
SUCCESFULL SURGICAL REPAIR OF AN ACCUTE TYPE A

DISSECTING AORTIC ANEURYSM ASSOCIATED WITH A
RIGHT-SIDED-AORTIC ARCH AND RIGHT SIDED DESCEND-
ING THORACIC AORTA
Konstantinos E. Paziouros MD* Stavros Siminelakis MD Sokrates Sis-
manidis MD Leonidas Disnitsas MD Miltiadis Matsagas MD George
Papadopoulos MD George M. Palatianos MD Onassis Cardiac Surgery
Center, Athens, Greece
INTRODUCTION: Acute Type A dissecting Aortic Aneurysm associ-

ated with a right-sided arch and descending Aorta is an extremelly rare
situation. One or two such cases have been reported at all times. The
situation is fatal especially when it is complicated with acute Aortic
Regurgitation and/or myocardial Ischemia.
CASE PRESENTATION: A 64-year-old man with a known history
of Aneurysmal dilation of the descending Thoracic Aorta, presented
with acute retrosternal pain and hemodynamic instability, at a rural
area Medical center. The patient being in cardiogenic shock was
succesfully resuscitated and transported to the University Hospital
where the CT scan revealed an acute Stanford A / DeBakey I Aortic
dissection with a right-arch and descending Aorta (Fig 1). TTE
findings -besides the dissection- were severe Aortic regurgitation,
2⫹/4⫹ Mitral regurgitation, LV hypertrophy and apical and inferior
wall hypokinesia. ECG revealed obvious new-onset Ischemia. The
patient was intubated in the ICU due to hypoxia and hemodynamic
instability. Risk factors included known COPD and acute Renal
Failure due to involvement of the Right Renal artery. The patient was
febrile due to active upper respiratoty Staphylococcal infection as
proven by subsequent culture; nevertheless he was scheduled for
emergent Surgery which was performed with a median Sternotomy,
cannulation of the right subclavian artery and a two staged right Atrial
venous cannula and performance of a Bentall procedure with distal
reinforcement of the Aortic wall with Teflon-felt rings placed inside
the intima and outside the adventitia. Fenestration was also performed
distally in order to equalise pressures between true-false lumens. It is
worth mentioning that intraoperative bleeding was controlled by felt
wrapping of the heart, a technique not known to the new generation of
cardiac surgeons. This is obvious as a white substernal rim in the
postoperative CT scan (Fig 2) and involves the innominate vein, the
superior vena cava, the pericardium next to the pulmonary artery and
the epicardium of the right ventricle all along from the superior vena DISCLOSURE: Konstantinos Paziouros, None.
cava to pulmonary artery. The patient recovered during a long ICU
stay and was finally discharged and went back to normal life. Chest SYNCOPE: A CASE OF A VEIN GRAFT RUPTURE
computed tomography at three months after surgery demonstrated a Jun R. Chiong MD* Prithviraj Rai MD Carmel Montiero MD Sergey
disappearance of the false lumen of the arch and descending Aorta (Fig Malykh MD Alan B. Miller MD University of Florida, Jacksonville, FL
2).
DISCUSSIONS: Acute Type A Aortic dissection associated with a INTRODUCTION: Spontaneous rupture of an aneurysmal bypass
right-sided arch and descending Aorta is an extremelly rare situation and graft is very rare. The time of presentation varies from 2 months to 21
to the best of our knowledge only two such cases have been reported years after the surgery with chest pain as the most common symptom. To
worldwide. This condition is fatal when complicated with acute severe date this is the only reported case that presented with a syncopal episode.
Aortic insufficiency and acute myocardial Ischemia. Surgery is idicated CASE PRESENTATION: This is a case of a 68-year-old man who
unless it is felt to carry a prohibitive risk because of medical debility, presented with syncope and cardiogenic shock. He had a 4 vessel
extensive renal, myocardial,or bowel infarction, or massive stroke. In our coronary bypass 15 years ago. A two dimensional echocardiogram
case we believe that surgery was indicated keeping in mind the heavy risk revealed a large extrinsic mass compressing the left atrium (Fig. 1). A
it carried for an unfavorable outcome. chest computed tomogram (CT) confirmed the findings. In addition ,
CONCLUSION: There seem to be some exceptions to the general the left pulmonary vein is also compromised(Fig. 2). Patient expired
rules of Cardiothoracic surgery concerning Risk stratification and decision during resuscitation. An autopsy was performed and revealed a
ruptured aneurysm of the bypass graft to the right coronary artery (Fig.
making. The unexpected results of prompt and aggressive Surgical
3) leading to hemopericardium with subsequent development of a
treatment of otherwise fatal situations make out for these exceptions. localized tamponade compressing the left atrium and the left pulmo-
REFERENCES: nary vein.
1 Moizumi Y., Komatsu T., Nagaya K., Sawamura Y., Sakurai M., DISCUSSIONS: In this case, the patient’s presentation was very acute
Tabayashi K.Type A aortic dissection involving a right-sided Aortic and his clinical status deteriorated rapidly. The initial working diagnosis
Arch, J. Cardiovasc. Surg (Torino) 1999 Feb; 40(1):117-9 was a metastatic mediastinal mass as he has a remote history colon cancer.
2 Nomoto T, Ueda Y, Sugita T, Izumi C. A case of type A dissection The persistent compression to the left atrium and the left pulmonary vein
involving a right aortic arch. Eur J Cardiothorac Surg. 1997 Dec;12(6): ultimately led to the patient’s death.The mechanism of the aneurysmal
922-4 dilatation of bypass grafts is unclear and the actual incidence is unknown.

The mortality is high as most cases are undiagnosed. Diagnostic modality for the treatment of disseminated fungal infections. Voriconazole may
for early detection includes coronary angiography, computed tomography cause visual and gastrointestinal disturbances but cardiac events are rare
scan, cardiac magnetic resonance imaging and in some cases, trans- and complete heart block (CHB) is exceptional. Caspofungin may cause
esophageal echocardiography. The treatment of choice is surgical resec- phlebitis and liver abnormalities but has not been known to cause any
tion with or with out the application of a new grafts. Trans-catheter cardiac side-effects. We present a case of CHB in a 44-year-old man with
embolization of the aneurysm has also been reported. With recent acute myeloid leukemia (AML) and no history of cardiac disease who
advances in coronary stents, implantation of polytetrafluoroethylene- received caspofungin and voriconazole as treatment for disseminated
covered stents (JOSTENTs) has been investigated. fungal infection. To our knowledge, this is the first case of CHB associated
CONCLUSION: Aneurysm of the saphenous venous graft is a very with the combination of these two agents.
rare finding. Of particular interest was the difficulty in establishing the CASE PRESENTATION: A 44-year-old man with a 10-year history of
correct diagnosis due to its rarity. Furthermore, presentation can be Crohn’s disease was diagnosed with AML. He received induction therapy
atypical, and therefore the diagnosis should be considered in all patients with idarubicin and cytarabine followed by consolidation with cytarabine.
who have had coronary surgery with saphenous vein grafts who present He was treated with empiric antibiotics for neutropenic fever. Computed
with atypical chest pain, superior vena caval obstruction, or mediastinal tomography (CT) revealed microabscesses of the lungs, liver, spleen and
mass. In spite of limited data, early intervention of SVG aneurysm appears kidneys suggestive of disseminated fungal infection. He was treated with
to be beneficial in most patient. voriconazole for one month without any side-effects. A follow-up chest CT
REFERENCES: showed enlarging microabscesses of the liver and spleen. Therefore, he
1 Davey P, Gwilt D, Forfar C. Spontaneous rupture of a saphenous was hospitalized for modified antifungal treatment using a combination of
vein graft. Postgrad Med J. 1999 Jun;75(884):363-4.2. intravenous (IV) voriconazole and caspofungin. The admission electrocar-
2 Toshihiro F, Shigefumi S, Toshihiko S. Aortocoronary saphenous diogram (ECG) revealed normal sinus rhythm (NSR) [Figure 1A]. A week
vein graft aneurysm in redo coronary artery bypass grafting: Jpn after hospital admission, he began complaining of chest discomfort. ECG
J Surg (1998) 28:321-324. revealed CHB (Figure 1B). Cardiac enzymes, serum electrolytes and
3 Kalimi R, Palazzo RS, Graver LM. Giant aneurysm of saphenous renal and hepatic function were normal. Because of the CHB, he was
vein graft to coronary artery compressing the right atrium. Ann transferred to the intensive care unit (ICU) for cardiac monitoring. In the
Thorac Surg. 1999 Oct;68(4):1433-7. ICU, the voriconazole was substituted for IV liposomal amphotericin and
4 Rogers JH, Chang D, Lasala JM. Percutaneous repair of coronary the caspofungin was continued. Transthoracic echocardiogram was nor-
artery bypass graft-related pseudoaneurysms using covered JOS- mal. Serum titers of antibodies against Borrelia burgdorferi, Coxsackie B
TENTs. J Invasive Cardol. 2003 Sep;15(9):533-5. virus, Echovirus, and of anti-nuclear antibodies were negative. Bacterial
and fungal cultures of blood, urine, and sputum were non-contributory.
Over the next several days, the ECG evolved from intermittent CHB to
type I second-degree atrioventricular block, and finally to NSR (Figures
2A-B). The chest discomfort resolved. The microabscesses disappeared on
caspofungin and liposomal amphotericin. He was not re-challenged with
voriconazole. He remained in NSR several months later.
CASE REPORTS
DISCLOSURE: Jun Chiong, None.

DISCUSSIONS: Cardiac side effects have been reported with vori-
COMPLETE HEART BLOCK IN A PATIENT RECEIVING COM- conazole in ⬍ 2.5% of patients in preclinical trials. These cardiac side
BINATION ANTIFUNGAL THERAPY WITH VORICONAZOLE effects have ranged from prolonged QT interval, bradycardia and bundle
AND CASPOFUNGIN: IS THERE A LINK? branch block to supraventricular and ventricular dysrhythmias, and even
Svetolik Djurkovic MD* Louis Voigt MD Eileen McAleer MD Brandi cardiac arrest; CHB was exceptionally rare. The mechanisms of these
Ross-Douglas MD Stephen M. Pastores MD Neil A. Halpern MD dysrhythmias are unclear since these cardiac events were demonstrated in
Memorial Sloan-Kettering Cancer Center, New York, NY severely ill patients with multiple medical problems and also receiving
several other medications. Thus, a direct relationship between the
INTRODUCTION: Voriconazole and caspofungin are two novel arrhythmias and voriconazole could not be firmly established. There have
antifungal agents recently approved by the Food and Drug Administration been no reported cardiac side effects with the use of caspofungin. While

there has been no documented adverse cardiac interaction of voriconazole activities prior to surgery. His chest pain has resolved initially, but persistent
and caspofungin, we hypothesize that the combination of voriconazole and retrosternal sharp pain, somewhat different from his presenting symptoms
caspofungin was responsible for the occurrence of CHB in our patient. He emerged at 6 months follow-up.
developed the CHB only after he was started on combination therapy with DISCUSSIONS: We reported the case of the largest (11 cm x 7cm)
voriconazole and caspofungin despite having received voriconazole for coronary artery aneurysm described. Nawwar et al. reported a 6 cm aneurysm
one month without side effects. He was not on any other medications and of the LAD (1) presenting with stroke, Banarjee et al. reported a 5.3 cm
the workup was negative for secondary causes of heart block. Additionally, aneurysm of the RCA presenting with acute myocardial infarction (2), and
once voriconazole was stopped, the CHB subsequently resolved over the Pinheiro et al described a giant aneurysm of left main aneurysm 5.6 cm in
course of two weeks despite ongoing treatment with caspofungin.
diameter (3). The natural history and prognosis of coronary aneurysm has not
CONCLUSION: Combination antifungal therapies for disseminated fun-
gal infections are being increasingly used, although the safety of these been delineated. Indeed, criteria for diagnosis have not been established,
regimens has never been established. Clinicians should be cognizant that perhaps this explains why the reported incidence on angiography varies
CHB is a potential and reversible side effect of voriconazole when combined between 0.2% and 4.9% in patients undergoing coronary angiography. There
with caspofungin for the treatment of disseminated fungal infections. is male predominance and a predilection for the right coronary artery, with
REFERENCE: left anterior descending artery involvement less common. The most common
1 Vfend (voriconazole) [package insert]. Pfizer Pharmaceuticals, 2002 etiology reported is atherosclerosis accounting for 50%, followed by congen-
ital origin and Kawasaki’s disease (4). Other etiologies include arteritis,
mycotic infections, connective tissue disorders, trauma and metastatic tumor.
Coronary aneurysms have also been associated with percutaneous translumi-
nal coronary angioplasty espeically after dissection, and with angioplasty using
an oversized balloon or directional atherectomy. There are no clinical features
of coronary artery aneurysm. Most patients are asymptomatic. Myocardial
infarction, thromboemboli, and sudden cardiac death with acute rupture have
been reported in association of coronary artery aneurysm. As for our patient,
was his presenting chest pain a clinical manifestation of the large coronary
aneurysm? And, is his recurrent chest pain a manifestation of a similar
disease? This will be clarified by the follow-up cardiac imaging studies plan.
CONCLUSION: Coronary artery aneurysm should be considered in
the differential diagnosis of chest pain. The size can be very large and its
diagnosis may require more than one imaging modality.
REFERENCES:
1 Nawwar FRCS, FETCS: Giant atherosclerotic aneurysm of the left
anterior descending artery. The Journal of Thoracic and Cardiovas-
cular Surgery 2003;126(3):888-890
2 Banaerjee p et al. Giant right coronary artery aneurysm presenting
as a mediastinal mass. Heart 2004;90;e50
3 Pinheiro et al. Surgical management of a giant left main coronary
aneurysm. The Journal of Thoracic and Cardiovascular Surgery
2004;128(5):751-752
4 Mushabbar Syed and Michael Lesch: Coronary Artery Aneurysm: A
Review. Progress in Cardiovascular Diseases, Vol. 40, No. 1, 1997; 77- 84
DISCLOSURE: Svetolik Djurkovic, None.
RIGHT CORONARY ARTERY ANEURYSM: THE LARGEST AN-

EURYSM REPORTED
Simon K. Topalian MD* Katherine Chiu MD Michael Reinig DO Steven
Werns MD Janah Aji MD Toby R. Engel MD Cooper University
Hospital, Camden, NJ
INTRODUCTION: Coronary artery aneurysm is dilatation greater

than 1.5 times an adjacent normal coronary artery. We report a case of an
unusually large right coronary artery aneurysm (RCA) presenting with
chest pain.
CASE PRESENTATION: A 61 year old male with hypertension and
hyperlipidimia had persistent retrosternal and epigastric pain over several
months. It had no relation to exertion. His blood pressure was 194/115 mm
Hg. The remainder of the physical examination was unremarkable. A 12-lead
electrocardiogram revealed normal sinus rhythm with left ventricular hyper-
trophy. On chest x-ray, a hiatal hernia was suspected but endoscopy was
normal. A chest CT and MRI suggested sinus of valsalva aneurysm. Coronary
angiography revealed diffuse ectasia in the left coronary artery. The right
coronary artery could not be engaged, but non-selective injection of the right
coronary artery suggested a large aneurysm . It was better visualized by a
trans-esophageal echocardiography (figure 1a,1b). An aneurysm of the right
coronary artery measuring 7 x 11cm (figure 2) was resected with saphaneous
vein bypass of the distal right coronary artery. Pathologic changes of the
resected right coronary artery were most consistent with cystic medial
necrosis. At 6 months follow-up, he has resumed most of his customary DISCLOSURE: Simon Topalian, None.

Critical Care
4:15 PM - 5:45 PM
QUETIAPINE OVERDOSE INDUCED ACUTE RESPIRATORY medications, it has an improved safety profile, particularly decreased
DISTRESS SYNDROME extrapyramidal symptoms and tardive dyskinesia, although there is still a
Paul Strachan MD* Brian Benoff MD Long Island Jewish Medical risk for neuroleptic malignant syndrome.1 This patient developed pro-
Center, New Hyde Park, NY gressive hypoxia with infiltrates, requiring mechanical ventilation within
24 hours of presentation. Respiratory depression has previously been seen
INTRODUCTION: A 41 year-old male, with bipolar disorder pre- with large ingestions of quetiapine. In a case series by Balit, four of
sented after ingestion of 4500 mg of quetiapine. Within 24 hours, eighteen patients with quetiapine overdose required mechanical ventila-
respiratory failure ensued, requiring intubation and mechanical ventilation. No patients developed ARDS. Our patient presented with minimal
tion. Chest radiograph demonstrated bilateral infiltrates, consistent with changes on his initial chest x-ray. Within 24 hours he had bilateral
Acute Respiratory Distress Syndrome (ARDS). infiltrates and was intubated for respiratory failure. He required 100%
CASE PRESENTATION: The patient is a 41-year old male with a FIO2 while on the ventilator, with an initial PaO2:FIO2 ratio of 90. The
history of bipolar disorder and generalized anxiety. He was recently CVP was 10 mmHg and the ejection fraction was normal. These findings
admitted to another hospital with depression and suicidal ideation. are all consistent with the diagnosis of ARDS. This is the first reported
Discharge medications included: quetiapine 25 mg twice daily, valproic case of such resulting from quetiapine overdose.
acid, gabapentin, desipramine and paroxetine. Two days post-discharge, CONCLUSION: As quetiapine is a relatively new medication, experi-
his parents observed clonic movements that were presumed to be ence with cases of overdose are limited. This patient’s respiratory status
seizures. Intravenous lorazepam was administered by EMS with no rapidly declined over the first twenty-four hours. Cases involving quetia-
response. A suicide note was found next to an empty bottle of quetiapine pine overdose warrant admission, with close monitoring of respiratory
that previously contained 180, 25 mg tablets. On examination: Vitals: HR status.
98, RR 20, BP 100/60, T 97.2 F. O2 Sat 100%. HEENT: Minimally REFERENCE:
reactive pupils, bilateral opsoclonus. Lungs: Poor inspiratory effort, 1 Mosbey Drug Consult 2004 2 Balit C. et al. Quetiapine Poisoning:
otherwise clear. Heart: Regular rate & rhythm. Abdominal: Normal active A Case Series. 2003 Annals of Emergency Medicine 42:6 751-758
bowel sounds, soft, non-tender. Extremities: No edema, clubbing or DISCLOSURE: Paul Strachan, None.
cyanosis. Neurological: Drowsy, but arousable to stimuli. Glasgow Coma
Scale ⫽ 8. Frequent myocolonic jerks in all extremities. Chest radiograph:
Minimally increased interstitial markings (Fig 1). ECG: Sinus Rhythm at HAZARDS OF OPIATE MISMANAGEMENT IN THE ICU
98 bpm. QT/QTc interval was prolonged at 536/684. EEG: No epilepti- Richard A. Mularski MD* Karen S. Mularski MD Steven M. Asch MD VA
form activity. Toxicology: Positive for tricyclic antidepressants (TCA). Gas Greater Los Angeles Healthcare System, Los Angeles, CA
chromatography showed desipramine and quetiapine (quantitative levels
were not available). Valproic acid level: 22 (ref 50-120). He received INTRODUCTION: The American College of Chest Physicians is
intravenous lorazepam, magnesium and sodium bicarbonate for electro- leading the charge to integrate the skills of pain management and
cardiographic changes and was admitted to the intensive care unit (ICU). palliative care into ICU practice. This case poignantly illustrates how
During the first twenty-four hours, the patient developed progressive opiate mismanagement can threaten patient safety. We describe how
hypoxemia, unresponsive to increased oxygen supplementation. There was suboptimal care transformed an otherwise successful laminectomy into a
no witnessed aspiration. He was intubated for hypoxemic respiratory protracted ICU stay complicated by acute respiratory distress syndrome
failure. Post-intubation, he required 100% FIO2 and high levels of (ARDS).
positive end-expiratory pressure (PEEP). Chest radiograph showed bilat- CASE PRESENTATION: A 50 year-old man who was failing conser-
eral infiltrates (Fig 2). Central venous pressure (CVP) measured 10 vative management for longstanding back pain was hospitalized for a
cmH20. Echocardiogram showed normal left ventricular function. The laminectomy under spinal anesthesia. He had used controlled-release oral
patient had a prolonged ICU course, complicated by the subsequent morphine at a dose of 100mg three times a day for several years.
development of gram-negative bacteremia. After five weeks of mechanical Post-operative pain management included only an epidural catheter with
ventilation, he improved clinically and was extubated. Once stable, he was bupivicaine infusion and oral codeine for break through pain. By the end
transferred to psychiatry for further care. of post-operative day one, increasing doses of codeine were ineffective at
relieving pain and intravenous morphine was administered. The patient
complained of nausea which was treated per a standing order with 1 cc
intravenous droperidol. Thirty minutes later he was noted to be somno-
lent, hypopneic, and hypoxic. Again following a standing order, 1 mg
intravenous naloxone was administered. The patient screamed, had
paroxysms of emesis, pulled out his intravenous line, experienced explo-
sive diarrhea, and aspirated. His symptoms subsided over the ensuing
thirty minutes, but he became increasingly somnolent and hypoxic despite
CASE REPORTS
receiving no additional medications; eventually an endotracheal tube was
placed. He was diagnosed with ARDS and required mechanical ventila-
tion for 14 days; he also manifested acute tubular necrosis with a peak
serum creatinine of 3.4. As his pulmonary and renal processes began to
reverse, he was noted to be persistently unresponsive, leading to a head
CT (negative). Sedative (propofol) and opiate medications (intravenous
morphine) were stopped; two days later he was noted to have a pulse of
148 with irregularity, a blood pressure of 180/100, and a temperature of
100.1 F. He experienced ventilator dyssynchrony, tachypnea at over 40
breaths per minute, and agitated delirium. Hydromorphone 1.5 mg
intravenous resulted in stabilization within 10 minutes and epidural
medications and opiates were restarted. Four days later he emerged from
his coma and was liberated from the ventilator; he complained of diffuse
abdominal pain and bloating. Review of nursing notes revealed no bowel
movement since the explosive diarrhea 22 days ago, despite nasogastric
feeding. Aggressive therapy for obstipation was initiated, and after a four
week hospital stay, the patient was discharged to a rehabilitation facility.
Although differential diagnoses are broad for many of the manifestations
in this case, opiate mismanagement was implicated for the complications
reviewed in this presentation.
DISCUSSIONS: Multiple opportunities for improved quality of care
for pain and symptom management exist in this case. Chronic opiate use
DISCUSSIONS: Quetiapine fumarate is an antipsychotic drug that is and physiologic dependence led to withdrawal on multiple occasions due
an antagonist at multiple receptors in the brain including: serotonin, to insufficient weaning of opiates. Over-medication with sedatives can
dopamine, adrenergic and histamine. Compared to older antipsychotic increase opiate respiratory depression. Use of naloxone in dose range

Critical Care, continued
0.04-0.1mg every five minutes can avoid the acute reaction that precipi-
tated aspiration and serious lung injury in this patient. Codeine was likely
ineffective, whereas hydrocodone and morphine produced expected
clinical responses, likely due a polymorphism in debrisoquin hydroxylase
which is required to convert codeine to its active metabolite, morphine
(6-10 % of Caucasians). Morphine generally has a half life of 2-4 hours;
however central nervous effects can be markedly prolonged by an active
metabolite, morphine-6-glucoronide, especially in renal insufficiency.
Bowel prophylaxis is essential in all patients on opiate medications and
should include both a stimulant agent and a stool softener.
CONCLUSION: Integration of palliative care and pain management
skills into ICU medical care can lead to improvements in patients’
symptom experience and, as demonstrated in this case, may help improve
patient safety and overall quality of care.
DISCLOSURE: Richard Mularski, Grant monies (from sources other
than industry) AHRQ, NINR, CMS, NCI, National Quality Forum,
American Lung Association of Oregon, & Northwest Health Foundation
DISCUSSIONS: This case report is notable for several reasons. First,

A PATIENT PRESENTING WITH LUNG CAVITIES, ENDO- C-ANCA-positive DAH was successfully treated with cylophosphamide,
BRONCHIAL NODULES, DIFFUSE ALVEOLAR HEMOR- corticosteroids and plasmapheresis as previously described in a limited
RHAGE, MICROTHROMBI AND A POSITIVE C-ANCA number of patients. Second, the pathology was notable for numerous
Lewis A. Eisen MD* Ya J. Chang MD Samuel O. Acquah MD Beth Israel microthrombi which is not a typical finding in WG. Only a few small
Medical Center, New York, NY studies have shown that C-ANCA stimulates tissue factor and the
coagulation cascade and can cause microthrombi. Finally, cyclophospha-
INTRODUCTION: Typical Wegener’s granulomatosis (WG) is a small mide was given despite the pathologist being unable to make a firm
and medium-sized vessel vasculitis that primarily affects the upper respiratory diagnosis of WG.
tract, lungs, and kidneys. It is associated with antibodies to proteinase-3 CONCLUSION: A C-ANCA positive patient with atypical clinical and
(C-ANCA). We describe a near-fatal presentation of a C-ANCA positive pathological features was treated successfully with immunosuppression
patient with atypical clinical and pathological features who responded to a and plasmapheresis. In extreme situations, treatment must be initiated
combination of immunosuppression and plasmapheresis. promptly even when diagnostic uncertainty remains.
CASE PRESENTATION: A 23-year-old woman with no significant DISCLOSURE: Lewis Eisen, None.
past medical history presented with three weeks of dyspnea, fevers and
non-productive cough. She denied weight loss, night sweats, hematuria,
joint pains, visual changes, or paresthesias. She had recently traveled to EXTRAGONADAL GERM CELL TUMOR PRESENTING WITH
Wisconsin and Southern California. Physical examination was notable for RESPIRATORY FAILURE
normal upper respiratory and pulmonary findings and the absence of signs Sam S. Parsia MD* Robert L. Smith MD Kevin J. Felner MD New York
of neuropathy, uveitis, rash or arthritis. The chest radiograph showed University School of Medicine, New York, NY
multiple large cavities and areas of dense consolidation. The white blood
cell count (WBC) was 10,300/mm3 and the hematocrit was 29%. Eryth- INTRODUCTION: Germ cell tumors occur in a younger population
rocyte sedimentation rate was 103 mm/hour. Creatinine was normal. and can present with aggressive metastatic disease. We are presenting an
Urinalysis showed hematuria without proteinuria or red cell casts. C- elderly gentleman with respiratory failure due to an extragonadal mixed
ANCA (ELISA to proteinase-3) was positive at 16 Elisa Units/ml. germ cell tumor.
Rheumatoid factor, antinuclear antibody, and antiglomerular basement CASE PRESENTATION: Patient was a 76 year old white male with
membrane antibodies were negative. The activated partial thromboplastin past medical history of hypertension and microscopic hematuria who was
time was 35.5 s. Bronchoscopy revealed numerous smooth, white endo- in his usual state of health until three days prior to admission when he
bronchial 1 cm nodules. Multiple bronchoscopic lung biopsies revealed developed acute back pain and gross hematuria. He was admitted to a
necrotizing pneumonitis; smears and cultures for acid fast bacilli (AFB), local hospital and was treated for an enterococcal urinary tract infection.
fungi and bacteria were negative. Due to persistent diagnostic uncertainty, Patient’s chest radiograph on admission was significant for bilateral
an open lung biopsy was performed. It showed small vessels with nodular infiltrates that were not present on a chest radiograph performed
inflammatory cells, a few giant cells, acute hemorrhage, and multiple two weeks prior to admission. Patient denied chest pain, shortness of
microthrombi. The patient was treated with empiric antibacterial and breath, fever, chills, night sweats, weight loss, or hemoptysis.The patient’s
antifungal agents, and methylprednisolone 1 gm/day for three days. The past medical history included benign prostatic hypertrophy, essential
patient improved symptomatically and was discharged on a tapering dose tremor, bladder diverticulae. His outpatient medications included aspirin,
of prednisone, moxifloxacin and itraconazole. Due to the lack of definitive clopidogrel, atenolol, finasteride, primidone, and terazosin. Patient was a
pathology for WG, cyclophosphamide was not initiated at that time. One former heavy smoker and alcohol user, but he denied any illicit drug use.
week later, the patient returned in severe respiratory distress requiring He had not traveled recently, had no pets, and denied ill contacts. He
intubation and mechanical ventilation. The chest radiograph showed new served in the military during the Korean War, and was a retired
cavities with worsening opacification of both lungs, sparing only the maintenance worker. Patient had a chest CT which showed multiple
apices. The WBC was 19,500/mm3 and the hematocrit was 26%. Bron- nodules (Figure 1), as well as bilateral pleural effusions without airspace
choscopy demonstrated diffuse alveolar hemorrhage(DAH). Repeated disease or mediastinal lymphadenopathy. On hospital day 3, patient
testing, including stains for pneumocystis, was negative. Bronchoalveolar developed a cough with brown sputum and became hypoxic; broad
lavage fluid showed hemosiderin-laden macrophages. Serology for anti- spectrum antibiotics were started. Flexible bronchoscopy revealed no
glomerular basement membrane antibodies was negative. Cultures from endobronchial abnormalities. Transbronchial biopsies and bronchoalveo-
the first admission showed no fungal or mycobacterial organisms. The lar lavage specimens were non diagnostic. Patient was referred to our
patient required ventilatory support with 100% oxygen. Broad spectrum institution for an open lung biopsy. Upon transfer, hospital day 7, patient
antibiotics were started, along with 1 gram of methylprednisolone daily for was in moderate respiratory distress, requiring a 100% non-rebreather
three days. After risks and benefits were discussed with the family, mask to achieve a oxygen saturation above 90%. He was afebrile and
cylcophosphamide was started at a dose of 2mg/kg. In light of the DAH, hemodynamically stable. Physical exam was significant for no palpable
plasmapheresis was initiated as well. Four days after this regimen was lymphadenopathy, decreased breath sounds at both bases, no murmurs,
started, the oxygen fraction was able to be reduced to 0.6. Repeat and no hepatosplenomegaly. His genitourinary exam was remarkable for
bronchoscopy showed continued hemorrhage, so plasmapheresis was an enlarged left testicle, which was smooth and without discrete mass. The
continued for a total of 10 sessions. Three weeks later, the patient was white blood cell count 8X103cells/mm3 with normal differential, hemo-
weaned from ventilatory support. Radiography showed improvement of globin 14.4 g/dL, lactate dehydrogenase 233U/L, ESR 27mm/60sec.
the pulmonary cavities and consolidation. Two months after admission, Urinalysis revealed no proteinuria or casts. HIV serology was negative, as
the patient had no pulmonary symptoms and had returned to work. were blood and urine cultures. Urinary legionella and histoplasma antigens

were negative, as was a serum cryptococcal antigen. Antinuclear cytoplasmic with transfusion of packed red blood cells. The dialysis nurse reported
antibodies were negative. The presence of asymmetric testes prompted a some difficulty with flow through the catheter and the renal team
serum beta HcG that was 2318 mIU/ml, however, a testicular ultrasound attempted to adjust SLED by pulling from the distal port and reinfusing
showed no testicular mass. The open lung biopsy revealed a mixed germ cell through the proximal port. However, effective SLED could not be
tumor which stained positive for beta HcG (Figure 2). Thyroid transcription achieved and the critical care team elected to remove the patient from
factor 1, alpha-fetoprotein, and CD 30 markers were negative. Despite SLED and place a new dialysis catheter at another site. Upon flushing the
treatment with cisplatin, patient remained on mechanical ventilation and his SLED circuit and removing the patient from the SLED unit, he acutely
radiographs revealed persistent infiltrates. Nine days after his last dose of became hypotensive, bradycardic, and progressed to asystole that was
chemotherapy patient had a PEA arrest and expired. refractory to advanced cardiac life support protocol. He subsequently
DISCUSSIONS: Mixed germ cell tumors account for one third of all underwent post-mortem examination which revealed 500ml of bloody
germ cell tumors and the average age at diagnosis is 30 years of age. Tissue fluid upon opening the pericardial sac, with the final position of the
diagnosis is imperative, due to favorable outcomes with chemotherapy. dialysis catheter in the pericardial space.
Extragonadal germ cell tumors usually present with a primary focus either DISCUSSIONS: Cardiac tamponade is a rare, but lethal complication
in the mediastinum or retroperitnoeum without evidence of malignancy in of misplaced central venous catheters. Standard bedside practices to
the testes or ovaries. In our patient no primary focus was identified. determine catheter placement may fail to disclose the true location of the
CONCLUSION: This case illustrates a rapidly progressive course of an catheter. The possibility of pericardial placement must be considered in
extragonadal mixed germ cell tumor associated with hypoxemic respira- order to avoid lethal cardiac tamponade. In this case, as the SLED circuit
tory failure. To our knowledge no cases of a mixed germ cell tumor with was flushed and the patient was removed from the dialysis unit, 500ml of
progressive respiratory failure have been reported. Our case illustrates fluid filled the pericardial space resulting in acute cardiac tamponade and
how germ cell tumors must be part of the differential diagnosis in a patient cardiac arrest. A definitive study such a contrast venography may be
with diffuse, rapidly progressive pulmonary lesions. required to confirm catheter location in special circumstances. A persis-
tent left SVC may have a radiograph identical to the radiograph in this
case, however, as illustrated here, simple measurements such as blood gas
analysis and pressure transduction may fail to confirm the true location of
the catheter.
CONCLUSION: In order to avoid potentially lethal complications of
central venous catheter placement, we must be aware of the potential
pitfalls of simple bedside tests such as venous blood return, blood gas
analysis, and pressure transduction to determine catheter location. When
there is suspicion of aberrant catheter placement, contrast venography
should be considered to verify the true catheter location.
DISCLOSURE: Sam Parsia, None.
ACUTE CARDIAC TAMPONADE AFTER REMOVAL FROM

SLOW LOW EFFECTIVE DIALYSIS (SLED) IN THE INTENSIVE
CARE UNIT
Susan M. Rohr DO* University of Nebraska Medical Center, Omaha, NE
INTRODUCTION: Central venous catheter placement is a common DISCLOSURE: Susan Rohr, None.
procedure performed in the intensive care unit with common and
uncommon complications. Standard post-procedure practices such as
CASE REPORTS
chest radiography, free venous blood return, and pressure transduction HYPEREOSINOPHILIC SYNDROME MIMICKING SEPSIS AND
may fail to detect catheter malposition in rare cases. The following case LUNG INJURY: DRAMATIC IMPROVEMENT AFTER ACTI-
illustrates a patient who underwent left internal jugular central venous VATED PROTEIN C THERAPY
dialysis catheter placement and suffered acute cardiac tamponade as a Aliya Noor MD* David E. Miller MD Patricia A. Smith BS Kenneth S.
consequence of a misplaced dialysis catheter into the pericardial space. Knox MD Indiana University, Indianapolis, IN
CASE PRESENTATION: A 69 year-old male physician was admitted
to the intensive care unit for severe community acquired pneumonia and INTRODUCTION: Hypereosinophilic syndrome (HES) is a systemic
sepsis that was found to be due to methicillin-resistant Staphylococcus illness that usually presents with nonspecific symptoms. However, HES
aureus. Despite aggressive antibiotic therapy, fluid resusitation, drotreco- can be fatal, particularly when eosinophils infiltrate vital organs. We
gin alfa infusion, hydrocortisone infusion, and vasopressor therapy, his report a patient with HES who presented with a sepsis-like syndrome and
sepsis progressed and he developed oliguric renal failure with metabolic rapidly improved with Drotrecogin-alpha therapy.
and respiratory acidosis. The renal team was consulted for evaluation for CASE PRESENTATION: A 52 yr old male presented to an outside
slow low effective dialysis (SLED). The drotrecogin alfa infusion was held hospital (OSH) with debilitating abdominal pain 12 days prior to his
as a left internal jugular dialysis catheter was placed utilizing bedside transfer to Indiana University. Two months ago he underwent an outpa-
ultrasound guidance. A post-procedure chest radiograph revealed an tient evaluation for fatigue, arthralgias, nonproductive cough and inter-
unusual position of the catheter consistent with either a persistent left mittent abdominal pain. Upon admission to the OSH he underwent an
superior vena cava (SVC) or arterial cannulation. Blood gas analysis exploratory laprotomy which revealed colonic perforation. A small portion
revealed a pH of 7.04, pCO2 58, pO2 34, HCO3 15, and a measured of bowel was excised with end to end anastomosis. Surgery went well but
saturation of 63% consistent with venous blood. Transduction of central he was reintubated 2 days later for increasing dyspnea. Upon transfer, the
venous pressure (CVP) via the left internal jugular dialysis catheter distal patient was intubated, on FiO2 of 100% and PEEP of 15. He was
port was 20cmH2O pressure and was equal to the CVP transduced by a hypotensive and required levophed. Except for a heart rate of 122, a
previously placed right internal jugular catheter. The patient was there- benign post op abdomen, and bilateral rhonchi his exam was normal.
fore presumed to have a dialysis catheter located in a persistent left SVC. Admission automated CBC showed a WBC count of 36,000 with 57N,
An echocardiogram obtained prior to initiation of SLED revealed a small 27B, 6E (manual differential showed 40% eosinophils, erroneously re-
pericardial effusion without evidence of tamponade. SLED was initiated ported as bands). His hemoglobin was 9.4. His transaminases were mildly

elevated and a troponin I was 25units. Chest radiograph revealed diffuse tion of human eosinophil chemotaxis by protein C. J Allergy Clin
bilateral airspace opacities. The patient was continued on broad spectrum Immunol. 2003 Aug;112(2):375-81
antibiotics. Drotrecogin-alpha was added for treatment of presumed DISCLOSURE: Aliya Noor, None.
sepsis on admission. Over the first 18 hours of Drotrecogin-alpha infusion,
the patient dramatically improved and at the end of the 96 hour infusion
the patient was weaned to 50% FiO2 and 8cm PEEP. Troponin had
normalized. Despite improvement, mechanical ventilation was unable to
be discontinued. CT scan of the chest showed extensive ground glass
opacities and bibasilar infiltrates (figure1). Bronchoscopy was performed
and revealed 94% eosinophils in the lavage fluid (figure2). Pathology from Infectious Disease I
the recent colon resection was reviewed and showed marked eosinophilic 4:15 PM - 5:45 PM
infiltration and eosinophilic vasculitis. Bone marrow biopsy showed
hypercellular marrow with eosinophilic precursors and no blasts. Hypere-
osinophilic syndrome was diagnosed and the patient was started on NOCARDIOSIS IN AN OTHERWISE IMMUNOCOMPETENT
steroids (60mg IV q6) and hydroxyurea. The patient was extubated 24 hrs PREGNANT FEMALE
after the initiation of steroids. He did well over the next few days and was Mihwa C. Pak MD* Seth Rivera MD University of California Los Angeles,
discharged home on 40 mg of prednisone. Los Angeles, CA
INTRODUCTION: Nocardiosisis a bacterial infection seen predomi-

nantly in patients with altered cell-mediated immunity (CMI). We
describe the first case of pulmonary Nocardiosis in an immunocompetent
pregnant female presenting as hemoptysis and discuss the pertinent
aspects of this patients care.
CASE PRESENTATION: A 32-year-old recently immigrated Chinese
woman in the first trimester of pregnancy presented with a 24-hour
history of intermittent hemoptysis. She denied fevers, chills, respiratory
symptoms and recent tuberculosis exposure although she had a newly
positive tuberculin skin test 5 months ago. She had lost 6 pounds in the
past two months due to hyperemesis. Her medical history was remarkable
for two spontaneous abortions, both during the first trimester, and
stress-induced gastritis. Physical examination was unremarkable. Labora-
tories revealed a hemoglobin of 11.3 g/dL and a D-Dimer of 337. A
computed tomography scan of the chest with angiogram (figure 1)
revealed ground glass opacifications with peribronchial thickening in the
right lower lobe and no pulmonary embolism. Bronchoscopy revealed
friable mucosa in the right lower lobe without active bleeding. A work-up
for connective tissue disease, HIV and tuberculosis was negative. Her
hematocrit remained stable and she was discharged.. Three days later, she
presented with recurrent hemoptysis and a new onset of frontal headaches
without other neurological symptoms. Her hemoglobin decreased to
9.0g/dL. Magnetic resonance imaging of the brain was negative. The BAL
culture returned positive for Nocardia astreroides. She was started on
trimethoprim/sulfamethoxazole (TMP-SMX) in consultation with obstet-
rics and ultimately terminated the pregnancy.
DISCUSSIONS: Nocardiosis an uncommon infection caused by gram
positive, weakly acid fast, filamentous, aerobic actinomycetes in the genus
Nocardia. They are relatively slow-growing and may require 5-21 days to
exhibit growth. Nocardiosis is typically seen in patients with altered CMI
(HIV, iatrogenic immunosuppression, and malignancy), but approximately
36% of patients are immunocompetent. Intact CMI in concert with
neutrophil activity is important in containing the infection (Filice et al,
JID 1987). In our patient, her sole risk factor was pregnancy. Two
DISCUSSIONS: Hypereosinophilic syndrome is a systemic illness previous cases of non-pulmonary nocardia in immunocompetent pregnant
which can be rapidly fatal. The criteria for diagnosis include a blood women have been described (Braun et al, RID 1991; Kannon et al, J Am
eosinophilia of ⬎1500/uL for more then six months, no other apparent Acad Derm 1996). This association, though extremely rare, is plausible.
etiologies for eosinophilia, and evidence of end organ dysfunction. Some Immunologic changes associated with pregnancy have been designated
of the common symptoms are fatigue-26%, cough-24%, dyspnea-16%, “pregnancy-associated immune deficiency syndrome” (Weinberg, RID
fever-12%, myalgia-14%, angioedema-14%, skin rash-12% and visual 1984). Increases in hydrocortisone, estrogen, progesterone, and other
problems-10 %. Involvement of skin, heart, nervous system, lungs and serum factors may modulate lymphocyte or macrophage synthesis, acti-
spleen occurs in 45-60% of cases. Thromboembolic events are common vation and function during pregnancy leading to depressed CMI.(Wein-
and anticoagulation is universally administered. Specific treatment regi- berg, RID 1984). There is also an imbalance between helper and
mens for HES include steroids, chemotherapeutic agents (hydroxyurea), suppressor T cells (Sridama et al, NEJM 1982) and several studies have
and immune modulators such as interferon-alpha and anti-interleukin 5 shown that total T cell number and cytotoxic lymphocyte activity are both
antibody. The tyrosine kinase inhibitor, Imatinib mesylate, has also been decreased during pregnancy(Davis et al, J Rep Immuno 1998). An
shown to be efficacious in the treatment of HES. Drotrecogin-alpha has important consideration from this case is the overall outcome of the
anti-inflammatory and anti-thrombotic effects, as well as recently recog- pregancy. The untoward affects of Nocardiosis in human fetuses is not
nized effects on eosinophil recruitment, which may explain its usefulness known. However, it is worth noting that Nocardia has been associated with
in the management of HES. placentitis in cattle, swine and equine populations and has been linked
CONCLUSION: Drotrecogin-alpha therapy was associated with a with increasing numbers of losses from late abortions, still-births, prema-
rapid and substantial improvement in hemodynamic parameters, static turity, and early neonatal deaths (Volkmann et al, J S Afr Vet Assoc 2001).
compliance, and cardiac function in the absence of any other therapy for Furthermore, antibiotic selection during pregnancy is challenging since
HES. Additionally, no identifiable source of infection was found. This case the teratogenic potential of most therapeutic agents in humans are largely
highlights the importance of early diagnosis of HES and the utility of APC unkown. TMP/SMX is the agent of choice in treatment of Nocardiosis.
therapy in SIRS related to HES. However, TMP/SMX has been reported to cause congenital anomalies
REFERENCE: such as cleft palate in rats, although no cases of human birth abnormalities
1 Feistritzer,et al. Endothelial protein C receptor-dependent inhibi- related to maternal ingestion have been recorded (Chow et al, RID 1985).

Infectious Disease I, continued
CONCLUSION: To our knowledge, this is the first reported case of DISCUSSIONS: Cavitary lung disease due to Histoplasma capsulatum
pulmonary nocardiosis in an immunocompetent pregnant female. This is well recognized, but complete destruction of the lung parenchyma is
case highlights the importance of bronchoscopy in hemoptysis and the rarely reported1. Cavitary histoplasmosis is more common in men and
subtle immune deficiency of pregnancy. those patients with underlying emphysema. Successful treatment of
cavitary histoplasmosis to preserve viable lung was reported in 29
patients2. Despite early evaluation in this case, the right lung was
completely destroyed. More importantly, FOB became a necessary
adjunct to drain infected secretions from the destroyed lung until
definitive surgery.
CONCLUSION: Development of this large chest cavity secondary to
histoplasmosis was treated by repeated FOB until definitive thoracotomy
to obliterate the infected space.
REFERENCES:
1 Diveley W, McCracken R. Cavitary pulmonary histoplasmosis
treated by pulmonary resection: 13-year experience with 29 cases.
Ann Surg 1966; 163: 921-930.
2. Ryu J, Swensen SJ. Cystic and cavitary lung diseases: focal and
diffuse. Mayo Clin Proc 2003; 78:74
DISCLOSURE: Mihwa Pak, None.
PROGRESSIVE CAVITARY DESTRUCTION OF THE RIGHT

LUNG
Jennifer L. Rippon DO* Lynn L. Horvath MD Robert G. Rivard MD
William C. Conner MD Michael J. Morris MD Brooke Army Medical
Center, San Antonio, TX
INTRODUCTION: Numerous malignancies and infectious etiologies

cause cavity formation but rarely does the process involve an entire lung.
We describe progressive right lung cavitary destruction requiring man-
agement with repetitive fiberoptic bronchoscopy (FOB).
CASE PRESENTATION: A 60 year old male presented in September
1998 with cough and pleuritic chest pain. Medical history was significant
for traumatic right rib fractures with pneumothorax and 40 pack year
smoking history. Radiography demonstrated a right upper lobe (RUL)
infiltrate and left exudative pleural effusion. After antibiotic therapy,
repeat evaluation revealed persistent RUL infiltrate with pleural thicken-
ing and interval resolution of the effusion. Fiberoptic bronchoscopy was
performed; stains and cultures were negative for infection. Skin testing
with purified protein derivative was negative. He was referred to our
institution in November 1998 with increasing chest pain, non-productive
cough and weight loss. Chest computed tomography (CT) showed an
increasing RUL infiltrate, pericardial effusion and extensive mediastinal
and cervical adenopathy. Mediastinal exploration, pericardial window with DISCLOSURE: Jennifer Rippon, None.
biopsy, and right apical lung biopsy were performed; all were negative for
malignancy or infection. The lung pathology was described as “diffuse
interstitial fibrosis with emphysematous changes, atelectasis, and paren- OCHROCONIS GALLOPAVUM INFECTION IN AN IMMUNO-
chymal hemorrhage suggestive of desquamative interstitial pneumonia.” COMPETENT HOST
The patient was returned to the referring hospital but failed to follow- Scott Shofer MD* Aimee Zaas MD John Hollingsworth MD Duke
CASE REPORTS
up.At another facility in July 2001, a right thoracotomy was attempted for University Medical Center, Durham, NC
the persistent RUL infiltrate, but was aborted due to chest wall adhesions
and excessive bleeding. He later developed hemoptysis, but had a INTRODUCTION: Ochroconis gallopavum (formerly Dactylaria gal-
nondiagnostic FOB in March 2002. The patient returned in November lopava) is a dematiaceous fungi which is an increasingly prevalent
2002 with progressive symptoms and cutaneous “bubble-like” lesions with opportunistic infection in the immunosuppressed population. However,
respiratory variations at the thoracostomy site. Chest CT demonstrated few reports exist of infections in immunocompetent hosts. We report the
progressive change with complete destruction of right upper and mid lung treatment of O. gallopavum infection in an immunocompetent host with
parenchyma and minimal intact basilar parenchyma. Centrilobular nod- voriconazole.
ules along with diffuse pleural thickening were noted bilaterally. Fiber- CASE PRESENTATION: This is a 79 year old Caucasian female who
optic bronchoscopy demonstrated thick secretions and right lung airway presents for evaluation of a two year history of shortness of breath.
erythema with normal left anatomy. Histoplasmosis capsulatum was Patient’s initial symptoms were breathlessness with exertion, however her
isolated from left upper lobe and tracheal aspirates, but biopsies had no dyspnea had progressed over 3 months prior to evaluation to being able to
granulomatous changes. He began oral itraconazole for chronic pulmo- climb only a single flight of stairs. She notes morning cough with chronic
nary histoplasmosis with stabilization of CT findings during regular production of thick yellow, sputum. Review of symptoms is notable for
evaluations.In August 2004, he was admitted with interval development of fatigue, low grade fevers, and intermittent night sweats. Past medical
a right lung air/fluid level and draining cutaneous fistula from the prior history includes hypothyroidism, basal cell skin cancer, and a remote
thoracostomy site (Figure 1). Chest CT showed complete right lung history of melanoma. Medications are synthroid, aspirin, and doxepin.
destruction, a large bulla with air/fluid level, and chest wall erosion with There was a family history of melanoma. Patient was a life-long non-
fistula formation. Bronchoscopy revealed the right bronchus intermedius smoker although she had significant exposure to second-hand smoke. She
opened into a large cavity with purulent secretions and fibrous tissue had lived in Florida, but currently lives in North Carolina. She had worked as a
(Figure 2). All right upper and lower lobe airways terminated into the clerk in a drugstore and at a cigarette manufacturer on the production line. She
cavity. After aspiration of 800 ml of secretions, the cutaneous fistula denied significant exposure to dust or toxins, and had not traveled recently.
opening was identified and later spontaneously closed. For the next six Physical exam revealed bilateral inspiratory crackles at the lung bases. There was
months, the patient required regular repeat FOB to aspirate secretions no adenopathy present. Ambulatory oximetry and pulmonary function testing was
while gaining weight to tolerate definitive thoracotomy. normal. Chest x-ray revealed interstitial prominence in the left mid, and lower

lung fields. Computed tomography of the chest revealed 2-3 mm nodules, LEPROSY MISTAKEN AS SARCOIDOSIS IN LARGE REFERRAL
ground-glass, and bronchiectasis in the right middle, and left lower lobes (fig. 1). SARCOIDOSIS CLINIC
The patient was taken for bronchoscopy which showed mild inflammation on Geneva B. Tatem MD* Bashar Bash MD Zachary Q. Morris MD Henry
transbronchial biopsies and late growth of O. gallopavum and Aspergillus Ford Hospital, Detroit, MI
versicolor. Computed tomography of the head to rule-out cerebral involvement
was negative and patient was started on voriconazole therapy, an advanced INTRODUCTION: Leprosy is a chronic granulomatous infection of
generation antifungal with activity against most dematiacious fungi, Aspergillus the skin and peripheral nerves with Mycobacterium leprae. It can be
species, Fusarium species and yeasts. Patient showed remarkable improvement difficult to distinguish from other granulomatous diseases, including
in her symptoms of dyspnea, cough, sputum production, and fevers. Her exercise sarcoidosis. This case describes lepromatous leprosy presenting as neu-
tolerance had improved to three fights of stairs within the first month of therapy,
ropathy and non-caseating granulomas of the skin presumed to be
and repeat CT of the chest showed resolution of the nodules and ground-glass
opacities (fig 2). The patient completed four months of anti-fungal therapy with neuro-cutaneous sarcoidosis.
resolution of her symptoms. CASE PRESENTATION: A 34 year-old male from India with a
DISCUSSIONS: Ochroconis gallopavum is a member of the dematia- five-year history of mononeuritis multiplex affecting the distal lower
ceous hyphomycetes which are best known in the veterinary literature as a extremities and diffuse rash was referred to our sarcoidosis clinic for
cause of epidemic avian encephalitis(1). Reports of human infections have management of his presumed neuro-cutaneous sarcoidosis. Physical
been primarily limited to immunocompromised hosts with history of solid examination revealed multiple indurated erythematous plaques on the
organ transplantation or hematologic malignancy. Standard treatment of cheeks, forehead and left ear, as well as violaceous and hypopigmented
systemic infections with dematiaceous molds involves surgical resection in patches on the bilateral upper and lower extremities. There was
combination with antifungal agents(1). Two previous reports have described decreased sensation on the dorsum of both feet and lateral aspect of
infections in immunocompetent hosts, both of whom had either predisposing the left leg. Left cheek biopsy demonstrated noncaseating granuloma.
environmental exposure or concomitant infection with structural lung dis- Sural nerve biopsy revealed very prominent round cell infiltration of
ease(2,3). Our patient had no history of environmental exposure or predis- the endoneuria and epineurium. Skin smear was negative for acid-fast
posing infections. She rapidly improved with oral therapy and made a clinical bacilli (AFB). Fite stain for Mycobacterium leprae was negative.
recovery with radiographic resolution in 4 months time. Serum angiotensin converting enzyme (ACE) level, vitamin D level,
CONCLUSION: Ochroconis gallopavum is a rare infection in immu- and urinary calcium were normal. Chest radiograph was normal, and
nocompetent patients. We report a case of pulmonary infection in a pulmonary function tests revealed a mild restrictive pattern with a mild
patient with no predisposing factors who had a rapid and complete
diffusion impairment. Prednisone was started with mild improvement
response to oral antifungal agents.
REFERENCES: of his neuropathy. Multiple attempts at weaning steroids caused
1 Kralovic, S and Rhodes, J. Phaeohyphomycosis caused by dactylaria worsening of his neuropathy, and steroid-sparing agents were added.
(human dactylariosis): report of a case with review of the literature. His rash persisted despite 5 years of treatment with varying doses of
J. Infec, 1995. 31: p.107-113 prednisone and hydroxychloroquine. Repeat biopsy of his skin rash
2 Odell, J., et al. Multiple lung abscesses due to ochroconis gallopa- revealed multiple large nodular histiocytic infiltrates throughout the
vum, a dematiaceous fungus, in a nonimmunocompromised wood dermis, and Fite stain revealed numerous intact AFB, consistent with
pulp worker. Chest, 2000. 118: p. 1503-1505. lepromatous leprosy. The patient was subsequently hospitalized at the
3 Bravo, L., et al. Ochroconis gallopavum and mycobacterium avium intra- National Hansen’s Disease Programs Center in Louisiana and after
cellulare in an immunocompetent patient. Chest, 2004. 126: p. 975s. additional skin biopsies the diagnosis of lepromatous leprosy was
confirmed. Monthly rifampin and clofazimine as well as daily dapsone,
clofazimine, thalidomide, and a slow prednisone taper were begun.
One month after instituting this regimen, most of his skin lesions
completely inverted. Despite therapy, however, his neuropathic symp-
toms have worsened.
DISCUSSIONS: The differential diagnosis of granulomatous disease
is large, and definitive diagnosis can be difficult. The comprehensive
initial evaluation of our patient revealed only non-caseating granulo-
mas, and the diagnosis of sarcoidosis was presumed after exclusion of
most alternate diagnoses. Tuberculoid leprosy was considered initially,
but was excluded based on negative microbiology and serology. A
caveat to the exclusion of tuberculoid leprosy is that although AFB
smears and Fite stain are highly specific, they are also insensitive,
being negative in at least 70% of all leprosy patients. As cell-mediated
immunity decreases, there is gradual progression to lepromatous
leprosy. The decreased cellular host response allows for proliferation
of Mycobacterim leprae bacilli and a high bacillary load in skin lesions,
which eventually yield positive AFB smear or Fite stain results. This is
a case of tuberculoid leprosy that was presumed to be neuro-cutaneous
sarcoid that progressed to lepromatous leprosy after many years of
immunosuppression.
CONCLUSION: The initial presentation of our patient with non-
caseating granulomatous patches of the skin and sensory loss, represented
tuberculoid leprosy, which progressed to lepromatous leprosy after years
of immunosuppression. Conversion to the latter form enabled the diag-
nosis to finally be made on biopsy. Our patient originated from an
endemic area and presented years after leaving the region. Though
leprosy is rare in the United States, this case emphases the importance of
recognizing the disease in appropriate populations. Clinicians need a
heightened awareness in order to diagnose this disease, which can cause
DISCLOSURE: Scott Shofer, Grant monies (from sources other than significant impairments if left untreated.
industry) This work was supported by grants from the National Institute REFERENCES:
of Environmental Health Sciences (ES12717, ES11961) and the National 1 Britton JW, Lockwood DN. Leprosy. The Lancet 2004;363:1209-19.
Institute of Allergy and Infectious Disease (AI58161, AI65837); Other 2 Lockwood DN. Leprosy elimination: a virtual phenomenon or a
The voriconazole to treat the patient discussed in the presentation was reality? BMJ 2002; 324:1516-18.
provided by Pfizer. DISCLOSURE: Geneva Tatem, None.

PAECILOMYCES: EMERGING FUNGAL PATHOGEN contaminated air exhaust systems, and in a bone marrow transplant unit
Catherine E. Grossman MD* Alpha Fowler MD Medical College of from sharing contaminated skin lotion. Paecilomyces has been also found
Virginia, Richmond, VA to contaminate prosthetic devices including lens and breast implants.
Prior reviews have noted this fungus to be resistant to some commercial
INTRODUCTION: Paecilomyces is a saprophytic fungus implicated sterilizing techniques, burgeoned by the fungus’ ability to thrive in a saline
in sporadic reports of serious infections involving almost all organ systems. environment and the p.variotti species being able to withstand very high
Mycetoma, empyema and pneumonias have been described. This case temperatures. The clinical risk factors for invasive paecilomyces infection
report describes an immunocompetent patient with a progressive lung are similar to that described for other fungal infections and center around
infection found at autopsy to have Paecilomyces variotii. immune compromise. Prosthetic devices also seem to carry risk.Several
CASE PRESENTATION: A forty nine year old woman who had been Paecilomyces species are known to produce human infections but by far
followed in our pulmonary clinic for emphysema presented in January the two most common are lilacinus and variotii. Antifungal susceptibilites
2004 with fatigue, fevers, sinus drainage and weight loss. She had reported vary significantly in the literature; susceptibility testing for each
undergone right upper and middle lobectomies for a non-resolving isolate found is supported by the literature.
pneumonia in 1993; pathology report from that operation revealed the CONCLUSION: Although bronchoscopy performed five months prior
absence of neoplasm or infection. She was a baker by trade, but had not to our patient’s death grew two colonies of paecilomyces, the resolution of
worked over the past two years. Radiographs revealed multiple cavitary symptoms on antibiotics for pneumonia during that period would favor
lesions in her right lower lobe with one cavity that had an air fluid level. colonization. The patient reported in this case was likely exposed to
She was admitted to the hospital, where bronchoscopy was performed and paecilomyces from her occupational history as a baker.Factors that may
she was started on broad spectrum antibiotics. Several weeks after her have led our patient to be at increased risk for infection were supplemen-
discharge two colonies of paecilomyces grew from bronchoscopy culture tal steroids for treatment of symptoms attributable to obstructive lung
- felt to be a contaminant as her symptoms and radiographs improved disease.
while on a six week intravenous antibiotic course. Symptoms of pneumo- DISCLOSURE: Catherine Grossman, None.
nia returned in June 2004. Radiographs revealed increasing cavitary
lesions in her right lung, and airspace disease in her left upper lobe.
Bronchoalveolar lavage returned clear fluid which was sent for culture- INFLAMMATORY PSEUDOTUMOR ASSOCIATED WITH MY-
.The patient was admitted to the hospital after the bronchoscopy for COBACTERIUM-AVIUM COMPLEX (MAC) INFECTION
increased work of breathing. On the second hospital day hypoxic respira- Vanessa A. Ribaudo MD* Mangala Narasimhan DO Samuel O. Acquah
tory failure and shock developed. Mechanical ventilation and resuscitative MD Beth Israel Medical Center, New York, NY
measures were initiated; high dose vasopressor was required, broad
spectrum antibiotics and amphotericin B were started. Care was with- INTRODUCTION: Pulmonary inflammatory pseudotumor is a rare
drawn by family request on intensive care unit day three after progressive lesion of the lung. It has been associated with EBV and HHV-8 but there
escalation of support and multiple organ system failure. Autopsy was not been a reported association with MAC infection.
performed. Cultures of her cavitary lung lesions, and her liver grew CASE PRESENTATION: A 64-year-old woman had a two-year
paecilomyces variotii. The amount of fungal forms seen on tissues from history of chronic productive cough and weight loss unresponsive to two
autopsy was overwhelming – but bronchoalveolar lavage cultures from her courses of treatment with a macrolide antibiotic. Chest radiographs over
final bronchoscopy remained sterile. the initial three-month evaluation period revealed a persistent infiltrate in
the superior segment of the right lower lobe, and a CT scan of the chest
revealed consolidation of the superior segment of the right lower lobe and
posterior segment of the right upper lobe. Transbronchial biopsy revealed
a dense inflammatory infiltrate with collagen fibrosis, proliferation of
lymphocytes and plasma cells consistent with inflammatory pseudotumor.
Culture of the specimen revealed numerous MAC organisms. Surgical
resection was recommended, but the patient refused. She was started on
rifabutin, ethambutol, and clarithromycin, with intention to reevaluate
after 3 months for surgical removal of the pseudotumor. The patient was
lost to follow up for 10 months; on return, she stated that her cough had
resolved while on the antibiotics, which she stopped taking after 3 months.
Following discontinuation of the antibiotics symptoms recurred. A fol-
low-up CT scan of the chest revealed a decrease in the size of the previous
right lung opacity. Surgery was again advised, and she was again lost to
follow-up for six months. At the time of the last admission, she was a thin,
CASE REPORTS
diaphoretic woman using accessory muscles of respiration. The pulse was
153/min, blood pressure of 118/78 mm Hg, respiratory rate 40/min, and
temperature of 39°C. Pulse oximetry showed an oxygen saturation of 84%
breathing room air. Coarse inspiratory rales were present bilaterally. A
chest radiograph revealed dense opacification of the right lung field,
sparing the apex, associated with shift of the mediastinum to the left. CT
scan revealed a large right lower lobe mass, with complete collapse of the
right lower and middle lobes. A moderate pleural effusion was present-
.The patient was admitted to the medical intensive care unit and started
on moxifloxacin for presumed pneumonia. On hospital day 2, endotra-
cheal intubation and flexible bronchoscopy were performed, revealing
obstruction of the right lower lobe superior segment and medial basal
segment bronchi by pearly white material that did not clear with
suctioning. Biopsy of one of those lesions showed inflammatory cells.
Methylprednisolone, 40 mg IV every 6 hours was started. Over the next
few days, oxygenation and the radiographic abnormalities improved. She
had a right thoracotomy on day 11, revealing for a large mass invading all
three lobes of the right lung. The mass was enucleated (along with a
DISCUSSIONS: Paecilomyces are soil saprophytes which are increas- portion of the chest wall), and the histology was consistent with an
ingly cited in cases of serious infections. This fungus is known to survive inflammatory pseudotumor. Giant cell granulomata with necrosis were
on a variety of surfaces/environments including plastics, saline, water also noted. Cultures of the lesion were MAC, and azithromycin, amikacin,
damaged wood, and decaying food matter. Paecilomyces is also found as rifampin and ethambutol were begun. After 50 days in the hospital, the
an airborne contaminant in graineries, sawmills, and water damaged patient was removed from the ventilator and discharged to a subacute
houses. Outbreaks of paecilomyces have been noted in dialysis units from rehabilitation unit. She was maintained on MAC treatment with complete
dialysate bags stored on water damaged wood, operating rooms from resolution of symptoms and the radiographic abnormalities.

single or multiple lesions, or involve the lung parenchyma as one or more

nodules. It only rarely causes diffuse infiltration of the lungs. Its presence
can easily be mistaken for other more serious etiologies as this case
illustrates.
CASE PRESENTATION: We present a 54 year old Caucasian female
diagnosed with infiltrative ductal carcinoma of the breast in 1997. She had
a lumpectomy with lymph node dissection which revealed two lymph
nodes positive for cancer. She received chemotherapy and hormonal
therapy. Radiation therapy was not given because of an underlying
diagnosis of SLE. She was diagnosed with SLE and Sjogren’s syndrome in
1990 at which time she had renal involvement and received cyclophos-
phamide for six months followed by azathioprine. More recently she has
been on methotrexate and hydroxychloroquine. She was referred to us
after a chest radiograph and CT of the chest showed right middle lobe
airspace disease and multiple nodules throughout both lung fields that
were highly suspicious for metastatic breast carcinoma. This was evaluated
previously at another hospital where she had a bronchoscopy that revealed
atypical cells. She subsequently underwent open lung biopsy, which
reported patchy lymphoid aggregates and chronic inflammation. We
obtained these biopsies taken from the right middle lobe and lower lobe.
The biopsies demonstrated a waxy nodular material. Alkaline Congo Red
stain of this material was positive for apple-green birefringence under
polarized light that was consistent with pulmonary nodular amyloidosis. It
also showed evidence of lymphoid hyperplasia and follicular bronchiolitis
consistent with her connective tissue disease. There were emphysematous
changes compatible with her past 30-40 pack year smoking history and
moderate airways obstruction noted on pulmonary function testing.
DISCUSSIONS: Amyloidosis is usually systemic in nature and when it
involves the lungs it is diffuse, infiltrative, and asymptomatic. It is can be
idiopathic in nature, hereditary, or associated with underlying inflammatory
or hematologic disorders such as myeloma. Pulmonary nodular amyloidosis,
though rare, is limited to the lungs. An extensive evaluation looking for other
predisposing causes for amyloidosis was negative, as was the evaluation
looking for other organ involvement, including core biopsy of the abdominal
fat pad. Pulmonary nodular amyloidosis is a rare condition, and even more
uncommonly it has been associated with Sjogren’s syndrome. The few reports
describe it as cystic changes, lymphoplasmocytic infiltrates, or irregular
noncavitary nodules. In this particular case, the appearance was compatible
with metastatic disease as well as a possible drug reaction.
CONCLUSION: Pulmonary nodular amyloidosis can mimic other
more serious entities such as metastatic breast cancer as in this case. It can
be missed on histopathology unless it is considered in the differential and
special stains are performed. The nodular distribution suggests it is not
secondary to the often more life threatening causes of systemic amyloid-
osis which include malignant disorders. This patient’s Sjogren’s syndrome
was the most likely condition that predisposed her to develop this rare
complication of this disease.
DISCLOSURE: Maria Carrillo, None.
DISCUSSIONS: Inflammatory pseudotumor is a rare lesion of un-
known etiology, characterized by proliferation of lymphocytes, plasma
cells, neutrophils and fibroblasts forming mass lesions. Some cases have
been confused with malignancy. In our case, the persistent positive LYMPHOCYTIC INTERSTITIAL PNEUMONIA (LIP) AND HU-
cultures for MAC and the initial regression on antimicrobial treatment MAN IMMUNODEFICIENCY VIRUS (HIV) IN THE MODERN
suggests that MAC infection was related to the development of the ERA
pseudotumor. Michelle Cao DO* Malini Soogoor MD Janine Vintch MD Harbor/UCLA
CONCLUSION: To our knowledge this is the first case of inflamma- Medical Center, Torrance, CA
tory pseudotumor associated with MAC infection.
DISCLOSURE: Vanessa Ribaudo, None. INTRODUCTION: Lymphocytic interstitial pneumonitis (LIP) is
classified under the idiopathic interstitial pneumonias. The incidence of
LIP is uncommon, mainly seen in immunocompromised patients espe-
cially infection with human immunodeficiency virus (HIV), and those with
connective tissue disease. We describe a young patient with HIV who
developed LIP with subsequent complete clinical and radiographic
resolution of the disease shortly after initiation of highly active antiretro-
It Is a Dangerous World viral therapy (HAART).
CASE PRESENTATION: A 29 year-old homosexual Hispanic male
4:15 PM - 5:45 PM with newly diagnosed HIV infection presented with 8 months of progres-
sive shortness of breath, nonproductive cough, 35-pound weight loss, and
PULMONARY AMYLOIDOSIS MIMICKING METASTATIC fevers. He grew up in southern California, denied recent travel, did not
BREAST CANCER IN A PATIENT WITH CONNECTIVE TISSUE own pets, and was not taking any medications. He denied use of
DISEASE recreational drugs or alcohol. On presentation he was febrile, hemody-
Maria Carrillo MD* Mihela Sescioreanu MD Zachary Q. Morris MD namically stable, room air pulse-oximetry was 90%. Physical exam was
Henry Ford Hospital, Detroit, MI remarkable for thrush, bibasilar crackles, and 2cm raised violaceous
lesions on chest wall and back. Laboratory values revealed a normal white
INTRODUCTION: Unlike systemic amyloidosis, when amyloidosis is blood cell count, hypoalbuminemia, lactate dehydrogenase of 365, and a
localized to the lungs, this uncommon condition usually involves the lungs CD4 count of 9. Arterial blood gas on room air revealed PaO2 of 64. Chest
in one of two ways. In can be confined to the tracheobronchial tree as CT was remarkable for bilateral patchy areas of ground-glass opacities and

It Is a Dangerous World, continued
nodularity most notable in left lower lobe. Sputum for acid-fast bacilli and veolar lavage showed no pathogens by stain or culture but revealed 80%
fungal serologies including coccidioidomycosis, histoplasma, and cryptococ- eosinophils. Duloxetine was discontinued and methylprednisolone started.
cus were negative. Bronchoscopy findings revealed normal airways, no Antibiotics and vasopressors were discontinued after two days; the patient
endobronchial lesions, and a bland bronchoalveolar lavage (BAL) fluid with was extubated in three. She developed peripheral eosinophilia while still
negative findings for infection or malignancy. Antibiotics as well as empiric receiving methylprednisolone. She was discharged after eight hospital
treatment for pneumocystis carinii pneumonia (PCP) with trimethoprim- days on prednisone, fluoxetine, and gabapentin. As an outpatient, eosin-
sulfamethoxazole and prednisone were initiated. Treatment for PCP was ophilia resolved then recurred. Eosinophilia finally normalized after
stopped after 5 days with negative BAL results. There was no improvement fluoxetine was discontinued. Three weeks after discharge, repeat chest
in his symptoms while on antibiotics. A repeat bronchoscopy with transbron-
x-ray was normal and she was doing Pilates.
chial biopsy revealed nonspecific interstitial pneumonitis. The patient subse-
quently underwent a video-assisted thoracoscopic (VATS) lung biopsy that DISCUSSIONS: Acute eosinophilic pneumonia was first described as
revealed lymphocytic interstitial pneumonitis. He was started on HAART “double pneumonia” with respiratory failure.[3] The AEP diagnostic crite-
with complete resolution of his clinical and radiographic findings. ria[4] are: (1) Acute febrile illness ⬍ 5 days duration, (2) Hypoxemic
DISCUSSIONS: LIP, although common in HIV children, is rare in the respiratory failure, (3) Diffuse alveolar or mixed alveolar-interstitial chest
adult HIV population. It is an AIDS defining illness in children ⬍ 13 years of X-ray infiltrates, (4) BAL eosinophils ⬎ 25%, (5) Absence of parasitic, fungal,
age. LIP occurrences in HIV are more common in men, African American, or other infection, (6) Prompt and complete response to corticosteroids, (7)
Afro-Caribbean, and homosexuals. It is rare in Caucasians. There is no Failure to relapse after discontinuation of corticosteroids. This case fulfills
relationship with CD4 count. There is suggestion of an association with these criteria, with duloxetine and secondarily fluoxetine the inciting agents.
Epstein Barr virus (EBV) and HIV infection itself inducing LIP. Symptoms Medications are well known causes of eosinophilic pneumonia.[5] Venlafaxine
include nonproductive cough, progressive dyspnea on exertion, fevers, weight was reported as an inciting agent in 2000.[6] Duloxetine and venlafaxine are
loss, and fatigue. Some patients are asymptomatic or have minimal symptoms. similar in mechanism and pharmacodynamics.[7].
Duration of symptoms can range from several months to several years. Lung CONCLUSION: This may be the first report linking duloxetine with
exam shows inspiratory crackles, wheezing, decreased breath sounds, in other AEP. Cessation of exposure and corticosteroid treatment produced
cases minimal physical findings are seen. Chest radiography and chest CT prompt and complete resolution of severe respiratory failure. The diag-
show predominant interstitial pattern with bilateral reticular and ground glass nosis of AEP was made only by bronchoalveolar lavage, emphasizing the
opacities, and centrilobular nodules. In advanced cases CT shows bronchiec- importance of bronchoscopy in selected patients despite the risk imposed
tasis and fibrosis. Pulmonary function tests show restriction and loss of
by severe hypoxemia.
diffusing capacity. No clinical or laboratory findings are characteristic for LIP,
tissue biopsy is ultimately required for diagnosis. Treatment is anecdotal; REFERENCES:
there are no controlled trials. Cyclophosphamide, chlorambucil, and azathio- 1 Buddharaju VL et al, Acute eosinophilic pneumonia associated with
prine have been tried with varying results. Highly active antiretroviral therapy shock, Crit Care Med 1999;27(9):2014-2016.
(HAART) with or without corticosteroids has been successful in very small 2 Badesch DB et al, Acute eosinophilic pneumonia: a hypersensitivity
studies. The dose of corticosteroids and duration of use are variable. HAART phenomenon? Amer Rev Respir Dis 1989;139:249-52.
therapy has been used alone with success in case reports, there is no clear-cut 3 Ibid.
evidence for non-nucleoside or protease inhibitor based regimens. 4 Allen JN and Davis WB, Eosinophilic Lung Diseases, Am J Respir
CONCLUSION: This case demonstrates successful treatment of lym- Crit Care Med 1994;150:1423-1438.
phocytic interstitial pneumonia with HAART in an individual newly 5 http://www.pneumotox.com/indexf.php?fich⫽clin0&lg⫽en
infected with HIV with complete clinical and radiographic resolution. 6 Fleisch MC et al, Eosinophilic pneumonia and respiratory failure
Further studies are needed to define the role of HAART with or without associated with venlafaxine treatment, Eur Respir J 2000;15:205-208.
steroids in the treatment of LIP for patients infected with HIV. 7 Sharma A et al, Pharmacokinetics and Safety of Duloxetine, a
DISCLOSURE: Michelle Cao, None. Dual-Serotonin and Norepinephrine Reuptake Inhibitor, J Clin
Pharmacol 2000;40:161-167.
DISCLOSURE: Antonio Salud II, None.
TOO MUCH OF A GOOD THING: ACUTE EOSINOPHILIC
PNEUMONIA WITH A “NEW” ANTI-DEPRESSANT
Antonio V. Salud II MD* Nathan Dean MD University of Utah, Salt Lake APPARENT LIFE THREATENING EPISODE: THE EARLIEST PRE-
City, UT
SENTATION OF NEUROENDOCRINE CELLS HYPERPLASIA
INTRODUCTION: The incidence of acute eosinophilic pneumonia Rasik V. Shah MD* Claire Langston MD Scott Schroeder MD Winthrop
(AEP) among patients admitted with presumed community acquired University Hospital, Mineola, NY
pneumonia is uncertain, but AEP associated with shock is rare.[1]
CASE REPORTS
Medications can cause AEP with the pathophysiology attributed to acute INTRODUCTION: We present a child whose symptoms began as an
hypersensitivity.[2] We report AEP possibly caused by duloxetine. apparent life threatening episode (ALTE), initially thought to be second-
CASE PRESENTATION: A 30 year old Caucasian woman was ary to gastroesophageal reflux but over time, she developed persistent
admitted with five day history of fever, chills, dyspnea, cough, and nausea. tachypnea and hypoxemia and she was ultimately found to have neuroen-
She was diagnosed with pneumonia by chest x-ray and prescribed docrine hyperplasia of infancy (NEHI).
antibiotics one day prior to admission. Past medical history was significant CASE PRESENTATION: A 9 week old girl was admitted for evalu-
for depression and chronic prescription narcotic use secondary to back ation of an ALTE. Her birth history, review of systems, family and
pain. She was recently institutionalized for depression, weaned off environmental histories were non-contributory to her present illness.
narcotics, and prescribed duloxetine. Medications on admission were During her evaluation, her lung fields were hyperinflated and she had
azithromycin, duloxetine, vitamin B12, multivitamin, and garlic. She radiographic evidence of increased bronchovascular marking and a barium
denied tobacco use but drank alcohol heavily prior to hospitalization for esophogram demonstrated gastroesophageal reflux. She was discharged
depression. She had no history of IV drug abuse or HIV exposures. She home on oral ranitidine. Over the next three months, she developed
denied occupational, chemical, dust, or asbestos exposures. She had a persistent wheezing and tachypnea which worsened despite aggressive
parrot for five years, but no recent travel. On admission, the patient was outpatient supportive management. She was readmitted and since an
in “mild distress” with temperature of 39.5°C, blood pressure 95/55
extensive work up was negative the decision was made to obtain an open
mmHg, heart rate 144 bpm, and respiratory rate 20. Her SpO2 was 90%
on room air. Lung exam revealed diffuse crackles. She had 20.4 103/uL lung biopsy. Routine histopathology and microbiology were non-conclu-
white blood cells with 86% segmented neutrophils. Comprehensive sive but immunohistochemical staining with bombesin showed the pres-
metabolic panel and lactate were within normal limits. Admission ABG ence of increased neuroendocrine cells in the bronchial epithelium,
showed pH 7.44, PaCO2 30 mmHg, PaO2 49 mmHg and SaO2 84%. confirming the diagnosis of NEHI (figure 1). She was treated with
Blood cultures were ordered. The admission chest x-ray showed worsen- intravenous methylprednisone 30 mg/kg once daily for three days every
ing consolidation bilaterally. She was placed on 4 liters oxygen, and month for six months and hydroxychloroquine (10mg/kg/day) orally daily
administered ceftriaxone combined with azithromycin. The following day, for six months with good response. She is now 30 months old, growing and
her clinical course deteriorated with development of respiratory failure developing normally, she takes inhaled budesonide twice daily and she has
and shock. She was intubated and treated with vasopressors. Bronchoal- needed two courses of oral corticosteroids in the last year.

pathic or occur in association with systemic disorders such as connective tissue

diseases or immunodeficiency syndromes. Relatively little is known regarding
prognostic implications and treatment of this disorder. Herein, we report a case
of successful treatment of this condition with a macrolide antibiotic.
CASE PRESENTATION: A 45 year old ex-smoker who initially presented to
her primary care physician with shortness of breath. She was treated at that time
for a presumed chest infection, but had no clinical improvement. Subsequently,
pulmonary function studies revealed a reduced diffusing capacity and oxygen
desaturation with exercise. A CT scan demonstrated a mosaic attenuation pattern.
Bronchoscopy was non-diagnostic with negative cultures. She experienced symp-
tomatic improvement with prednisone treatment but her dyspnea and fatigue
worsened again as the prednisone dose was tapered. She had no significant
environmental or occupations exposures. She had no clinical or laboratory
features to suggest an underlying systemic connective tissue disorder or immu-
nodeficiency syndrome. Surgical lung biopsy revealed histopathologic features of
follicular bronchiolitis. Clinically, she had done relatively well, but was experienc-
ing the side effects of chronic corticosteroid therapy including weight gain and
DISCUSSIONS: This case describes the presentation of an ALTE that hyperglycemia. A trial of azithromycin was initiated along with further tapering of
evolved into an interstitial lung disease (ILD) that was ultimately diagnosed as the prednisone dose. After 3 months of azithromycin therapy, she was noticing
NEHI. Although her initial symptoms were characteristic of an ALTE, her initial improvement in her respiratory symptoms. After one year of therapy, she had
chest radiograph had some subtle signs suggestive of an interstitial process. When normalization of her oxygen saturation with exercise and slight improvement of
she developed tachypnea and hypoxemia that was only corrected by supplemen- the diffusing capacity.
tal oxygen therapy, a computerized tomographic (CT) scan of the chest (Figure DISCUSSIONS: Follicular bronchiolitis is an uncommonly form of bron-
2) showed bilateral ground glass opacities, confirming our suspicions of ILD. In chiolar lesions that can be idiopathic or occur in association with connective-tissue
pediatrics, acute interstitial lung diseases are usually due to viral pneumonias and disorders – particularly rheumatoid arthritis, immunodeficiency syndromes in-
less commonly, pulmonary hemorrhage and edema. This child had repeated cluding AIDS, pulmonary infections, or ill-defined hypersensitivity reactions.
negative cultures for viruses and other microorganisms and her echocardio- Patients usually present with progressive dyspnea and variable pulmonary func-
graphic evaluation was normal. Since her symptoms persisted and the interstitial tion abnormalities have been reported, including obstructive, restrictive, and
changes on her chest radiograph did not improve, a lung biopsy was necessary for mixed patterns. The predominant finding on chest radiography is bilateral, small
diagnosis and determination of the best therapy.Neuroendocrine (NE) cells are nodular, or reticulonodular infiltrates with intrathoracic adenopathy, though
granulated epithelial cells that produce bioactive products such as bombesin, occasionally chest radiographs may look normal. The cardinal features of follicular
serotonin and calcitonin that are capable of causing bronchoconstriction, vasoac- bronchiolitis on HRCT consist of bilateral and diffusely distributed centrilobular
tivity, epithelial differentiation and smooth muscle alteration. NE cells appear in nodules measuring 1 to 12 mm in diameter, variably associated with peribronchial
developing conducting airways of fetuses by 10 weeks’ gestation and increase in nodules and patchy areas of ground-glass opacity. Treatment is generally directed
number primarily in bronchioles as gestation progressed. They are felt to play a to the underlying disease when such association is recognized. Those patients with
role in lung development and gradually disappear by 12 months of age. NE cells no identifiable underlying cause have generally been treated with bronchodilators
and bodies hyperplasia in the lungs have been reported in patients with SIDS, and corticosteroids. More recently, Hayakawa and colleagues reported on the
BPD, cystic fibrosis, prolonged ventilation, congenital diaphragmatic hernia, and therapeutic benefits of chronic erythromycin therapy in patients with follicular
congenital central hypoventilation syndrome. There are recent reports of patients bronchiolitis associated with rheumatoid arthritis. Based on this preliminary data
with persistent tachypnea whose lung biopsies showed NE cell hyperplasia. Our our patient was also treated with chronic macrolide therapy and had experienced
patient had a negative sweat test, normal alveolar ventilation studies and abnormal improvement. Macrolide antibiotics possess non-bactericidal immunomodulatory
chest radiograph. She has no identifiable cause for NE cells and bodies properties that include inhibiting inflammatory cell chemotaxis, cytokine synthe-
hyperplasia. The mainstay of treatment for ILD of unknown origin is glucocor- sis, adhesion molecule expression, and reactive oxygen species production.
ticosteroids and/ or hydroxychloroquine. She responded well to these treatments CONCLUSION: Follicular bronchiolitis is a bronchiolar lesion that can be
and now she is asymptomatic. idiopathic or occur in association with systemic disorders. Relatively little is known
about the treatment or prognostic implications of this disorder. Our case suggests
that chronic macrolide therapy may be useful in the treatment of follicular
bronchiolitis in the non-rheumatoid population.
REFERENCES:
1 Am J Respir Crit Care Med. 2003 Dec 1;168(11):1277-92.
2 Howling SJ, et al. Follicular bronchiolitis: thin-section CT and
histologic findings. Radiology. 1999;212:637-642.
3 Romero S, et al. Follicular bronchiolitis: clinical and pathologic
findings in six patients. Lung. 2003 181:309-319.
4 Hayakawa H et al. Bronchiolar disease in rheumatoid arthritis. Am J
Respir Crit Care Med 1996 154(5):1531–1536.
DISCLOSURE: Michelle Aerni, None.
ROTAVIRAL RNA IN TYPE II PNEUMOCYTES AND ALVEOLAR

MACROPHAGES IN A PATIENT WITH ACUTE INTERSTITIAL
CONCLUSION: In an infant with ALTE and lower respiratory tract PNEUMONIA
symptoms of undefined etiology with progressive deterioration, NEHI and other Martin A. Valdivia-Arenas MD* Cynthia M. Magro MD Nuovo J. Gerard
interstitial lung diseases should be included in the differential diagnosis since MD Clay B. Marsh MD John G. Mastronarde MD The Ohio State
there are potential therapies available and NEHI has a good prognosis. University, Columbus, OH
DISCLOSURE: Rasik Shah, None.
INTRODUCTION: The etiology of acute interstitial pneumonia (AIP)
remains elusive since its original description. It has only recently described
SUCCESSFUL TEATMENT OF FOLLICULAR BRONCHIOLITIS that rotavirus may cause interstitial pneumonia. We describe a case of fatal
WITH MACROLIDE acute interstitial pneumonia in which rotaviral RNA was isolated by reverse
Michelle R. Aerni DO* Robert Vassallo MD Jay H. Ryu MD Mayo Clinic, transcription in-situ-PCR in alveolar macrophages and pneumocytes.
Rochester, MN CASE PRESENTATION: A 57-year-old white female with a 3-week
history of progressive dyspnea on exertion preceded by 2 days of diarrhea and
INTRODUCTION: Follicular bronchiolitis is a bronchiolar lesion character- malaise. Her medical history was significant for ischemic cardiomyopathy and
ized by the presence of hyperplastic lymphoid follicles with reactive germinal diabetes. She denied alcohol, illegal drugs or cigarrete smoking.Vitals on
centers distributed along bronchovascular bundles. This disorder can be idio- admission: respiratory rate 22 breaths/min, blood pressure 105/54 mmHg,

heart rate 75 beats/min temperature 96.8 F, O2sat 98% room air. There was had an initial clinical improvement, however, three weeks prior to admission
no jugular venous distention. She had bilateral coarse crackles. Heart exam his symptoms recurred despite compliance with his medical regimen. Med-
disclosed regular rate and rhythm. There was no hepato-splenomegaly. She ical history included multiple myeloma (IgA lambda) diagnosed 4 months
had bilateral lower extremity edema. Laboratory data: WBC 8,500 cells/ul, prior and treated initially with doxorubicin, prednisone, and vincristine;
hemoglobin 10 mg/dl, arterial blood gas on room air revealed a pH of 7.49, coronary artery disease status post bypass grafting; hypertension; and diastolic
pCO2 37 mmHg, pO2 74 mmHg. Chest X-ray showed interstitial pattern and dysfunction. Medications included thalidomide, metoprolol, furosemide, and
bilateral pleural effusions.After a 6-day therapy with furosemide and empiric erythropoietin. Social history was notable for previous tobacco use. Physical
antibiotics her hypoxemia progressed. All subsequent cultures remained exam revealed a chronically ill appearing man who was in no acute distress.
negative. A chest CT showed thickening of both inter and intralobular septa. Respirations were unlabored and his oxygen saturation was 92% on room air.
A bronchioalveolar lavage showed 71% alveolar macrophages, 27% neutro- Breath sounds were decreased on the right with dullness to percussion and
phils, 2% lymphocytes and negative bacterial, fungal and viral cultures. decreased tactile fremitus. Wheezing was also noted intermittently. Cardiac
Autoimmune panel was negative.The open lung biopsy showed extensive exam was unremarkable. Initial laboratory exam revealed a WBC 1.3k,
acute interstitial pneumonitis in an organizing fibroblastic phase with pre- Hemoglobin of 7.9 g/dL, and platelets of 32k. Chest radiograph showed a
dominant type II pneumocyte hyperplasia. Reverse transcriptase in-situ-PCR right pleural effusion that had increased in size on serial films. Non-contrast
studies were negative for adenovirus, Epstein-Barr virus, cytomegalovirus and CT of the chest revealed a large loculated right pleural effusion with
herpes virus but strikingly positive for rotavirus RNA in macrophages and associated lower lobe atelectasis, and extrinsic compression of the trachea
pneumocytes. After the biopsy the patient remained hypoxemic and despite (Figure 1). Ultrasound guided thoracentesis revealed bloody fluid, with WBC
treatment with high dose steroids she expired. 1.5K (differential 88% plasma cells and 6% neutrophils), an LDH of 221 U/L
DISCUSSIONS: Acute interstitial pneumonia is characterized by diffuse (80% of serum value), a protein of 7.4 g/dL (68% of serum), and glucose of
alveolar damage similar to the Acute Respiratory Distress Syndrome. It has an 157 mg/dL. Due to the possibility of airway compression and the atypical
initial exudative phase that progresses to a proliferative phase within one pleural effusion, the patient underwent bronchoscopic airway inspection and
week. As in the majority of reported cases, our patient’s biopsy showed video assisted thorascopic surgery. Findings included mild external compres-
prominent type II pneumocyte proliferation. Although AIP is idiopathic a sion of the trachea, and firm, round nodules throughout the pleural surface.
number of clinical situations such as infections, drugs, connective tissue Tumor was debulked and talc pleurodesis was performed. Pathology revealed
diseases, and vasculitides may cause a pattern of diffuse alveolar damage atypical plasma cells consistent with myeloma (Figure 2). Due to the failure
similar to AIP. Viral infections may cause pneumonitis, however there have of previous chemotherapy regimen, the patient elected to forgo additional
been only two reported cases of fatal pneumonits in which rotaviral RNA was treatments and received palliative care. He expired at home 6 weeks later.
isolated in the lungs. One had a similar clinical presentation, an upper
respiratory illness that progressed to a fatal respiratory failure over several
weeks. His biopsy showed acute interstitial pneumonitis in the proliferative
phase, and rotaviral RNA was localized in alveolar macrophages and pneu-
mocytes. The other reported patient had a more acute course and he died 2
days after admission. His autopsy showed marked septal capillaritis and
prominent denudement of the alveolar lining. Viral RNA was found in
endothelial cells, pneumocytes and alveolar macrophages. Although all
cultures were negative for viruses a recent study demonstrated that RT-PCR
was more sensitive identifying viruses causing lower respiratory infections
compared with the traditional cell culture methods. In our patient the
presence of viral RNA most likely represents an acute infection, as rotavirus
does not cause latent infections.
CONCLUSION: The strong presence of rotaviral RNA in alveolar macro-
phages and pneumocytes suggests its pathogenic role in our patient’s AIP.
However the virus could only be an innocent bystander or a contributing factor
in the progression to respiratory failure in a patient with established idiopathic
interstitial pneumonia. More studies using RT in- situ-PCR would be helpful to
understand the roll of viruses in the pathogenesis of AIP.
REFERENCE:
1 Nuovo GJ, Owor G, Andrew T, Magro C. Histologic distribution of
fatal rotaviral pneumonitis: an immunohistochemical and RT in-situ-
PCR analysis. Diagn Mol Pathol 2002; 11:140-5
DISCLOSURE: Martin Valdivia-Arenas, None.
CASE REPORTS
Pleural Disease I
4:15 PM - 5:45 PM
MYELOMATOUS PLEURAL EFFUSION AND AIRWAY COM-
PRESSION COMPLICATING MULTIPLE MYELOMA
Steven M. Rowe MD* University of Alabama at Birmingham, Birmingham, AL
INTRODUCTION: Myelomatous pleural effusion is a very rare DISCUSSIONS: Multiple myeloma is a neoplastic disorder caused by
manifestation of multiple myeloma, and represents the small minority of the proliferation of a single plasma cell clone, and is associated with the
pleural effusions associated with this disorder. Pleural myeloma has also production of monoclonal immunoglobulin. Pleural effusions occur in
rarely been associated with mediastinal involvement of malignant plasma approximately 6% of patients with myeloma due to a variety of causes;
cells. We report a case of pleural myeloma associated with mediastinal however, myelomatous pleural involvement occurs in less than 1% of
disease and extrinsic compression of the airway. cases. Mediastinal involvement is also quite rare, and may be the source
CASE PRESENTATION: A 72 year-old white male with a history of of pleural disease. As in this case, the majority of myelomatous effusions
coronary artery disease, hypertension, and multiple myeloma was in his usual are due to those that produce IgA, as this type tends to invade extraosse-
state of health until eight weeks prior when he developed slowly progressive ous structures. Diagnosis requires immunoelectrophoresis of the pleural
dyspnea, orthopnea, and wheezing. He denied fever, chills, cough, or chest fluid or histologic confirmation. Treatment is directed at the underlying
pain. Four weeks prior he was hospitalized for pneumonia and anemia, and disease, but may also include pleurodesis for symptomatic control and
treated with levofloxacin, corticosteroids, and packed red cell transfusions. He appropriate airway management.

Pleural Disease I, continued
CONCLUSION: Myelomatous pleural effusion with mediastinal in-

volvement is a rare manifestation of multiple myeloma, and may mimic
infectious complications of the disease. This entity should be considered
in those with advanced disease.
REFERENCES:
1 MG Alexandrakis, FH Passam, DS Kyriakou, D Bouros. Pleural
Effusions in Hematologic Malignancies. Chest 2004; 125:1546 –52.
2 JS Kintzer, EC Rosenow, RA Kyle. Thoracic and pulmonary abnor-
malities in multiple myeloma. Arch Intern Med 1978; 138:727–73.
DISCLOSURE: Steven Rowe, None.
TENSION PNEUMOTHORAX FOLLOWING COLONSCOPY

Brian R. Zeno MD* Steven Sahn MD Medical University of South
Carolina, Charleston, SC Table 1
INTRODUCTION: Little has been published on the thoracic compli- Pneumomedi-
cations following bowel perforation with colonoscopy. We report a woman astinum
with tension pneumothorax following this procedure.
Author Year Sex Age Mechanism of injury present
CASE PRESENTATION: A 64-year-old woman presented to the
emergency department with severe lower abdominal pain with nausea and Meyers 1975 M 68 polypectomy yes
vomiting for one day. Her pain was intermittent and described as sharp
and cramping; the vomitus was not bilious or bloody. During her initial Thomas* 1979 F 47 biopsies of transverse yes
evaluation, she was found to have a large fecolith in her sigmoid colon with colon
multiple air fluid levels, likely as a result of mechanical obstruction. A Schmidt 1986 F 59 possibly biopsy of ileum yes
colonoscopy was attempted to remove the fecolith. During the introduc- Ho* 1996 M 68 polypectomy yes
tion of the colonoscope, the patient developed an acute worsening of her Tam 1996 F 65 polypectomy no
abdominal pain. The procedure was immediately terminated, and the
Webb* 1998 F 72 diagnostic colonoscopy yes
patient was moved to the holding room. The patient had a BP of 170/90
and an O2 saturation of 85-90% on a 100% non-rebreather. An acute Baumann 1999 M 65 diagnostic colonoscopy no
abdominal series revealed a large, right-sided pneumothorax with con- Hearnshaw 2003 F 80 possibly spontaneous no
tralateral shift (Figure 1). The left lateral decubitus film revealed a large pneumothorax
amount of free intraperitoneal air (Figure 2).A small bore chest tube was
subsequently placed with partial resolution of the pneumothorax prior to *Denotes bilateral pneumothoraces
having an emergent laparotomy. At surgery, she was found to have a large, DISCLOSURE: Brian Zeno, None.
luminal defect in her sigmoid colon. The patient required a left hemico-
lectomy with ostomy diversion. She had a prolonged postoperative course
and was discharged following 16 days of hospitalization. CHYLOTHORAX AND HYPERPLASTIC MESOTHELIAL CELLS
DISCUSSIONS: Colonic perforation is a rare but serious complication of ASSOCIATED WITH OVARIAN HYPERSTIMULATION SYN-
colonoscopy, occurring in 0.14% to 0.2% of diagnostic colonoscopies with a DROME
rate of up to three times that for therapeutic colonoscopies. This may be Shirley F. Jones MD* Robert I. Garver MD Jennifer J. Davis MD Jeffrey
caused by direct manipulation, use of electrocautery devices, or excessive Reid MD Unviersity of Alabama at Birmingham, Birmingham, AL
intraluminal pressure from colonic insufflation. Depending on the site and
mechanism of injury, intraluminal air may escape into either the peritoneum INTRODUCTION: Ovarian hyperstimulation syndrome (OHSS) is an
increasingly recognized complication associated with in vitro fertilization
or retroperitoneum. Once air escapes from the bowel, it may induce a
(IVF). Hyperplastic mesothelial cells in lymph nodes is a rare entity often
pneumothorax through a variety of mechanisms. First, gas may traverse from simulating metastatic carcinomas. We report a case of chylothorax, medias-
the peritoneum through small fenestrations in the diaphragm and enter the tinal mass, and hyperplastic mesothelial cells associated with OHSS.
pleural space along a pressure gradient. Aside from minute diaphragmatic CASE PRESENTATION: A 34 year old white female with history of
fenestrations, there is a subset of patients who have undiagnosed diaphrag- infertility and polycystic ovarian syndrome (PCOS) presented with 2 week
matic defects which allow the transmission of air also via a pressure gradient. history of increasing dyspnea, orthopnea, and decline in activity. In Decem-
Depending on the site of injury, it is also possible for air to enter the ber 2004 she underwent successful IVF with subsequent twin intrauterine
retroperitoneum. When this occurs, a direct communication exists to the pregnancy. Shortly afterwards, she was admitted for OHSS and massive
mediastinum; a pneumomediastinum can lead to a pneumothorax when the ascites requiring paracentesis. She complained of dyspnea which resolved
mediastinal parietal pleura ruptures. This mechanism may also account for with treatment. She had returned to her usual state of health until 2 weeks
the advent of bilateral pneumothoraces and also may predispose a patient to prior to admission at our hospital when she noted pain, redness, and swelling
pneumopericardium. To date, there have been only eight reported cases of of her left arm. She suspected a spider bite but did not seek medical care. Her
pneumothorax resulting from colonoscopy. These are listed in Table 1. Of the complaints resolved however were followed by respiratory problems. Perti-
reported cases, it appears that the majority occur via air dissection through the nent exam findings included tachycardia, tachypnea, and hypoxia. There were
mediastinum, presumably from the retroperitoneum. diminished breath sounds, dullness to percussion, and decreased tactile
CONCLUSION: Although there is a relative paucity of reports of fremitus over the left hemithorax. Laboratory evaluation was notable for
leukocytosis and anemia. A chest radiograph showed a large left pleural
iatrogenic pneumothorax following colonoscopy, this potential complica-
effusion. A thoracentesis was performed with drainage of milky white pleural
tion exists whenever a colonic perforation occurs given the relatively high fluid consistent with chylothorax. She was transferred to our facility where a
pressure system of the colon during insufflation and the negative pressure left thoracostomy tube was placed. A contrasted CT of the chest showed
of the pleural space. Consequently, during a problematic colonoscopy bilateral upper extremity venous thrombi, left axillary adenopathy and fluid
with the use of high insufflation pressures, the possibility of colonic attenuated structures located in the prevascular mediastinum. These were
rupture and its consequence, free air in the abdomen, needs to be suggestive of adenopathy, but were not located along the thoracic duct. A left
investigated. The clinician needs to be cognizant that, on occasion, a thoracotomy with lymph node sampling was done. This showed large
pneumothorax under tension may develop with potentially serious conse- numbers of proliferating well differentiated mesothelial cells within the sinuses of
quences. the lymph nodes, a rare entity known as hyperplastic mesothelial cells.

DISCUSSIONS: Hyperplastic mesothelial cells in lymph nodes have examination showed the tumor to consist almost entirely of malignant
been described mainly in case reports. They have been identified in osteoid, with only a few small foci of undifferentiated spindle cell tumor.
mediastinal lymph nodes associated with pericardial and pleural effusions DISCUSSIONS: Four histologic mesothelioma subtypes exist (WHO
(1,2). Their presence can simulate malignancy by demonstrating atypical 2004 classification): epithelioid, sarcomatoid, desmoplastic and biphasic.
cytological and architectural features(2). We postulate the hyperplastic The sarcomatoid type, seen in 15% of cases, is characterized pathologically
cells resulted in the formation of her chylothorax, although we are by spindle-shaped cells similar to those seen in fibrosarcomas. Rarely, foci
unaware of any previously reported cases. OHSS could also be an etiology of cartilagenous and/or osseous differentiation, as in our case, are seen.
as well. The prevalence of moderate to severe OHSS ranges from 1-10% The extent of osseous metaplasia here is exceptional. The principal
in major IVF programs (3). Risk factors include young age, low body mass differential diagnostic considerations are primary sarcoma of the pleura
index, history of PCOS, atopy, and the use of GnRH antagonists in IVF(4). and extension of a sarcoma of the rib or a sarcomatoid carcinoma of the
Clinical features include ascites, pleural and pericardial effusions. Patients lung into the pleural space. Keratin expression on immunohistochemical
complain of nausea, vomiting, diarrhea, dyspnea, and abdominal discom- study will usually be sufficient to exclude the first two of these. However,
fort. Lab abnormalities can include hyponatremia, hyperkalemia, a small peripheral pulmonary tumor may show an immunohistochemical
hemoconcentration, leukocytosis and abnormal liver function tests. Re- profile similar to sarcomatoid mesothelioma and also may be difficult to
spiratory distress can ensue and is due to ascites, pulmonary edema, and detect by radiologic imaging. Definitive exclusion of such a lung primary
hemorrhage. OHSS is also associated with thromboembolic disease and may require autopsy examination.
sepsis. Treatment is supportive. The goal is to maintain intravascular CONCLUSION: Osteosarcomatous differentiation of mesothelioma is
volume and systemic perfusion. Paracentesis, thoracentesis and anticoag- rare and the diagnosis can be challenging. While the sarcomatoid type
ulation can be done in these patients. mesothelioma portends a poorer prognosis in general, it is unclear if
CONCLUSION: As the number of patients undergoing assisted fertiliza- osteosarcomatous differentiation heralds an even worse outcome.
tion techniques increase, physicians should recognize the clinical manifesta- REFERENCES:
tions of OHSS. Complications can lead to significant morbidity and mortality 1 Antman KH. Natural history and epidemiology of malignant me-
whereas the presence of hyperplastic mesothelial cells can also provide a sothelioma. Chest 1993;103(4Suppl):373S-376S
diagnostic challenge in the diagnosis of underlying malignancy. 2 Raizon A, et al Calcification as a sign of sarcomatous degeneration
REFERENCES: of malignant pleural mesotheliomas: new CT finding. Journal of
1 Argani P, Rosai J. Hyperplastic mesothelial cells in lymph nodes. Coumputed Assisted Tomography. 1996;20:42-44.
Human Pathology 1998;29:339-346. 3 Quoix E et al. A left pleural effusion with a calcified tumoral mass and left
2 Isotalo PA, Veinot JP, Jabi M. Hyperplastic Mesothelial Cells in hemithoracic uptake on bone scan. Lung Cancer 2001;32:203-305.
Mediastinal Lymph Node Sinuses with Extranodal Lymphatic In- 4 Pistolesi M, Rusthoven J. Malignant Pleural Mesothelioma: Update,
volvement. Arch Pathol Lab Med 2000;124:609-613. Current Management, and Newer Therapeutic Strategies. Chest
3 Brinsden PR, Wada I, Tan SL, Balen A. Diagnosis, prevention and 2004;126:1318-1329.
management of ovarian hyperstimulation syndrome. Br J Obstet
Gynaecol 1995;102:767-72.
4 Avecillas JF, Falcone T, Arroliga AC. Ovarian hyperstimulation
syndrome. Crit Care Clin 2004;679-695.
DISCLOSURE: Shirley Jones, None.
A CASE OF A PLEURAL EFFUSION, CALCIFIED PLEURAL

PLAQUES AND AN ABNORMAL BONE SCAN
Mariam Louis MD* James Gruber MD Richard Fraser MD McGill
University, Montreal, PQ, Canada
INTRODUCTION: The annual incidence of malignant mesothelio-

mas in the United States is 2200 cases per year, of which 70% are linked
to asbestos exposure (1). Osteosarcomatous differentiation of this tumor
has been reported in 14 cases (2, 3). We report a case of a patient who
presented with a large pleural effusion, very densely calcified pleural
plaques and an abnormal bone scan.
CASE PRESENTATION: The patient was a 79 year old man,
CASE REPORTS
non-smoker, who before retiring, had worked in construction and had
been employed for 2 years as a shipyard worker unloading bags of
asbestos. He had a history of pseudobulbar palsy and of prostate cancer
that was treated successfully with surgery alone 2 years before the current
illness.On routine blood testing, an elevated alkaline phosphate was noted
and a bone scan showed intense uptake along the sternum and the entire
adjacent right ribcage, which was interpreted as possible bony metastasis.
The patient complained of increasing dyspnea, pleuritic chest pain and
cough. Computed tomography of the thorax (figure 3) showed a large
right pleural effusion with almost complete collapse and retraction of the
right lung. Additionally, there was a 2.5 cm low density soft tissue mass
attached to thickened and irregular calcified pleura. Analysis of fluid
obtained by thoracocentesis showed an exudate with lymphocytosis and no
malignant cells. Microscopic examination from a video-assisted thoracos-
copy (VATS) and pleural biopsy showed a malignant tumor composed of
spindle and polygonal cells, the latter intimately associated with osteoid DISCLOSURE: Mariam Louis, None.
(Figure 2). No epithelioid cells were identified. Immunohistochemical
study showed strong positive reactions for keratin AE1 and AE3 in the
spindle cells; the reaction for calretinin was negative. These findings were ENLARGING PLEURAL NODULES DIAGNOSED NONINVA-
consistent with a mesothelioma with osteosarcomatous differentiation. SIVELY AS THORACIC SPLENOSIS
The patient elected for palliative treatment. Three months after his VATS, Jaspal Singh MD* Meg McCormack BS Duke University Medical Center,
the patient was admitted for pneumonia and passed away. At autopsy, Durham, NC
tumor completely surrounded the right lung (figure 3) and spread along
the major fissure and focally into the superficial lung parenchyma. A INTRODUCTION: The workup of pleural-based nodules often requires a
Faxitron image (Figure 4) confirmed extensive ossification. Microscopic biopsy to exclude malignancy. Thoracic splenosis, a rare entity that can occur in

patients who have had simultaneous splenic and diaphragmatic trauma, can thickening of the visceral and parietal pleura. Biopsy displayed dense
occasionally present as left-sided pleural-based nodules. We describe a case in fibrous tissue with scattered lymphohistiocytes and no malignant cells.
which slowly enlarging pleural nodules were diagnosed noninvasively as thoracic Despite talc pleurodesis, the left effusion returned, and repeated biopsy
splenosis by radionuclide imaging without the need for a biopsy. showed fibrous tissue with histiocytes and fibroblast proliferation. He
CASE PRESENTATION: A 58-year-old male Vietnam veteran was referred returned months later with severe dyspnea and CT-chest showing peri-
for incidental findings on chest radiograph of 2 large pleural nodules in the left cardial and right pleural effusions. VATS illustrated thickening of both the
hemithorax. The patient remained asymptomatic. His past medical history right pleura and pericardium, with fragments of fibroadipose tissue and
included medication-controlled hypertension, sickle cell trait, alcoholism and aggregates of histiocytes. No medical treatment was initiated during these
diverticulosis. He smoked 1 pack-per-day for 42 years and worked a variety of
hospitalizations.On this admission, physical examination revealed swelling
custodial jobs; he was unaware of specific exposure to asbestos. Of note, 40 years
ago the patient sustained extensive combat wounds and relates multiple and proptosis in both eyes. Visual acuity was 20/25 in right and 20/20 in
surgeries to his left chest, stomach, intestines, and left arm. He vaguely left. The eyes were midline, with restricted motility in all directions. The
recalls having a prolonged ventilator wean, tracheostomy, gastrostomy, heart sounds were distant and breath sounds were decreased in bilateral
and a left thoracostomy tube. Eventually he had a full recovery. Upon bases. Labs were normal. Magnetic resonance of the orbit demonstrated
evaluation by our service, a full review of systems was unremarkable. bilateral diffuse infiltrates. A biopsy revealed many lipid-laden histiocytes
Exam was notable only for multiple healed incisions in the mid- that were immunopositive for CD68 and negative for CD1a and S100.
abdomen and left chest wall. Chest X-ray and CT scans showed 2 large Thus, the diagnosis of Erdheim-Chester disease was made. The patient
left pleural-based nodules, the largest of which was 6 cm x 2.9cm. The was started on prednisolone 40mg/day and a 2-month follow-up showed
nodules were homogeneous in appearance and not calcified. When improved proptosis and stabilization of pleural effusions.
compared to a CT scan performed 10 years prior (performed at the DISCUSSIONS: Erdheim-Chester disease (ECD) is a rare, non-Langerhans
time for diverticular bleeding) the nodules had nearly doubled in size. cell histiocytosis characterized by diffuse infiltration of affected organs
To confirm our initial impression of thoracic splenosis, a liver-spleen with lipid-laden histiocytes and Touton-type giant cells, histochemi-
sulfur-colloid scan was performed. This demonstrated 2 small foci of cally distinct from Langerhans’cell histiocytosis. The most common
enhancement that correlated anatomically with the nodules on the CT presentation is bone pain, followed by diabetes insipidus, exophthal-
scan. The diagnosis of thoracic splenosis was thereby confirmed by mos, retroperitoneal masses, and pulmonary or cardiac involvement.
radionuclide imaging without the need for a biopsy.
Radiologically, the most specific finding is bilateral and symmetrical
DISCUSSIONS: First described in 1937 by Shaw and Shafi [1],
thoracic splenosis is a rare condition that usually follows simultaneous cortical osteosclerosis affecting mainly the diaphyseal and metaphyseal
splenic and diaphragmatic injury, with autotransplantation of splenic regions of long bones with sparing of the epiphyses. Characteristic
tissue into the left hemithorax [2]. The resulting nodules are usually histopathology includes a dense infiltrate of foamy histiocytes accom-
incidental findings from chest imaging, but because they can radio- panied by lymphocytes, monocytes, Touton-giant cells and variable
graphically mimic malignancy, biopsy is often needed. Biopsy in this amounts of fibrosis. These non-Langerhans cells are immunopositive
setting is often nondiagnositic, may have complications, or may result for CD68 and factor XIIIa, and negative for CD1a and other dendritic
in resection of functional splenic tissue with an associated theoretical cell markers. There is variable response to S100 and Birbeck granules
increased incidence of infection [3]. Therefore in the appropriate are not detected under electron microscopy. ECD may involve the
clinical setting, a noninvasive means of diagnosis is preferred. Radio- pituitary causing diabetes insipidus, and other endocrine abnormali-
nuclide imaging was first reported in 1971 to diagnose abdominal ties. It may cause interstitial lung disease with a restrictive pattern.
splenosis, and recently has been used in scattered reports for thoracic Frequently, the thoracic CT shows reticular and centrilobular nodular
splenosis [2]. As the nodules in our patients slowly enlarged over the opacities, scattered ground glass shadowing, and interlobular septal
years, it slightly raised the possibility of the nodules being malignant in and pleural thickening. It can involve the pericardium, resulting in
nature. With nodule enhancement on the liver-spleen scan, however, symptomatic pericardial effusion. Fibrosis of retorperitoneal fat can
the patient and our staff were reassured that the nodules are indeed lead to hydronephrosis and renal failure. The dense fibroxanthagranu-
splenic tissue, which is benign in nature.
lomatous can extend into the retro-orbital area with resulting exoph-
CONCLUSION: The finding of multiple pleural-based nodules in a
patient with a history of splenic and diaphragmatic trauma should raise the thalmos and visual defects. Demyelination in the cerebellum and pons
possibility of thoracic splenosis. Diagnosis of this rare entity can be leads to ataxia, while lesion in the dura can cause seizures. Treatment
confirmed by noninvasive radionucleotide imaging. has been attempted with steroids, cyclophosphamide, vinblastine,
REFERENCES: vincristine, adriamycin, colchicines and radiotherapy in varying com-
1 Shaw AFB, Shafi A. Traumatic autoplastic transplantation in man bination, with minimal clinical response. Steroid therapy has been
with observations on the late results of splenectomy in six cases. reported to improve exophthalmos, renal and bone involvement.
J Pathol 1937;45:215-235. Radiotherapy transiently relieves bone pain, but not exophthalmos.
2 Yammine JN, Yatim A, Barbari A. Radionuclide imaging in thoracic Improvement in pulmonary symptoms has been documented with
splenosis and a review of the literature. Clin Nucl Med 2003;28(2):121-123. steroids alone or in combination with cyclophosphamide.
3 Pearson HA, Johnston D, Smith KA, Touloukian RJ. The born again CONCLUSION: The prognosis is usually poor, most patients die of
spleen: return of splenic function after splenectomy for trauma. congestive heart failure, lung fibrosis or renal insufficiency. This may
N Engl J Med 1978;298:1389-1392. be due to delayed diagnosis, which requires an alert physician to
DISCLOSURE: Jaspal Singh, None. interpret the histological appearance and unique pattern of multi-
organ involvement seen in ECD.
A UNIQUE CASE OF EXOPHTHALMOS IN A PATIENT WITH

RECURRENT PLEURAL EFFUSIONS
Raymond Khan DO* Vasilios Sierros MD Thu Yein MD New York
Hospital Queens/Weil Medical College Cornell, Flushing, NY
INTRODUCTION: Erdheim-Chester disease is a rare histiocytosis,

which must be considered in patients with diffuse xanthogranulomatous
infiltrations. The diagnosis of ECD requires a vigilant physician to
interpret both the histological appearance and multi-organ involvement
seen in this disease.
CASE PRESENTATION: A 64-year-old Greek male with multiple
admissions for pleural effusions presented complaining of progressive
swelling of both eyes and intermittent double vision for four months.
Eighteen months prior he was hospitalized for an exudative left pleural
effusion, with negative work-up for infections and malignancies. Two
months later, he had recurrence of this effusion and under went
Video-Assisted Thoracic Surgery (VATS), which revealed generalized DISCLOSURE: Raymond Khan, None.

Pulmonary Hypertension
4:15 PM - 5:45 PM
AN UNUSUAL COMPLICATION OF THE ROSS OPERATION Right Heart Catheterization Pressures
CAUSING SYMPTOMATIC PULMONARY HYPERTENSION
Timothy S. Mooring MD* Victor J. Test MD Scott and White Hospital Site Pressure (mm Hg) Mean (mm Hg)
and Clinics, Temple, TX
Aortic 165/50 107
INTRODUCTION: Unilateral pulmonary artery stenosis(PAS) is an Right Ventricle 76/9 31
uncommon but reported complication of many cardiothoracic surgeries.
We present an unusual presentation of PAS following the Ross operation
Pulmonary Artery 44/9 24
that ultimately leads to symptomatic pulmonary hypertension. Pulmonary Capillary Wedge 13
CASE PRESENTATION: A 24-year-old Hispanic male was re- Right Atrium 9
ferred to the pulmonary hypertension clinic with symptoms of pro- Left Pulmonary Artery 48/13 25
gressive right heart failure. Two years prior he developed endocarditis Right Pulmonary Artery 16/10 12
complicated by embolic strokes and seizures. He later underwent a
Ross operation. The postoperative course was difficult but he had
gradual improvement in his functional status. Upon presentation to our DISCLOSURE: Timothy Mooring, None.
clinic he complained of severe dyspnea at approximately 100 yards of
ambulation with occasion midsternal chest pain. He denied dyspnea at
rest, palpitations, orthopnea, and paroxysmal nocturnal dyspnea. Past
medical history was otherwise unremarkable. No other surgeries had
been performed. He denied other risk factors for pulmonary hyper- 18-YEAR-OLD WITH HYPOXEMIA AND CHEST PAIN
tension. Physical examination demonstrated normal blood pressure Michael S. Plisco MD* Linda M. Lam DO George M. Matuschak MD
Saint Louis University, Saint Louis, MO
and pulse. His general appearance was only noted for multiple tattoos.
There was no jugular venous distention. Lungs were clear. Cardiac INTRODUCTION: Hypoxemic respiratory failure is a common rea-
exam revealed a normal S1, prominent S2 and a holosystolic murmur son for admission to the intensive care unit. The etiology has an extensive
along the left sternal border. Extremities were without cyanosis, differential diagnosis.
clubbing, or edema. He had a moderate left hemiplegia. Laboratory CASE PRESENTATION: 18-year old female smoker with history of
data including serologic screening for pulmonary hypertension were asthma presented to the emergency department with progressive dyspnea
unrevealing. Ventilation/Perfusion lung scan showed decreased perfu- and chest pain for one week. Past medical history included polycythemia
sion to the right lung. Pulmonary angiography (Figure 1) demonstrated treated with serial phlebotomies. Also, episodic cyanosis often accompa-
stenosis at the level of the distal anastomosis of the pulmonary nied previous asthma exacerbations and physical exertion. Initial improve-
homograft as well as tight stenosis within the right pulmonary artery. ment of dyspnea and cyanosis were noted with an albuterol inhaler.
Right heart catheterization (Table 1) demonstrated elevated right Systems review included progressive wheezing, productive cough and
ventricular pressures (mean of 31 mm Hg) and less markedly elevated three syncopal episodes in the past year. Neither edema, calf pain, nor
pulmonary artery pressures (mean 25 mm Hg) distal to the main artery hemoptysis were noted. Admission vital signs were: blood pressure,122/80
stenosis. Pulmonary artery saturation was 65.2%. Also noted was a mmHg, heart rate,120 beats per minute, respiratory rate, 24 respirations
pressure drop-off across the stenosis of the right pulmonary artery. per minute, temperature, 38.0C. Oxygen saturation of 74% on room air.
Pulmonary artery occlusion pressure and cardiac output were normal The patient was placed on a non-rebreather mask with FIO2⫽1.0, which
improved oxygenation to 92%. Physical exam revealed a petite female in
(8.4L/min). The patient underwent cardiac reoperation for tricuspid
minimal distress. Jugular venous pressure was 12cm H20. Cardiac exam
valve repair and direct evaluation of the pulmonary artery stenosis. The disclosed an increased pulmonic component of the second heart sound,
distal anastomosis on the pulmonary homograft was stenotic. The right but no murmur. Bilateral expiratory wheezes were noted over the lungs,
pulmonary artery was noted to be severely stenotic. Further dissection and digital clubbing was present. Desaturation to as low as 60% with
revealed the presence of a Prolene suture placed through the proximal minimal exertion occurred. Arterial blood gases showed a pH of 7.42,
right pulmonary artery causing the stenosis. This suture was removed pCO2, 31 mmHg, PO2, 65 mmHg, with sPO2 of 90% on FIO2 of 1.0.
and the artery was successfully dilated to the size of a #10 dilator. The Enlarged pulmonary arteries with no infiltrates were noted on chest
main pulmonary artery at the site of stenosis was enlarged with a radiography and electrocardiographic right ventricular strain was evident.
Dacron patch. The tricuspid valve was repaired. The postoperative Transthoracic echocardiogram (TTE) study indicated an estimated right
course was uncomplicated and exercise tolerance continues to im- ventricular systolic pressure⫽120 mmHg with no evidence of an intracar-
prove. His limitations due to dyspnea upon exertion have been diac shunt. Computed tomography of the chest and extremeties to
evaluate for pulmonary thromboembolism excluded vascular occlusion,
CASE REPORTS
dramatically decreased with relief of the pulmonary artery stenoses.
DISCUSSIONS: Our case is a unique cause of pulmonary hyper- but deep vein thrombosis was diagnosed. Upon further review, a patent
tension due to an unreported complication of the Ross operation. Main ductus arteriosus (PDA) was found (image 1). On sagittal cuts, contrast
PAS is a well-recognized complication of placement of a pulmonic was noted flowing from the pulmonary artery directly into the aorta with
homograft. Unilateral stenosis is also a reported complication of lung retrograde flow into the ascending aorta (image 2). The patient’s condition
deteriorated rapidly despite aggressive treatment with bronchodilators,
transplantation, including contralateral PAS following single lung
antibiotics, glucocorticoids and anticoagulation. A cardiac arrest occurred
transplantation. Unilateral stenosis has been previously reported with following intubation from which the patient could not be resuscitated.
other cardiothoracic surgeries. In our case lung perfusion scanning,
pulmonary angiography, and right heart catheterization demonstrated
the presence of hemodynamically significant unilateral stenosis in
addition to an anastomotic stenosis. A suture as the etiology was
surprising. After surgical correction, the patient was able to make
expected gains in exercise tolerance. It can be inferred that both areas
of stenosis contributed to the patient’s symptoms based upon hemo-
dynamic data, intraoperative findings, and post-operative improve-
ment.
CONCLUSION: Pulmonary artery stenosis is an unusual complication
of the Ross Procedure. Unilateral pulmonary artery stenosis has not been
reported but should be considered when pulmonary hypertension devel-
ops after this procedure.

Pulmonary Hypertension, continued
aggressive diuresis, PA pressures and oxygen requirements improved.

Repeat bronchoscopy revealed less bloody return. Patient was successfully
extubated 5 days post-delivery. Follow-up echocardiogram was unchanged
except for significant reduction in PA pressures. Pediatric cardiology
evaluated the patient for possible PDA closure.
DISCUSSIONS: Pregnancy is associated with significant alterations in
the cardiovascular system. A 50% increase in blood volume and cardiac
output (CO) may occur. In the 1st trimester, the increase in CO is due to
larger stroke volume, while higher heart rates account for later rise.1
While most patients tolerate these hemodynamic changes, those with
underlying congenital heart disease are at increased risk for cardiovascular
complications. In general, PDA in gravid patients carries a favorable
outcome. There is increased risk if pulmonary hypertension is present, as
often occurs by the time adulthood is reached. If the systemic blood
pressure falls and elevated pulmonary pressures are present, shunt
reversal may occur. Overall, by age 40, one-third of patients with
uncorrected PDAs succumb to heart failure, endarteritis or PHTN. Our
DISCUSSIONS: The patient had severe pulmonary hypertension with
patient developed alveolar hemorrhage in association with PHTN both of
Eisenmenger syndrome secondary to a large PDA. Increased pulmonary
which resolved post-partum. Based on studies by West et al. this is likely
arterial pressure from progressive increases in pulmonary vascular resistance
due to “stress failure” of pulmonary capillaries. In animal models, when
due to hypoxia most likely led to further reversal of blood flow across the
capillary pressures rise, there is microscopic evidence of breakdown of the
PDA. PDA comprises 10% of congenital heart disease cases. Individuals with
alveolar-capillary membrane, or “stress failure”. This produces edema
a small PDA may be asymptomatic; exercise intolerance may develop in older
fluid with higher protein concentrations or “high-permeability” edema
children. Notably, subjects with large undiagnoses PDAs such as in our
rather than “hydrostatic” edema. Development of “high-permeability”
patient seldom reach adulthood because of supervening endarteritis and
edema, which includes leakage of red blood cells (RBC’s) and alveolar
sudden death from cardiac failure. Considering that a cardiac murmur may
hemorrhage, increases as capillary pressures rise. Although not specifically
not be audible with a PDA once pulmonary vascular resistance is abnormally
recognized in patients with PDA, similar phenomena are well described in
elevated, echocardiography plays a key diagnostic role in establishing the
mitral stenosis, whereby increased pulmonary venous pressures lead to
diagnosis. Transesophageal echocardiography has a higher sensitivity and a
edema and extravasation of RBC’s, manifesting as hemoptysis. Athletes
greater negative predictive value than TTE. Poor functional class, syncope,
who train intensely, raising their CO and necessitating higher filling
pulmonary hypertension, and lack of pulmonary vasodilatory response during
pressures, thus creating increased capillary pressures, have also presented
right heart catheterization are associated with a poor outcomes.
with hemorrhagic pulmonary edema.2.
CONCLUSION: Clinically occult congenital heart disease with Eisen-
CONCLUSION: The interplay of pregnancy related hemodynamic
menger syndrome is an infrequent cause of hypoxemic respiratory failure. Of
changes and underlying PDA can lead to significant PHTN and increased
particular concern is when the cause is a large, previously undiagnosed PDA
capillary pressures which may present as pulmonary edema and alveolar
without an accompanying cardiac murmur. Our case demonstrates the need
hemorrhage. Knowledge of the hemodynamic changes associated with
to consider this possibility, and confirms the high mortality observed when
congenital heart disease and pregnancy will aid in understanding the
pulmonary hypertension supervenes in this condition.
pathophysiology of resulting alveolar hemorrhage and its treatment.
REFERENCES:
REFERENCES:
1 Perloff. Congenital heart disease in adults. WB Sauders Co 1998.
1 Hess, D. Management of cardiovascular disease in pregnancy.
2 Brickner ME et al. Medical Progress: Congenital Heart Disease in
Obstetrics and Gynecology Clinics of NA 1992; 19: 679-93.
Adults. NEJM 2000; 342: 334-342.
2 West, J. Vulnerability of pulmonary capillaries in heart disease.
3 Shyu et al. Diagnostic Accuracy of TEE for detecting PDA in
Circulation 1995; 92: 622-31.
adolescents and Adults. CHEST1995; 108:1201-1205.
4 McLaughlin et al. Prognosis of pulmonary arterial hypertension.
CHEST 2004; 126:78-92S.
DISCLOSURE: Michael Plisco, None.
PULMONARY HYPERTENSION (PHTN) AND ALVEOLAR HEM-

ORRHAGE IN PREGNANCY: A CASE OF MATERNAL PATENT
DUCTUS ARTERIOSUS (PDA)
Amee Patrawalla MD* Doreen Addrizzo-Harris MD Rany Condos MD
Bellevue Hospital/NYU, New York, NY
INTRODUCTION: One of the most common cardiac problems seen

in pregnancy is maternal congenital heart disease. We describe a PDA in
a pregnant patient and its effect on cardiopulmonary pathophysiology.
CASE PRESENTATION: A 24 y.o. gravid woman with an uncor-
rected PDA presented at term with severe dyspnea. Vital signs: blood
pressure 149/65, pulse 105, respirations 25, SaO2 97% on room air. On
exam a 5/6 continuous murmur, palpable thrill and bilateral crackles were
noted. A chest roentogram was consistent with congestive heart failure.
Due to preeclampsia and fetal distress, caesarean section was performed.
Upon intubation, frank blood was noted in the endotracheal tube. Oxygen
saturations fell to 50%-70% and ventilation was difficult. A chest roento-
gram demonstrated bilateral fluffy infiltrates. Mechanical ventilation was
initiated with high oxygen requirements. ABG revealed mild respiratory
acidosis and a PaO2/FiO2 ratio of 125 mmHg. Bronchoscopy revealed
blood clots throughout mainstems and in distal airways. Sequential
bronchoalveolar lavage (BAL) had consistently bloody return, compatible
with alveolar hemorrhage. Cytopathology revealed hemosiderin-laden
macrophages. Laboratory studies revealed normal platelet count, coagu-
lation, and renal function. Pulmonary artery (PA) pressures were elevated
at 88/58 mmHg with wedge systolic of 32 mmHg. Echocardiogram
showed left atrial and ventricular (LV) dilatation, dilated PA, mild LV
hypertrophy, PHTN, and large PDA with left-to-right shunting. After DISCLOSURE: Amee Patrawalla, None.

LEFT MAIN CORONARY ARTERY COMPRESSION BY AN EN- thromboembolic pulmonary hypertension. Eur J Cardiothor Surg
LARGED PULMONARY ARTERY IN PULMONARY HYPERTEN- 2005;27:512.
SION DUE TO DIFFUSE PARENCHYMAL LUNG DISEASE AND 5 Fujiwara K, et al. Left main coronary trunk compression by dilated
SUCCESSFUL TREATMENT BY LUNG TRANSPLANTATION pulmonary artery in atrial septal defect. Report of three cases.
Caralee E. Caplan-Shaw MD* Steven M. Kawut MD Joshua R. Sonett J Thorac Cardiovasc Surg 1992;104(2):449-52.
MD Gregory D. Pearson MD Anna Rozenshtein MD Mark A. Apfelbaum 6 Rich S, et al. Stenting to reverse left ventricular ischemia due to left
MD Selim M. Arcasoy MD Jessie S. Wilt MD Columbia University main coronary artery compression in primary pulmonary hyperten-
Medical Center, New York, NY sion. Chest 2001;120:1412-5.
DISCLOSURE: Caralee Caplan-Shaw, None.
INTRODUCTION: Extrinsic compression of the left main coronary
artery (LMCA) by an enlarged pulmonary trunk has been reported in
patients with pulmonary hypertension. We report the first case of SEVERE DIFFUSION CAPACITY REDUCTION IN A CASE OF
symptomatic LMCA compression in a patient with pulmonary hyperten- SYSTEMIC ONSET JUVENILE RHEUMATOID ARTHRITIS
sion associated with diffuse parenchymal lung disease successfully treated WITH MILD PULMONARY HYPERTENSION
with bilateral lung transplantation. Paul K. Nolan MD* Curt Daniels MD Fredrick Long MD Megan K.
CASE PRESENTATION: A 60-year-old man was referred to our center Dishop MD Peter Baker MD Margarita Guarin MD Elizabeth D. Allen
with pulmonary hypertension resulting from diffuse parenchymal lung disease MD Robert Rennenbohm MD Columbus Children’s Hospital, Columbus,
due to beryllium exposure. He reported substernal chest pain during exercise. OH
The pain was dull, radiated to the left arm, persisted throughout the day, and was
associated with dyspnea on exertion. A resting myocardial perfusion scan with INTRODUCTION: Pulmonary Artery Hypertension (PAH) with se-
thallium-201 showed perfusion defects in the LMCA territory. Right heart vere diffusion capacity (DLCO) reduction complicating Systemic Onset
catheterization revealed a right atrial pressure of 3 mm Hg, a right ventricular Juvenile Rheumatoid (SOJRA) is extremely rare, only one case being
pressure of 76/2 mm Hg, a pulmonary artery pressure of 78/31 mm Hg, and a reported. (1) In children with JRA, Pulmonary Function Tests (PFT)
pulmonary artery occlusion pressure of 11 mm Hg. Coronary angiography rarely show DLCO impairment and if present is usually only to a mild to
showed a 70% smooth tapered narrowing of the LMCA without other coronary moderate. (2) We describe a case of SOJRA complicated by a severely
artery abnormalities (Figure 1). Computerized tomography (CT) of the chest reduced DLCO with mild PAH.
revealed bilateral coarse fibrotic changes with traction bronchiectasis and honey- CASE PRESENTATION: A 13 year old female with SOJRA and
combing and a main pulmonary artery diameter of 4.7 cm. CT angiography partial Macrophage Activation Syndrome (MAS) developed dyspnea and
revealed LMCA compression by the main pulmonary artery with no evidence of tachycardia during the 5th month of active disease. During the 9th month,
coronary artery atherosclerosis (Figure 2a, arrow ⫽ LMCA, P ⫽ pulmonary she experienced a life threatening MAS episode. PFT showed hypoxia and
artery, A ⫽ aorta). Three months later, the patient underwent successful bilateral severe hemoglobin (hgb) corrected DLCO at 24% without obstruction or
lung transplantation. Postoperatively, the patient had no recurrence of chest pain, restriction. High Resolution Chest CT with angiography and Ventilation
and exercise stress testing with technetium99m-sestamibi revealed normal exer- Perfusion scan showed no interstitial pulmonary fibrosis or macrovascular
cise performance and normal myocardial perfusion at a heart rate of 171. thromboembolism. Despite aggressive immunosuppressive therapy with
Follow-up CT angiography showed complete resolution of the LMCA compres- pulsed methylprednisolone, cyclosporine A and methotrexate, the pa-
sion (Figure 2b). tient’s dyspnea and hypoxia worsened. Heart catheterization at month 13
DISCUSSIONS: LMCA compression by an enlarged pulmonary demonstrated mild PAH with mean pulmonary artery pressure (mPAP) of
artery may cause angina, left ventricular ischemia, and sudden death. 37 mm Hg without intrapulmonary or significant intracardiac shunting.
LMCA compression has been reported in patients with idiopathic Bosentan was started in month 14. The patient experienced a second life
PAH, PAH associated with congenital systemic-to-pulmonary shunts, threatening MAS episode in month 15. Etoposide was instituted to
and pulmonary hypertension due to chronic thromboembolic disease suppress the activated macrophages, which was followed by rapid clinical
(1-4). Although optimal management of this entity is unknown, improvement in the MAS. Open lung biopsy at month 16 showed changes
successful treatment of LMCA compression has been reported after on light microscopy (LM) of preplexogenic pulmonary hypertension with
surgical correction of atrial septal defects (5), LMCA stenting (6), and intimal thickening and on electron microscopy (EM), multilamellated
pulmonary thromboendarterecomy with concurrent coronary artery thickening of the alveolar capillary basement membrane (BM). There was
bypass grafting (4). We report the first case of LMCA compression in no evidence of pulmonary fibrosis or thrombosis. The patient’s hgb
association with pulmonary hypertension due to diffuse parenchymal DLCO reached it’s nadir of 21% by month 16 and has improved to 52 %
lung disease with clinical, radiologic, and functional improvement after by month 21. Repeat heart cath at month 18 showed normalization of the
bilateral lung transplantation. mPAP at 25 mm Hg.
DISCUSSIONS: The severe hypoxia and diffusion capacity limitation
described were markedly out of proportion to degree of PAH found.The
CASE REPORTS
etiology of the hypoxia is the ultramicroscopic structural changes at the
level of the alveolar capillary endothelium. Similar pathologic microvas-
cular endotheliopathy lesions have been described on electron microscopy
of the pulmonary alveolar capillary basement membrane in patients with
plexogenic primary pulmonary hypertension. (3) The likely pathologic
cascade that led to these BM changes was likely excess release of
endothelin-1 and cytokines from the activated macrophages, which trig-
gered further endothelin-1 synthesis and release from the vascular
endothelium. (4,5,6).
CONCLUSION: PAH and pulmonary microvascular endotheliopathy
should be suspected when hypoxia and DLCO reduction are present in
systemic rheumatologic disease. Early lung biopsy with LM and EM
should be pursued to allow expeditious implementation of potentially life
CONCLUSION: LMCA compression by an enlarged pulmonary artery saving medications before irreversible pathology develops in the lungs.
may occur in pulmonary hypertension due to diffuse parenchymal lung REFERENCES:
disease and may be treated successfully with lung transplantation. 1 Padeh S, Laxer RM, Silver MM, Silverman ED. “Primary Pulmo-
REFERENCES: nary Hypertension in a patient with Systemic-Onset Juvenile Arthri-
1 Kawut SM, et al. Extrinsic compression of the left main coronary tis.” Arthritis Rheuma 1991; 34(12):1575-79.2.
artery by the pulmonary artery in patients with long-standing 2 Pelucchi A, Lomater C, Gerloni V, Foresi A, Fantini F, Marazzini L.
pulmonary hypertension. Am J Cardiol 1999;984-6. ”Lung function and diffusing capacity for carbon monoxide in
2 Patrat JF, et al. Left main coronary artery compression during patients with juvenile chronic arthritis: effect of disease activity and
primary pulmonary hypertension. Chest 1997;112:842-3. low dose methotrexate therapy.“ Clin Exp Rheuma
3 Bonderman D, et al. Left main coronary artery compression by the 3 Villaschi S, Pietra GG. ”Alveolo-capillary membrane in primary
pulmonary trunk in pulmonary hypertension. Circulation 2002;105:265. pulmonary hypertension.“ Appl Pathol. 1986;4(3):132-7.
4 Ngaage DL, et al. Left main coronary artery compression in chronic 4 Li Z, Niwa Y, Rokutan K, Nakaya Y. ”Expression of endothelin-1 in

Tuesday, November 1, 2005
macrophages and mast cells in hyperplastic human tonsils.“ FEBS at a dose of 50 mg thrice daily. She discontinued bosentan therapy on her own.
Lett. 1999 Sep 3;457(3):381-4. After 12 weeks of treatment with sildenafil alone, she had significant clinical
5 Molet S, Furukawa K, Maghazechi A, et al. ”Chemokine and improvement in WHO Functional Class (from III to II) and 6MWD (190 to 295
cytokine induced expression of endothelin 1 and endothelin con- meters). Her pedal edema had disappeared. Her hemodynamic parameters
verting enzyme 1 in endothelial cells. J Allergy Clin Immunol 2000; showed MPAP of 55 mmHg, PCWP of 10 mmHg, CI 2.3 L/min/m2, and PVR
105:333-8. of 9.5 Woods units.
6 Simonson MS, Herman WH, Knauss TC, Schulak JA, Hricik DE. “ DISCUSSIONS: Our patient had sarcoidosis-associated pulmonary
Macrophages– but not T-cell– derived cytokines stimulate endothe- hypertension and, unlike our previous case report, showed no clinical or
lin-1 secretion by endothelial cells” Transplant Proc. 1999 Feb-Mar; hemodynamic improvement with bosentan. In contrast, she had consid-
31(1-2):806-7. erable clinical and hemodynamic benefit with sildenafil monotherapy.
CONCLUSION: Sildenafil is expected to be approved for the treat-
ment of pulmonary arterial hypertension (PAH) soon. SAPH is not
considered PAH in the most recent classification of pulmonary hyperten-
sion(8). Our patient showed significant clinical and hemodynamic im-
provement with sildenafil. Others have demonstrated improvement of
SAPH with iNO(4) and epoprostenol(5). Taken together, these reports
suggest that SAPH has similar underlying vasoresponsive component as
PAH. If further pathophysiological evidence is found, re-classification of
SAPH as PAH should be considered.
REFERENCES:
1 Mitchell DN, Scadding JG. Sarcoidosis. Am Rev Respir Dis 1974;
110:774-802
2 Rosen Y, Moon S, Huang CT, et al. Granulomatous pulmonary
angiitis in sarcoidosis. Arch Pathol Lab Med 1977; 101:170-174
3 Hietala SO, Stinnett RG, Faunce HF, 3rd, et al. Pulmonary artery
narrowing in sarcoidosis. Jama 1977; 237:572-573
4 Preston IR, Klinger JR, Landzberg MJ, et al. Vasoresponsiveness of
sarcoidosis-associated pulmonary hypertension. Chest 2001; 120:866-872
5 Jones K, Higenbottam T, Wallwork J. Pulmonary vasodilation with
prostacyclin in primary and secondary pulmonary hypertension.
Chest 1989; 96:784-789
6 Rodman DM, Lindenfeld J. Successful treatment of sarcoidosis-
associated pulmonary hypertension with corticosteroids. Chest
1990; 97:500-502
7 Sirithanakul K, Nadeem S, Potts KE, Pitta SR, Mubarak KK.
Sarcoidosis-related pulmonary hypertension improved with bosen-
tan therapy: hemodynamic evidence. Chest 2004; 126(4):935S-936S
8 Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of
pulmonary hypertension. J Am Coll Cardiol 2004; 43:5S-12S
DISCLOSURE: Paul Nolan, None. DISCLOSURE: Haroon Faraz, Grant monies (from industry related
sources) Actelion, Inc.; Encysive, Inc.; Myogen, Inc.; United Therapeu-
tics, Inc.; Consultant fee, speaker bureau, advisory committee, etc.
CASE REPORT: BOSENTAN-RESISTANT SARCOIDOSIS-ASSO- Actelion, Inc.; CoTherix, inc.; Intermune, Inc.; Pfizer, Inc.; Product/
CIATED PULMONARY HYPERTENSION RESPONSIVE TO SIL- procedure/technique that is considered research and is NOT yet approved
DENAFIL for any purpose. Sildenafil is approved for erectile dysfunction, but not for
Haroon A. Faraz MD* Yelena Selektor MD Muhammad A. Ehtesham PAH so far. It probably will be approved for PAH before this case is
MD Michael Harbut MD Kamal K. Mubarak MD Wayne State Univer- presented. Bosentan is approved for PAH, but not for sarcoidosis-
sity, Detroit, MI associated pulmonary hypertension.
INTRODUCTION: Pulmonary hypertension in sarcoidosis has been

attributed to end-stage lung fibrosis with resultant destruction of pulmo-
nary vasculature(1), granulomatous pulmonary angiitis(2), or extrinsic
compression of major pulmonary vessels(3). Sarcoidosis-associated pul-
monary hypertension (SAPH) has been shown to be responsive to Bronchology I
vasodilator therapy with inhaled nitric oxide (iNO)(4), intravenous 4:15 PM - 5:45 PM
epoprostenol(5), and corticosteroid therapy(6). Our group has previously
reported that SAPH can be treated successfully with endothelin-receptor
antagonist bosentan(7), signifying the role of altered endothelium-derived “ASTHMA” CAUSED BY TRACHEAL TUMORS
vasoactive mediators in pulmonary vasculature of such patients. We now Atasha Asmat MB, BCh* Poo-Sing Wong MB, BCh National University
report a case of SAPH that was resistant to bosentan but showed marked Hospital, Singapore, Singapore
improvement with phosphodiesterase-5 inhibitor sildenafil.
CASE PRESENTATION: A 41-year-old African-American female with INTRODUCTION: Tracheal tumors are exceedingly rare and often
sarcoidosis was referred to our Pulmonary Hypertension Clinic for worsening overlooked as a cause of pulmonary symptoms until they reach an
dyspnea (WHO Functional Class III). She had a history of mild obstructive sleep advanced stage. Most patients present with symptoms suggestive of
apnea treated with nocturnal CPAP and systemic hypertension. On examination, asthma and the correct diagnosis is often made when the patient fails to
she had a loud P2, right ventricular heave, jugular venous distension, bilateral lung respond to conventional asthma treatment.
crackles, and pitting edema. A cardiac catheterization at baseline showed mean CASE PRESENTATION: Patient 1 is a 66 year old gentleman who
pulmonary artery pressure (MPAP) of 56 mmHg, pulmonary capillary wedge presented with nocturnal “noisy breathing” and dyspnoea. He had been
pressure (PCWP) of 18 mmHg, cardiac index (CI) of 2.7 L/min/m2 and treated for “uncontrolled” asthma for 3 years. When his symptoms
pulmonary vascular resistance (PVR) of 6.6 Woods units, with no response to worsened, a computed tomography (CT) scan of the thorax was performed
adenosine. Her 6-minute walk distance (6MWD) was 175 meters. She was and showed a tumor measuring 5 cm below the vocal cords, located
started on bosentan with standard dosing and oxygen. She remained clinically posterolaterally and extending extraluminally outside the trachea. The
stable with some improvement in her pedal edema. After 1 year of treatment with lesion was occupying 60% of the lumen. Tracheal resection with primary
bosentan, we repeated her studies. These showed MPAP of 57 mmHg, PCWP anastomosis following suprahyoid release and mobilization of the thyroid
of 14 mmHg, CI of 1.7 L/min/m2, PVR of 11.9 Woods units, and 6MWD of 190 gland was performed. Operative findings were of a hard but well-
meters. When no significant improvement was seen, she was started on sildenafil encapsulated tumor located 5 cm below the vocal cords and attached to

Bronchology I, continued
the right lateral wall. The tumor extended about 3 cm distally. Histopatho-
logical diagnosis was pleomorphic adenoma. The patient made an uneventful
postoperative recovery and remains well 2 years after surgery. Patient 2 is a
38 year old lady who was treated for daily attacks of “asthma” and presented
with “status asthmaticus” requiring endotracheal intubation and ventilatory
support. Her peak airway pressures were persistently high and a flexible
bronchoscopy showed an endobronchial tumor in the distal third of the
trachea about 10 cm above the carina. Subsequently, a CT thorax showed an
eccentric narrowing of the distal trachea consistent with tumor. A right
thoracotomy and distal tracheal resection with primary anastomosis was
performed. At operation, a polypoidal tumor measuring 2 x 2 cm with a broad
base was noted arising from the left posterolateral aspect of the trachea.
Histological diagnosis was mucoepidermoid carcinoma. She went on to make
an uneventful recovery. Patient 3 is a 14 year old girl who had “poorly
controlled” asthma for 2 years. CT thorax showed a tumor in the distal
trachea. Flexible bronchoscopy and biopsy showed mucoepidermoid carci-
noma. The patient underwent right thoracotomy and distal tracheal resection
with primary anastomosis. Post-operative recovery was uneventful and the
patient continues to be well 5 years after surgery.
DISCUSSIONS: Primary tracheal tumors are uncommon. Most pa-
tients are misdiagnosed as asthma and undergo numerous trials of
unsuccessful conventional asthma treatment before further evaluation is
undertaken leading to the correct diagnosis. In 2 of our patients, the DISCUSSIONS: Hamartomas are the most common form of benign
diagnosis was initially made with CT imaging. The other patient required lung tumors, with an incidence ranging between 0.025% and 0.32%.
intubation and ventilatory support for “status asthmaticus”. She had However, endobronchial hamartomas (EH) are a very rare entity. In the
persistently high peak airway pressures on ventilatory support and bron- largest review series, only 1.4% of hamartomas had an endobronchial
choscopy revealed the tumor. Other diagnostic modalities include mag- location, the remainder being located within the lung parenchyma.
netic resonance imaging and fluoroscopy. Flow-volume curves may Whereas patients with intra-pulmonary hamartomas are usually symptom-
provide valuable data and aid in the diagnosis. Once the diagnosis is made, free, those with EH typically are symptomatic, and therefore, require
patients should be referred for surgery. treatment. EH are generally broad-based lobulated nodules, resulting in
CONCLUSION: The majority of tracheal tumors misdiagnosed as asthma symptoms of airway obstruction. Cough, hemoptysis, dyspnea, and post
reported in the literature were diagnosed when flow-volume curves suggested a obstructive pneumonia are the main clinical features. Histologically, the
fixed airway obstruction resulting in further imaging studies for the patient. Our tumors consist of varying combinations of benign elements including
cases did not have flow-volume curve studies with further imaging studies cartilage, connective tissue, fat, and smooth muscle. Most tumors grow
performed when they failed to respond to conventional treatment. Whilst slowly (average of 3 mm/year) during follow up.Different treatment
tracheal tumors are rare, they should be considered in the differential diagnosis in modalities are available for the management of EH. Surgical resection
any patient who presents with late-onset asthma or patients with “asthma” who (wedge resection, lobectomy and in extreme case, pneumonectomy) has
fail to improve despite appropriate treatment. been recommended for these patients, but carries the surgical risks
DISCLOSURE: Atasha Asmat, None. inherent to a thoracotomy. When completely resected, pulmonary hamar-
tomas rarely recur. Less invasive, bronchoscopic techniques have been
used, allowing the preservation of normal lung parenchyma. These are
ENDOBRONCHIAL HAMARTOMA TREATED WITH BRON- performed with flexible or rigid bronchoscopy, typically using a ND-YAG
CHOSCOPIC ARGON PLASMA COAGULATION laser and forceps. To our knowledge, this is the first report of the use of
Tanveer Ahmed MD* Walid G. Younis MD Kellie R. Jones MD Gary T. APC as a treatment modality in this type of benign endobronchial tumor.
Kinasewitz MD Jean I. Keddissi MD University of Oklahoma Health CONCLUSION: Endobronchial hamartoma remains a rare entity.
Sciences Center, Oklahoma City, OK Patients are frequently symptomatic, due to airway obstruction. The
benign nature of the lesion makes surgical resection, with its risks and
INTRODUCTION: We present a case of endobronchial hamartoma, potential complications, less appealing. The use of endoscopic techniques,
CASE REPORTS
which was resected and ablated with loop electrocautry and Argon Plasma including Argon Plasma Coagulation, should be considered in the appro-
Coagulation using therapeutic fiberoptic bronchoscopy. priate setting. The long term outcome after endoscopic resection remains
CASE PRESENTATION: A 55-year-old white male with a 30 pack-year to be determined.
smoking history was referred to pulmonary clinic after failing two cycles of
antibiotic treatment for a right upper lobe “pneumonia”. The patient denied
dyspnea, chest pain, sputum production, hemoptysis, weight loss or night sweats.
He worked as a salesperson in an auto shop, and denied any occupational or
environment exposure to allergens, irritants or toxins. Chest radiograph after the
two courses of antibiotics showed a persistent triangular infiltrate in the right
upper lobe, extending to the hilum suggesting an obstructive lesion. CT of the
chest showed an endobronchial density, mucus plug vs. mass, in the right upper
lobe anterior segmental bronchus with post-obstructive atelectasis.A fiberoptic
bronchoscopy was performed which demonstrated a pedunculated, spindle
shaped, occluding mass protruding from the right upper lobe orifice into the right
main bronchus. No nodularities or ulcerations were noted. Endobronchial
biopsies indicated a benign papillomatous growth. The patient underwent a
repeat fiberoptic bronchoscopy, during which a heated loop electrocautry was
used to remove the mass. It was found to originate from the anterior segment of
the right upper lobe. Argon Plasma Coagulation (APC) was then used to cauterize
the remaining base. Pathological examination of the mass indicated it was a
benign hamartoma.Two additional therapeutic bronchoscopies, using APC, were
performed to further open the anterior segment of the right upper lobe. This
objective has been only partially achieved thus far and additional bronchoscopies
are planned to completely open the airway. DISCLOSURE: Tanveer Ahmed, None.

SUCCESSFUL LASER PHOTO RESECTION OF ENDOBRON- tracheobronchial tree: radiographic and CT findings in 12 patients.
CHIAL MUCOEPIDERMOID TUMOR Radiology 1999; 212: 643-648.
Mohamed S. Soliman MD J. Chandrasekhar MD Jeffrey Nascimento 2 Duhamel, DR, Harrell, JH. Laser bronchoscopy. Surg Clin North
DO* Trajko Bojadzeski MD David Posner MD Klaus Lessnau MD Am 2001;11: 769-789.
Murray Rogers MD Lenox Hill Hospital, New York, NY 3 Leonardi HK, Jung-Legg Y, Legg MA, Neptune WB. Tracheobron-
chial mucoepidermoid carcinoma: clinicopathological features and
INTRODUCTION: Mucoepidermoid tumors of the lung arise from results of treatment. J Thorac Cardiovasc Surg 1978; 76:431-438.
tracheobronchial mucous glands and are similar in morphology to muco- 4 Payne, WS, Ellis, FH, Woolner, LB, et al. The surgical treatment of
epidermoid tumors arising from oropharyngeal salivary glands. They are cylindroma (adenoid cystic carcinoma) and muco-epidermoid tu-
extremely rare, composing 0.1-0.2% of primary lung cancers. A mixture of mors of the bronchus. J Thorac Cardiovasc Surg 1959.
epithelial, mucin-secreting, and intermediate cells is seen microscopically. DISCLOSURE: Jeffrey Nascimento, None.
The biologic behavior depends on the histologic appearance; tumors with
increased mitosis, necrosis and nuclear pleomorphism are considered high
grade and are usually lethal. The majority are low-grade malignancies with A MULTIMODAL TREATMENT USING ARGON PLASMA CO-
a benign clinical course without recurrence or metastases (1). AGULATION (APC) AND ALPHA INTERFERON FOR RECUR-
CASE PRESENTATION: A 65 year-old non smoker female presented RENT RESPIRATORY PAPILLOMAS
with hemoptysis. She had a history of shortness of breath and cough for the Mudusar I. Raza MD* Thomas A. Dillard MD Muhammad A. Kaleem
previous 6 months. On physical examination, vital signs were normal except for a MD Christine Gourin MD Medical College of Georgia, Augusta, GA
respiratory rate of 24 breaths per minute. There was diminished air entry with
faint wheezing on the right side of the chest. The rest of the examination was INTRODUCTION: Recurrent respiratory papillomas (RRP) pose a
unremarkable. Chest radiography showed right middle lobe collapse with a challenging management problem. In up to 20% of cases papillomas
suspicion of adjacent mass. CT scan of the chest revealed an enlarged mass extend below the vocal cords to involve the lower respiratory tract causing
extending into the right main stem bronchus and the subcarina, enlarged right cough, dyspnea and other symptoms on occasion. Permanent and com-
infrahilar mass with right middle and lower lobes atelectasis. Emergency bron- plete eradication of RRP is still not considered possible at this time but
choscopy revealed a tumor arising from the medial wall of the right main stem remission of disease can occur in a few cases.
bronchial orifice, obstructing the bronchus and involving the main carina CASE PRESENTATION: A 56 year old male presented with increas-
(figure1). The histopathology of the tumor revealed low grade mucoepidermoid ing shortness of breath and paroxysmal cough. Bronchoscopy revealed
tumor. The patient was referred to our institution. We used Nd-YAG laser photo upper and lower respiratory papillomas in 1995. Human Papilloma Virus
resection via flexible bronchoscope to control the bleeding and relieve the serotypes 16 and 18 were confirmed on tissue biopsy. The patient
obstruction. Over the period of 6 months, we achieved excellent results with only underwent 43 CO2 laser treatments between 1995-2004 via rigid bron-
a small residual tumor left behind (figure 2). The patient is currently asymptom- choscopy and direct laryngoscopy to treat his lesions. CO2 laser controlled
atic and doing well. lesions as far as the distal trachea but could not reach the distal trachea
DISCUSSIONS: In the past twenty years, the use of lasers for treating and right main bronchus where residual papillomas were noted. He had
endobronchial disease has been tested and accepted as an important therapeutic also received methotrexate therapy for approximately 1 year but this was
modality for obstructive endobronchial or tracheal lesions. Although the primary discontinued for lack of any improvement. He continued to have cough
role of laser resection in oncology patients is a palliative one, this technique may and shortness of breath due to distal airway disease (Figure 1) and was
allow a prolonged survival when combined with other therapies as subsequent referred to the Pulmonary Critical Care Division in May 2004. At
surgical excision and/or radiation therapy (2). Resection of the bronchus or a bronchoscopy the right main bronchus was extensively involved with 40%
lobectomy with clear surgical margins in the absence of disease in the lymph narrowing by papillomas which also involved the lateral wall of the right
nodes is usually curative in patients with low-grade mucoepidermoid tumors. One mainstem bronchus continuously down to the level of the right upper lobe
study showed long-term survival ranging from 5 to 23 years, averaging 12.8 years which was almost completely obstructed. Due to extensive disease he
(3). In this patient, because of the location of the tumor and expected morbidity, received multimodal therapy with argon plasma coagulation (APC) to
surgery was not performed. One of the earliest series of bronchial adenoid cystic ablate papillomas and Interferon 2 alpha starting in June and July 2004,
carcinoma (cylindroma) and mucoepidermoid carcinoma found that some pa- respectively. Control of disease was significant after three APC treatments
tients had a prolonged survival and minimally associated morbidity, even when (Figure 2). The bronchoscopic approach used the side fire probe on two
residual tumor was knowingly left behind (4). occasions and straight fire probes at other times. The latest bronchoscopy
CONCLUSION: Low grade mucoepidermoid tumors have excellent in April 2005 showed complete resolution of disease in the distal trachea
response to laser resection which is minimally invasive with low morbidity. and orifice to right main bronchus (Figure 2). The right upper lobe was
Long term survival of these slowly growing tumors following laser fully patent with minimal residual disease proximal to the lobar orifice
resection is yet to be studied. which was treated with APC. Interferon 2 Alpha dosing started in July
2004 initially on 9 million units three times a week. This was later reduced
to 4.5 million units three times a week and subsequently to 4.5 million
units twice a week. The dosage reduction was elected due to clinical
control of RRP as assessed by bronchoscopy and to reduce systemic
symptoms of fever, malaise, fatigue and myalgias following Interferon 2
Alpha injections.
DISCUSSIONS: There have been earlier case reports of APC being
used to treat lower respiratory disease with good results. Presumably APC
would also be effective in the upper respiratory tract. A comparison
between the two methods may be warranted. The use of Interferon 2
Alpha also needs to be assessed independently of APC and CO2 Laser.
CONCLUSION: The combination of argon plasma coagulation with
Interferon 2 Alpha injections has produced substantial improvement in a
relatively short interval of 10 months of treatment. A declining dose of
Interferon 2 Alpha has been used and appears adequate to control disease.
DISCLOSURE: Mudusar Raza, None.
ENDOBRONCHIAL ACTINOMYCOSIS ASSOCIATED WITH A

PISTACHIO NUTSHELL
Manish Joshi MD* Sunil Sharma MD Basil Varkey MD Kavita Mundey
MD William O’Neil MD Valarie Bonne MD Lee Hranicka RRT Veterans
Affairs Medical Center and Medical College of Wisconsin, Milwaukee, WI
REFERENCES: INTRODUCTION: Endobronchial actinomycosis is a rare condition

1 Kim, TS, Lee, KS, Han, J, et al. Mucoepidermoid carcinoma of the often associated with a foreign body aspiration. We report such a case in a

45-year-old man who presented with dyspnea and cough. Bronchoscopy

revealed a mass obstructing the bronchus intermedius and biopsy of the
lesion revealed actinomycosis. On repeat bronchoscopy after 4 weeks of
antimicrobial treatment, a pistachio nut shell was seen and successfully
removed.
CASE PRESENTATION: A 45-year-old man, who never smoked, pre-
sented with dyspnea and cough that was attributed to worsening asthma. He had
no other symptoms. There was no history of cerebrovascular event, swallowing
difficulty, dental or periodontal problem. He had a clinical diagnosis of asthma for
9 years. He was treated with inhaled steroids and bronchodilators and oral
corticosteroid without relief of his symptoms.On examination his vital signs were
normal and oxygen saturation by pulse oximetry was 97%. No lymphadenopathy
or clubbing was detected. Auscultation of the lung fields showed wheezing more
prominent on the right side. The rest of his physical examination was unre-
markable.Laboratory results revealed normal complete blood count and serum
chemistries. Chest radiograph showed nonspecific right sided parenchymal
infiltrate. Computed tomography (CT) of the chest revealed marked narrowing of
the bronchus intermedius, bronchiectasis of the right lower lobe with an infiltrate.
Fiberoptic bronchoscopy revealed an endobronchial mass causing near total
occlusion of the bronchus intermedius with severe mucosal inflammation and
edema, mimicking endobronchial carcinoma. The bronchial mucus membrane
was very friable and bled easily. Endobronchial biopsy revealed large multiple DISCLOSURE: Manish Joshi, None.
colonies of Actinomyces sp. (“sulphur granules”) surrounded with inflammatory
cells (Fig 1).He had allergy to penicillin. Intravenous ceftriaxone via an indwelling BRONCHOSCOPIC LUNG BIOPSY EVIDENCE OF CALCIUM
catheter was started. Bronchoscopy repeated 4 weeks after antibiotic treatment OXALATE CRYSTALS SIGNALS ASPERGILLUS NIGER INFEC-
revealed a pistachio nutshell lodged in the bronchus intermedius (Fig 2) which TION
was successfully removed using endobronchial biopsy forceps. Two weeks after Septimiu D. Murgu MD* Henri Colt MD UCI Medical Center, Orange, CA
bronchoscopic removal of the nutshell the patient was completely free of
symptoms. He was continued on antibiotic for 3 months. INTRODUCTION: Calcium oxalate crystals (COC) have been reported on
DISCUSSIONS: Actinomycosis is most frequently caused in humans by surgical or autopsy specimens or in association with hyphae or conidia in tissue
Actinomyces israelii and the usual route of lung infection is by aspiration of and cytology specimens from patients with aspergillosis. We report here a case of
oropharyngeal or gastrointestinal secretions. Endobronchial actinomycosis is Aspergillus niger (A. niger) infection in which the presence of crystals on
a rare form of pulmonary actinomycosis most often associated with foreign transbronchial biopsy specimens was the only laboratory indication of aspergillosis
body aspiration. Chouabe et al reported the largest series of endobronchial before fungal cultures became positive.
actinomycosis cases in predominantly men with known risk factors – poor CASE PRESENTATION: A 71 year-old woman presented with two
dental hygiene and debilitation- for actinomycosis (1). The presenting month history of fevers, night sweats, hemoptysis and chest pain after one
symptoms in these patients were cough, hemoptysis and recurrent pneumo- year of fatigue, decreased appetite and a 35 lb weight loss. Past medical
nia. The initial bronchoscopy findings in all the cases revealed endobronchial history is significant for diabetes and pulmonary tuberculosis treated with
mass obstructing the bronchial lumen suggestive of malignancy. Foreign body four drug therapy nine months earlier. Physical examination disclosed
was detected during initial bronchoscopy in 50% of cases; chicken bone being crackles at the right lung base.Computed tomography showed lingular,
most common. Our case differs from previously reported cases. Our patient right middle lobe and lower lobe thick walled cavities with intracavitary
is young, without debilitation or predisposing conditions. Our literature solid lesions and focal bronchiectasis (Figure 1). Bronchoscopy demon-
review did not reveal any previous case of endobronchial actinomycosis strated inflammation and necrosis of the mucosa of the right middle and
associated with pistachio nutshell aspiration. However our patient is similar to lower lobe bronchi. Bronchoscopic lung biopsies (Figure 2) revealed acute
the cases cited previously in having an endobronchial mass mimicking tumor. and chronic inflammation with the presence of oxalate crystals. Giemsa
(GMS) and acid fast bacilli stains showed no fungal or acid -fast organisms.
CONCLUSION: In conclusion, endobronchial actinomycosis associ-
Fungal serology, blood culture, brushings and biopsy cultures were
ated with foreign body aspiration is a rare cause of endobronchial mass nondiagnostic but the bronchioloalveolar lavage (BAL) and sputum
and atelectasis. In our patient, prominent wheezing on the right side and cultures eventually grew A. niger. Six weeks after initiating antifungal
CT finding of a nearly occluded bronchus intermedius raised the diag- therapy with voriconazole the patient became asymptomatic.
CASE REPORTS
nostic consideration of bronchogenic carcinoma. Histopathologic exami- DISCUSSIONS: Oxalosis is the deposition of calcium oxalate in
nation confirmed the diagnosis of actinomycosis. The important learning tissues. It results from a variety of causes including nephropathies and
point in this case is the importance of repeating fiberoptic bronchoscopy uremia, primary hyperoxaluria, ileal diseases and jejunoileal bypass,
after few weeks of antibiotic therapy to specifically look for foreign body excessive intake of oxalate-rich food, ethylene glycol poisoning, methoxy-
which may be missed on the initial bronchoscopy. flurane anesthesia, glycerol and xylitol administration, vitamin C intoxica-
REFERENCE: tion and pyridoxine and thiamine deficiency. It is usually systemic but may
1 Chouabe, S, Perdu, D, Deslée, C, et al (2002) Endobronchial be localized with the renal tubules most commonly involved. In 1891
actinomycosis associated with foreign body: four cases and a review oxalic acid was demonstrated as a mycotoxin produced by A. niger.
of the literature. Chest 121, 2069-2072 Starting in the 1960s, COC have been reported in association with
pulmonary fungal infection, especially A. niger and were found to be more
common in patients with diabetes. Oxalate, produced through the tricar-
boxilic acid cycle, combines with calcium ions to form calcium oxalate
crystals (COC). Oxalic acid has been incriminated in blood vessel
destruction, tissue damage and necrosis which seem to be more severe in
immunosuppressed states. The presence of COC in pulmonary cytology
specimens has been considered a reliable marker for the presence of
Aspergillus infection, which may be detected before cultures are positive.
Previous case reports, however, revealed the COC only in surgical or
autopsy specimens or along with fungal elements (hyphae or conidia) in
tissue or cytology specimens. In our patient, histology and cytology
specimens failed to reveal any fungal elements. The bronchoscopic biopsy
specimens showed crystals consistent with calcium oxalate. Sputum and
BAL culture confirmed A. niger infection and patient responded well to
voriconazole. Patient had no clinical or laboratory evidence of systemic
oxalosis. We consider that previous tuberculosis infection and diabetes

predisposed this patient to aspergilloma and chronic necrotizing aspergil- We present a case of granulocytic sarcoma presenting as a single necrotic
losis (CNA) due to A. Niger. The most common predisposing factor for pulmonary mass.
aspergilloma is the presence of a preexisting lung cavity. On a background CASE PRESENTATION: A 39-year-old recently immigrated Jamaican male
of diabetes, the metabolic by –product oxalic acid may have caused further presented to an outside institution with three weeks of fever, chills, night sweats,
tissue destruction leading to CNA, which is mostly seen in fact, in mildly weight loss, and productive sputum. The patient was a 25-pack-year smoker, and
immunosuppressed hosts such as this patient. he also smoked marijuana and “crack” cocaine. He denied a history of active TB
CONCLUSION: In summary, this case demonstrates that calcium or TB exposure. He was noted to have a LUL lung cavity and left pleural effusion.
oxalate crystals in bronchoscopic lung biopsy specimens can signal A. niger At the outside institution he was diagnosed with AML and received treatment for
infection even in the absence of identified fungal elements. community-acquired pneumonia with subsequent clinical improvement. The
pleural fluid analysis for cytology was non-diagnostic. He was transferred to our
hospital for further evaluation and treatment. Physical examination was unreveal-
ing. An initial CT scan showed a moderate sized pericardial effusion, mediastinal
adenopathy, and a left-upper lobe cavity at the left heart border with a pleural
effusion. Laboratory evaluation was significant for a white-blood cell count of
106,500 cells/mm3 with 65% blasts and hemoglobin of 7.4 g/dl. A bone marrow
biopsy was consistent with a diagnosis of acute myelogenous leukemia. The
patient underwent bronchoscopy that showed a mild amount of blood-tinged
secretions from the left-upper lobe. Special stains of bronchial washings and
lavage were negative for fungus and acid-fast bacilli. An endobronchial biopsy was
unrevealing, and so a transthoracic needle biopsy was performed. The fungal and
AFB stains were again negative, but cytology showed a mass of necrotic tissue
with cells that were immunohistochemically consistent with myeloblasts. The
patient was started on a seven-day induction chemotherapy protocol of Dauno-
rubicin and Cytarabine. It was started thirteen days after transfer. The patient had
transient bone marrow suppression but blasts were still present on recovery.
Nevertheless, there was an improvement in the necrotic cavity and adenopathy
seen on a subsequent CT scan about one month later.
DISCUSSIONS: Granulocytic sarcomas are associated with myelopro-
liferative disorders or leukemias. They have been reported both before
and after the hematological diagnosis(1). Usually, there is known bone
marrow involvement at the time of presentation and a clinical diagnosis is
made. Immunohistochemical staining will reveal myeloperoxidase positiv-
ity though it is not universally present, and the tumor will share receptors
with the myeloblasts in bone marrow. In this case, the patient was at risk
for active TB and had supportive radiographs. Therefore, a definitive
diagnosis had to be made before chemotherapy began. In a large case
series, the most common sites were the soft tissue, bone, lymph nodes,
and skin(2). Granulocytic sarcomas have been much less frequently noted
in the thoracic cavity, and of those, the majority have solely involved the
mediastinum or pleural space(3).There have been cases of these granu-
locytic sarcomas presenting as endobronchial tumors, multiple bilateral
parenchymal nodules and interstitial infiltrates.(3-6).
CONCLUSION: A young male presented with constitutional symp-
toms and was found to have an abnormal chest radiograph and peripheral
smear. The evaluation revealed AML with granulocytic sarcoma in the
form of a necrotic lung nodule with mediastinal adenopathy and pleural
effusion. Involvement of the lung is less common with granulocytic
sarcoma, and this particular presentation is distinctly unusual.
REFERENCES:
1 Menasce LP. Histopathology.1999 May;34(5):391-8.
2 Neiman RS. Cancer. 1981 Sep 15;48(6):1426-37.
3 Takasugi JE. J of Thorac Imaging. 1996 Summer;11(3): 223-30.
4 Hicklin GA. Chest. 1988 Sep;94(3):655-6.
5 Dugdale DC. Am Rev Respir Dis. 1987 Nov;136(5):1248-50.
DISCLOSURE: Septimiu Murgu, None. 6 Lee DA. J Pediatr Hematol Oncol. 2004 July;26(7):431-434.
Cancer Cases II
4:15 PM - 5:45 PM
GRANULOCYTIC SARCOMA PRESENTING AS A NECROTIC
LUNG NODULE
Vivek Kaul MD* Joseph Cicenia MD Patricia Tietjen MD St. Vincent
Catholic Medical Center, New York, NY
INTRODUCTION: Granulocytic sarcoma is a mass of myeloid pre-

cursor cells that are extra-medullary in location. The masses of leukemia
cells have also been called chloroma because of their green hue on
exposure to air. They have been reported in many sites in the human body. DISCLOSURE: Vivek Kaul, None.

Cancer Cases II, continued
CYSTIC AND SOLID BRAIN METASTASES IN SMALL CELL

LUNG CARCINOMA: A CASE REPORT
Vijay P. Balasubramanian MD* Mehmet Kocak MD Ralph Schapira MD
Medical College of Wisconsin, Milwaukee, WI
INTRODUCTION: Cystic brain metastases are unusual. Case series

and reports have described cystic brain metastases in association with
non-small cell lung cancer (NSCLC). We present the first case of a
cystic brain metastasis associated with small cell lung cancer (SCLC).
The metastasis was associated with the initial presentation of a lung
mass. Biopsy of both the cystic brain lesion and lung mass demon-
strated SCLC.
CASE PRESENTATION: A 70-year-old male was admitted for a
total knee replacement. He had a 75 pack-year history of smoking and
denied any respiratory symptoms. On admission, the respiratory exam
was unremarkable but the neurological examination demonstrated a
left temporal field defect and left-sided weakness, both attributed to a
prior stroke. Preoperative chest radiograph revealed a right perihilar
opacity. Post-operative evaluation of the lung opacity by chest CT scan
demonstrated a right middle lobe mass (3.7x4.1x3.5 cm) and right hilar
and mediastinal lymphadenopathy. Post-operatively, the patient devel-
oped worsening left-sided weakness. A brain MRI showed a large, DISCLOSURE: Vijay Balasubramanian, None.
fluid-filled cystic lesion with a small, enhancing mural nodule
(6.9x5.4x5.4 cm) in the right temporal region with mild edema but with
midline shift and effacement of hemispheric sulci (Figure 1a).
The cystic mass revealed increased T1 signal on the pre-contrast image
as opposed to decreased signal expected from pure cystic fluids or CSF
(Figure 1b). This increased signal may be explained by its protein- TESTICULAR EMBRYONAL RHABDOMYOSARCOMA WITH
aceous content, cellular infiltrate, and/or subacute blood products. In METASTASIS TO THE LUNG: FIRST REPORTED PEDIATRIC
addition, multiple small solid lesions were noted within the brain with CASE
surrounding edema (Figure 2). The patient underwent a craniotomy Saleh Alharbi MD* Raquel Consunji-Araneta MD Faisal Almohammadi
with decompression of the brain cyst and biopsy of the cyst MD University of Manitoba, Winnipeg, MB, Canada
wall. Cytological analysis of the cyst fluid showed SCLC. The biopsy of
the cyst wall also showed SCLC. The patient’s neurological INTRODUCTION: We report a rare case of testicular rhabdomyo-
examination significantly improved following cyst decompression. A sarcoma with endobronchial metastases in a 14-year old male who
CT-guided biopsy of the right middle lobe mass confirmed a pure presented with shortness of breath and fatigue. Multiple nodular densities
SCLC. were seen on chest x-ray (CXR), with chest CT showing multiple
DISCUSSIONS: Brain metastases are an important cause of mor- metastatic lesions.
bidity and mortality in cancer patients, occurring in approximately 10 CASE PRESENTATION: A 14-year-old-male presented with a
to 30% of adults with cancer. The lung is the primary site of 6-month history of persistent asthma-like symptoms and easy fatiguability,
approximately 70% of cancers that initially present with symptomatic despite treatment with appropriate anti-asthma medications. On admis-
sion, he was tachypneic (35/min), with intercostal retractions. There was
brain metastases. Metastases to the brain are usually symptomatic, the
no cyanosis or significant lymphadenopathy. The chest was dull on
most common being headache (40 to 50%), motor deficit (20 to 40%), percussion. Breath sound intensity was generally diminished; there were
cognitive or affective disturbance (30 to 35%) and seizures (10 to 20%). no adventitious sounds. There was no hepato-splenomegaly or ascites.
CASE REPORTS
Metastases from breast, colon, and renal cell carcinoma are often Chest radiograph (CXR) and chest CT showed multiple nodular lesions,
single, while lung cancer and malignant melanoma have a greater with the largest measuring approximately 3 cm. A thorough physical
tendency to produce multiple metastases.Cystic brain metastases examination revealed a swollen right testicle, which the patient had first
from lung carcinoma are unusual. In a series of 25 consecutive patients noticed 6 months earlier; he was reluctant to disclose this information to
with NSCLC undergoing open resection of one or more symptomatic anyone. Initial work-up (complete blood and differential count, urea,
brain metastases, 19 were solid and 9 were cystic. In another report of creatinine, glucose, serum electrolytes, sedimentation rate, liver enzymes,
cystic brain metastases preceding a diagnosis of lung carcinoma, bilirubin, alkaline phosphatase, urinalysis and protein electrophoresis) was
only NSCLC was described. A further case report described a cystic normal. Capillary blood gas result showed pH: 7.43, pCO2: 35, HCO3: 25.
lesion with enhancing mural nodule associated with metastatic adeno- Human Chorionic Gonadotropin and ␣-fetoprotein levels were normal.
carcinoma with no localization of the primary. An analysis of cytology Lactate dehydrogenase (LDH) was elevated (962-IU/L). Sputum speci-
from 115 consecutive biopsies of intracranial lesions (95 solid men was negative for malignant cells. A combined restrictive-obstructive
and 20 cystic) demonstrated that 3 of the 20 cystic lesions pattern was evident on pulmonary function testing (body plethysmogra-
were malignant of which 2 were primary brain malignancies phy). Enlarged mesenteric and retroperitoneal lymph nodes were seen on
and the remaining one was a metastatic adenocarcinoma (site unspec- abdomino-pelvic CT scan. A cystic and solid mass measuring 4.4 x 4.4 cm
arose from within the right testis with infiltration into the adjacent
ified).1.
epididymis and spermatic cord and extension into the right inguinal canal.
CONCLUSION: We describe the first reported case in which SCLC Biopsy of the testicular mass revealed embryonal rhabdomyosarcoma. His
was associated with a cystic brain metastasis. Although cystic brain bone scan was abnormal and bone marrow aspirate showed infiltration
metastases are associated with NSCLC, this case demonstrates that and abnormal cells. He was classified as having Stage 3C (metastasis above
consideration should be given to SCLC when evaluating a cystic brain the diaphragm) testicular cancer. After orchiectomy, the Oncology service
lesion. began chemotherapy with alternating cycles of Ifosfamide and Etoposide
REFERENCE: with Vincristine, Actinomycin and Cyclophosphamide followed by Radio-
1 Collaco LM, Tani E, Lindblom I, et al. Stereotactic biopsy and therapy. Ten months into therapy, his repeat CXR and chest CT showed
cytological diagnosis of solid and cystic intracranial lesions. Cytopa- marked improvement; his bone marrow findings, LDH (196-IU/L) and
thology 2003; 14:131-5 lung function were normal.

METASTATIC BASAL CELL CARCINOMA PRESENTING AS A

SOLITARY PULMONARY NODULE
Jeffrey S. Kim MD* Kristin Fless MD UMDNJ -Newark, Newark, NJ
INTRODUCTION: Metastatic basal cell carcinoma is a rare entity,

having only 230 reported cases in the last century. Pulmonary complica-
tions while also reported are rarer still. We present a case of a metastatic
basal cell carcinoma presenting as a solitary pulmonary nodule.
CASE PRESENTATION: An 80 year-old male was noted to have an
elevated right hemidiaphragm as well as right middle lobe atelectasis during a
preoperative chest radiograph for an Achilles tendon repair (figure 1). Three
months subsequently, the patient complained of a persistent cough. A repeat
chest radiograph demonstrated this persistent abnormality and the patient was
referred for pulmonary evaluation. The patient had a past medical history
significant for hypertension, prostate cancer, as well as a 20 pack-year smoking
history with a moderate obstructive defect on pulmonary function testing.
Additionally, the patient had a basal cell carcinoma of the scalp removed nine
years prior. Computed tomography(CT) of the chest revealed a 1.5 centimeter
irregular nodule within the right middle lobe as well as atelectasis versus scarring
of the right middle and lower lobes. The nodule demonstrated an intense uptake
on positron emission tomography(PET). The patient underwent a right thoracot-
omy with wedge resection of the nodule. Initial frozen section identified the
nodule as a possible adenoid cystic carcinoma. Further immunohistochemical
staining revealed the nodule to be a basal cell carcinoma, morphologically similar
to the lesion resected nine years earlier.
DISCUSSIONS: When a child presents with discrete lung masses, DISCUSSIONS: Metastatic basal cell carcinoma is a rare clinical entity,
with likely a distinct pathophysiology. The incidence ranges from 0.01-0.1%,
apart from the more common differential diagnoses (infectious and
and the median time of onset from primary diagnosis appears to be nine
immunologic causes), neoplasms (benign, malignant or metastatic) must years. While basal cell carcinoma typically is slow growing and has an
also be considered. Majority of malignant lung lesions in children are excellent prognosis, metastatic basal cell growth is aggressive and portends a
metastatic, so it becomes essential to search for a primary site. Testicular poor prognosis. Median survival after metastasis has been reported to be eight
tumors constitute a very small percentage of all malignant tumors in men, months, with rare patients surviving more than 1.5 years. Primary tumor size
and account for 11.4% of deaths from cancer in males between 20-35 appears to correlate with likelihood of metastasis. This is thought to be
years old. Trauma, cryptochordism, and exogenous maternal estrogen (in primarily from lymphatic and or hematogenous spread. Basal cell carcinoma
utero) have all been associated with its development. The most common however, displays a stromal dependency, that is, successful implantation to
presentation is pain, swelling or hardness of the testis. A few patients may other tissues occurs only if the stroma is included. Interestingly, there is little
already have signs or symptoms of metastatic disease such as back pain, stroma in the lung and liver, though metastasis to these organs have been
cough and dyspnea (indicating pulmonary metastasis), nausea and vomit- described. This suggests that metastatic cancer cells to these organs may have
ing, bone pain, or central nervous system manifestations. Diagnosis is developed a mechanism for stromal independence.
made by CT scans, serum tumor markers and surgical biopsy. Orchiec- CONCLUSION: Basal cell carcinoma is a rare clinical entity. We
tomy followed by chemotherapy and/or radiation is the treatment of present a case of metastatic basal cell carcinoma presenting as a solitary
choice for nonseminomatous tumors. Recurrence may occur within 2 pulmonary nodule. The time course from primary disease to onset of
years so intensive surveillance and follow up is necessary. Overall survival metastasis in our patient was nine years, which is consistent with current
literature. Metastatic basal cell to the lung carries a poor prognosis, and
rate for this patient’s disease stage is 48% at 5-years.
may suggest a novel adaptation, that is, stromal independence.
CONCLUSION: 1. This is the first reported pediatric case of embry- REFERENCES:
onal rhabdomyosarcoma with metastasis to bone marrow and lung (Stage 1 Robinson, J.K., Dahiya, M., Basal cell Carcinoma with Pulmonary
3C). 2. When respiratory symptoms in a child do not respond to and Lymph Node Metastasis Causing Death. Arch Dermmatol.
treatment, and discrete lung masses are seen on CXR, a thorough clinical 2003;139:643-648.
history and physical examination is essential for diagnosis. 3. Most young 2 von Domarus, Stevens, H., Metastatic basal cell carcinoma. Report
males are unaware of testicular cancer, a highly curable neoplasm that can of five cases and review of 170 cases in the literature. J Am Acad
be detected by self-examination. Dermatol. 1984 Jun;10(6):1043–1060
DISCLOSURE: Saleh Alharbi, None. DISCLOSURE: Jeffrey Kim, None.

OVARIAN METASTASIS OF SMALL CELL LUNG CARCINOMA Five of these cases have been small cell carcinoma(2,3). Based upon the
Steven Kadiev MB, BCh* Chitra Uppaluri MD Kamran Quraishi MD rarity of the metastasis of SCLC to the ovary, initial assumptions lead to
Ohio State Medical Center, Columbus, OH the incorrect diagnosis of a primary ovarian carcinoma. Frozen section of
the adnexal tumor at surgery revealed small cell carcinoma (SCC).
INTRODUCTION: Small cell lung cancer (SCLC) occurs almost exclu- Differential diagnosis of ovarian SCC deposits includes pulmonary and
sively in smokers and invariably presents with a central pulmonary mass. In the extra-pulmonary metastases (intestine, thymus, skin, salivary glands,
majority of cases, it is metastatic on presentation, with predilection for spread to esophagus, bladder, prostate, cervix and undetermined) as well as primary
the liver, adrenal glands, bone and brain. We report an unusual case of SCLC ovarian cancer(4). In view of the latter being rare and having a defined
presenting with a massive left adnexal mass. pulmonary source with corroborative histological staining, it was con-
CASE PRESENTATION: A 53-year-old post menopausal caucasian cluded that this patient has SCLC with an ovarian metastasis(5).
woman with a history of smoking, hypertension, mild intermittent asthma and CONCLUSION: To our knowledge, this is a rare report of SCLC with
dyslipidemia presented to the ER with a one month history of lower ovarian metastases.
abdominal fullness, pain and recent vaginal bleeding. Review of systems REFERENCES:
revealed one month of intermittent fevers, chills, nausea and vomiting as well 1 Guinee DG Jr; Fishback NK et al. The spectrum of immunohisto-
as a 40 pound weight loss over the last six months. She also described mild chemical staining of small-cell lung carcinoma in specimens from
exertional dyspnea but no other cardiopulmonary symptoms. Initial examina- transbronchial and open-lung biopsies. Am J Clin Pathol 1994 Oct;
tion revealed wasting, pallor, and a tender lower abdominal mass. Chest 102(4):406-14.
auscultation was normal. Abdominal CT scan revealed a 20 cm heteroge- 2 Yeh KY, Chang JW, et al. Ovarian metastasis originating from
neous left adnexal mass which was initially believed to be a primary ovarian bronchioloalveolar carcinoma: a rare presentation of lung cancer.
cancer. She was discharged for an outpatient workup. A week later the patient Jpn J Clin Oncol. 2003 Aug;33(8):404-7.
returned with progressive symptoms. A chest CT indicated a 6 cm left hilar 3 Sukumvanich P, et al. Recurrent small cell lung cancer presenting as
mass with significant compression of the left pulmonary artery and left main bilateral adnexal masses. Gynecol Oncol. 2005 Jan;96(1):232-4.
stem bronchus resulting in almost complete left sided atelectasis. Subse- 4 Eichhorn JH, et al. Nonpulmonary small cell carcinomas of ex-
quently she developed hypoxic respiratory failure requiring intubation. tragenital origin metastatic to the ovary. Cancer. 1993 Jan 1;71(1):
Pulmonary embolus was excluded. When stable, she underwent abdominal 177-86
hysterectomy with bilateral salpingo-oophorectomy and tumor removal. 5 Mebis J, De Raeve H, et al. Primary ovarian small cell carcinoma of
Bronchoscopy noted a large friable fleshy mass occupying most of the left the pulmonary type: a case report and review of the literature. Eur
main stem bronchus. Histology from both sites indicated small cell carci- J Gynaecol Oncol. 2004;25(2):239-41.
noma. Immunostains of the endobronchial lesion were positive for chromo- DISCLOSURE: Steven Kadiev, None.
granin, neuron specific enolase and synaptophysin which indicated an almost
certain diagnosis of primary small cell carcinoma of the lung(1). Further
staging was negative. Once extubated, our patient underwent focal thoracic MICROEMBOLI OF TRANSITIONAL CELL CARCINOMA
radiation therapy directed at the compressive pulmonary lesion and then (TCC) CAUSING RAPIDLY PROGRESSIVE DYSPNEA AND
began an outpatient systemic chemotherapy regimen. RIGHT HEART FAILURE
Timothy P. Collins DO* Carla R. Lamb MD Anthony W. Gray Jr MD
Lahey Clinic Medical Center, Burlington, MA
INTRODUCTION: Tumor microemboli to the pulmonary vasculature

is described in many different cancers. This case illustrates the diagnostic
and clinical challenges associated with acute cor pulmonale and rapidly
progressing dyspnea in a man with a history of treated bladder cancer.
CASE REPORTS
CASE PRESENTATION: A 70 year old man presented with a six month

history of nonproductive cough and progressive dyspnea. He had been
treated for community acquired pneumonia, hypersensitivity pneumonitis
and asthma with no improvement. Accompanying his symptoms were
episodes of pleuritic chest pain and a 30 pound weight loss. His past medical
history was significant for coronary artery disease, hypertension and type 2
diabetes. He was diagnosed cystoscopically with superficial bladder cancer
nine months prior to presentation and treated with 5 weeks of Bacille
Calmette-Guerin washings. Medications included metoprolol, salmeterol,
aspirin, lansoperazole and glyburide. His occupation was as a construction
worker. He had a 20 pack year tobacco history and quit 20 years ago. He had
pet parakeets. He traveled to Bermuda anually. Physical exam: blood pressure
150/90, heart rate 110, respirations 24, pulse oximetry 90% on room air and
81% with exertion. Jugular venous pressure was estimated at 6cm. There was
no heave or accentuated P2. Lung exam revealed crackles at the bases and
DISCUSSIONS: This case is highlighted by a typical central SCLC mid lung fields. There was 1⫹ lower extremity pitting edema without
with a massive adnexal tumor deposit. Review of the literature has clubbing. Diagnostic data included a negative serological workup for autoim-
identified only ten cases where lung cancer has spread to the ovaries(2,3). mune and connective tissue disease. Purified protein derivative skin test for

tuberculosis was positive. Pulmonary function testing revealed a mixed obstruc- inhibits the T lymphocyte proliferation that occurs in response to
tive and restrictive defect and a severely diminished diffusing capacity. Electro- antigenic and cytokine stimulation (2). This case describes a female
cardiogram revealed a new right bundle branch block. Chest radiograph revealed patient that developed a unilateral lung infiltrate related to sirolimus use.
bilateral lower lobe predominant patchy interstitial disease. Computed tomogra- CASE PRESENTATION: A 54 year-old Afro-American female was
phy anigiogram of the chest was negative for pulmonary emboli. Bronchoscopy seen in the pulmonary clinic due to a persistent right lower lobe infiltrate
was performed one day later, and revealed a monohistiocytic predominant lavage. (Figure 1). She had six years before a motor vehicle accident that caused
Stains and cultures for bacteria, fungi and acid fast bacillus were negative. end stage renal disease and got a cadaveric renal transplant at that time.
Biopsies were precluded by refractory hypoxemia. He was admitted to the She was on chronic immunosuppressive therapy with cyclosporine and
hospital and a video assisted thoracoscopic surgical (VATS) lung biopsy was tacrolimus but due to impaired renal function the first drug was changed
scheduled. 18 hours after admission, he was transferred to the ICU for tracheal to sirolimus eighteen months before she present to us. One month before
intubation. Refractory hypoxemia and hypotension complicated his ICU course. she went to an emergency department with cough and was diagnosed as
Additional diagnostic data in the ICU included persistent chest radiograph having pneumonia, but despite adequate antibiotic therapy the right lung
abnormalities and negative lower extremity Doppler ultrasounds. An echocardio- infiltrate did persist. The patient was asymptomatic at that time and the
gram revealed a hypertrophied, dilated and hypokinetic right ventricle and no physical exam was unremarkable except for mild inspiratory rales in the
shunt. Pulmonary artery (PA) catheterization revealed right atrial pressures of 22 right lung base. A ventilation perfusion scan was done and resulted as low
mmHG, PA pressures of 95/45 and PA wedge pressure of 12 mmHG. 48 hours probability for pulmonary embolism. She then got a bronchoscopy as an
after admission, he suffered an asystolic arrest and was not able to be resuscitated. outpatient. The study of the bronchoalveolar fluid did show a mild
A post mortem examination was performed. neuthrophilic predominance and no malignancy or infection. Transbron-
DISCUSSIONS: The final cause of death was acute on chronic right chial biopsy of the right lower lobe revealed interstitial pneumonitis with
heart failure secondary to diffuse pulmonary metastasis of high grade organizing pneumonia and mild fibrosis compatible with sirolimus toxicity.
transitional cell carcinoma of the bladder with tumor microembolization Sirolimus was held, and the patient was kept on tacrolimus and low dose
to the pulmonary vasculature. This case of tumor microembolization prednisone. In a computer tomography of the chest two months latter
represents a diagnostic challenge first described in 1897 by Schmidt in a there was near complete resolution of the chest infiltrate (figure 2) and
small autopsy series(1). Similarly, over a century later, this diagnosis is the patient did remain asymptomatic.
rarely made ante mortem. Common sources include liver, breast, stom-
ach, renal, prostate and choriocarcinoma(2). This patient was diagnosed
with superficial bladder cancer by cystoscopy 9 months prior to admission.
Ironically, after a 5 week course of immunotherapy, his repeat cystoscopy
was precluded by dyspnea and hypoxemia.
CONCLUSION: Tumor microembolization is diagnostically challeng-
ing and almost always fatal. Diagnosis by careful aspiration of blood for
cytological examination from a wedged pulmonary artery catheter has
shown promise in small case report series1. Tumor microembolization
should be suspected in patients with a history of malignancy, clinical signs
of right heart failure, hypoxemia and a negative thromboembolic workup.
REFERENCE:
1 Masson RB NEJM 1989 321:71-762.King MB Clin Chest Med 1994
15:61-80
DISCLOSURE: Timothy Collins, None.
DISCUSSIONS: The use of sirolimus is not without potential morbid

side effects. Sirolimus has been shown to cause dose-dependent hyper-
Drugs and the Lung cholesterolemia, hypertriglyceridemia and thrombocytopenia. Since the
year 2000 pulmonary toxicity has been recognized as another potential
4:15 PM - 5:45 PM adverse effect, with more than 40 cases reported in the literature so far
(1). The case described here is remarkable due to the paucity of symptoms
SIROLIMUS LUNG TOXICITY AS A UNILATERAL LUNG INFIL- and the unilateral, rather than bilateral, lesion on the lung. All the cases
TRATE of sirolimus lung toxicity described so far have bilateral lesions in the
Daniel A. Salerno MD* Thomas Jefferson University Hospital, Philadel- lungs. Most of the cases in the medical literature of drug-induced
phia, PA pneumonitis are bilateral (3,4).
CONCLUSION: With the more common use of sirolimus in
INTRODUCTION: Sirolimus was introduced in transplant medicine transplant patients physicians must be aware of the possibility of lung
in 1999. It was designed to provide adequate immunosupression to the toxicity related to this drug. The diagnosis can be a challenge due to
transplant recipient while avoiding the nephrotoxic side effects associated the concomitant use of other pneumotoxic drugs and the need of
with calcineurin inhibitor therapy: cyclosporine, tacrolimus (1). Sirolimus aggressive exclusion of infection in this immunosupressed population.

Drugs and the Lung, continued
The resolution of symptoms and lung infiltrates usually is fast after the within 6 weeks, and ultimately discharged her without any oxygen
discontinuation of the drug, but there are some reports that claim a therapy.
better outcome with the addition of corticosteroids (5). DISCUSSIONS: HA main toxicity is a chemical pneumonitis which
REFERENCES: can progress to acute respiratory distress syndrome (ARDS). The mainstay
1 Pham PT, Pham PC, Danovitch GM, Ross DJ, Gritsch HA, of HA is supportive therapy. The use of steroids is not fully supported in
Kendrick EA, Singer J,Shah T, Wilkinson AH. Sirolimus-associated the literature. Surfactant has been demonstrated to be helpful in experi-
pulmonary toxicity. Transplantation. 2004 Apr 27;77(8):1215-20. mental animals, but not in humans. The use of iNO in adult patients with
2 Morelon E, Stern M, Israel-Biet D, Correas JM, Danel C, Mamzer- ARDS is controversial. We demonstrate the successful treatment of a
Bruneel MF,Peraldi MN, Kreis H. Characteristics of sirolimus- pediatric case of ARDS secondary to HA using iNO. In this patient, iNO
associated interstitial pneumonitis in renal transplant patients. was effective in improving oxygenation and pulmonary hypertension with
Transplantation. 2001 Sep 15;72(5):787-90. subsequent improvement in hemodynamics. Further studies are needed
3 Cleverley JR, Screaton NJ, Hiorns MP, Flint JD, Muller NL. to determine if this specific subset of patients with ARDS from HA have
Drug-induced lung disease: high-resolution CT and histological a unique therapeutic benefit from iNO.
findings. Clin Radiol. 2002 Apr;57(4):292-9. CONCLUSION: iNO may be effective for management of pediatric
4 Akira M, Ishikawa H, Yamamoto S. Drug-induced pneumonitis: patients with ARDS and refractory hypoxemia secondary to HA.
thin-section CT findings in 60 patients. Radiology. 2002 Sep;224(3):
852-60.
5 Haydar AA, Denton M, West A, Rees J, Goldsmith DJ. Sirolimus-
induced pneumonitis: three cases and a review of the literature.
Am J Transplant. 2004 Jan;4(1):137-9.
DISCLOSURE: Daniel Salerno, None.
USE OF INHALED NITRIC OXIDE FOR HYDROCARBON AS-

PIRATION
Pallavi P. Patwari MD* Kelly Michelson MD Children’s Memorial
Hospital, Chicago, IL
INTRODUCTION: Hydrocarbon aspiration (HA) is known to cause

significant pulmonary disease by inducing an inflammatory response,
hemorrhagic exudative alveolitis, and loss of surfactant function. We
describe a pediatric case of HA which caused hypoxia and pulmonary
hypertension that improved after administration of inhaled nitric oxide
(iNO).
CASE PRESENTATION: A previously healthy 18-month-old girl
was found by her parent coughing and gagging, and had a bottle of
“ultra-pure” lamp oil in her hand. She was taken to the emergency DISCLOSURE: Pallavi Patwari, None.
department, quickly became obtunded, was intubated, and then
transported to the PICU for further management.Upon arrival, her
weight was 9kg, temperature 37.3°C, pulse 191, blood pressure 103/51, PULMONARY EDEMA AFTER GRANULOCYTE-COLONY STIM-
respirations 48, and oxygen saturations 90% on 100% FiO2. On ULATING FACTOR TREATMENT IN A BONE MARROW DO-
examination, she had tachypnea, coarse breath sounds with inspiratory NOR
and expiratory crackles throughout, and no wheezing. We suctioned Courtney S. Lucado MD* Kevin Cooper MD VCU-MCV Campus,
copious bloody secretions from her endotracheal tube. On cardiac Richmond, VA
exam, she had tachycardia, no murmur, 5 second capillary refill, and
1⫹ peripheral pulses. The remainder of her exam was unremarkable INTRODUCTION: We report a case of non-cardiogenic pulmonary
Due to persistent hypoxia using conventional ventilation (maximum edema following granulocyte-colony stimulating factor (G-CSF) adminis-
settings on pressure regulated volume control mode were tidal volume tration in a bone marrow donor. During leukapheresis the patient was
100, peep 14, rate 24, and FIO2 100%), we switched to high frequency asymptomatic, but the next day she developed dyspnea, hypoxia, and
oscillator ventilation (HFOV) with initial settings of Hz 9, MAP 35, bilateral pleural effusions. The patient clinically improved with diuretic
CASE REPORTS
deltaP 38, and FIO2 100%. On HFOV she continued to have a and steroid therapy. We propose the significant leukocytosis triggered an
PaO2/FiO2 ratio ⬍100. She developed hypotension and coagulopathy, inflammatory response leading to acute lung injury.
requiring fluid boluses, pressor support, fresh frozen plasma, and CASE PRESENTATION: A 21 year old white female at 28 weeks
cryoprecipitate. We started stress dose hydrocortisone for hypotension gestation had G-CSF administration for bone marrow donation for her
refractory to escalating pressor support. We also started her on sister. She was healthy and her pregnancy had been unremarkable. Her
prophylactic antibiotics. ECMO was contraindicated because of pul- only medications were prenatal vitamins and iron. She received G-CSF
monary hemorrhage.On hospital day 2, we administered inhaled 900 ␮g (10 ␮g/kg/day) intravenous daily for three days via a #12 French
surfactant. Her PaO2 increased from 63 to 71 and FiO2 decreased catheter in the internal jugular vein. On day four, the WBC peaked at 40.0
from 97% to 95%. An echocardiogram showed mild left ventricular 10e9/L and the patient underwent leukapheresis. The next morning, the
dysfunction and mild right ventricular enlargement with a tricuspid catheter was removed taking care to avoid air embolism. Minutes later
regurgitation (TR) gradient of 30 - 35 mmHg. To support her blood while walking to her car, the patient developed shortness of breath and
pressure she required epinephrine at 0.8mcg/kg/min and dopamine at cough. She denied any associated chest pain. She had a presyncopal
15mcg/kg/min. Her urine output was 1.5ml/kg/hr on a furosemide episode and was taken to the emergency room. She was hypoxic with
infusion at 1mg/hr. On hospital day 3, we started iNO at 10ppm. Her arterial oxygen tension of 88 mmHg on 100% fractional inspired oxygen
oxygenation improved immediately as demononstrated by a change in via nonrebreather mask. On examination she was normotensive, alert and
PaO2 from 56 to 172 and ability to wean FiO2. Within the next 24 oriented, with diffuse bilateral rales. Laboratory data showed normal
hours we weaned her dopamine to 10mcg/kg/min and epinephrine to kidney and liver function. WBC had decreased to 33.0 10e9/L. Hemoglo-
0.1mcg/kg/min. Her urine output improved to 6ml/kg/hr. An echocar- bin was 12.6 g/dL. Platelets were at a nadir of 83 10e9/L following
diogram on hospital day 4 demonstrated trivial TR. By hospital day 5, apheresis. A CT angiogram performed immediately on arrival to emer-
we were able to stop the dopamine and epinephrine infusions. A repeat gency room showed bilateral pleural effusions without evidence of clot or
echocardiogram on day 23 showed trivial TR and lack of systolic septal air embolism. Echocardiogram showed normal systolic function. Serial
wall flattening consistent with resolution of pulmonary hypertension. cardiac enzymes and electrocardiogram were normal. The patient re-
We continued the iNO until hospital day 35. She also developed a ceived lasix and methylprednisone 40 mg intravenous every 12 hours.
pneumothorax and pneumatoceles which required no intervention. We After several days she had normal oxygen saturation on room air.
switched her to conventional ventilation within a month, extubated her Methylprednisone was stopped and the patient was discharged home.

DISCUSSIONS: Pulmonary edema has been reported in patients ALT was mildly elevated (67 U/L). A nonenhanced chest computed
receiving G-CSF. Capillary leak sydrome has been described in bone tomography (CT) scan showed an irregular right upper lobe mass
marrow recipients pretreated with G-CSF and is thought to be due to measuring 3.0 x 2.3 cm (Figure 1). A smaller (1.8 x 1.2 cm) right lower
leukocyte activation with recruitment of inflammatory mediators leading lobe peripheral wedge shaped lesion was also noted. There was increased
to systemic inflammation and increased capillary permeability. Acute lung density of both lesions (80 Hounsfield units) as well as hyperdensity of the
injury in bone marrow donors pretreated with G-CSF has been associated liver. Percutaneous fine needle aspirate of the right upper lobe mass was
with increased levels of interleukin-1 beta as in the case described by performed. Pathology revealed chronic interstitial inflammation, organiz-
Arimura et al. We are not aware of any reported cases of non-cardiogenic ing pneumonia, intra-alveolar aggregates of foamy macrophages and type
pulmonary edema after G-CSF in pregnant donors. The differential 2 pneumocyte hyperplasia. There was no evidence of malignancy. These
diagnosis also included embolic phenomenon. Air embolism was ruled out results are consistent with pulmonary changes due to amiodarone therapy
by CT, but remains a diagnositc consideration in the appropriate clinical (1). Repeat imaging three months after discontinuation of amiodarone
setting. Air embolism causes the formation of platelet-fibrin aggregates showed complete resolution of radiologic abnormalities (Figure 2).
and microthrombi leading to pulmonary hypertension and ultimately
capillary leak. Although G-CSF and air embolism can both result in
capillary leak, the treament is very different for each of these conditions
depending on the underlying mechanism.
CONCLUSION: In this case, marked leukocytosis following G-CSF
mobilization of peripheral blood progenitor cells may have triggered the
activation of inflammatory mediators in the lung resulting in non-
cardiogenic pulmonary edema. Our patient clinically improved with
diuretic as well as with the anti-inflammatory effect of steroids. Thus we
propose the diagnosis of pulmonary edema due to G-CSF induced
capillary leak.
REFERENCES:
1 Arimura K, et al. Acute Lung Injury in a healthy donor during
mobilization of peripheral blood stem cells using G-CSF factor
alone. Haematologica. 2005 Mar;90(3):ECR10.
2 Azevedo, AM et al. Life-treatening capillary leak syndrome after
G-CSF mobilization and collection of peripheral blood progenitor
cells for allogenic transplantation. Bone Marrow Transplantation.
2001; 28:311-312.
3 Kitamura S, et al. A risk of pulmonary edema associated with G-CSF
pretreatment. Masui 1997;46:946-950.
4 Murray and Nadel. Textbook of Respiratory Medicine, 3rd ed,
Saunders, Philadelphia, 2000.p.465
DISCUSSIONS: This case represents an unusual presentation of

amiodarone pulmonary toxicity. There have been isolated case reports of
similar presentations over the last 20 years, but none of these have
correlated radiographic and CT imaging with light and electron micros-
copy (2-4). Previous reports have postulated that these solitary depositions
are due to increased localized accumulation of the drug in an area of
previous inflammation (5). Interestingly, all previous reported cases
describe masses in the upper lobes, particularly right upper lobe. Our case
highlights the fact that primary lung cancer needs to be excluded in a long
standing smoker with a lung mass. Also, one needs to consider amiodarone
DISCLOSURE: Courtney Lucado, None. toxicity as a potential, usually reversible, etiology. With an increasing
population at risk for both lung cancer and cardiovascular disease, the
need for this distinction will become increasingly important. It also
AMIODARONOMA: ONE OF MULTIPLE FORMS OF AMIODA- demonstrates that amiodarone toxicity can occur after many years of low
RONE PULMONARY TOXICITY dose therapy. Previous studies support that toxicity correlates more closely
Julie Jarand MD* Jessi Minion MD Andrew Lee MD Francis Green MD with total cumulative dose and duration than with serum drug levels (1).
Richard Leigh MD University of Calgary, Calgary, AB, Canada This case is in keeping with previous reports who had concomitant thyroid
disease and/or liver toxicity. Abnormalities in other organ systems should
INTRODUCTION: Amiodarone pulmonary toxicity most commonly prompt physicians to look for pulmonary amiodarone toxicity in asymp-
takes the form of interstitial pneumontitis (1). Here we report a case of tomatic patients. Diagnosis can be made by combination of clinical,
amiodarone-induced lung injury presenting as a pulmonary mass. radiologic and/or pathologic findings with improvement after discontinu-
CASE PRESENTATION: A 66 year old woman presented to hospital ation of the drug.
with dyspnea secondary to congestive heart failure and high grade CONCLUSION: Amiodarone-induced toxicity should be included in
atrioventricular block. She underwent insertion of a biventricular pace- the differential diagnosis of pulmonary mass lesions in patients receiving
maker without complication. A routine post-procedure chest radiograph amiodarone.
revealed a right upper lobe mass. She was referred for further pulmonary REFERENCES:
assessment. She has a 50 pack year smoking history. Her past medical 1 Camus P et al. Amiodarone pulmonary toxicity. Clin Chest Med
history is significant for severe left ventricular dysfunction, supraventric- 2004;25:65-75.
ular tachycardia (SVT), recently diagnosed hypothyroidism, hyperlipid- 2 Arnon R et al. Amiodarone pulmonary toxicity presenting as a
emia and remote pulmonary embolism. SVT had been treated with solitary lung mass. Chest 1988;93:425-427.
amiodarone 200mg daily for four years (total cumulative dose approxi- 3 Piccione W Jr et al. Amiodarone-induced pulmonary mass. Ann
mately 300g). Her dyspnea had resolved following pacemaker insertion Thorac Surg 1989;47:918-919.
and medical management of heart failure. She denied any other respira- 4 Rodriguez-Garcia JL et al. Pulmonary mass and multiple pulmonary
tory or constitutional symptoms. Physical examination was unremarkable. nodules mimicking a lung neoplasm as amiodarone-induced pulmo-

nary toxicity. Eur J Intern Med 2001;12:372-376. variety of distinct forms including: subacute cellular interstitial pneumo-
5 Kuhlman JE et al. Amiodarone Pulmonary Toxicity: CT findings in nitis, pulmonary infiltrates with eosinophilia, desquamative interstitial
symptomatic patients. Radiol 1990;177:121-125. pneumonia, bronchiolitis obliterans organizing pneumonia, pulmonary
DISCLOSURE: Julie Jarand, None. fibrosis, acute pulmonary edema, eosinophilic pleural effusion, pleural/
pericardial thickening or effusion with positive antinuclear/antihistone
antibodies, and vasculitis. The majority of patients recovered after termi-
SULFASALAZINE-INDUCED PULMONARY DISEASE nation of the drug. Clinical improvement usually occurs between 1 and 32
Victor K. Salloum MD Sam Ancheril MD Rosa Estrada-Y-Martin MD* weeks, with an average time of 6.5 weeks. In contrast, our patient rapidly
University of Texas Health Science Center Houston, Houston, TX improved in only four days after termination of the drug. Sequential
pulmonary function tests up to three years after intervention clearly
exhibited improvement and stabilization of lung function and diffusion
capacity. More importantly, pathology demonstrating well formed granu-
INTRODUCTION: Sulfasalazine-induced pulmonary disease is a rare lomas and Schaumann bodies mimicking sarcoidosis has not previously
entity. We describe the first patient with psoriatic arthritis to develop been described.
pulmonary toxicity with unique pathology and an interesting clinical CONCLUSION: Sulfasalazine-induced pulmonary disease may mas-
course. querade in different forms. The importance of early recognition remains
CASE PRESENTATION: A 38 year old Caucasian woman with paramount. Proper identification may not only spare the patient from
psoriatic arthritis presented with a five month history of progressively severe lung damage, but also prevent an untimely death and unnecessary
worsening dyspnea on exertion and dry cough. Of note, her psoriatic complications from other drug therapies. Prompt recognition of the
arthritis was initially diagnosed in September 2000, and sulfasalazine (3 disease, followed by immediate cessation of the drug, should result in an
grams/day) was started in October 2000. She was evaluated in February excellent prognosis.
2002, 17 months after her initial diagnosis, and 16 months after initiation DISCLOSURE: Rosa Estrada-Y-Martin, None.
of therapy. Initial pulse oximetry on room air was 95%, however with
ambulation, desaturation to 91% was noted. Physical examination revealed
bibasilar inspiratory crackles in the posterior lung fields and psoriatic
plaques over the extensor surfaces of the elbows. Chest radiograph
displayed bilateral patchy infiltrates in the lower lobes. Pulmonary
function tests demonstrated restrictive lung disease with a significantly
reduced diffusion capacity (44% of predicted). Subsequent chest com- VINORELBINE INDUCED INTERSTITIAL PNEUMONITIS: A
puted tomography showed bilateral lower lobes ground glass alveolar CASE REPORT
opacities. Transbronchial biopsy revealed pulmonary parenchyma with the Mayank Vats MD* Rakesh C. Gupta MD Deepa V. Khandelwal MBBS
interstitium fraught with granulomas. Several granuloma giant cells Neeraj Gupta MD Pramod Dadhich MD J.L.N. Medical College, Ajmer,
contained refractile and calcified material, resembling Schaumann bodies. Rajasthan, India
Stains and culture for acid fast bacilli and fungi were negative. Given the
possibility of sulfasalazine toxicity, the medication was discontinued. INTRODUCTION: Vinorelbine (Navalbine), a newer Vinca alkaloid
Significant improvement of symptoms was noted within four days after has been increasingly used in the chemotherapy of non-small-cell Bron-
cessation of the drug. Repeat pulmonary function tests two months after chogenic carcinoma & metastatic breast carcinoma. Initially, it appeared
intervention demonstrated improvement of the total lung capacity and to have a favorable side-effect profile with dose & duration limited
diffusion capacity (69% predicted). Repeat chest computed tomography myelo-suppression (Granulocytopenia, Thrombocytopenia) being the ma-
also showed clearing of the ground glass opacities. Pulmonary function jor toxicity1. With more frequent use of Vinorelbine, new side effects of
tests obtained three years after intervention showed a near normal this drug are being reported. We are reporting a case of Interstitial
diffusion capacity (74% of predicted). Pneumonitis developing after Vinorelbine therapy for Bronchoalveolar
carcinoma.
CASE PRESENTATION: K.K. 46 year old farmer smoker presented
to our OPD with complaints of dull diffuse chest pain on right side, cough
with mucoid expectoration ⬎200 ml/d & breathlessness. On physical
examination breath sounds were diminished in intensity on right side,
coarse crept was present in mid & lower chest. Chest X-Ray revealed
diffuse heterogenous infiltrates with air-bronchogram in right lung.
Patient was previously treated for bronchopneumonia with broad-spec-
trum antibiotics elsewhere but had no improvement. Considering the
possibility of malignancy, Fibre-Optic Bronchoscopy revealed excessive
CASE REPORTS
mucoid secretions. Bronchial-brush and Bronchoscopic-lavage was nega-
tive for microbiology & cytology, so lung biopsy was done, it showed
bronchoalveolar carcinoma. CT-scan chest revealed, focal area of appar-
ent parenchymal consolidation with linear strands extending to the
periphery. After complete evaluation & basic investigation {Complete
Blood Count (CBC), Liver Function Test, Renal Function Test}, we
planned chemotherapy with Vinorelbine, hence Vinorelbine 50 mg (as the
body surface area was 1.68m2) over 10 minutes was infused after proper
hydration, patient tolerated the drug well & after observation for 1 day, he
was discharged on request. After 3 days, patient returned with complaints
of increasing dyspnoea, dry cough & severe distress. He was tachycardiac,
accessory muscles of respiration was working, breath sounds were dimin-
ished and of bronchial nature on right side, Chest X-Ray revealed
extensive consolidation on whole of right lung. His CBC was normal &
sputum for Gram stain/culture was negative. However because of suspi-
cion of community-acquired-pneumonia, broad-spectrum antibiotic &
intravenous steroids were started, patient responded well with clearance
of radiological-infiltrates. Next week, IInd cycle of Vinorelbine 50 mg over
10 minutes was given & on IInd day of IInd cycle patients again developed
breathlessness & dry cough. CXR showed homogenous consolidation with
DISCUSSIONS: Review of the literature between 1972 and 1999 air-bronchogram in right lung.This time considering the possibility of
identified 50 patients with possible sulfasalazine-induced lung toxicity. Vinorelbine induced pneumonitis, Trans-bronchial-lung-Biopsy was done
The daily dose of sulfasalazine ranged from 1 to 8 grams (mean of 3 revealing focal fibrosis, eosinophilic-proteinaceous exudate & reactive
grams). The average duration of exposure to sulfasalazine was 17.8 months proliferation of type-II-pneumocytes, diffuse alveolar damage & hyaline-
with a range of 0.5 to 120 months. Sulfasalazine toxicity may present in a membrane. Patient was put only on intravenous methyl-prednisolone

500-mg 8-hourly & symptomatic treatment. After 3days of treatment he granulomas and fibrosis were more frequent in CS. The differentiation
responded with some clearance of radiographic infiltrates, hence confirm- between CS and IGCM is clinically important, because the natural history
ing the diagnosis of Vinorelbine induced Interstitial Pneumonitis. of the disease, survival, and the treatment are different. For example, CS
DISCUSSIONS: This is probably the first case-report documenting has better prognosis than IGCM, the 5 years survival is 80% for CS versus
Interstitial Pneumonitis after Vinorelbine administration.The clinical 20% for IGCM.The treatment for CS is corticosteroids. However, the
course, radiological & pathologic characteristics, rapid response to steroid treatment for IGCM is the combination of corticosteroids and cyclospor-
& absence of other potential causes are suggestive of drug-induced ine which showed a survivor benefit compared with corticosteroids only.
pneumonitis. Many chemo-therapetic agents like bleomycin, busulfan CONCLUSION: The clinical cardiac involvement occurs in fewer than
have been implicated with development of interstitial pneumonitis, but 5% of patients with sarcoidosis.CS and IGCM can look similar on the
this is probably the first case-report of Vinorelbine induced interstitial histopathology. Lung biopsy may help to Cardiac sarcoid patients treated
pneumonitisThe mechanism is unknown, but is believed to be an with had 5-year survival rates 59-89%. However, the combination of
allergic-immunological process as is shown by rapid & near-complete corticosteroids and cyclosporine for patients with Giant Cell Myocarditis
response after drug cessation & steroids. had 5-year survival rates of 10-18%.
CONCLUSION: Vinorelbine toxicity should be strongly considered in REFERENCES:
diagnosis of interstitial pneumonitis developing after short period of 1 Cooper et al. New England Journal of Medicine 1997;336(26):1860-
administration, urgent diagnostic workup is essential to exclude other 1866.
etiology & to establish drug as a cause of interstitial pneumonitis & to 2 Okura et al. American College of Cardiology. 41(2):322-9, 2003 Jan
enable early institution of steroid therapy which given prompt response. 15.
REFERENCES:
1 Roland T. Skeel. Anti-Neoplastic drugs & biologic response modi-
fiers: classification use & toxicity of clinically useful agents. In
Handbook of Cancer Chemotherapy. Ed. Roland T. Skeel. V ed.
P.142, 1999. Lippincott, Williams & Wilkins.
DISCLOSURE: Mayank Vats, None.
Miscellaneous Cases
4:15 PM - 5:45 PM
CARDIAC SARCOIDOSIS COULD MIMIC GIANT CELL MYO-
CARDITIS IN A PATIENT PRESENTING WITH VENTRICULAR
ARRHYTHMIAS
Bassel Ramadan MD* Emory University, Atlanta, GA
INTRODUCTION: Giant cell myocarditis (GCM) is a rare, rapidly

fatal myocarditis with histologic features that have some similarities to
cardiac sarcoidosis (CS). We report a case of cardiac saecoidosis which
initially was diagnosed as GCM. Howevere after a carefull review of the
histopathlogical feature of the endomyocardial biopsy, and the presence of
pulmonary granulomas on the transbronchial biopsy the diagnosis con-
firmed as a cadiac sarcoiodosis.
CASE PRESENTATION: 38 Year-old African American male, with
no known medical problems, was transferred from an outside hospital to
our institution for further workup of his ventricular arrhythmias. He had
a syncopal episode while walking to the TV in his living room. He lost
consciousness for 5-6 minutes and woke up without confusion, but he was
complaining of chest pain. On admission the patient was noted to have
multiple arrhythmias including second degree AV Block, nonsustained
ventricular tachycardia, and torsades. He was complaining of some chest
discomfort, cough, dyspnea, and 10 lbs weight loss. His physical exam was
remarkable for elevated JVD; third heart sound, systolic murmur over the
apex, bibasilar end inspiratory crackles, and 2 ⫹ pitting edema over the
lower extremities. The electrolytes were within normal limits. Urine drug DISCLOSURE: Bassel Ramadan, None.
screen was negative and his TSH as well. Arterial blood gas on room air
showed moderate degree of hypoxemia. Chest Ct with PE protocol did
not show pulmonary embolus, but it showed diffuse pulmonary nodules HEREDITARY ANGIOEDEMA PRESENTING AS ACUTE PAN-
with hilar and mediastinal lymphadenopathy. He underwent a cardiac CREATITIS
catheterization which showed normal coronaries and ejection fraction of Evans R. Fernández MD* Damir Matesic MD Nicholas E. Vlahakis MD
15%. He had an endomyocardial biopsy as well and initially showed Giant Mayo Clinic, Rochester, MN
Cell Myocarditis. Subsequently he had a bronchoscopy with transbron-
chial biopsy showing non-caseating granulomas suggestive of sarcoidosis INTRODUCTION: Hereditary angioedema (HAE) is an infrequent
(see figure 1). In view of the lung biopsy results the pathologist reevalu- disorder characterized by abnormal serum levels and/or function of
ated his endomyocardial biopsy (see figure 2)and due to presence of complement C1q esterase inhibitor (C1q INH). Clinically it presents with
non-caseating granulomas on endomyocardial biopsy, he revised the recurrent attacks of subcutaneous and/or submucosal swelling. When the
diagnosis to cardiac sarcoidosis.Patient underwent a placement of a gastrointestinal (GI) tract is involved, an exacerbation of HAE results in
cardiac defibrilator for his arrhythmias. Prednisone of 60mg a day was numeorus GI symptoms but rarely acute pancreatitis.
given with a marked improvement of his heart failure symptoms. CASE PRESENTATION: A 40-year old man presented to our
DISCUSSIONS: Idiopathic giant-cell myocarditis IGCM is a fre- institution with a 1-month history of increasing anxiety and a 1-day history
quently fatal type of myocarditis. The clinical course, unlike that of sarcoid of intermittent abdominal pain, nausea, and vomiting. He described the
myocarditis CS, is usually characterized by progressive congestive heart pain as severe with radiation through to his back. He denied melena,
failure, frequently associated with refractory ventricular arrhythmia. Most hematochezia or previous gallbladder disease. He had a past medical
patients die of congestive heart failure. On the bases histopathology CS history of peptic ulcer disease, generalized anxiety disorder and C1q
and IGCM may have similar characteristic. Eosinophils, myocyte damage, esterase inhibitor deficiency, diagnosed at the age of two. His HAE
and foci of lymphocytic myocarditis were more frequent in IGCM, while attacks, occurring 5 to 6 times per year, generally lasted 1 to 2 days and

Miscellaneous Cases, continued
were characterized by recurrent episodes of painless, nonpruritic swelling PARAGANGLIOMA: A CASE OF PULMONARY NODULES
of the skin, abdominal pain and nausea. His medications included Jose A. Delgado MD* Ganesan Murali MD Albert Einstein Medical
esomeprazole, sertraline, danazol and ondansetron, dyphenhydamine and Center, Philadelphia, PA
hydromorphone as needed for angioedema flares. He was a nonsmoker
and denied use of alcohol. On admission to the ICU, he was afebrile INTRODUCTION: We report a case of an asymptomatic male
(98°F), hemodynamically stable with minimal sinus tachycardia and presenting with bilateral, disseminated and well-defined pulmonary nod-
tachypnea. His exam was remarkable for epigastric tenderness but no ules. The patient had undergone resection of a retroperitoneal paragan-
hepatosplenomegaly or palpable abdominal masses. Laboratory studies glioma 6 years earlier; close follow-up had revealed no evidence of
recurrence. After our evaluation, a VATS with wegde resection of the
revealed a white blood cell count of 19,300/ cu mm, a hematocrit of 55 %,
lingula was performed and histologic diagnosis of metastatic paragangli-
a serum amylase of 1,176 IU/L, a serum lipase of 11,485 IU/L, a serum oma was established.
aspartate and alanine aminotransferase of 68 IU/L and 105 IU/L respec- CASE PRESENTATION: This is a 51-year-old African-American male seen
tively and a serum alkaline phosphatase of 138 IU/L. Other laboratory in consultation for bilateral lung nodules. Six years ago he was diagnosed with an
values, including serum total bilirubin, electrolytes, creatinine and trig- abdominal mass during a routine clinical evaluation. Subsequent imaging studies
lycerides were normal. The C1q esterase inhibitor antigen was low at 7 confirmed the presence of a 10x10x10cm retroperitoneal tumor between the
mg/dl (normal, 19-37 mg/dl). With aggressive intravenous hydration, aorta and left ureter, and resection of a 700g mass was performed. The histologic
antibiotic prophylaxis, gastric decompression, pain control, and continu- examination revealed an extra-adrenal paraganglioma with evidence of capsular
ation of his home medications including esomeprazole and danazol, the and extracapsular infiltration and angiolymphatic invasion. Immunohistochemical
patient improved and his pancreatic enzymes began to normalize within stains for chromogranin were positive. The patient underwent radiation therapy,
72 hours. Abdominal ultrasonography revealed no biliary obstruction or and follow-up octreotide scan and CT abdomen revealed no evidence of tumor
gallbladder pathology. Contrast-enhanced computed tomography (CT) of activity or recurrence. Hormonal assays were negative for elevated 24-h urinary
the abdomen confirmed acute pancreatitis with patchy areas of pancreatic metanephrine and for serum catecholamines on two separate occasions. The
necrosis, mild bowel swelling and no intra or extrahepatic bile duct patient had undergone a follow-up MRI of the abdomen 1 month before our
dilatation (Figure). Before discharge he underwent upper GI endoscopy, evaluation; disseminated nodules were visualized in the liver and lung bases. His
endoscopic retrograde cholangiopancreatography and analysis of bile for past medical history is also remarkable for chronic active hepatitis C, for which he
had received treatment with interferon alfa-2b and ribavirin as well as pegylated
microlithiasis. These failed to explain the cause of pancreatitis thus
interferon in the past 2 years; the patient has remained well compensated. He is
favoring HAE as the most probable etiology. a former smoker and has a remote history of IV drug use. At the time of our
DISCUSSIONS: HAE affects between 1/10000-1/50000 people evaluation the patient was asymptomatic, and the review of systems was negative.
worldwide and can present after stressful triggering events, such as He was normotensive, hemodynamically stable and there were no stigmata of
severe anxiety as seen in our patient. Besides subcutaneous swellings chronic liver disease. His liver function studies and coagulation parameters were
and occasionally fatal upper airway edema, it can also cause severe GI normal except for an elevated alfa-fetoprotein level of 20.8. The patient subse-
symptoms. However, a thorough literature search revealed only one quently underwent VATS with wedge resection of the lingula, which confirmed
case report describing HAE associated with pancreatitis. A high degree the suspected diagnosis of metastatic paraganglioma. The immunohistochemical
of clinical suspicion for possible pancreatitis should be maintained stains were positive for chromogranin and synaptophysin. As of this writing, no
when facing a patient with history of HAE and abdominal pain. One further intervention has been considered.
quarter of HAE cases represent a new gene mutation so when no DISCUSSIONS: Extra-adrenal pheochromocytomas, or paraganglio-
obvious cause for pancreatitis is found and recurrent episodes of mas, are rare neuroendocrine tumors with infrequent pulmonary involve-
pancreatitis occur then HAE should be considered in the differential ment. This case reveals one of the possible intrathoracic manifestations:
diagnosis. Finally, treatment options are limited since in the United metastatic pulmonary disease. Although most cases present with the
States C1q INH concentrate use has not been approved. Moreover, classical paroxysmal attacks or symptoms related to catecholamine excess,
treatment with fresh frozen plasma (FFP) should be undertaken with our patient interestingly never exhibited any of those symptoms. We based
the diagnosis on the MRI and CT scan findings, as well as on the
caution since a number of cases have been reported with worsening of
histopathologic exam – with positive immunohistochemical markers such
angioedema symptoms. as synaptophysin and chromogranin. Paragangliomas are potentially ma-
CONCLUSION: HAE, although rarely recognized and likely under- lignant 40% of the time, with a mean survival of 1 to 2 years in metastatic
reported can cause pancreatitis via either swelling of the pancreas per se disease. Surgical resection has been advocated in well-localized tumors.
or by causing edema interfering with normal pancreatic drainage. Current The role of radiation and combination chemotherapy has been reported to
treatment options for acute angioedema are limited with observation be effective in disseminated disease; however, this role is still under
being the most common form of treatment since most attacks are self investigation.
limited and of short duration.
CASE REPORTS
CONCLUSION: Paragangliomas are tumors with infrequent intratho-

racic manifestations. The related classical symptoms may be absent, and
DISCLOSURE: Evans Fernández, None. the hormonal assays may be negative as well. In such cases, we recom-

mend basing the diagnosis on MRI and CT imaging findings, as well as on .Stein in 1982 discovered a cytokine receptor CD 30 on large lymphoid
histopathologic exam with immunohistochemical markers. In addition, we cells. It is absent in normal tissue and expressed on HL. In 1994 some
believe surgery should be considered in cases involving well-demarcated cells were found to express the ALK-1 protein. There are 3 types, primary
lesions. systemic (ALK⫹ and ALK-) and primary cutaneous, which is ALK-. The
REFERENCES: secondary ALCL arises from HL and peripheral T- cell lymphomas. They
1 Sandur S, Dasgupta A et al. Thoracic involvement with pheochro- are usually ALK-. The absence of ALK is associated with a poor prognosis,
mocytoma.Chest 1999;115:511-521 unless it is the primary cutaneous form.
2 Edstrom E et al.The management of benign and malignant pheo- CONCLUSION: The case is of interest for several reasons. It repre-
chromocytoma and abdominal paragangliomas.Eur J Surg Onc sents an unusual presentation of an uncommon lymphoma. It has all the
2003;29:278-83 features of primary cutaneous (ALK negative) ALCL, which rarely
3 Shibahara J et al.Primary pulmonary paraganglioma.Am J Surg disseminates to the lungs. Also, this patient may have had secondary
Pathol 2004;28:825-29 ALCL arising from prior lymphoma and/or its treatment. This is the
second reported case of cutaneous ALCL associated with Hepatitis C.
Fourthly this is the first case report of ALCL mimicking WG, with skin
nodules, nasal ulceration and thick walled cavitary lung nodules. However
pathology was absent. Lastly, cavitation is rare in secondary lung lym-
phoma, which again makes the case an interesting pulmonary finding.
DISCLOSURE: Reverly John, None.
MEDIASTINAL SARCOIDOSIS IN A PATIENT RECEIVING IN-

TERFERON-BETA FOR MULTIPLE SCLEROSIS
Sean O’Reilly MD* Alexander White MD Agustin Florian MD Tufts-New
England Medical Center, Boston, MA
INTRODUCTION: A female with multiple sclerosis presented to

pulmonary clinic with a mediastinal mass.
CASE PRESENTATION: A 52-year-old female was referred to
pulmonary clinic having developed right-sided chest pain which radiated
DISCLOSURE: Jose Delgado, None. to both shoulders. The sensation was described as dull and pressure-like.
The pain was pleuritic and made worse by swallowing both solids and
liquids. It was not exertional. A two week course of ibuprofen reduced, but
ANAPLASTIC LARGE CELL LYMPHOMA OF THE LUNG did not eliminate the pain. There were no associated symptoms. She
Reverly John MBBS* Babak Shokrani MD Howard University Hospital, specifically denied cough, fevers, chills, night sweats, or weight loss. Her
Washington, DC past medical history was significant for multiple sclerosis, diagnosed 28
years ago. The MS was well controlled with 30 mcg of weekly, self-
INTRODUCTION: Only four percent of patients with non-Hodgkin’s administered interferon-beta for the past three years. She had been very
lymphomas (NHL) present with lung involvement (12% with HL), but the active, walking two miles daily without difficulty. The patient was afebrile,
disease should be included in the differential of lung lesions as its heart rate 84, blood pressure 126/80 in both arms, respiratory rate 14, and
presentations are myriad. A rare case of anaplastic lymphomas kinase pulse oximetry of 98% while breathing room air. There was no cervical or
negative (ALK-) anaplastic large cell lymphoma (ALCL) of the skin axillary lymphadenopathy. The lungs were clear to auscultation and heart
involving the lung and mimicking Wegener’s granulomatosis (WG) is exam was normal. Neurologic, musculoskeletal, and skin exams were
presented. unremarkable. Results of a CBC, serum chemistries, liver function tests,
CASE PRESENTATION: The patient is a 54 yo man with a 4-month and serial troponins were all within normal limits. ECG showed normal
history of ulcers on his nose bridge, left leg and arms. They started as sinus rhythm at a rate of 65. Chest radiograph showed a widened
round nodules. He had testicular cancer with left orchidectomy in 1987. mediastinum and a small right pleural effusion. Computerized tomogra-
Two years ago he received chemotherapy for NHL involving the neck and phy of the chest revealed a 3cm mass in the superior mediastinum in
was in remission. He had weight loss and anorexia for 6 months. An HIV addition to the small effusion. Positron emission tomography showed
test was negative 2 years ago. He had an 80-pack year smoking history.He moderately intense tracer uptake in the mediastinum, corresponding to
was thin, had dry, pale mucous membranes, no icterus, thrush or the lesion seen on CT. PPD testing was negative and a serum angiotensin
adenopathy. Temperature was 101.1F, pulse 120 regular, respiratory rate converting enzyme level was normal at 20 IU/ml. Mediastinoscopy
16 and BP 94/78. He had a nasal bridge ulcer involving the cartilaginous revealed multiple enlarged mediastinal and right paratracheal lymph
septum. There were 1 to 2 cm, hyperpigmented, stage 2 ulcers on the nodes. Pathology showed non-caseating granulomas, and was consistent
extensor surface of the extremities. The remainder of the exam was with sarcoidosis. Acid fast and fungal stains were negative for organisms.
normal.Labs are shown in table1. The diagnoses were sepsis, infected A diagnosis of sarcoidosis was made, possibly relating to her use of IFN-␤.
ulcers and dehydration. The differentials were syphilis, leprosy, HIV This medication was discontinued. Repeat CT of the chest at six months
infection and WG. He received ceftriaxone and intravenous fluid. Skin demonstrated complete resolution of both the mediastinal lymphadenop-
and nasal biopsies were performed. He developed respiratory distress, was athy and the right sided pleural effusion. Her MS remained stable after
intubated and treated for septic shock. (Lab trends, graphs 1-5). The discontinuation of the IFN-␤.
initial chest radiograph showed bilateral thick-walled nodules of varying DISCUSSIONS: Sarcoidosis is a systemic granulomatous disease of
sizes with cavitation, a right pleural effusion and a broken port-a-cath in unknown etiology. Common manifestations include hilar lymphadenopa-
the right descending pulmonary artery (figure 1). Later, there were thy, pulmonary infiltrates, ocular and cutaneous lesions, although it can
superimposed alveolar infiltrates (figure 2, 3). Chest CT revealed the same affect any organ system. Neurosarcoidosis rarely mimics multiple sclero-
findings (figure 4,5,6). Bronchoscopy was performed. His catheter was sis, and this patient’s clinical course was more suggestive of MS. Numer-
removed. He developed candida albicans urinary infection and sepsis. ous reports have linked exogenously administered interferons to the
Bronchial washings and catheter were culture negative. Despite aggres- development of sarcoidosis. The majority of cases involve patients using
sive therapy, he died of septic shock on day 34. IFN-␣; and ribavirin for chronic hepatitis C infection. Interferons are
DISCUSSIONS: The biopsies (figure7) revealed atypical, lymphoid cytokine mediators involved in the early, innate immune response to viral
infiltrates of large pleomorphic cells with abundant cytoplasm and pathogens. They are also crucial to the development of virus-specific
prominent nucleoli with admixed neutrophils (below). The neoplastic cells adaptive immunity and can enhance antigen presentation to specific T
stained positive with CD3 and CD 30 markers. The CD 20 and CD 15 cells. Type I interferons (IFN-␣ and IFN-␤) have been shown in vitro to
stains were negative. CD 3 stains T-lymphocytes, while CD15 stains promote Th1 cell differentiation. In sarcoidosis, an exaggerated Th1
neutrophils and Reed-Sternberg cells (RS). CD 20 stains B-cells and CD immune response, to an unknown antigen, is thought to promote macro-
30⫹ cells are HL, particularly RS and ALCL, the latter comprising 2-8% phage activation and result in the pathologic granulomatous response.
of adult lymphomas. The ALK was negative. These findings were This misdirected immune response results in tissue damage in the
consistent with ALK - ALCL. There were no granulomas or vasculitis- affected organs.

CONCLUSION: Sarcoidosis is a recognized complication of IFN-␤ disease and negative mediastinoscopy. He eventually underwent en bloc
therapy. While the exact etiology of sarcoidosis remains unknown, this resection of the LUL mass and chest wall. On detailed pathologic examina-
unusual case provides evidence of a contributing role of interferon. The tion of the specimen, no histologically viable or clearly necrotic malignant
use of IFN-␤ as a treatment for multiple sclerosis may be associated with tumor was identified in either lung or chest wall tissue. Necrotizing granu-
an increased risk of sarcoidosis. lomatous inflammation highly suggestive of tuberculosis was noted in the
REFERENCES: lung, and a single weakly acid fast structure suggestive of Mycobacterium
1 Antoniou KM et al. Interferons and Their Application in the tuberculosis was identified.
Diseases of the Lung. Chest, Jan 2003; 123: 209 - 216. DISCUSSIONS: Although 95% of cases are due to malignancy, a variety
2 Marzouk K et al. Interferon-induced granulomatous lung disease. of other lesions can arise in the superior sulcus and cause Pancoast syndrome.
Current Opinion in Pulmonary Medicine 2004, 10:435-440. We report a case where Pancoast syndrome may have resulted from the
3 Sinigaglia F et al. Type I Interferons and the Th1/Th2 paradigm. combination of lung cancer and tuberculosis, or tuberculosis alone. The
Developmental and Comparative Immunology 1999. 23:657-663. pathologic specimen revealed areas of granulomatous inflammation, but also
foci of fibrosis and chronic inflammation without granulomas. It is possible
that these were sites of carcinoma sterilized with radiotherapy. Tuberculosis
presenting as Pancoast tumor in a non-smoking man from Cameroon was
recently reported. Tuberculosis and Pancoast tumor presenting synchro-
nously has also been reported. In our case, however, the BAL was negative for
AFB. In retrospect, histologic diagnosis of malignancy should have been more
aggressively pursued. But in addition to suggestive cytology, several features
suggested the diagnosis of lung cancer, including the patient’s smoking history
and the presence of clubbing, rarely seen in TB. The apparent response of the
lesion to radiotherapy is surprising. Some authors have suggested an associ-
ation between radiotherapy and reactivation of tuberculosis. We found older
references of radiotherapy being used therapeutically for pulmonary TB.
Postoperatively, the patient completed a course of antituberculous therapy.
CONCLUSION: We report a case of Pancoast syndrome which may
have represented tuberculosis in isolation, or tuberculosis combined with
lung cancer. The patient’s symptoms and the lesion responded to initial
palliative radiotherapy, given for presumed isolated lung cancer. Given
the differential diagnosis, a histologic diagnosis should be obtained before
proceeding with therapy in this context.
REFERENCE:
1 Beshay M. Ann Thorac Surg 2003; 76: 1733-5 2. Vishak Acharya K.
Indian J Tuberc 2004; 51: 89-91
DISCLOSURE: Anne Gonzalez, None.
Non-Pulmonary Critical Illness

4:15 PM - 5:45 PM
A CASE OF CENTRAL CORD SYNDROME CAUSED BY EMER-
GENT REINTUBATION
Mark B. Napier MD* Erica F. Bisson MD William D’Angelo MD Diana
L. Wilson MD Maine Medical Center, Portland, ME
DISCLOSURE: Sean O’Reilly, None. INTRODUCTION: Central cord syndrome (CCS), an injury often
seen in the elderly, is caused by hyperextension of the cervical spine
resulting in weakness and sensory changes with the upper extremities
AN UNEXPECTED RESPONSE TO RADIOTHERAPY! more affected than the lower. Recovery is variable and residual deficits are
Anne V. Gonzalez MD* Christian Sirois MD Richard S. Fraser MD James common. Three reports of CCS as a result of endotracheal intubation exist
CASE REPORTS
Gruber MD McGill University, Montreal, PQ, Canada in the literature.[1-3] We present a fourth case that occurred when a
patient was emergently re-intubated.
INTRODUCTION: The vast majority of cases of Pancoast syndrome CASE PRESENTATION: A 77-year-old physically active woman with
are due to lung cancer. Rarely, other lesions arising in the superior sulcus a history of an ischemic cardiomyopathy presented to an outside hospital
can cause Pancoast syndrome. This case emphasizes the importance of with fever and respiratory distress. The patient was intubated in the
securing a histologic diagnosis before proceeding with treatment. The emergency department, and improved slowly with treatment of commu-
effect of radiotherapy on the underlying lesion is discussed. nity acquired pneumonia. The patient was noted to be neurologically
CASE PRESENTATION: A 49 year old man presented with 3 weeks of intact at the time of admission and immediately prior to extubation. The
severe left sided chest pain, and minor hemoptysis. He had lost 40 lbs over patient was extubated on hospital day four but developed respiratory
the preceding year, and complained of severe anorexia and fatigue. He denied distress within fifteen minutes and was re-intubated. The re-intubation
any fever; there was no history of tuberculosis. He lived in Canada since 1987, was uncomplicated with a full view of the vocal cords utilizing a Macintosh
after having left Romania. He was a longstanding heavy smoker. The physical #3 blade on the first attempt and easy passage of the endotracheal tube.
examination revealed a cachectic man, who had finger clubbing. There was no The patient was transferred to our institution on hospital day eight.
lymphadenopathy, and the neurological exam was unremarkable. The CXR Admission examination revealed that although she was able to weakly
revealed a left upper lobe (LUL) mass. On CT scan, a large irregular mass in move her legs, her arms were nearly plegic. On questioning, the patient’s
the LUL with destruction of the second and third ribs was noted, as well as family recalled noting diminished movement of her upper extremities
severe emphysema. No endobronchial lesion was seen on bronchoscopy. The following re-intubation. In consultation, neurology and neurosurgery
BAL cytology revealed atypical cells, suggestive of malignancy but insufficient found the patient to have severe flaccid paresis of her bilateral upper
to establish a diagnosis. No acid-fast bacilli (AFB) were seen and mycobac- extremities, which was more severe on the left, and antigravity strength in
terial culture was negative. Based on the above findings, the diagnosis of lung her bilateral lower extremities with flexor plantar responses and no clonus.
cancer was accepted by the lung tumor board. Given his severe pain and poor An MRI revealed diffuse spondylosis with moderate to severe stenosis at
performance status, the patient received a course of palliative radiotherapy C4-5 with anterolisthesis and associated cord deformity. Although the
(17 Gy in 2 fractions). He reappeared in our thoracic surgeon’s office 9 patient’s neurological status improved modestly, she had a prolonged
months later. A repeat CXR revealed a shrunken LUL lesion compared to hospital course and failed to wean from mechanical ventilation. The family
previous films. The patient was restaged, with no evidence of metastatic decided not to pursue aggressive care when the patient progressed to

Non-Pulmonary Critical Illness, continued
aneuric renal failure, supportive care was withdrawn, and the patient DISCUSSIONS: CPSI is the first enzyme involve in conversion of
expired. ammonia to urea through the urea cycle. Partial enzyme deficiencies may
DISCUSSIONS: CCS, the most common form of incomplete spinal present in late adulthood as seen in our patient. CPSI deficiency can
cord injury, is a well-described phenomenon that produces asymmetric present at almost any time of life with a stressful triggering events.
and incomplete tetraplegia and sensory deficit disproportionately affecting Prevention of systemic stress and early intervention of hyperammonemic
the upper extremities. In patients with cervical spondylosis, hyperexten- crisis is paramount. Clinical features include nausea, vomiting, somno-
sion causes compression of the cord between the ligamentum flavum lence and seizures. Neurologic symptomatology has been linked to
posteriorly and anterior osteophytes resulting in contusion of the cord cerebral injury during hyperammonemic crisis which is associated with
centrally.[1]Initial theories to explain the degree of involvement of the glutamine accumulation. Intratraneuronal glutamine may serve as an
arms claimed somatotopic organization of the lateral corticospinal tracts, osmole causing alterations in neurotransmitter metabolism and brain
with the hand and arms based medially, near the site of the contused cord, swelling (1). The diagnosis is based on enzymatic assay of liver tissue and
with the legs laterally [4] have been rejected after animal models proved treatment involves nitrogen restriction and enhancement of nitrogen
no basis for this type of organization. The current thinking is that the excretion using sodium benzoate or phenylbutyrate. Continuous arterio-
upper limb, in particular the hand, is more represented in the lateral venous or venovenous hemodialysis (HD) at high flow rates should be
corticospinal tract than the leg, which has the majority of its descending started in the setting of severe hyperammonemia or absence of clinical
fibers from other tracts. improvement. HD is continued until the ammonia concentration is lower
CONCLUSION: We present a case of CCS in a patient with occult than 200 ␮mol/L. Below this level HD appears to be of no benefit.
cervical stenosis following urgent re-intubation. Extension of the cervical Alternatively, successful treatment with orthotopic liver transplantation
spine during direct laryngoscopy for intubation is capable of producing has also been described in the literature.
CCS, in both patients with preexisting cervical pathology, as in our CONCLUSION: Urea cycle disorders should be part of the differential
patient, as well in patients with no demonstrable pre-existing cervical diagnosis of isolated hyperammonemia if no obvious cause is identified
pathology. Caution should be used when intubating the elderly and regardless of the patient’s age. Hyperammonemic encephalopathy may
patients with known cervical spine pathology. result in severe brain dysfunction and a rapidly fatal course. A high degree
REFERENCES: of clinical alertness is a prerequisite since prompt diagnosis and treatment
1 Yan K, Diggan MF. A case of central cord syndrome caused by may be of benefit in CPSI deficiency before brain injury develops.
intubation. J Spine Cord Med 1997;20:230-232 REFERENCE:
2 Buchowski JM, et al. Central cord syndrome after total hip arthro- 1 Takeoka M, Soman TB, Shih VE, et al. Carbamyl phosphate
plasty. Spine 2005;30:E103-E105 synthetase 1 deficiency: a destructive encephalopathy. Pediatr Neu-
3 Clinchot DM, et al. An unusual case of traumatic spinal cord injury. rol. 2001; 24(3):193-9.
Spinal Cord 1997;35:181-2 DISCLOSURE: Teck-Kim Khoo, None.
4 Levi AO, et al. Clinical syndromes associated with disproportionate
weakness of the upper versus lower extremities after cervical spinal
cord injury. Neurosurgery 1996;38:179-85
DISCLOSURE: Mark Napier, None.
POTENTIAL BROMIDE TOXICITY ASSOCIATED WITH MYAS-

THENIA GRAVIS TREATMENT IN A 22 MONTH OLD CHILD
Aaron J. Godshall MD* Cyrus Rangan MD John T. Li MD Childrens
SEVERE HYPERAMMONEMIA FROM AN INHERITED UREA Hospital Los Angeles, Long Beach, CA
CYCLE DISORDER PRESENTING IN LATE ADULTHOOD
Evans R. Fernández MD Teck-Kim Khoo MD* Tiffany Priester MD INTRODUCTION: Treatment strategies for Myasthenia gravis (MG)
Bekele Afessa MD Mayo Clinic, Rochester, MN are directed at reducing acetylcholine receptor antibody production
(immunosuppression, thymectomy, corticosteroids) and increasing avail-
INTRODUCTION: Dietary protein is metabolized and then excreted ability of acetylcholine at the neuromuscular junction with acetylcholines-
as urea via the urea cycle. Urea cycle enzymatic deficiencies are usually terase inhibitors. Pyridostigmine bromide (PB) is an acetylcholinesterase
inherited and rare, and generally present in newborns. We present a case inhibitor used to treat MG. Toxicity from PB overdose has focused on
of carbamyl phosphate synthetase deficiency in an elderly woman admit- cholinergic toxicity, but bromide toxicity (bromism) can be underappre-
ted to the intensive care unit (ICU) for decreased consciousness with ciated. We present one of the first reported pediatric cases suspicious for
hyperammonemia. bromism from PB use.
CASE PRESENTATION: A 65 year old woman was transferred to our CASE PRESENTATION: A 22-month-old male with a history of MG
institution soon after abdominal hernia repair surgery in an outside presented to the emergency department with bradycardia and respiratory
hospital with severe progressive decrease in mentation. On presentation, distress. He was in his usual state of health and feeding normally at home
her Glasgow Coma score was 3, and she was endotracheally intubated for when he developed increased salivation and difficulty breathing. There
airway protection. Her past medical history was significant for two was no report of choking or coughing surrounding the dyspnea. At home
previous unexplained episodes of nausea and nonspecific abdominal pain when he became ashen, emergency medical services was activated and
associated with vomiting. On admission to the ICU, she was unresponsive CPR was initiated. Upon arrival, paramedics noted bradycardia and
without focal neurological abnormalities. Her admission blood ammonia respiratory arrest. On hospital presentation, he was bradycardic (pulse ⫽
level was 196 ␮mol/L (normal for our laboratory is 10 to 47 ␮mol/L) and 30), breathing shallow and had a Glasgow Coma Scale score of 3. There
increased to 305 ␮mol/L the following day. Other laboratory studies was no evidence of increased lacrimation, excessive urination, miosis,
including liver transaminases, creatinine and spinal fluid were normal. CT diarrhea, nor emesis. He was intubated after receiving atropine, etomidate
of the head was unremarkable and CT of the abdomen and pelvis and rocuronium. Heart rate increased transiently after intubation; how-
disclosed changes suggestive of fatty liver. A urea cycle disorder was ever, bradycardia returned after 30 minutes. A chemistry panel revealed
suspected. The serum amino acid panel demonstrated high plasma an elevated chloride level of 121 mEq/L with a low anion gap of 3. A
glutamine levels (5531 ␮mol/L; reference range: 205-756 ␮mol/L). Be- bromide level obtained two days after presentation was elevated at 14
cause of the persistent hyperammonemia and lack of improvement in mg/dL (normal ⬍0.5 mg/dL). Clinical course was significant for 3 days of
mental status, she was initiated on a low-protein enteral diet and persistent CNS depression. Head CT was within normal limits. Past
hemodialysis. With dialysis, her ammonia level decreased to 86 ␮mol/L. medical history was significant for MG diagnosed 5 months prior to
However, she remained unconscious and showed no sign of improvement. admission for persistent ptosis and difficulty holding his head upright. His
On day 7 of ICU stay, she developed ventilator-associated pneumonia, evaluation included an abnormal tensilon test and an elevated serum
growing methicillin-sensitive staphylococcus aureus. Blood cultures also acetylcholine receptor antibody titer. PB therapy was initiated with partial
grew a gram-negative bacillus. Antimicrobial therapy was initiated. Be- improvement in weakness and ptosis. Thymectomy was performed 2
cause of the lack of clinical improvement, the family decided to withdraw months prior to admission. Due to persistent symptoms, PB doses were
therapy. Hemodialysis was discontinued and her ammonia increased to a increased independently by ophthalmology and neurology.
high of 686 ␮mol/L. On day 10 of hospital stay, she passed away. A DISCUSSIONS: Signs of cholinergic toxicity include diarrhea, exces-
transcutaneous needle biopsy was obtained which provided the diagnosis sive urination, miosis, bradycardia, emesis, lacrimation and salivation. Our
of partial carbamyl phosphate synthetase I (CPSI) deficiency. patient displayed two of these, but also displayed evidence of CNS

depression; a sign not typically associated with cholinergic crisis. Further- asone also increases Ca-ATPase activity and level of Ca2⫹/calmodulin-
more, pyridostigmine does not readily cross the blood brain barrier, thus dependent protein kinase II in cardiac sarcoplasmic reticulum vesicles in
raising concerns for another factor to account for the CNS depression. adrenalectomized rats (3).
Our suspicion for bromism stemed from the high chloride level of 121 CONCLUSION: Depressed levels of cortisol can lead to the impair-
mEq/L with a low anion gap. Many hospital laboratories utilize electrolyte ment of both contraction and relaxation of the cardiac myocytes, resulting
panels that interpret halide ions as chloride; therefore, an elevated in impaired cardiac function. Cardiomyopathy may be more prevalent in
bromide level would be falsely reported as an elevated chloride level. If patients with adrenal insufficiency and it may play a larger role in the
we approximate a normal chloride level of 110 mEq/L then 11 mEq of acute presentation of adrenal crisis. As it is resolved with administration of
Bromide could be present. This level translates into a toxic level of 88 corticosteroids the cardiomyopathy may simply go unnoticed. This finding
mg/dL (toxic level ⬎ 50 mg/dL). Unfortunately, a specific bromide level may also explain why patients in shock from adrenal crisis respond so well
was not measured until after two days of resuscitation with normal saline to corticosteroids.
and maintenance of brisk urine output, maneuvers that increase excretion REFERENCES:
of bromide. Bromism has previously been associated with pyridostigmine 1 Afzal A, Reversible cardiomyopathy associated with Addison’s dis-
[1]. To our knowledge, this is the first reported suspicious case occurring ease. Can J Cardiol 2000; 16(3):377-379.2.
in a child. In cases of long-term exposure to PB, such as treatment for 2 Eto K, Adult reversible cardiomyopathy with pituitary adrenal
MG, bromism should be considered in the differential diagnosis; espe- insufficiency caused by empty sella“. Angiology , 2000; l 51,319-323.
cially when the clinical picture is not one of typical cholinergic toxicity. 3 Rao MK, Glucocorticoid modulation of protein phosphorylation and
Clinical suspicion and prompt laboratory confirmation can assist in sarcoplasmic reticulum function in rat myocardium. Am J Physiol
establishing inpatient treatment plan and on-going pyridostigmine treat- Heart Circ Physiol 2001;281: H325-H333
ment. Other agents are available that are not complexed with bromide and
may present a viable alternative.
CONCLUSION: Bromide toxicity should be considered in patients on
PB who present with depressed mental status.
REFERENCE:
1 Rothenberg, D.M., et al., Bromide intoxication secondary to pyri-
dostigmine bromide therapy. Jama, 1990. 263(8): p. 1121-2.
DISCLOSURE: Aaron Godshall, None.
REVERSIBLE CARDIOMYOPATHY IN A 35-YEAR-OLD MALE

WITH ADRENAL INSUFFICIENCY
Rana Y. Ali MD* Basir Haque MD Kevin Irish S Hilwa Farhad Arjomand
MD The Brooklyn Hospital Center, Brooklyn, NY
INTRODUCTION: Adrenal crisis is a true medical emergency requir-

ing prompt recognition and treatment. It is characterized by insufficient
steroid hormone release to meet the body’s physiological needs. However,
cardiomyopathy in adrenal crisis is not well described.
CASE PRESENTATION: 35-years old Ecuadorian male presented to
the ER with complaints of nausea, vomiting, diarrhea and vague epigastric
discomfort for four days. He also had significant weight loss and lack of
energy over the past 2 months. Past medical and family histories were
unremarkable. On physical exam he was cachectic. Blood pressure
80/42mmHg; heart rate was 130 beats/min, respirations 26 breaths/min
and oral temperature of 97.2oF. Cardiovascular and lung examination was
normal. Abdomen was soft, mildly tender in the epigastrium. He was alert
and orientated with no focal neurological deficits. EKG showed sinus
tachycardia. Serum sodium-124mmol/L; potassium-5.9mmol/L; calcium-
10.0mmol/L; BUN-44mg/ml: creatinine-1.9mg/dl, glucose-46mg/dl, Hb-
14.3gm/dl, WBC-16000/mm3 with 49% neutrophils, and CK-116U/L with
CKMB-1.6ng/mL and troponin I-0.0ng/ml. Despite aggressive IV fluid DISCLOSURE: Rana Ali, None.
CASE REPORTS
resuscitation, blood pressure continued to drop and norepinephrine was
added. As Adrenal crisis was suspected, IV hydrocortisone was started. CT
scan of the chest showed patchy areas of scaring and infiltrate in the lung WERNICKE’S ENCEPHALOPATHY IN A PATIENT AFTER GAS-
apices. Upper abdominal cuts showed bilateral calcification of adrenal TRIC BYPASS SURGERY
glands.On the second day he complained of chest pain. EKG was Jonathan P. Parsons MD* Clay B. Marsh MD John G. Mastronarde MD
unremarkable. However, Troponin-I increased to 26.8ng/dl. Echocardio- The Ohio State University Medical Center, Columbus, OH
gram revealed an ejection fraction (EF) of 37%, with dilated left ventricle.
Cardiac catheterization revealed normal coronary arteries, EF of 15% and INTRODUCTION: Obesity is a major public health crisis. There were
pulmonary artery wedge pressure of 22mmHg. Patient’s cortisol level approximately 414,000 deaths attributed to obesity in 2000.1 Bariatric
came back as ⬍0.5ug/dl. PPD was positive. Because of high clinical surgery for morbid obesity has become more common as a result of the
suspicion for tuberculosis, he was started on Rifampin, Isoniazid, Pyra- increasing prevalence of obesity. There are many complications that can
zinamide, and Ethambutal.On subsequent days he showed significant occur related to bariatric surgery; however, malnutrition and essential
clinical improvement. A repeat echocardiogram on day 10 revealed an EF vitamin deficiencies are not commonly recognized. We present a case of
of 50%, with improvement in Left ventricular diameter (LVD). Sputum Wernicke’s encephalopathy most likely secondary to thiamine deficiency
cultures came back positive for Tuberculosis. in a patient who had a recent history of gastric bypass surgery.
DISCUSSIONS: Cortisol is an important mediator in maintenance of CASE PRESENTATION: A 24 year-old female with a history of
homeostasis and an essential component to stress. Depressed levels are morbid obesity presented with neurologic dysfunction to the general
associated with collapse of vascular tone, derangements of vascular medical ward. She had gastric bypass surgery four months prior to
permeability and disruption of distribution of total body water. These presentation. She had been frequently admitted to hospitals post-opera-
abnormalities lead to a state of shock that is not responsive to fluids or tively for intractable nausea and vomiting and received intravenous (IV)
pressors.Cardiomyopathy in adrenal insufficiency has previously been dextrose. She had lost 100 pounds since surgery and had been non-
reported in adults (1,2). Cortisol has many well documented physiological compliant with her vitamin supplementation. Her medical and social
effects. Seldom described is its effect on the myocyte. Dexamethasone has histories were otherwise unremarkable. She presented with weakness,
been shown to significantly enhance the contractile tension and increase encephalopathy, ataxia, and visual changes. Her family stated her symp-
the velocity of contraction and relaxation in cardiac muscles. Dexameth- toms were gradual in their onset, but progressive. She developed acute

hypercapnic respiratory failure and was transferred to the intensive care arteries. We report a case of acute hypernatremia after discontinuation of
unit. Initial labs including a toxicology screen were unremarkable. Cere- vasopressin for the treatment of septic shock.
bral spinal fluid analysis revealed an elevated protein at 135, but gram CASE PRESENTATION: A 34 year old male presented to our
stain, cell counts, cultures, and serologies, including arboviruses and intensive care unit with hypercarbic respiratory failure due to obesity-
herpes simplex virus, were negative. Electroencephalogram (EEG) re- hypoventilation syndrome and pneumonia. On hospital day #3, he devel-
vealed diffuse slowing, but no epileptiform activity. Electromyelogram oped hypotension requiring norepinephrine and eventually, vasopressin.
(EMG) showed a mild sensory neuropathy not specific for Guillain-Barre Empiric antifungal therapy was started given extensive epidermal yeast
Syndrome or multiple sclerosis. Magnetic resonance imaging (MRI) of the infection. Blood cultures eventually grew Candida glabrata.On hospital
brain showed bilateral lesions involving the brainstem and the thalami. day #6, norepinephrine and vasopressin were discontinued. A brisk
This pattern was most likely related to toxic or metabolic disorder and was diuresis followed: the patient urinated 12 Liters in 8 hours, serum sodium
not consistent with lacunar or embolic infarcts. Magnetic resonance climbed from 146 to 171 mmol/L and urine osmolarity fell to 116
angiography was normal.Her clinical presentation was thought to be mOsm/kg (normal 250-1200) (Figure 1). This profound hypoosmotic
secondary to Wernicke’s encephalopathy based on her constellation of diuresis ceased with exogenous DDAVP, consistent with an acquired
neurologic symptoms (ataxia, encephalopathy, and visual complaints) and diabetes insipidus. To maintain eunatremia, he required scheduled and
characteristic MRI findings. She was treated with intravenous thiamine then intermittent doses of DDAVP until hospital day #47. Head computed
and her encephalopathy improved dramatically. She was able to be tomography revealed no pituitary or hypothalamic lesions.
weaned from mechanical ventilation after unsuccessful attempts previ-
ously. She was transferred to an acute rehabilitation hospital for recovery
and eventually to home. Repeat MRI approximately 4 weeks later showed
nearly complete resolution of the thalamic lesions.
DISCUSSIONS: Wernicke’s encephalopathy occurs both in alcoholics
and nonalcoholic subjects and it likely is an underrecognized cause of
encephalopathy in the intensive care unit. In non-alcoholic patients it may
be seen in patients who are fasting, receiving parenteral nutrition,
recovering from gastrointestinal surgery, or undergoing hemodialysis.
Wernicke’s encephalopathy occurs in patients who have undergone gastric
bypass surgery2, because thiamine is absorbed predominately in the
stomach and proximal small bowel which is bypassed surgically. Devel-
opment of neurologic symptoms such as confusion or ataxia in these
patients post-operatively should raise the possibility of Wernicke’s en-
cephalopathy. If patients have vomiting post-operatively and receive IV
dextrose-containing fluid, thiamine deficiency can be exacerbated as
glucose increases metabolic demands for thiamine. Additionally, their
nutritional deficiencies may go untreated as they may still be obese and go
unrecognized as “malnourished.” These patients are routinely instructed
to take additional iron, multi-vitamin, and mineral supplementation
post-operatively to prevent significant deficiencies from developing.
CONCLUSION: This case underscores the importance of a high-index
of suspicion for vitamin and mineral deficiencies in patients who have had
gastric bypass surgery. Thiamine supplementation should be considered in
all patients who have encephalopathy in an ICU setting, particularly those DISCUSSIONS: Vasopressin is a peptide hormone secreted by the
who have had gastric bypass surgery. posterior pituitary involved in both the regulation of serum osmolality and
REFERENCES: maintenance of adequate perfusion pressure. High serum osmolality and
1 Mokdad AH MJ, Stroup DF, Gerberding JL. Actual causes of death hypotension stimulate vasopressin release, but hypotension is a more
in the United States, 2000. JAMA 2004; 291:1238-1245 potent stimulus. Vasopressin acts on the endothelium causing vasocon-
2 Escalona A PG, Leon F, et al. Wernicke’s encephalopathy after striction and in the distal convoluted tubule and collecting ducts to
Roux-en-Y gastric bypass. Obes Surg. 2004; 14:1135-1137 facilitate reabsorption of free water. Vasopressin has been used to treat
nocturnal eneuresis, GI hemorrhage, diabetes insipidus, some forms of
von Willebrand’s disease, hemophilia A, and as an alternative to epineph-
rine in cardiac arrest. Recently vasopressin has been used at physiologic
doses for vasodilatory shock: post CABG or in sepsis. Investigators
rationalize that low doses of vasopressin replete vasopressin stores in the
pro-inflammatory state, improving sensitivity to cathecholamines. In
small, randomized controlled trials, vasopressin infusion allowed greater
dose reductions of other vasopressors when compared to placebo. Re-
ported side effects of vasopressin include: arterial and venous thrombo-
embolism, pseudotumor cerebri, torsades des pointes, myocardial infarc-
tion, rhabdomyolysis, skin necrosis, and disorders of sodium homeostasis.
Most of these adverse events were observed with the higher doses of
vasopressin used for GI hemorrhage, but some have been reported with
the doses used in sepsis. One prior report described hypernatremia
following discontinuation of vasopressin therapy, but the patient had a
history of SIADH. We believe our patient’s central diabetes insipidus was
iatrogenic–related to the discontinuation of a continuous vasopressin
DISCLOSURE: Jonathan Parsons, None. infusion. The mechanism is speculative, but may be due to antibody-
mediated competitive inhibition of the hormone which may be overcome
by additional exogenous replacement.
TRANSIENT ACQUIRED DIABETES INSIPIDUS AFTER VASO- CONCLUSION: The phenomenon of acquired transient diabetes
PRESSIN THERAPY FOR HYPOTENSION: A CASE REPORT insipidus may represent a rare adverse reaction to vasopressin therapy in
Christian Ramers MD* Joseph A. Govert MD Alison S. Clay MD Duke patients with septic shock.
University, Durham, NC REFERENCES:
1 Holmes CL, Patel BM, Russell JA, et al. Physiology of vasopressin
INTRODUCTION: Vasopressin use has increased after being shown relevant to management of septic shock. Chest 2001; 120:989-1002
to be an effective adjunct for adrenergic-refractory septic shock. Adverse 2 Sharshar T, Carlier R, Blanchard A, et al. Depletion of neurohy-
events from vasopressin infusions included decreased cardiac output and pophyseal content of vasopressin in septic shock. Crit Care Med
vasoconstriction causing hypoperfusion to the skin, gut and coronary 2002; 30:497-500

3 Patel BM, Chittock DR, Russell JA, et al. Beneficial effects of POST IRRADIATION ANGIOSARCOMA OF BREAST META-
short-term vasopressin infusion during severe septic shock. Anes- STATIC TO PLEURA
thesiology 2002; 96:576-582 Rahat Salamat MBBS Dominic R. DeKeratry MD Nikhat Salamat MD*
4 Holmes CL, Walley KR, Chittock DR, et al. The effects of Scott and White Hospital, Temple, TX
vasopressin on hemodynamics and renal function in severe septic
shock: a case series. Intensive Care Med 2001; 27:1416-1421 INTRODUCTION: We present a rare case of irradiation induced
5 Kristeller JL, Sterns RH. Transient diabetes insipidus after discon- angiosarcoma of breast metastasing to pleura .
tinuation of therapeutic vasopressin. Pharmacotherapy 2004; 24: CASE PRESENTATION: A 67 year old white female was diagnosed
541-545 with stage I infiltrating ductal carcinoma of the right breast in 1997 and
treated with lumpectomy, lymph node dissection and radiation. She
DISCLOSURE: Christian Ramers, None.
completed 5 years of Tamoxifen. In Sept. 2004 she presented with right
breast pain and violaceous nodules. She underwent a biopsy that showed
angiosarcoma. Epithelia tumor markers were negative. CD34 was nega-
tive, however CD31, factor VIII and vimentin were positive suggesting an
endothelial origin. She was treated with simple mastectomy and chemo-
therapy.Two months later she presented with shortness of breath and
Pleural Disease II clinical exam showed recurrence of angiosarcoma as violaceous nodules on
the muscle flap and chest wall. She had developed a large right pleural
4:15 PM - 5:45 PM effusion. CT scan of chest showed right sided effusion, bilateral lung
nodules, mediastinal lymphadenopathy and axillary lyphadenopathy along
PLEURAL EFFUSION DUE TO SALINE BREAST IMPLANT with nodules on the flap. After fluid reaccumulation despite two thora-
centesis, she underwent medical thoracoscopy for pleurodesis. Biopsies
WITHOUT RUPTURE MIMICKING MALIGNANT EFFUSION
were taken from the nodular parietal pleura revealing angiosarcoma. She
Mihaela Sescioreanu MD* Zachary Q. Morris MD Henry Ford Hospital,
was treated with palliative chemotherapy but died one month later.
Detroit, MI DISCUSSIONS: Pleural angiosarcomas are malignant vascular tu-
mours. Because of epitheliod appearance these are also called epitheloid
INTRODUCTION: Breast cancer is common in women and often angiosarcomas. Confirnation of endothelial origin is by immunohisto-
treated with radiation therapy to the chest wall, which is known to cause chemical staining with Factor VIII, CD34, CD31 related antigens.
injury to the underlying ribs. It is also common for these women to Epithelial markers like keratin are used to exclude epithelial origin.
undergo reconstructive surgery of the breast. We believe this is the first Primary and metastatic pleural involvement by lung angiosarcoma as been
reported case of over filling of a breast implant causing pleural effusion well documented, but post-irradiation angiosarcoma of the breast with
without the implant rupturing. The mechanism was from abnormal pleural metastases has not been reported.Angiosarcoma of breast can
pressure within the chest wall impairing normal lymphatic drainage. occur spontaneously (primary) or after local radiation and in association
CASE PRESENTATION: The patient is a 48 year old female who was with lymphedema (secondary). The first reported case of post irradiation
diagnosed three years earlier with left sided breast cancer. She underwent breast angiosarcoma was described in 1987. Total of 100 cases of
a lumpectomy with lymph node dissection because of a positive sentinel post-irradiation breast AS have been reported in English literature, but
node. She had subsequent chemotherapy and radiation therapy. Recently, none reported with metastases to pleura. One case has reported recur-
because of biopsy proven DCIS on the left side, she underwent total rence with pleural lesions. Pleural metastases are rare at initial presenta-
mastectomy with contra lateral prophylactic mastectomy. Bilateral breast tion (1%).Criteria for radiation induced AS were described in 1948 by
reconstruction was performed with saline filled implants. The left breast Cahan et al 1) tumour arising in an irradiated area ; 2) tumor is
required multiple additional saline installations every couple of weeks over histologically different from first tumor ; 3)A long period of latency
several months, because of asymmetry in breast size. This was caused by between irradiation ad occurance of AS.Treatment is with primarily
surgical resection, radiation and chemotherapy with little success and has
fracture and inward displacement of an underlying atrophic rib injured
poor prognosis high rate of recurrence. Most patients die within 3-5 years
from radiation therapy. She then developed shortness of breath and was .Average interval between irradiation and dignosis of angiosarcoma is 7
found to have a large left sided pleural effusion. A Chest CT scan of the years with range 3 - 20 years.These are highly aggressive tumours and has
chest showed the implant was bulging into and compressing the left mid high rate of recurrence with in months of radical resection .
lung. Aside from the effusion, there was no other sign of malignancy. CONCLUSION: Breast AS has a poor prognosis. As more women are
Several days later a thoracentesis was cancelled because a radiograph treated with breast conservation therapy for early breast cancer the
performed prior to the procedure showed a significant decrease in size of incidence is expected to rise. Early clinical detection will require high
the effusion. A follow up film two weeks later showed the effusion had index of suspicion by health care providers.
CASE REPORTS
again increased in size, so a thoracentesis was performed. The fluid was DISCLOSURE: Nikhat Salamat, None.
negative for malignancy. It was not bloody, ruling out trauma as a cause.
Leakage of saline was also excluded as a cause because surgery was able
to recover all of the saline used to fill the implant. After the implant was
completely emptied of saline, within one week the effusion resolved with DESMOID TUMOR DISGUISED AS A PLEURAL LESION
total resolution of symptoms. Theophilus T. Ogungbamigbe MB, ChB*U. D. Bayraktar MD Sharon
DISCUSSIONS: The treatment of breast cancer is associated with a Ngan MD Marie F. Schmidt MD Interfaith Medical Center, Brooklyn,
number of complications related to surgery as well as radiation. Surgery is NY
associated with local complications including infection, hematoma, im-
plant rupture, seroma, lymphedema of the arms, muscle and nerve INTRODUCTION: We report a case of a desmoid tumor or a
destruction, and chest wall injury. Radiation therapy is known to cause desmoplastic fibroma, an extremely rare benign bone tumor that pre-
complications to the skin, lungs, and ribs. Breast implants have only been sented as a pleural based density radiologically.
reported to cause pleural effusion when the implant ruptured. CASE PRESENTATION: A 61-year-old African American male
CONCLUSION: Because the normal drainage of pleural fluid occurs presented with left-sided sharp chest pain for a month. Chest radiog-
through the lymphatic drainage of the parietal pleura, we believe the raphy and chest Computerized Axial Tomography Scan showed a 3x2
cm pleural based density in the posterior aspect of the left upper lobe
mechanism of this exudative effusion was due to partial obstruction of the
(Fig 1). Adjacent rib showed bone destruction with thin sclerotic rims.
lymphatic drainage from increasing pressure in the chest wall caused by Bone imaging with Technetium 99m-HDP showed increased focal
over filling of the implant. Contributing factors were the atrophic radiated uptake in the region of 5th rib adjacent the tumor. Video-assisted
rib and possibly the previous lymph node dissection. This is supported by thoracoscopic surgery was performed but the firm consistency of the
waxing and waning of the effusion, possibly due to positional changes. The mass made frozen section technically impossible. The patient under-
complete recovery of all the fluid used to fill the implant ruled out went thoracotomy and the lesion was excised totally with posterior
rupture, and the absence of hemothorax ruled out trauma from the broken segments of the 3rd, 4th and 5th ribs. Histological examination showed
rib as causes for the effusion. sheets of spindle cells partially encircling the bone and focally
DISCLOSURE: Mihaela Sescioreanu, None. involving extensive areas of cortical and medullary bone (Fig 2&3).

Pleural Disease II, continued
Cells showed moderate amount of hyperchromaticity without high- desmoplastic fibroma histologically, although the radiological features
grade anaplasia or atypical mitotic figures. Immunohistochemical favor desmoid tumor. Management does not differ in both entities and
staining showed scattered areas of positivity with smooth muscle actin consists of wide local resection. Local recurrence is a definite possi-
and desmin 33. Staining for S-100 and CD34 was equivocal. Tumor bility and metastatic potential is negligible.
extended very near but without involvement of the pleura. Resection CONCLUSION: In this case, the lesion, which presented as a pleural
margins were free of tumor. Post-operatively patient developed acute based density, was likely a desmoid tumor although its bone counterpart,
renal failure requiring hemodialysis for uremic encephalopathy that desmoplastic fibroma, could not be ruled out.
eventually resolved. Patient was discharged on post-operative 27th day DISCLOSURE: Theophilus Ogungbamigbe, None.
still complaining of mild intermittent chest pain.
A CASE OF PRIMARY TULAREMIC PNEUMONIA DIAGNOSED

BY PLEURAL FLUID CULTURES
Anthony J. Perella MD* Wissam Abouzgheib MD Rania Aboujaoude MD
Henry Fraimow MD Ramya Lotano MD Cooper University Hospital,
Camden, NJ
INTRODUCTION: Tularemic pneumonia is often complicated by

pleural involvement and effusion.1 The causative pathogen, Francisella
tularensis, rarely has been cultured form pleural fluid.1 The microbiolog-
ical diagnosis of tularemia relies mainly on serology.
CASE PRESENTATION: A healthy 31 year old male, construction
worker, was admitted to an outlying emergency room for the sudden onset
of fever, shaking chills, productive cough and dyspnea. A Chest Roent-
genogram documented right middle and lower lobes consolidations with
right pleural effusion. Diagnostic thoracentesis yielded a sero-sanguineous
effusion with 1313 UI LDH, 2.9 protein , 48 glucose, and a negative Gram
stain. Pleural fluid cultures were sent. He was initially treated for
community acquired pneumonia. but increasing oxygen requirements
resulted in broadening of antimicrobials to include imipenem, azithromy-
cin, moxifloxacin and fluconazole and he was transferred to Cooper
University Hospital.Physical exam upon arrival revealed a tachypneic
young man on 100% oxygen non re-breather. No cervical or inguinal
lymphadenothy were appreciated, and there were no skin lesions or rashes
noted. Diffuse rhonchi were heard over bilateral lung fields. The remain-
der of physical examination was unremarkable. A chest CT scan showed
right apical area necrosis, left lower lobe consolidation and moderate left
pleural effusion. A repeat thoracentesis yielded similar results to the initial
thoracocentesis.Antibiotics were changed to levofloxacin and vancomycin.
His clinical condition markedly improved and after 48 hours supplemental
oxygen was no longer need. He was discharged on P.O levafloxacin. Three
days after discharge, the pleural fluid from the initial right sided
thoracentesis grew Francisella tularensis. Indentity of the isolate was
confirmed by PCR by the New Jersey State Department of Health
Laboratory. Treatment was switched to doxycycline for two weeks, he
then completed an additional three weeks of levafloxacin due to Doxycy-
cline induced GI Discomfort.
DISCUSSIONS: This case illustrates pneumonic tularemia; one of the
six distinct clinical syndromes of Francisella tularensis infection. Pneumo-
nic tularemia refers to an illness with an initial presentation dominated by
pulmonary infection. Two clinical forms are recognized. The primary form
occurs by direct inhalation of aerosolized Francisella tularensis. It includes
DISCUSSIONS: Desmoid tumors are benign but locally aggressive many of the most fulminate cases encountered.2 The secondary form can
myofibroblastic neoplasms originating from the muscle aponeurosis or complicate any of the other clinical syndromes, mainly typhoidal and
fascia constituting 3% of all soft tissue tumors. They are primarily ulceroglandular disease.2 and is believed to occur by hematogenous
located on abdominal wall. Extra-abdominal sites include shoulder spread . Our patient may have become infected by inhaling aerosolized
(20%), chest wall and back (15%), thigh (12%), mesentery (10%), neck Francisella tularensis while clearing his work site with a power mower.The
(10%), and knee (7%). Desmoid tumors are composed of abundant pleural effusion in pleuropulmonary tularemia is described as turbid or
collagen surrounding poorly circumscribed dense bundles of eosino- sero-sanguineous exudate with a predominance of lymphocytes or neu-
philic spindle cells with regular nuclei, pale cytoplasm and absence of trophils and a high Adenosine Deaminase level.1 Pleural granulomas can
mitoses. Tumor cells can be reactive for vimentin, desmin, and smooth be found on pleural biopsy specimens.4 Findings that mimic tuberculous
muscle actin. Radiographs may show a non-specific soft-tissue mass pleurisy.1,4Francisella tularensis has rarely been cultured from pleural
and underlying bone involvement in 6-37% of patients. Bone scintig- fluid.1 Culture of Francisella tularensis is not routinely performed due to
raphy usually demonstrates increased uptake on blood pool and static special media requirements and potential hazard to laboratory personnel.
images in areas adjacent the tumor. Desmoplastic fibroma, which is When growth is suspected, a reference laboratory should be consulted for
considered as the bone counterpart of desmoid tumor, is a very rare safe handling and confirmation by detection of DNA encoding for a 17-kD
primary tumor of bone with a reported incidence of 0.06% of all bone lipoprotein of Francisella tularensis, as occurred in our case.2.
tumors. First described by Jaffe in 1958, it is most commonly found in CONCLUSION: Pneumonic Tularemia can cause pleural effusion.
the long tubular bones (56%), mandible (26%), and pelvis (14%). When suspected, Adenosine deaminase level and culture for Francisella
Histologically similar to desmoid tumor, it shows prominent bundles of tularensis should be included in the analysis of the pleural fluid specimen.
fibrous tissue composed of slim spindle shaped fibroblasts without REFERENCES:
mitoses or atypical cells, and variable amounts of bonds of collagen 1 Pettersson T, Nyberg P et al. Similar pleural findings in pleuropul-
fibers. Radiologically, tumors are expansile lytic lesions often with monary tularemia and tuberculous pleurisy. Chest. 1996 Feb:
internal trabeculation (91%) and soap bubble appearance. Desmoplas- 109(2):572-5
tic fibroma in the rib is extremely rare, with only four cases reported 2 Tärnvik A, Berglund L. Tularaemia. Eur Respir J 2003; 21: 361-373
in the literature up to date. In our case, thoracic wall mass that first 3 Evans ME, Gregory DW et al. Tularemia: a 30-year experience with
appeared to be pleural in origin resembles to both desmoid tumor and 88 cases. Medicine (Baltimore). 1985 Jul;64(4):251-69.

4 Schmid GP, Catino D et al. Granulomatous pleuritis caused by on her right side and was progressively increasing in size. On exam, she
Francisella tularensis: possible confusion with tuberculous pleuritis. had a 2 by 1.5 cm firm subcutaneous nodule along the site of the old
Am Rev Respir Dis. 1983 Aug;128(2):314-6 tunnel. A CT of the chest done 4 weeks prior showed the presence of a
small subcutaneous nodule. A fine needle aspiration was performed and
confirmed the presence of metastatic adenocarcinoma.
DISCUSSIONS: Local tumor recurrence or seeding following pleural
drainage, pleural biopsy, tube thorascopy and surgical video assisted
thoracoscopic surgery has been described but it is uncommon in non-
mesothelioma malignancies234. PleurX catheters, a relatively newer de-
vice, are an option in the palliative management of recurrent malignant
effusions. These catheters offer the advantage over tube thorascopy and
chemical pleurodesis of being an outpatient procedure and allowing the
patients to rapidly return to their pre-procedure functional status. They do
require some training of the patients and their caregivers so that they can
be intermittently drained using vacuum bottles when the patients become
symptomatic. Approximately 40% of patients will achieve successful
pleurodesis and can have their catheter removed1. Thus far, there had
been no reported cases of tumor seeding from removal of a PleurX
catheter. The clinical significance is uncertain since most of the patients
undergoing placement of PleurX catheter already have metastatic disease.
However, as it was the case in our patient, pain secondary to the
subcutaneous nodule can be an issue.
CONCLUSION: Seeding of the tunnel tract and subcutaneous metas-
tasis after removal of PleurX catheters can occur. It should be considered
in a patient presenting with pain and a palpable nodule at the prior site of
a PleurX catheter.
REFERENCES:
1 Wyckoff CC, Anderson ED, Read CA. The PleurX Catheter for the
Management of Symptomatic, Recurrent Malignant Pleural Effu-
sions: The Georgetown Experience [abstract]. Chest 2003; 124
(suppl):130S
2 Jones FL. Subcutaneous implantation of cancer: a rare complication
of pleural biopsy. Chest 1970; 57; 2: 189-190
3 Kumar UN, Varkey B. Case report: subcutaneous metastasis. Rare
complication of drainage of malignant pleural fluid. Postgrad Med
1976; 60: 253-255
4 Yim AP. Port-site recurrence following video-assisted thoracoscopic
surgery. Surg Endosc 1995; 9: 1133-1135
DISCLOSURE: Cristina Reichner, None.
EOSINOPHILIC PLEURAL EFFUSION DUE TO ARTEMISNIN:

A CASE REPORT
Mayank Vats MD* Rakesh C. Gupta MD Deepa V. Khandelwal MBBS
DISCLOSURE: Anthony Perella, None. Manohar L. Gupta MD Neeraj Gupta MD Mukesh Tailor MBBS J.L.N.
Medical College, Ajmer, Rajasthan, India
SUBCUTANEOUS METASTATIC SEEDING AFTER REMOVAL INTRODUCTION: Drug induced eosinophilic pleural effusion (EPE)
OF A PLEURX CATHETER is well documented in literature. EPE is not a disease rather an interesting
Cristina A. Reichner MD* Charles A. Read MD Georgetown University laboratory finding, defined as ⬎10% eosinophils in pleural fluid, exclusive
Hospital, Washington, DC of erythrocytes. Pleural fluid eosinophilia may be associated with blood
CASE REPORTS
eosinophilia e.g. Loeffler syndrome, Churg Strauss syndrome etc. Con-
INTRODUCTION: Tunneled PleurX catheters are an alternative versly EPE can also occur without blood eosinophilia e.g. Pulmonary
outpatient option for the palliation of recurrent malignant effusions. They Infarction, Pneumonia or Trauma. We report a case of bilateral EPE
are associated with very little morbidity. Forty percent of patients achieve secondary to Artemisnin with no peripheral eosinophilia.
pleurodesis with the catheter in place, allowing for removal of the CASE PRESENTATION: AM 46-year-old female presented to OPD
catheter1. We report the first development of subcutaneous metastasis in because of bilateral dull aching chest pain, which increased, on taking deep
the tract of a PleurX catheter following its removal after successful breath. Five days before the presenting illness she also had high grade fever
pleurodesis. with chills for which she received injection Artemisinin on the suspicion of
CASE PRESENTATION: The patient is a 43 year old Puerto Rican malaria in another tertiary health care center. The patient demonstrated no
female with a history of recurrent metastatic adenocarcinoma of the right symptom, sign or laboratory data of any infectious process, Chest X-Ray PA
breast originally diagnosed in 1994. At the time of diagnosis, she revealed bilateral pleural effusion more on right side & no parenchymal
underwent right mastectomy and lymph node dissection followed by infiltrates. Patient underwent right thoracentesis, revealing protein-4.2 g/dl,
adjuvant chemotherapy and tamoxifen. She had a chest wall recurrence in 60%-lymphocyte, 26%-eosinophils, 10%-mesotheilial cells & 4%-poly-
2001 treated with additional chemotherapy. In 2003, she developed a morphs. Next day, left thoracentesis revealed protein-4.4 g/dl, 56%-lympho-
right-sided malignant pleural effusion requiring 3 thoracenteses, each cytes, 32%-eosinophils, 8%-mesothelial cell & 4%-polymorphs. Pleural bi-
with radiographically proven reexpansion of her right lung and improve- opsy showed mixed lymphocytic & eosinophilic infiltrates within pleura. The
ment in dyspnea. She underwent an uncomplicated right PleurX catheter bacterial, fungal & mycobacterial smear & culture of fluid & biopsy specimen
placement in August of 2004. She drained her effusion every 3 to 4 days were negative after 6 weeks. Her serology was negative for Anti-Nuclear
initially and then noted progressively decreased drainage. The PleurX Antibody, LE cells, & Rheumatoid factor. There was no history of contact of
catheter was removed in the outpatient office 8 weeks after it had been pulmonary tuberculosis & Montoux test was negative. A detailed history of
placed. A CXR at that time showed minimal residual pleural effusion. illness (including absence of any significant past medical history), drug intake
Since then, the patient has been asymptomatic and has not required any & complete evaluation strongly pointed out towards a possible drug (Ar-
thoracentesis on the right side. Four months after the removal of the temisnin) induced pleural effusion, hence after informed consent & with
catheter, the patient noticed a painful lump on her right chest, at the site permission of ethical society of institution, injection Artemisnin 40mg IM x 5
of the old PleurX catheter. The lump was more noticeable when she slept days was given, as challenge dose & on 7th day patient again developed

bilateral chest pain & Chest X-Ray revealed bilateral minimal pleural 76.5%, FVC; 69%, FEV1/FVC; 85%. TLC; 84.4%, VC; 71%, RV;
effusion. Patient was started on prednisolone 60 mg/day for 7 Days & pleural 104%. MIP; 79%, MEP; 66%. DLCO corrected for Hgb was normal.
effusion disappeared completely, hence confirming the diagnosis. Brain and cervical spine MRI showed white matter attenuation.
DISCUSSIONS: Air & Blood are the most common cause of EPE. Echocardiography showed severe right ventricle enlargement. Poly-
Other causes of EPE1 are Bronchial or Pleural malignancy, hypersensi- somnogram demonstrated a baseline sleep SaO2 of 85% and an SaO2
tivity reactions, pulmonary infarction & infection with viruses, fungus2 nadir of 64%. Sleep architecture was fragmented with a respiratory
(Coccidiodomycosis, Histoplasmosis) & parasites (Echinococcus, Amoe- arousal index of 88. There was severe snoring and dysrhythmias. The
biasis, Ascariaris, Schistosomiasis, Ankylostomiasis etc.).Drug reactions apnea-hypopnea index (AHI)was 87. The average duration of apneas
like Dantrolene, Bromocriptine, Nitrofurantoin. Procarbazine, Ergot, was 14s with a maximum of 46.5s. He was initiated on BIPAP at an
Methotrexate has been implicated in EPE. Allergic disease like Asthma, IPAP pressure of 20cm H20 and EPAP of 10 cm H2O. Following 6
Tropical Pulmonary Eosoniphilia, Churg-Strauss syndrome may also lead months of BIPAP therapy, the hematocrit normalized without re-
to Eosinophilic Pleural Effusion.3In this case the absence of other causes peated phlebotomy. The hypersomnolence also improved.
for the EPE & its complete resolution after withdrawal of Artemisnin, DISCUSSIONS: NMO is characterized by the preferential demyeli-
reappearance on challenging with low dose supports a causative relation- nation of the spinal cord and optic nerves and clinically unravels as a
ship of this drug with the development of EPE. This is probably the first monophasic or relapsing neurological disease.The precise pathogenesis of
case report of EPE secondary to Artemisnin. NMO is unknown. However, NMO is considered to be a separate
CONCLUSION: Artemisnin must be included in the list of drugs clinico-pathological entity from MS. SDB/OSA syndromes associated with
causing EPE, however the exact mechanism is not known, but, if the drug demyelinating diseases are complex in their genesis, evolution and
is considered as a cause of EPE, the drug should be immediately management owing to a complex interplay of the primary neurological
discontinued. Clinical Implications: a high degree of suspicion should pathology and socio-biological sequel to neuro-functional impairment.
always be kept in mind to prove the drug as an etiologic agent in Inflammatory damage to the central cardiopulmonary control centers
undiagnosed EPE, and a challenge test with all due precautions should impairs the automated regulation of respiration during sleep and may
always be done to prove or disprove this etiology. cause sudden nocturnal death.In addition, myelitis mediates complex limb
REFERENCES: movement disorders that increases arousal during sleep. Moreover,the
1 Campbell GD, Webb WR: Eosinophilic Pleural Effusion, Amer. neuromuscular weakness associated with NMO impairs respiratory me-
Rev. Resp. Dis. 1964, 90, 194. chanics and augments sleep related pharyngeal hypotonia thus further
2 Curran WS, Williams AW: Eosinophilic Pleural Effusion, Arch. reducing ventilatoty efficiency as well as mediating upper airway obstruc-
Inern. Med. (Chiacgo) 1963, 111, 809. tion.These factors occur in tandem with pain syndromes,fatigue and the
3 Erzurum SE, Underwood GA, Hamilos DL, Waldron JA: Pleural eff psychological consequences of chronic illness and thus further distorting
DISCLOSURE: Mayank Vats, None. sleep architecture.
CONCLUSION: SDB/OSA should be considered in demyelinating
diseases that have fatigue and unexplained polycythemia.
REFERENCE:
1 Wingerchuk DM. Neuromyelitis optica: current concepts. Front
Biosci. 2004 (1);9:834-40.
DISCLOSURE: Daniel Dube, None.
Sleep Apnea/Pulmonary Hypertension
4:15 PM - 5:45 PM
FAILURE TO THRIVE DUE TO OBSTRUCTIVE SLEEP APNEA
IN A CYSTIC FIBROSIS PATIENT
SEVERE OBSTRUCTIVE SLEEP APNEA (OSA) ASSOCIATED Ignacio E. Tapia MD* Suzanne E. Beck MD St. Christopher’s Hospital
WITH SEVERE SECONDARY POLYCYTHEMIA AND PULMO- for Children, Philadelphia, PA
NARY HYPERTENSION IN A PATIENT WITH DEVIC’S SYN-
DROME INTRODUCTION: In Cystic Fibrosis (CF) poor nutritional status is
Daniel S. Dube MD* Priscilla Sarinas MD The Department of Veteran associated with decreased pulmonary function and increased Pseudomo-
Affairs and Palo Alto Health Care System and Stanford, Stanford, CA nas aeruginosa (PA) colonization; recognizing failure to thrive (FTT) and
its causes is of paramount importance so that an aggressive and early
INTRODUCTION: Neuromyelitis optica(NMO)or Devic’s syndrome treatment can be instituted. Obstructive sleep apnea syndrome (OSAS) is
is an idiopathic demyelinating disease characterized by a variable course a well-recognized cause of FTT in non-CF children, and catch-up growth
of relapsing optic and spinal neuritis that occur conjointly or separately has been observed in pediatric patients after the resolution of OSAS. At
and interspaced with asymptomatic interludes. Although sleep disordered present time however, there are no reported cases of OSAS as a cause of
breathing (SDB) is an established complication of demyelinating syn- FTT in CF. We report a case of FTT secondary to OSAS in a CF patient.
dromes such as multiple sclerosis (MS),there are no reports of severe OSA CASE PRESENTATION: BS is a 3-1⁄2 y.o. twin boy, full term (BW
in cases of NMO culminating in severe polycythemia and pulmonary 2496 G) with CF and pancreatic insufficiency (PI). He and his twin sister
hypertension. were diagnosed with CF by sweat test (99 mEq Cl/139.3 mg sweat) after
CASE PRESENTATION: A 48-year old male was referred to the a positive neonatal screening. Genotyping revealed one copy of Delta F
pulmonary clinic for the evaluation of a 1 year history of worsening 508 mutation and one copy of K710X mutation, and pancreatic fecal
fatigue and hypersomnolence. His Epworth sleepiness score was 18/24. elastase was elevated.Pancreatic enzyme replacement was started at 1
He had been evaluated by his physician for similar symptoms and was month of age, with an average daily weight increase of 50 g until 6 months
noted to have a hematocrit of 67% and he was started on periodic of age, when weight gain fell to 11.5/day, despite CF therapy with daily
phlebotomy. Eight years previously, he was diagnosed with NMO DNAase, chest physiotherapy, nebulizations, and oral antibiotics. There
following a consultation for the management of variant MS that was were many factors identified that may have contributed to poor weight
typified by recurrent optic neuritis and global extremity motor weak- gain, such as malabsorption, poor social situation (low family income, and
ness and Lhermitte’s phenomenon. He was legally blind and mildly older sister with Down syndrome having several needs), recurrent low
obese (BMI; 28.4). He denied the use of alcohol or illicit drugs. Blood grade URI. Enzyme replacement was increased to 1000 U/kg, and caloric
pressure was 130/76, pulse; 96/minute, respiratory rate; 19/minute and density of feeds increased to 27 cal/oz. In addition daily in-house nursing
resting SaO2;98%.Core body temperature was normal. Physical exam- visits (8 hours/day x 5 days/week) were instituted to help the family take
ination revealed a middle aged male with a depressed affect. The nose care of the twins increased nutritional and respiratory needs.At 14 months
exhibited mild turbinate hypertrophy and inflammation. The airway of age, despite the interventions, weight persisted along the 0-5th
was Malampati class 3. Cardiac revealed a loud S2. Chest was clear. percentile. At this time, PA was retrieved from a throat culture; TOBI™
Neurological examination revealed bilateral optic atrophy.Power was and ciprofloxacin were started in an effort to eradicate it. A feeding
3⫹ in all extremities.There was 2⫹ peripheral leg edema. Abdomen gastrostomy was considered, but it was hoped that PA eradication would
was normal. Laboratory data revealed Hgb; 20.4 mg/dl, hematocrit alleviate the poor weight gain.Despite PA eradication, proper enzyme
(HCT); 59%, erythropoietin; 50 mU/ml, ABG; PH; 7.39, PCO2 52 replacement, and caloric supplementation, as well as in-home nursing,
mmHg, PO2; 66 mmHg. PFT showed the following indices of weight gain did not improve. Although subsequent cultures showed
pulmonary function (expressed as percentages of predicted): FEV1; absence of PA, symptoms of nasal congestion, mouth breathing, and

Sleep Apnea/Pulmonary Hypertension,
continued
snoring persisted, so at 17 months of age a sleep study was performed to prostacyclin synthesis by endothelial cells in vitro, therefore suggesting a
evaluate for OSAS as a contribution to FTT and snoring. In fact, it showed possible relationship between the two2. Notably, her ANCA titer was
several obstructive apneas (5.3/hr), most of which were brief in duration. negligible prior to initiation of epoprostenol. Finally, the vasculitis could
Episodes of desaturation were short and related to obstructive apnea.The be induced by bosentan. Bosentan has also been reported to be associated
study confirmed the suspicion of severe OSAS, based on the number of with necrotizing LCV3. Our patient had been on a stable dose of 125mg
events, so adenotonsillectomy was performed. After surgery, snoring twice daily throughout the entire course of her vasculitis; however her
resolved, work of breathing, growth parameters, and appetite all improved rash intensity and ANCA levels varied throughout.
considerably, and at 29 months old, BS’s weight was in 32nd %ile, height CONCLUSION: In summary, we describe a patient who developed
was in 57th %ile. Wegener’s granulomatosis while undergoing treatment with epoprostenol
DISCUSSIONS: This patient represents the point that OSAS is a and bosanten for her PAH. Although a direct causal relationship can not
contributor to FTT in CF patients. There are several publications pointing be asserted, an association between epoprostenol or bosentan and ANCA
out FTT as secondary to OSAS in non-CF infants and children, showing vasculitis is possible.
improvement in weight and height after surgery. The cause of poor REFERENCES:
growth is not known, the most cited causes are increased sleep energy 1 Myers SA et al. Cutaneous findings in patients with pulmonary
expenditure (SEE) and impaired growth hormone secretion. OSAS could arterial hypertension receiving long-term epoprostenol therapy.
be overlooked in the CF population, because of many other common J Am Acad Dermatol 2004;51(1):98-102
reasons for FTT, such as PI, chronic infection, increased work of 2 Sibelius U et al. Wegener’s granulomatosis: anti-proteinase 3 anti-
breathing, increased energy expenditure, feeding behavior problems, bodies are potent inductors of human endothelial cell signaling and
social issues. Physicians may also be reluctant to overwhelm the family leakage response. J Exp Med 1998;187(4):497-503
with secondary diagnosis. These same reasons may increase the risk of 3 Stefan G et al. Severe necrotizing leucocytoclastic vasculitis in a
non-treated OSAS sequelae in CF patients. patient taking bosentan. BMJ 2004;329:430
CONCLUSION: We stress the point of early recognition of OSAS in
the differential diagnosis of FTT in CF children.
DISCLOSURE: Ignacio Tapia, None.
C-ANTINEUTROPHIL CYTOPLASMIC ANTIBODY VASCULITIS

IN A PATIENT WITH PRIMARY PULMONARY HYPERTENSION
ON PROSTACYCLIN AND BOSENTAN
Aneesa M. Das MD* Linda Paradowski MD University of North Carolina
Hospitals, Durham, NC
INTRODUCTION: Pulmonary arterial hypertension (PAH) and We-

gener’s granulomatosis are both rare diseases. To our knowledge these
have not been described in the same patient. Although there is an
association of skin manifestations with epoprostenol and bosanten, there
are no known associations of C-antineutrophil cytoplasmic antibody
(ANCA) vasculitis with the use of these drugs.
CASE PRESENTATION: A thirty-nine year old woman, previously
diagnosed with PAH, presented with a two day history of a non-pruritic,
non-painful rash over her lower extremities. She denied any fevers,
flushing episodes, hematuria, presyncope, or the use of new medications
or new skin products. Her past medical history included seizures and
tracheotomy for bilateral ankylosed arytenoids identified after an intuba-
tion three years prior. Her medications included prostacyclin, bosentan,
warfarin, and depakote. She had been seen 3 weeks prior to presentation
to increase her epoprostenol from 12 to 13 ng/kg/min. On physical exam,
she had a non-blanching purple maculopapular rash over her lower
extremities bilaterally with some confluent areas. She had minimal
peripheral edema. She had a normal pulmonary exam and a pronounced
P2 on cardiovascular exam. A biopsy of the rash confirmed leukocytoclas-
CASE REPORTS
tic vasculitis (LCV) (Figure 1). Her serologic work up included the
following: normal liver enzymes, erythrocyte sedimentation rate 87 mm/
hr, anti-nuclear antibody 1:80, platelets 113 M/mL, negative myeloperox-
idase-ANCA, positive proteinase 3 (PR 3) ANCA at 173.1 u/mL. Her
creatinine was 0.9 mg/dL and she had 2⫹ protein and 4⫹ blood in her DISCLOSURE: Aneesa Das, None.
urine with dysmorphic red cells present. A subsequent renal biopsy
showed focal necrotizing pauci-immune, PR3-ANCA associated glomer-
ulonephritis. She subsequently began treatment with intravenous cyclo- PULMONARY TUMOR EMBOLI FROM METASTATIC PANCRE-
phosphamide and prednisone. After one month of this therapy, she ATIC CARCINOMA: A RARE CAUSE OF PULMONARY HYPER-
presented with hemoptysis. Her computed tomography scan (Figure 2) TENSION
was consistent with pulmonary alveolar hemorrhage. She received plas- Alexis H. Meredith MD* Timothy Williamson MD University of Kansas,
mapheresis and pulse steroids. Her hemorrhage stabilized and she was Kansas City, KS
discharged. Unfortunately, the patient’s PAH has been too unstable to
wean or discontinue the epoprostenol or bosentan. She continues to have INTRODUCTION: Disseminated microvascular pulmonary tumor cell
dysmorphic red cells on urinalysis despite treatment. embolism is a known cause of pulmonary hypertension, however it is fairly
DISCUSSIONS: This woman could have developed two distinct life uncommon. Nevertheless, in an older patient without a clear cause of
threatening diseases or relationships could potentially exist between this pulmonary hypertension, it should be included in the differential diagnosis.
patient’s ANCA vasculitis, her PAH and its treatment. First, the patient CASE PRESENTATION: A previously healthy sixty-one year old male
could have had an initial smoldering vasculitis causing endothelial damage presented with a two month history of generalized fatigue and low grade
and predisposing the patient to PAH. Although the patient was intubated fevers. While undergoing evaluation of his fatigue and fevers, he had an
for less than 2 weeks in 2001, she underwent tracheotomy for ankylosed echocardiogram performed that showed elevated pulmonary artery pres-
arytenoids which could have been associated with Wegener’s granuloma- sures. Right heart catheterization was performed and demonstrated
tosis. Secondly, the vasculitis could have been induced by the epoproste- pulmonary artery pressures of 72/30 mmHg with a normal pulmonary
nol. LCV has been previously reported to be associated with epoprostenol, artery occlusion pressure. Further workup revealed a positive anti-nuclear
however system vasculitis was not reported1. PR3-ANCA has an effect on antibody, symptoms consistent with Raynaud’s disease, and a Factor VII

continued
deficiency. CT of the chest was done and was clear. Other workup
including ventilation perfusion scan, pulmonary function testing, exercise
and overnight oximetry, C-ANCA, P-ANCA, rheumatoid factor, and
human immunodeficiency virus testing was all unremarkable. The pa-
tient’s pulmonary hypertension was attributed to an underlying connective
tissue disorder, and he was started on corticosteroids. After a remarkable
improvement of two World Health Organization (WHO) functional
classes he was discharged home. Two weeks later he returned with
complaint of increasing shortness of breath, fevers and night sweats, and
cough productive of blood tinged sputum. Admission radiography showed
bilateral cavitary pulmonary infiltrates. PPD was negative. Sputum culture
and AFB were also negative. The patient was treated empirically with
broad spectrum antibiotics with improvement in his symptoms and was
again discharged home. The patient returned two weeks later with left
sided chest pain and worsening dyspnea. CT chest was performed on
admission and showed no change in the patient’s cavitary lesions. Flolan
was initiated based on the patient’s severe pulmonary arterial hyperten-
sion and his WHO functional class IV symptoms. The patient initially
improved on Flolan, but despite aggressive titration, he suffered progres-
sive cardio-pulmonary collapse and died. Post mortem examination was
performed and revealed a small pancreatic mass that was found by
microscopy to be poorly differentiated pancreatic adenocarcinoma. In
addition to the pancreatic mass, there were parenchymal masses seen in
the liver and lung, and widespread angiolymphatic dissemination to
multiple organs. Sections of the lung showed that the cavitary lesions were
areas of infarction and necrosis with tumor emboli occluding many
pulmonary vessels.
DISCUSSIONS: When evaluating possible causes of pulmonary hy-
pertension, the differential diagnosis is quite large. In this patient several
things confounded the ultimate diagnosis. The patient’s positive anti-
nuclear antibody in addition to his Raynaud’s symptoms led to a mistaken
diagnosis of a connective tissue disease. In addition, with the cavitary
lesions on chest x-ray and no history of malignancy, the patient’s
constitutional symptoms were wrongly attributed to either connective
tissue disease or infectious causes. Also, with the patient’s severe pulmo-
nary hypertension and rapidly deteriorating course, invasive procedures
such as lung biopsy and bronchoscopy were deemed too risky to perform.
In retrospect, both the constitutional symptoms and abnormal lung DISCUSSIONS: PCH involves capillary proliferation within: 1) alve-
findings on radiography were all attributable to the patient’s underlying olar septa, 2) bronchial and venous walls, 3) pleura, and sometimes 4)
pancreatic malignancy. If tumor cell embolism had been suspected, a regional lymph nodes [1]. Both clinical presentation and histologic
pulmonary capillary wedge aspirate could have been used for diagnostic features have similarities with PVOD. Patients usually present with
purposes.. progressive dyspnea, often with pleuritic chest pain and hemoptysis. Chest
CONCLUSION: Although rare, tumor cell emboli should be included radiographs usually show a diffuse, bilateral, reticulonodular interstitial
in the differential diagnosis of cryptic pulmonary hypertension. pattern. Examination of vasculature often reveals enlarged pulmonary
DISCLOSURE: Alexis Meredith, None. arteries on chest radiograph. Histologically, PCH manifests as capillary
proliferation in the areas noted above. There are many similar histologic
features between PCH and PVOD, with two distinct difference being
capillary growth into the bronchial and venous walls (occurring in PCH
PULMONARY CAPILLARY HEMANGIOMATOSIS: A CONSID- but not PVOD) and occlusion of the pulmonary vein lumen occurring
ERATION IN UNEXPLAINED PULMONARY HYPERTENSION more often in PVOD. Although potential therapies include medications
D. L. Bedsole MD* Katrin Klemm MD George L. Zorn MD Keith M. such as doxycycline [2] and interferon alpha-2a [3] which disrupt capillary
Wille MD University of Alabama at Birmingham, Birmingham, AL growth, only bilateral lung transplant currently offers potential long-term
survival. Prostacyclin therapy is considered contra-indicated secondary to
INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a a reported risk of pulmonary edema [4]. Interestingly, a hereditary form
rare condition which causes severe pulmonary hypertensive disease. of PCH has been reported [5].
There are overlapping features with other conditions, particularly pulmo- CONCLUSION: PCH is a rare disorder causing significant pulmonary
nary veno-occlusive disease (PVOD), adding to the difficulty with early hypertension. It both clinically and histologically resembles PVOD, and
suspicion and diagnosis of this disorder. Our case illustrates the value of clinical suspicion along with close histologic examination is required for
including PCH in the differential diagnosis of unexplained pulmonary differentiation. Consideration of this diagnosis is vital when evaluating a
hypertension. patient with pulmonary hypertension, particularly when investigation of
CASE PRESENTATION: KB is a 35 year old man referred to UAB another etiology such as PVOD yields conflicting findings as it did with
for lung transplant evaluation. 18 months prior to referral this previously KB. Diagnosing PCH allows for early evaluation for bilateral lung
healthy man was admitted to a local hospital with acute dyspnea. Chest transplantation, avoidance of prostacyclin therapy, and potential family
radiograph revealed a diffuse, bilateral reticulonodular pattern. Supple- genetic counseling.
mental oxygen, antibiotics, and nebulizer therapy were begun. With only REFERENCES:
minimal improvement, evaluation continued including a 2-D echocardio- 1 Yi ES: Tumors of the pulmonary vasculature. Cardiol Clin 22 (2004)
gram revealing pulmonary artery systolic pressures above 90 mm Hg. 431-440.
Open lung biopsy demonstrated “microemboli consistent with veno- 2 Ginns LC, Roberts DH, Mark EJ, et al: Pulmonary capillary
occlusive disease.” After learning this information, KB recalled that his hemangiomatosis with atypical endotheliomatosis. Successful anti-
father and two maternal aunts had pulmonary hypertension. A thorough angiogenic therapy with Doxycycline. Chest 124(5): 2017-22, 2003.
search for a source of emboli was unsuccessful. KB was treated with 3 White CW, Sondheimer HM, Crouch EC, et al: Treatment of
warfarin, inhaled beta-agonists, inhaled steroids, and epoprostenol ther- pulmonary hemangiomatosis with recombinant interferon Alpha-2a.
apy prior to referral. After transfer and lung transplant procedure, surgical N Engl J Med 320: 1197-1200, 1989.
pathology reported impressive capillary proliferation in the pulmonary 4 Humbert M, Maitre S, Capron F, et al: Pulmonary edema compli-
interstitium of the ex-plant. The proliferation impinged on small airways cating continuous intravenous prostacyclin in pulmonary capillary
and blood vessels, consistent with the diagnosis of PCH. hemangiomatosis. Am J Respir Crit Care Med 1998; 157:1681-5.

continued
5 Langleben D, Heneghan JM, Batten AP, et al: Familial capillary
hemangiomatosis resulting in primary pulmonary hypertension. Ann
Inter Med 1988; 109:106-9.
DISCLOSURE: D. Bedsole, None.
A CASE OF VIOLENT NON-REM PARASOMNIAS THAT RE-

SOLVED WITH TREATMENT OF OBSTRUCTIVE SLEEP AP-
NEA
Omar Lateef DO* James Wyatt PhD Rosalind Cartwright PhD Rush
University Medical Center, Chicago, IL
INTRODUCTION: We present a case of a woman suffering from

violent recurrent non-REM parasomnias with co-morbid obstructive sleep
apnea that responded to treatment with nocturnal non invasive mechan-
ical ventilation.
CASE PRESENTATION: A 54 yr female with a history of seizure
disorder, systemic hypertension and “blackouts” was referred to our sleep
center. Her seizure disorder had been well controlled on valproic acid
with no signs of seizure activity by EEG evaluations. Her blood pressure DISCLOSURE: Omar Lateef, None.
had been closely monitored and was well controlled on atenolol and
enalapril. She had no history of childhood parasomnias. Her nighttime
symptoms had started 5 years prior to her presentation. She had been
taking a nap in the car while her daughter went to the grocery store. She
awoke to find herself driving her car into the middle of a busy intersection
3 miles away. She promptly returned to the store to meet her daughter.
Approximately 5 times a month over the next five years the patient
Unusual Presentations of Cardiovascular
experienced similar episodes while “sleeping”. Several times she would Disease
awaken to horn sounds while driving her car, often miles away from her 4:15 PM - 5:45 PM
house. Other times she awoke to find herself barefoot in the snow in a
neighbor’s backyard and at a convenient store 3 blocks from her home
BIVENTRICULAR FAILURE POST TAMPONADE DRAINAGE
in her pajamas. Once she was found by police wandering in a IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS
neighboring town. Although she was apparently initially confused, she AND PULMONARY HYPERTENSION
awoke while they were driving her to the station and was able to tell Michael Liou MD Rana L. Adawi MD* Mark Rosen MD Jenny Diep MD
them where she lived. The patient was coerced by her daughter to see Beth Israel Medical Center, New York, NY
medical attention after her most recent disturbing incident. She
describes herself falling asleep around 10 PM while watching a horror INTRODUCTION: Systemic lupus erythematosus (SLE) involves the
movie. She awoke extremely fatigued around 6 AM. She found her heart or lungs in greater than 50% of cases. We describe a case of
hands covered in dried blood. She was then horrified to find blood worsening biventricular dysfunction after drainage of a large pericardial
stains on a cutting board in the kitchen and her cat’s remains next to effusion in a twenty-two year old female with SLE and secondary
the trash can. A detailed sleep history revealed non-restorative night- pulmonary hypertension.
time sleep and excessive daytime sleepiness. She was noted be a loud CASE PRESENTATION: A 22 year-old African-American female
snorer. She had been experiencing increased frequency of daytime with SLE presented with a three-day history of fever to 39° C, chills, left
dozing over the past several years which correlated with a period of thigh swelling and erythema. SLE had been diagnosed two years previ-
increased weight gain. Split-night polysomnography revealed severe ously, when she presented with symptoms consistent with Raynaud’s
OSA with marked oxygen desaturations (Figure 1). She received CPAP phenomenon, at which time she was begun on prednisone and hydroxy-
chloroquine. The latter had recently been discontinued secondary to
titration, which significantly reduced the number apneas and hypop-
ocular complications. Pulmonary hypertension with a mild pericardial
neas. She noted that this was her first restful night of sleep in years. In
CASE REPORTS
effusion was identified on transthoracic echocardiogram (TEE) six months
the four months after starting treatment for OSA she has not suffered prior to admission. The physical examination revealed a cachetic, ill-
any further parasomnias. appearing female. The pulse was 118/min, the blood pressure 120/75, and
DISCUSSIONS: Parasomnias secondary to OSA have been described the temperature 39.4°C. She had distended neck veins and a right
in the literature (1). Increased parasomnias following treatment with ventricular heave, but no pulsus paradoxus. There was a 10 cm area of
CPAP in previously untreated patients have also been well described (2). non-blanching macular erythema on the left anterior thigh. Extremities
No violent parasomnias have been linked to OSA. To our knowledge, this had symmetric, but weak pulses and fingertips were cyanotic. The
is the first case report of a violent parasomnia that responded to effective electrocardiogram demonstrated sinus tachycardia at 126 beats/min with a
treatment for OSA. rightward axis deviation, right atrial enlargement, and right ventricular
CONCLUSION: This case report suggests that violent non-REM hypertrophy. The chest radiograph showed an enlarged, globular heart
parasomnias may be linked to untreated OSA and that effective OSA with no evidence of pulmonary edema. She was initially managed with
control may improve symptoms of the parasominias. Sleep deprivation has intravenous steroids and broad-spectrum antibiotics for cellulitis versus
been shown to be an effective tool for inducing somnambulistic episodes myositis. A TTE revealed severely reduced left ventricular function with
in the laboratory, thereby facilitating the diagnosis of sleepwalking in an estimated ejection fraction of 20%, a dilated right ventricle with
predisposed individuals (3). We theorize the contrary is true, that by reduced ejection fraction, severe tricuspid regurgitation with an estimated
pulmonary artery systolic pressure (PASP) of 60 mm Hg, a dilated right
decreasing sleep deprivation by decreasing sleep fragmentation using
atrium, and moderate pericardial effusion without evidence of tampon-
CPAP, a person is less likely to have parasomnias. ade. There was septal motion abnormality consistent with RV volume and
REFERENCES: pressure overload. On hospital day three, she became acutely dyspneic
1 Pressman M R et al. Night Terrors in an Adult Precipitated by Sleep and blood pressure fell to 54/30 mmHg. A repeat TTE showed marked
Apnea. Sleep 18(9):773-7752. respiratory variation in mitral inflow consistent with cardiac tamponade.
2 Millman R P, Kipp GJ, Carskadon M.A. Sleepwalking precipitated She was taken to the operating room for emergency pericardial drainage
by treatment of sleep apnea with nasal CPAP. Chest 1991; 99 and biopsy via the subxyphoid approach. Five hundred milliliters of
3 Joncas S et al. The value of sleep deprivation as a diagnostic tool in serosanguinous fluid was removed and a pericardial biopsy was obtained.
adult sleepwalkers. Neurology. 2002 Mar 26;58(6):936-40. Despite this intervention, she became more hypotensive, requiring endo-

Disease, continued
tracheal intubation and high doses of norepinephrine, epinephrine, and ities. The exact cause of this apical balloning is not known but multivessel
dobutamine. Despite drainage of the pericardial effusion, a transesopha- coronary artery spasm, catecholamine induced cardiomyopathy, adrenocep-
geal echocardiogram showed no improvement of right or left ventricular tor-hyperactive cardiomyopathy and neurogenically mediated myocardial
function, RV dilated, small LA and LV, and LV appeared hypocontractile. stunning have all been proposed as a possible mechanism. The overall
Thermodilution cardiac index was 1.18 L/min/m2, the CVP was 25 mm prognosis is good with a low mortality and a low risk of recurrence. There has
Hg, and the PAOP 35 mm Hg, and the PASP 90 mm Hg. Once the patient been a case report of cardiac rupture and sudden cardiac death. Other
was stabilized, she was transferred to another facility for prostacyclin potential complications include left heart failure, ventricular arrhythmia,
infusion and inhaled nitric oxide. As a Jehovah’s Witness, she was unable mural thrombus formation, mitral regurgitation, and dynamic intraventricular
to undergo ventricular-assist device placement. She expired with refrac- obstruction. Treatment is mainly supportive with beta blockers and angioten-
tory shock. sin converting enzyme inhibitors. Anticoagulation is often used in the setting
DISCUSSIONS: The accumulation of fluid in the pericardium in an of significant left ventricular systolic dysfunction.
amount sufficient to cause severe obstruction to blood inflow to the CONCLUSION: Takotsubo is a rare syndrome with a relative lack of
ventricles results in cardiac tamponade. In most cases, removal of publised data and dignostic criteria. It should be considered in the
pericardial fluid significantly improves cardiac output and thus can be differential diagnosis of acute myocardial infarction. Additional informa-
lifesaving. The current case report describes a patient with pericardial tion is needed to determine the precise pathophysiology of this disease.
tamponade who underwent surgical decompression only to develop REFERENCES:
rapid hemodynamic instability, progressive heart failure and eventually 1 Bybee KA, et al. Systematic Review: Transient LV Apical Balloon-
expire. This paradoxical response to pericardial decompression may be ing; a syndrome that mimics ST-segment elevation myocardial
related to a phenomenon called “low output syndrome,” in which the infarction. Ann Intern Med.2004;141:858-865.
sudden removal of pericardial effusion results in acute ventricular 2 Akashi YJ, Sakakibara M, Miyake F. Reversible LV dysfunction“ta-
dilatation and failure. Unlike most previous case reports, this patient kotsubo”cardiomyopathy associated with pneumothorax;Y J Akashi-
had biventricular dysfunction before cardiac tamponade developed. .Heart 2002;87(2):E1.
CONCLUSION: It is particularly important to be aware of the 3 Akashi YJ, et al. The clinical features of takotsubo cardiomypathy.
possibility of biventricular failure postpericardiocentesis in the setting of QJM. 2003;96(8):563–73.
severe pulmonary hypertension.
DISCLOSURE: Rana Adawi, None.
TAKOTSUBO CARDIOMYOPATHY: TRANSIENT LEFT VEN-

TRICULAR APICAL BALLOONING, A SYNDROME MIMICK-
ING ST-ELEVATION MYOCARDIAL INFARCTION
Nikhat Salamat MBBS* Victor J. Test MD Scott E. Young DO Scott &
White Hospital, Temple, TX
INTRODUCTION: Takotsubo cardiomyopathy is a unique acute cardiac

syndrome characterized by typical chest symptoms,elevated ST segment on
electrocardiograms,elevated cardiac markers and is usually misdiagnosed as
acute myocardial infarction. Coronary angiography usually reveals no evi-
dence of obstructive atherosclerotic coronary disease. Left ventriculography
reveals a peculiar regional systolic dysfunction with hyperkinesis of the basal
ventricular segment and akinesis of the mid-ventricle and apex.The syndrome
was initially recognized in Japanese patients and has been named so after the
fishing pot with a round bottom and narrow neck that is used for trapping
octopus in Japan (octopus is “tako,” and pot is “tsubo” in Japanese).We report
a case of this rare syndrome in our critical care setting and discuss the the
pathophysiology involved .
CASE PRESENTATION: Patient is a 67 year old female with history of
end stage renal disease on hemodialysis who was admitted to intensive care
unit with 1 day complaint of fever(102’F),altered mental status and hypoxic
respiratory failure for which she required intubation. Her examination was
unremarkable except for fever and crackles on right side of chest and
confusion. Laboratory demonstrated a white blood cell count of 16,000 The
serum cardiac markers and electrocardiography were unremarkable EKG.
Chest radiograph demonstrated a right lower lobe infiltrate and fluid
overload. Blood cultures subsequently grew Streptococcus Bovis and the
urinary streptococcal antigen was positive. She was successfully extubated but
then she developed sinus tachycardia with rate of 122 beats/min and
electrocardiography demosntrated 3mm of elevation in Leads V1 to V5 and 1
and avL with an acute anteroseptal and lateral injury pattern. The troponin I
increased to 64 ,CK 763 , MB 54 and index of 7.1.Bedside echocardiogram DISCLOSURE: Nikhat Salamat, None.
showed akinesis of the mid distal anteroseptum and apex.Because of the
concern of an acute coronary event , she was taken to the cardiac catheriza-
tion laboratory. Coronary angiogram showed normal coronary arteries and a
A VIEW THROUGH THE HANGMAN’S NOOSE: VICHOW’S
left ventriculogram revealed anteroseptal akinesis and basal hyperkinesis. The
TRIAD REVISITED
patient was treated with beta blockers and ACE inhibitors and remained
Avelino Verceles MD Justin Sebastian MD* Siva Ramachandran MD
stable without any further complications.
Drexel University College of Medicine, Philadelphia, PA
DISCUSSIONS: Reversible left ventricular asynergy,known as “Takot-
subo” cardiomyopathy is rare and most often affects postmenopausal women
with a mean age between 62 to 75 years of age and mimics an acute coronary
syndrome in presentation despite the absence of obstructive coronary disease. INTRODUCTION: Pulmonary venous thromboembolism (PVTE) is
An episode of emotional or physiologic stress frequently has been reported commonly encountered in hospitalized patients. Risk increases follow-
prior to the presentation. We believe that pneumonia and streptococcal ing trauma, surgery, immobilization and malignancy. Most often, the
bacteremia was the precipitating event in our patient. It has been described origins of the emboli are from the deep veins of the lower extremities.
in patients with pneumothorax,alcohol withdrawl and head trauma,intracra- We present an unusual case of recurrent PVTE originating from the
nial or subarachnoid bleed ,pheochromocytoma,myocarditis ,hpertrophic Right Internal Jugular Vein (RIJV) in a patient following self-strangu-
cardiomyopathy can also present with similar regional wall motion abnormal- lation.

Disease, continued
CASE PRESENTATION: A 21 year-old male with no prior medical
history presented to the emergency department following a suicide
attempt by self-strangulation. The patient was found hanging from his
neck, apneic, obtunded, hypotensive and bradycardic. Despite success-
ful resuscitation efforts, the patient developed ARDS requiring me-
chanical ventilation. Physical examination revealed coarse breath
sounds and a distinctive circumferential excoriation on his neck. On
the 5th day the patient demonstrated improved neurological function
and good weaning parameters. A trial of extubation resulted in hypoxia
and reintubation. A chest radiograph revealed only resolving bilateral
infiltrates. A CT pulmonary angiogram revealed bilateral subsegmental
PVTEs. Simultaneously preformed doppler ultrasounds of the lower
and upper extremities were normal. Intravenous anticoagulation was
started and maintained at therapeutic levels. Three days later appro-
priate spontaneous breathing parameters prompted extubation. Within
24 hours of extubation the patient once again became hypoxic. On this
occasion, hypoxemia was further complicated by hypotension. An arterial
DISCLOSURE: Justin Sebastian, None.
blood gas demonstrated respiratory alkalosis and an Arterial-alveolar oxygen
gradient of 426 while breathing 100% oxygen. Following reintubation and
fluid resuscitation, a second CT pulmonary angiogram was performed. New,
bilateral central and segmental PVTE were demonstrated (Figure 1). Once
more, doppler ultrasonography of the extremities remained normal. Interest- YOLK SAC CARCINOMA OF MEDIASTINUM CAUSING RIGHT
ingly, doppler ultrasonography of the neck discovered the presence of a near VENTRICULAR OBSTRUCTIVE CARDIOMYOPATHY
occlusive thrombus of the RIJV. Echocardiogram demonstrated mild right Yu Ya Huang MD* Brody School of Medicine, Greenville, NC
ventricular dilatation with an estimated right ventricular systolic pressure of
60mm of Hg. Apart from strangulation injury to the neck, the RIJV remained INTRODUCTION: Extragonadal germ cell tumor is a rare tumor and
naive to central lines and intravenous canulations. The combination of high usually characterized by their location on the midline from pineal gland to
oxygen requirements, hemodynamic instability and extensive clot burden coccyx. Yolk sac carcinoma is a very virulent carcinoma with early
prompted thrombolysis with alteplase. A post-thrombolysis doppler ultra- hematogenous dissemination potential and high affinity for liver metasta-
sound study of the patient’s neck revealed persistent clot in the RIJV (Figure sis. Treatment modalities include radiation, chemotherapy, and surgery
2). In an attempt to prevent further thromboembolic events the RIJV was for residual masses. Obstructive cardiomyopathy is a well described left
surgically ligated. Two days after the RIJV ligation the patient was successfully ventricular entity causing significant reduction in cardiac output.
extubated after a 30 minute T-piece trial. In the days following the patient’s CASE PRESENTATION: Patient is 19 years old white male with out
oxygen requirement was brought down to room air. any significant past medical history presented with complaint of 2-3 weeks
history of left chest pain with worsening short of breath and fever. On
physical examination, pt was in moderate respiratory distress with increase
work of breathing. Clinical findings demonstrated reduced air entry to the
left hemi-thorax. Patient’s clinical status deteriorated required intubation
for air way protection. Hypotension also occur requiring large volume of
fluid to maintain blood pressure. Computerized Tomography of chest
showed a large mediastinal mass @ 15x11 cm extending across Left
hemi-thorax as well as a hepatic mass measuring @ 18x 11 cm. Tran
esophageal echocardiography show enlarge right ventricle with extra-
cardiac mass compressing on right ventricular outflow tract creating
outflow tract obstruction. Fine needle aspiration of liver mass revealed a
yolk sac tumor.Clinical course was complicated via acute respiratory
failure secondary to the mass effect on the left lung. Repeated hypotensive
collapse secondary to right ventricular outflow tract obstruction. Patient
received 4 cycle of chemotherapy and radiation therapy with substantial
CASE REPORTS
clinical response. PET scan shows persistence activity of hepatic mass w/o
any activity of mass of left hemi-thorax.
DISCUSSIONS: This patients mediastinal lesion compressing the
DISCUSSIONS: In 1856 Rodolf Virchow described a triad of predis- right ventricular outflow tract causing right ventricular failure in a manner
posing factors necessary for the formation of thrombus – abnormal flow, which clinically seem analogous to left ventricular outflow tract obstruc-
vessel injury, and hypercoagulability. We report a unique case of PVTE tion. This patient required intensive and prolonged respiratory and
originating form the RIJV following trauma to the neck. It is postulated hemodynamic support with emergent radiation and chemotherapy for
that the injury sustained by strangulation predisposed our patient to relief of right ventricular and left lung compression. Yolk sac carcinoma
endothelial injury in the RIJV, creating a nidus for persistent thromboses s/p chemotherapy with hepatic metastasis placed patient at high risk for
and recurrent PVTE. In addition, the strangulation effect caused by the thromboembolic complication. Given his low pulmonary reserved and
ligatures around our patient’s neck likely prevented venous return for an high propensity for pulmonary embolic events, patients also received
unknown amount of time, thus causing blood stasis. The injury that superior vena cava and inferior vena cava filter placed with long term
occurred before the patient was hospitalized predisposed him to eventual anticoagulation. Surgical removal of residual tumor with salvage chemo-
thrombosis, with ensuing recurrent PVTE. Our patient did not have a therapy is required in nonseminomas germ cell tumor secondary to high
known genetic hypercoagulable state or malignancy, according to our propensity for recurrence and malignant transformation.
work up. This is the only known reported case of PVTE originating from CONCLUSION: Mass effect secondary to mediastinal yolk sac carci-
a RIJV thrombus as a result of self-strangulation. Anticoagulation likely noma with right ventricular outflow tract obstruction requiring prolonged
plays a role in the therapy of these patients, however, there is no literature and aggressive fluid and vasopressor support, whilst awaiting outcome of
to support the use of upper extremity, or superior vena cava filters to cytotoxic therapies.
prevent clot propagation. REFERENCE:
CONCLUSION: Embolic events originating from the upper extremity 1 American College of Chest Physicians, Pulmonary Board Review
and neck veins are phenomena that must always be considered in patients 2003Cancer6 Medicine via Holland. FreiUptoDate online 12.3
with PVTE and injury to the neck. DISCLOSURE: Yu Ya Huang, None.

Disease, continued
PLATYPNEA ORTHODEOXIA SYNDROME IN AN 85 YEAR OLD CARDIAC TAMPONADE AS A RARE PRIMARY PRESENTA-
PATIENT WITH PATENT FORAMEN OVALE AND SEVERE TION OF SARCOIDOSIS
TRICUSPID REGURGITATION Dmitry Lvovsky MD* Eusebio Desuyo MD Ashok Fulambarker MD
Maja Zaric MD* Daniel Soffer MD Pragnesh Gadhvi MD Neil L. Coplan Joseph Rosman MD Rosalind Franklin University of Medicine and
MD Lenox Hill Heart and Vascular Institute of New York, New York, NY Science, Chicago Medical School, North Chicago, IL
INTRODUCTION: Platypnea orthodeoxia syndrome is a rare disorder INTRODUCTION: Sarcoidosis is a multisystem granulomatous disease
which includes dyspnea associated with standing up and concomitant of unknown etiology. Symptomatic cardiac involvement is present in up to 5%
hypoxia. The following is a case report of platypnea orthodeoxia in an of patients, mostly as heart blocks.1 Massive pericardial effusion leading to
elderly patient with patent foramen ovale (PFO) and severe tricuspid cardiac tamponade is extremely rare. Only 2 cases of cardiac tamponade as a
regurgitation (TR), which was successfully treated with percutaneous presenting symptom of sarcoidosis were reported in the literature.2,3.
closure of the PFO with a Cardioseal device . CASE PRESENTATION: A 32 year old African American obese male
CASE PRESENTATION: J.N. is an 85 years old white male with medical presented to the hospital with insidious onset of dyspnea over 6 week period.
history significant for hypertension and chronic atrial fibrillation. He was active He denied fever, chills, cough, hemoptysis, weight loss. Past medical, social
and fully functional when he presented to the ER complaining of weakness and and family history was non-contributory. There was no tuberculosis exposure.
exertional dyspnea, which had worsened over the prior 3 months. At the time of His PPD was negative. Physical examination revealed a well-developed man
presentation, he had dyspnea with minimal effort which was promptly relieved by in mild respiratory distress with blood pressure of 105/60. He was afebrile
resting in bed. The patient denied chest pain, orthopnea or palpitations and with respiratory rate of 24 breaths per minute. Jugular venous distention,
reported no fever or cough. The blood pressure in the ER was 138/87 mm Hg distant heart sounds and pulsus paradoxus were present. EKG showed
and the oxygen saturation on nasal oxygen (4 L) was 98%. The pulse was electrical alternans. CBC and chemistry were normal. Chest X-ray revealed
irregularly irregular, and cardiac examination was notable for a widely fixed and massive cardiomegaly with mediastinal adenopathy (Figure 1). Transthoracic
split second heart sound with 2/6 holosystolic murmur along the left sternal echocardiogram was positive for massive pericardial effusion with right
border. Breath sounds were distant but clear. Mild acral cyanosis was present ventricular and left atrial collapse in diastole. Pericardiocentesis drained 1500
while lying down, and this significantly worsened while standing up. Chest cc of bloody exudative fluid. CT-guided pericardial biopsy was negative for
roentgenogram on admission showed cardiomegaly. The electrocardiogram AFB and malignancy. Biopsy of mediastinal lymph nodes by anterior
revealed atrial fibrillation and right bundle branch block. Routine blood work mediastinoscopy showed granulomatous disease and ACE level was 63.
revealed mild microcytic anemia and mild renal insufficiency with serum Extensive work up with AFB cultures and multiple viral, fungal and HIV
creatinine of 1.2 mg/dl. Other blood tests including liver function tests and serologies remained negative. Patient remained asymptomatic over next 14
coagulation panel were within normal limits. Room air pulse-oximetry performed months without any treatment. He then presented with a month long gradual
while supine, standing up and walking 10 steps showed saturation trend of 93%, onset of dyspnea, cough and blood-tinged sputum. He denied fever, chills or
88% and 78%, respectively. Echocardiogram showed normal left ventricular weight loss. He was afebrile with normal physical exam. Chest X-ray and CT
function, severe right atrial (RA) and right ventricular (RV) dilatation and severe scan showed extensive right lung infiltrate with enlarged mediastinal lymph
tricuspid insufficiency with pulmonary artery (PA) systolic pressure of 40 mm Hg. nodes (Figure 2). ACE level was 101. Bronchoscopic transbronchial biopsy
Trans-thoracic echocardiogram done in supine and standing position with revealed multiple non-caseating granulomas. Patient was started on Pred-
contrast using agitated saline (‘bubble injection‘) showed minimal shunting while nisone 80 mg daily with complete resolution of symptoms and radiological
supine, and significant worsening of right to left heart shunting while upright findings as well as a drop of ACE level to 29 after 2 months.
(Figure 1). Right heart catheterization revealed: mean RA pressure 5 mmHg, RV
pressure 39/4 mmHg, PA pressure 39/12 mmHg and wedge pressure of 10
mmHg. There was no step-up in oxygen saturation. Intervention was done
percutaneously under general anesthesia. Closure of the PFO was successfully
performed using 33mm Cardioseal device. Final trans-esophageal echocardio-
gram showed no evidence of shunting. In contrast to presentation, post-
procedural oxygen saturation remained 98% both in supine and standing
positions. Patient noted significant symptom relief and improvement in functional
status.
DISCUSSIONS: Platypnea orthodeoxia is an unusual syndrome which
has most commonly been described post-pneumonectomy and in patients
with atrial septal defect / PFO and elevated right heart pressures. There
have been very few cases describing patients with platypnea orthodeoxia
in the setting of PFO and normal right heart pressures. Platypnea
orthodeoxia is postulated to occur because of stretching of the inter-atrial
septum in the upright position, resulting in relative increase in defect size.
The decreased RV compliance associated with advancing age might
explain increase in prevalence of this syndrome in the elderly. Symptoms
in our patient were thought to occur due to severe TR and presence of
uncoiled aorta which might have caused anatomical distortion causing the
TR jet to be directed towards the PFO, resulting in significant right to left
heart shunting with cyanosis when standing up.
CONCLUSION: Trans-catheter closure in this case was the preferred
treatment, given that a surgical approach potentially carried higher
peri-procedural risk. After the procedure, patient‘s symptoms promptly
resolved and there were no residual signs of oxygen desaturation associ-
ated with standing upright.
DISCUSSIONS: The usual clinical features of cardiac sarcoidosis, in

order of decreasing frequency, are complete heart block, ventricular
DISCLOSURE: Maja Zaric, None. irritability, congestive heart failure, sudden death, first-degree heart block

Wednesday, November 2, 2005
Unusual Presentations of Cardiovascular Airway II
Disease, continued 2:00 PM - 3:30 PM
or bundle branch block, supraventricular arrhythmias, mitral valve dys- PERSISTENT HOARSENESS OF VOICE IN A SARCOID PA-
function and pericarditis.1 Prevalence of asymptomatic small pericardial TIENT: A CLUE TO LARYNGEAL SARCOIDOSIS; A POTEN-
effusion in diagnosed cases of sarcoidosis is 19% to 21% detected by TIAL CAUSE OF UPPER AIRWAY OBSTRUCTION
echocardiography.4 Sarcoidosis involves the inflammation of both visceral Vijay A. Rupanagudi MD* Kartikeyan Kanagarajan MD Suriya Jayawar-
and parietal pericardium, resulting in gross thickening of the pericardium dena MD Muhammad U. Rehman MD Rashmikant Doshi MD Padma-
and accumulation of pericardial fluid.1 Pathologically, tiny nodules may be nabhan Krishnan MD Coney Island Hospital, Brooklyn, NY
seen in a patchy distribution which may explain the presence of a negative
pericardial biopsy in our patient.Massive effusions are rare, with only INTRODUCTION: Sarcoidosis is a multi-system disorder with lung as
the prime target organ. Laryngeal involvement is seen in only 0.5% to
about 10 cases found in the literature.1-3 Effusion may be secondary to
8.3% of patients and if unrecognized and untreated may progress to upper
systemic or pericardial disease, cor pulmonale, pericardial granuloma or airway obstruction. Hoarseness is the usual early symptom. We describe a
left ventricular dysfunction.2 The previous documentation of pulmonary patient with sarcoidosis of 13 years duration, who complained of hoarse-
involvement helps to establish the diagnosis. Steroids have been the ness for 2 years and was found to have laryngeal sarcoidosis with upper
mainstay of medical treatment of sarcoidosis, including pericardial effu- airway obstruction.
sions, but it is difficult to assess its role in treating pericardial effusions. CASE PRESENTATION: A 41-year-old non smoking Hispanic female
Most patients with cardiac tamponade due to sarcoidosis were placed on complained of hoarseness of voice for 2 years and was treated for allergic
steroids but the control of their pericardial effusion required surgical rhinitis for 9 months with no benefit. She also received multiple courses of
intervention.1-3. antibiotics for presumed upper respiratory tract infection and received high
CONCLUSION: Cardiac tamponade is a rare manifestation of sarcoid- dose proton pump inhibitors for 12 months with no effect on hoarseness.
osis. Our case stands out since it is the third documented case in literature Sarcoidosis was diagnosed 13 years ago when chest radiograph showed
of cardiac tamponade as the presenting feature of sarcoidosis. bilateral hilar lymphadenopathy and transbronchial biopsy revealed non-
REFERENCES: caseating granuloma. Her disease was characterized by hypercalcemia,
1 Israel RH, et al. Massive pericardial effusion in sarcoidosis. Respi- hypercalciuria with multiple renal calculi, high angiotensin converting en-
ration. 1994; 61(3):176-80. zyme levels and generalized lymphadenopathy (with non-caseating granu-
2 Rubinstein I, et al. Cardiac tamponade as the presenting symptom loma on lymph node biopsy). 8 years ago, she was treated with steroids for one
year for hypercalcemia and renal calculi. Spirometry and flow volume loops 5
of sarcoidosis. Am Heart J. 1985; 109(6):1387-8.
years ago were normal.Examination did not reveal stridor or generalized
3 Bai LY, et al. Sarcoidosis presenting as copious, bloody pericardial lymphadenopathy. Spirometry was normal. Flow volume loop showed de-
effusion. Am J Med. 2001; 111(6):507-8. crease in peak inspiratory and expiratory flow rates and plateauing of both
4 Angomachalelis N, et al. Pericardial effusion concomitant with expiratory and inspiratory loops suggestive of fixed upper airway obstruction.
specific heart muscle disease in systemic sarcoidosis. Postgrad Med Rigid laryngoscopy under general anesthesia revealed narrowed glottic area
J. 1994; 70 Suppl 1:S8-12. and vocal cord rigidity suggestive of an infiltrative process. Biopsies from
glottic area, anterior commisure and subglottic area showed granulomatous
inflammation consistent with sarcoidosis. AFB was smear negative and
bacterial, mycobacterial and fungal cultures were all negative. A diagnosis of
laryngeal sarcoidosis with upper airway obstruction was made. 3 months of
oral steroids treatment, resulted in marked improvement in hoarseness and
flow volume loops. See figures.
DISCUSSIONS: Laryngeal involvement has been described in 0.5 to
8.3% of patients with sarcoidosis. It is usually associated with multi-system
involvement and only rarely presents as isolated laryngeal disease. Supra-
glottic and subglottic areas are usually affected and primary vocal cord
involvement is rare. The most common presenting features are hoarseness
and stridor.Less common symptoms include dysphagia and cough.Laryn-
goscopy findings include mucosal alterations with erythema and edema,
punctate nodules, and mass lesions. The epiglottis is the most frequently
affected area, but any portion of the larynx may be involved. Vocal cord
dysfunction by direct involvement or Vocal cord paralysis by involvement
of the neural pathways, including the nucleus ambiguous, the 10th cranial
nerves, and the superior and recurrent laryngeal nerves have also been
described. The diagnosis is established by demonstrating granulomatous
CASE REPORTS
inflammation on laryngeal biopsy and excluding other causes of granulo-
matous laryngitis.Treatments used for laryngeal sarcoidosis have included
systemic steroids, intralesional steroids, laser resection, surgical excision,
low-dose radiation and tracheotomy. Improvement with use of inhaled
steroids and Clofazimine (an anti-leprosy agent) has been reported.
CONCLUSION: In patients with sarcoidosis, persistent hoarseness

should prompt an evaluation for laryngeal sarcoidosis by laryngoscopy.
Early detection and treatment may prevent development of upper airway
DISCLOSURE: Dmitry Lvovsky, None. obstruction.

Airway II, continued
REFERENCES: Samter’s syndrome.Clin Chest Med. 1988;9(4):567-76.

1 Gallivan GJ, Landis JN. Sarcoidosis of larynx: preserving and DISCLOSURE: Daniel Dube, None.
restoring airway and professional voice. J Voice 1993;7:81-94,
2 Neel HB III, McDonald TJ. Laryngeal Sarcoidosis. Report of 13
patients. Ann Otol Rhinol Laryngol 1982;91:359-62
HOW COMPLICATED ONE’S COUGH CAN BE!
3 Bower JS, Belen JE, Weg JG, Dantzker DR. Manifestations and
Ioana G. Amzuta MD* Tajender S. Vasu MD Robert Lenox MD SUNY
treatment of laryngeal sarcoidosis. Am Rev Respir Dis. 1980;122(2):
Upstate Medical University, Syracuse, NY
325–332.
DISCLOSURE: Vijay Rupanagudi, None.
INTRODUCTION: Cough is a complex physiologic response that
protects tracheobronchial airway from unwanted secretions and foreign
materials. In this report, we describe a patient with dural tear and CSF
MEGA-POLYP AS AN ETIOLOGY OF ACUTE UNILATERAL leak as a result of coughing.
PROPTOSIS IN A PATIENT WITH SAMTER’S SYNDROME CASE PRESENTATION: A 19-year-old female with a history of cystic
Daniel S. Dube MD* Priscilla Sarinas MD The Department of Veteran fibrosis came in the ER (Emergency Room) of our hospital complaining of
Affairs and Palo Alto Health Care System and Stanford, Palo Alto, CA severe bifrontal headache. She was having positional headache for the last
three weeks. Her Review of Systems was unremarkable except for frequent
INTRODUCTION: Samter’s syndrome is a clinical designation of the bouts of coughing. Examination of central nervous system was unremarkable
triad of bronchial asthma, nasal polyposis and allergic intolerance to except for neck stiffness. Examination of eyes including intraoccular pressure
salicylate derivatives. Persistent inflammatory rhino-sinusitis is regular and fundoscopy were unremarkable. CT scanning of her head did not reveal
feature of this syndrome and leads to mucosal engorgement and nasal any abnormality. Examination of cerebrospinal fluid (CSF) including cultures
obstruction. Polyps are frequently restricted to the sinus spaces. The was unremarkable. She was discharged but after two weeks came to ER with
occurrence of large polyps leading to mechanical displacement of the orbit persisting headache. MRI (magnetic resonance imaging) of her brain showed
has not been reported previously. a 4 mm subdural fluid collection overlying the right cerebral convexity.
CASE PRESENTATION: A 51-year old male with a life long history of Considering the possibility of CSF leak, MRI of her thoracic spine was done
nasal polyps, asthma and salicylate intolerance presented to the emergency that showed fluid collection posterior to thecal sac within the spinal cord with
room with a several day history of diplopia, rhino-sinusitis and frontal signal characteristic consistent with CSF fluid on T2 weighted imaging. Later
headache. There was no fever, chills, or nocturnal diaphoresis. Until the on Computed Tomography (CT) myelogram (Fig. 1) confirmed CSF leak at
current presentation, he denied any headaches, visual disturbance or neuro- the level of L1 and L2. A diagnosis of right L1 spontaneous CSF leak from
logical symptoms. Past medical history included hepatitis C infection and dural tear was confirmed during surgery. After the repair of tear, her
chronic allergic rhinitis and salicylate induced urticaria. There was no family headache disappeared completely.
history of asthma. He was taking albuterol for asthma on as required basis.
Physical Examination revealed an adult male in moderate distress with
increased nasal lacrimation and nasal phonation. Blood pressure was 134/75,
pulse; 89/min, respiration; 21/minute. Room air saturation was normal. He
was afebrile. Head and neck revealed no jugular venous distension, thyro-
megaly or adenopathy. The nose revealed generalized mucosal inflammation
and hypertrophic turbinates and multiple polyps occluding the both nasal
cavities. The left eye demonstrated proptosis and inflammation with in-
creased lacrimation. Chest revealed bilateral polyphonic wheezes. Cardiac
showed regular rate and rhythm, normal heart sounds no murmurs and no
gallops. Extremities; no tremor, dry palms, no finger clubbing, cyanosis or
edema. Cranial examination was remarkable for diplopia in all planes of
vision. The rest of the neurological examination was unremarkable. A
computer tomogram (CT) scan of sinuses performed in the emergency room
showed bilateral nasal polyps with a large left sided maxillary polyp measuring
1cm by 1.5 cm in a cranial caudal dimension and encroaching the left orbit
supero-laterally causing orbital and superior rectus muscle displacement.
Laboratory data; showed WBC; 96000/␮l, Hgb; 15.9g/dl, platelet; 198,000/␮l.
He was evaluated by otolaryngologist in the emergency room and given
hydrocortisone 100 mg intravenously and initiated on prednisone 40 mg qd.
He underwent nasal polypectomy and orbital decompression with a resultant
restolation of normal vision.
DISCUSSIONS: Samter’s syndrome comprises of the triad of asthma,
nasal polyps and salicylate intolerance. The pathogenesis of Samter’s DISCUSSIONS: During the bouts of coughing intrathoracic pressures
syndrome is thought to be caused by abnormalities of the cyclo-oxygenase of up to 300 mm Hg (1) may be achieved and it has been shown to be
enzyme system. It is considered that genetically mediated perturbations in associated with serious complications like pneumomediastinum (2) or
cyclo-oxygenase bioactivity channel the metabolites of eicosapentanoic pneumothorax, fracture of ribs, herniation of lung through intercostal
acids into the leukotriene pathway. These metabolic derangements cul- space (3), tracheal wall rupture (4), and diaphragmatic rupture (5).Most
minate in the amplification of the biological effects of leukotrienes. The spinal CSF leaks are iatrogenic, occurring after lumbar puncture or spinal
pathological effects of excess leukotrienes include bronchospasm, inflam- surgery, or they may be due to dural laceration caused by a blunt trauma
mation and the development of polyps. A preponderance of cell adhesion or penetrating spinal trauma. Spinal CSF leaks without any obvious
molecules on polyp surfaces has also been noted and suggests that preceding trauma, however, are increasingly recognized as a cause of
pro-inflammatory cytokines play a role in the pathogenesis of Samter’s postural headaches associated with intracranial hypotension (6). The exact
triad. The management of giant polyps with symptomatic mechanical cause of spontaneous CSF leak often remains unclear; however, the
effects includes evaluation for sinus fungal infection and the use of high combination of a trivial precipitating event and an underlying weakness of
dose corticosteroids as a bridge to polypectomy and sinus debridement. In the spinal meninges is generally suspected. A large majority of patients
the long term, leukotriene modifiers should be included in the treatment reports a previous trivial trauma such as coughing, lifting, pushing, sports
regimen for Samter’s syndrome. Despite aggressive treatment,polyps have activities, roller coaster ride (7), and the like. The disease is usually
a recurrent course. self-limiting, but in selected cases, surgical ligation of leaking dura has
CONCLUSION: This case suggests that in patients with a known been found to be satisfactory (6).
history of polyps who present with ocular complaints, diagnostic consid- CONCLUSION: Our patient had spontaneous CSF leak that we
eration should be given to the possibility of mechanical effects of large believe was secondary to bouts of violent coughing. One should suspect
polyps. High dose corticosteroids should be used while awaiting the the possibility of spontaneous CSF leak in a patient with positional
surgery which is the definitive treatment. headache. MRI of spine and CT myelogram may help us in confirming the
REFERENCES: diagnosis. Most of the leaks resolve spontaneously but in few patients
1 Zeitz HJ. Bronchial asthma, nasal polyps, and aspirin sensitivity: surgical repair of the dura may be necessary.

REFERENCES: included tapering of oral prednisone, weight reduction, speech therapy

1 Sharpey-Schafer EP.The mechanism of syncope after coughing. Br and reassessment for possible resection of the occluding supraglottic
Med J 1953; 2:860-63. structures. The patient refused to consider the surgical option.
2 Chiba Y, Kakuta H. Massive subcutaneous emphysema, pneumo- CONCLUSION: There are many symptoms and patient complaints
mediastinum, and spinal epidural emphysema as complication of that may be mistaken for bronchospasm and asthma. A variety of upper
violent coughing: a case report. Auris Nasus Larynx 1995; 22:205– and lower airway obstructive processes may produce audible inspiratory or
208. expiratory breath sounds. It is important to take an accurate history,
3 Sloth-Nielson J, Jurik AG. Spontaneous intercostal pulmonary her- complete appropriate diagnostic studies and interpret those studies
nia with subsegmental incarceration. Eur J Cardiothorac Surg 1989; correctly. Here we demonstrate the utility of routine office based flexible
3:562–564. rhinolaryngoscopy in pulmonary practice for evaluating patients with
4 Rousie C, Van Damme H et al. Spontaneous tracheal rupture: a case refractory dyspnea and atypical symptoms of asthma.
report. Acta Chir Belg 2004; 104(2): 22204-208.
5 Liziamma Geaorge, Shakib Rehman, Faroque Khan. Diaphragmatic
Rupture: A complication of violent cough. Chest 2000; 117: 1200-
1201.
6 Schievink WI, Meyer FB et al. Spontaneous spinal cerebrospinal
fluid leaks and intracranial hypotension. J Neurosurg 1996; 84:
598-604.
7 Schievink WI, Ebersold MJ, Atkinson JLD: Roller-coaster headache
due to spinal cerebrospinal fluid leak. Lancet 1996; 347: 1409.
DISCLOSURE: Ioana Amzuta, None.
SUPRAGLOTTIC CLOSURE DURING INHALATION AS A

CAUSE OF EPISODIC DYSPNEA ON EXERTION
Michael P. Pietila MD* Timothy I. Morgenthaler MD Udaya B. Prakash
MD Kaiser G. Lim MD Mayo Clinic, Rochester, MN
INTRODUCTION: A forty-four year old woman was referred for

refractory asthma characterized by fifteen years of variable dyspnea, worse
on exertion and associated with audible wheezing. She had responded
poorly to inhaled and oral glucocorticoids. Flexible rhinolaryngoscopy
showed collapse of the arytenoids during sniff maneuver resulting in
supraglottic airway obstruction. A diagnosis of laryngomalacia was made.
The otorhinolaryngologists recommended resection of redundant aryepi-
glottic folds and cuneiform cartilages but the patient refused the proce-
dure.
CASE PRESENTATION: A 44 year-old white woman presented for
evaluation of uncontrolled asthma with persistent dyspnea on exertion and
wheezing. Her current treatment regimen included Advair 500/50 BID,
Combivent four puffs QID, DuoNeb QID, Singulair 10 mg daily and
prednisone 20-60 mg as directed. Despite this aggressive medication
protocol she continued to experience frequent symptoms and had devel- DISCLOSURE: Michael Pietila, None.
oped medically complicated obesity related to prednisone use. She
suffered from frequent attacks of dyspnea requiring multiple emergency
room evaluations and unscheduled urgent clinic visits consulting her local OBLITERATIVE BRONCHIOLITIS DUE TO DIFUSE IDIO-
allergists and pulmonologists. She was referred to our institution with PATHIC PULMONARY NEUROENDOCRINE CELL HYPERPLA-
“refractory asthma.” Clinically, she described an unpredictable pattern of SIA
symptoms consisting of the abrupt onset of dyspnea associated with air Luigi Terminella MD* Alex Duarte MD University of Texas Medical
hunger and a sensation of chest/throat tightness during activity. Pulmo- Branch, Galveston, TX
nary function tests (PFTs) showed mild restrictive lung disease without
CASE REPORTS
obstruction. Her vital signs were normal. Her BMI measured 56. Auscul- INTRODUCTION: Bronchiolitis is a fibrotic lung disease involving
tation of the chest revealed upper airway wheeze. Initial ear, nose, throat the small airways characterized by cough, dyspnea and irreversible airflow
and neck exam was unremarkable. Flexible fiberoptic rhinolaryngoscopy obstruction. Idiopathic bronchiolitis is frequently confused for common
was performed to evaluate for chronic rhinosinusitis or vocal cord conditions as asthma or chronic obstructive pulmonary disease. Diffuse
dysfunction. The nasal passages showed purulent secretions from the right idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a
maxillary sinus os. The scope was advanced into the oropharynx. The rare cause of bronchiolitis. We describe a male with asthma subsequently
laryngeal complex was evaluated for laryngopharyngeal reflux, growths, diagnosed with bronchiolitis due to diffuse idiopathic pulmonary neuroen-
and vocal cord mobility. When the patient was asked to perform the sniff docrine cell hyperplasia.
maneuver, the arytenoids and both proximal aryepiglottic folds collapsed CASE PRESENTATION: A 42-year-old male with asthma was re-
medially covering the laryngeal opening and inducing dramatic airway ferred for evaluation of cough and exertional dyspnea. He developed a
obstruction. This is seen at real time speed and at half speed (sees images). nonproductive cough 10 years prior to referral and had been treated with
Due to tongue traction supporting the arytenoids at the time of rigid bronchodilators and corticosteroids without improvement. The cough was
laryngoscopy, the otolaryngologists initially failed to observe this obstruct- paroxysmal, aggravated by fumes and physical activity, but not relieved
ing phenomenon. The collapse of the arytenoids and medialization of the with antihistamines, antacids or corticosteroids. He smoked tobacco and
aryepiglottic folds and cuneiform cartilages with resultant airway obstruc- quit 8 years ago. He denied prior respiratory infections, inhalational
tion and audible wheezing was easily demonstrated with the flexible injury, substance abuse, aspirin sensitivity, or heartburn. Medications
fiberoptic rhinolaryngoscope. included amilodipine, theophyline, albuterol and fluticasone. Physical
DISCUSSIONS: This is an unusual presentation of laryngomalacia as examination revealed a healthy appearing male with a respiratory rate 20
a cause of steroid refractory asthma and is different from vocal cord min-1 and SpO2 98%. Neck was supple without stridor. Lung fields were
dysfunction. Her lack of response to conventional asthma treatments and clear. Heart examination did not reveal murmurs or extra heart sounds.
the unpredictable pattern of dyspnea were a result of a functional and Peripheral edema was absent. Chest radiography revealed peribronchial
anatomical abnormality. Laryngeal obstruction due to medialization of the thickening. Pulmonary function tests demonstrated airway obstruction
aryepiglottic folds and cuneiform cartilages resulting in stridor and air with a normal inspiratory flow-volume loop. Review of past pulmonary
hunger during sniff maneuver is an unusual cause of upper airway function studies demonstrated progressive airway obstruction (table). A
obstruction masquerading as steroid refractory asthma. Recommendations high resolution chest CT to evaluate for small airway disease revealed

bronchial wall thickening and focal areas of ground glass opacities. A CASE PRESENTATION: A 76 years old African American male was
bronchoscopy revealed no evidence of vocal chord dysfunction and the diagnosed by a polysomnogram 3 years ago with OSA and CPAP was
transbronchial biopsies were nondiagnostic. Subsequently, an open lung prescribed. He was intolerant of it and therefore noncompliant. He had
biopsy revealed neuroendocrine cell proliferation in the epithelium as well worsening of his excessive daytime hypersomnia, snoring and apneas. His
as submucosal and peribronchial fibrosis with little active inflammation weight had been stable. He had history of coronary artery disease, diabetes
indicative of obliterative bronchiolitis. Immunostains confirmed the pres- mellitus, stroke and depression. Physical examination revealed muffled
ence of neuroendocrine cell hyperplasia. Other etiologies for obliterative speech with normal voice. He had an elongated facial height, slight anterior
bronchiolitis were excluded, leading to the diagnosis of diffuse idiopathic overbite and a mild left septal deviation of the nose. The soft palate and uvula
pulmonary neuroendocrine cell hyperplasia. He received a 6 week course were elongated but not notably obstructed. Polysomnography identified
of steroids that failed to improve symptoms and pulmonary function significant hypoxia reaching 61% with an AHI of 33.3/hr. Treatment with
testing continued to demonstrate a decline. A six-minute walk demon- CPAP and even BiPAP at multiple settings were unsuccessful to completely
strated new onset exercise-induced hypoxemia. Oxygen was prescribed abolish REM associated desaturations and hypopneas. After failure of
and a lung transplantation evaluation initiated. acclimatization to Bilevel pressure he was referred to Otolaryngology for
possible surgery. Fiberoptic laryngoscopy revealed normal lingual tonsil,
Test date FEV1 (L) FVC (L) FEF 25-75(L/s) DLCO larynx and supraglottic area. Hypopharyngeal examination showed fullness in
the left lateral wall of hypopharynx in sitting position, but a marked
1999 2.62 3.95 1.35 29.94 obstruction was noted when supine with severe obstruction at the level of the
2004 2.15 3.40 0.91 27.34 epiglottis. CT scan followed by MRA revealed a 3X 2 cm growth with a mass
2005 1.74 2.51 0.91 20.14 effect on the lateral hypopharyngeal wall consistent with a carotid body
tumor. His 24-hour urine epinephrine and plasma metanephrine and
normetanephrine levels were all normal.A multidisciplinary tumor board
advised resection but the patient declined the surgical option
.
DISCUSSIONS: Bronchiolitis is frequently overlooked as a cause

for chronic cough and dyspnea. Classification of bronchiolitis has been
based on clinical history, radiology and histopathology. Diffuse idio-
pathic pulmonary neuroendocrine cell hyperplasia is a rare cause of
bronchiolitis characterized by cough, longstanding dyspnea and an
unrevealing chest radiograph. High resolution chest CT may demon-
strate nodules or patchy areas of hyperlucency indicative of air
trapping. Pulmonary function studies reveal irreversible airflow ob-
struction. Open lung biopsy is required to confirm the diagnosis and
the histopathologic findings include submucosal bronchial fibrosis with
diffuse hyperplasia of neuroendocrine cells in the bronchioles. Neu- DISCUSSIONS: OSA has been reported to occur with lipomas, retention
roendocrine cell hyperplasia is postulated to be a tissue response to cysts, palatine hemangioma, tonsillar lymphoma and salivary gland tumors.
hypoxemia. Neuroendocrine release of bombesin, serotonin and calci- 1/30000 of head and neck tumors are a paraganglioma, making it a very rare
tonin-related gene protein leads to airway injury and fibrosis and may tumor. Carotid body tumors are usually asymptomatic, grow slowly, originat-
have a role in the development of chronic obstructive lung disease (1). ing in the chemoreceptors of the carotid body and can go unnoticed for many
CONCLUSION: Obliterative bronchiolitis may produce persistent years. Mayer Brix et al reported pharyngeal tumors to be cause of OSA in 3
cough and dyspnea that may be mistaken for asthma or chronic obstruc- out of 336 patients who underwent thorough evaluation for the cause of OSA.
tive pulmonary disease. Neuroendocrine cell hyperplasia is a rare form of Hoijer et al evaluated 6 patients with pharyngeal tumors for evidence of OSA
bronchiolitis that responds poorly to conventional therapies and and found that all patients had either polysomnographic or oximetric
progresses to worsening airflow obstruction. evidence of OSA with average ODI of 24(range 10-58) with complete
REFERENCE: resolution after treatment. This is one of two cases reported of OSA caused
1 Aguayo SM et al. Idiopathic diffuse hyperplasia of pulmonary by carotid body tumor. As in the initial case report no resection was attempted
neuroendocrine cells and airways disease. NEJM 1992; 327: 1285- at the patient’s request. It is suggested by expert opinion not to operate on the
1288. patients ⬎50 years of age because of high morbidity associated with surgery
DISCLOSURE: Luigi Terminella, None. and low chance of growth and malignancy of these tumors. Rare cases of a
swinging epiglottis that totally occluded the upper airway during forced
inspiration and got further provoked by CPAP have been reported, impress-
OBSTRUCTIVE SLEEP APNEA CAUSED BY A CAROTID BODY ing the benefit of direct laryngoscopy.
TUMOR: CASE REPORT CONCLUSION: This rare secondary cause of refractory OSA as in our
Muhammad A. Raza MD Tarif Smadi MD* Rose A. Franco MD B. T. patient, demonstrates the value of direct laryngoscopy in both sitting and
Woodson MD Medical College of Wisconsin, Milwaukee, WI supine position for the detection of unusual causes of OSA.
REFERENCES:
INTRODUCTION: Obstructive sleep apnea (OSA) results from a 1 Mayer-Brix; HNO 1989;37:511-6
structural compromise of the upper airway combined with decrease in 2 Hoijer ;Acta Otolaryngol 1992; 112:138-43
muscle tone during sleep. Overt upper airway pathology is rare, 3 Metersky Mark ;Sleep 18(1):53-54
however a variety of pharyngeal tumors have been well described as a 4 Kuauhyama Luna;Oral Oncology 2005;41: 56-61
cause of OSA. We describe a case of a mass originating in the carotid 5 Fernandez Julian; Acta Otorrinolaringol Esp;53(9):701-6
body presenting as a case of severe OSA with hypersomnia. DISCLOSURE: Tarif Smadi, None.

Bronchology II
2:00 PM - 3:30 PM
AIRWAY EROSION OF EPICARDIAL DEFIBRILLATOR PATCH vascular structures. Pulmonary complications associated with epicardial
AND WIRE defibrillator hardware can occur early in the post-operative period and
Nguyen-Steve D. Vu MD* Michael A. Jantz MD University of Florida, include pleural effusion, atelectasis, pneumothorax, and pneumonia (most
Gainesville, FL commonly left-sided). These events are usually nonfatal. Late pulmonary
complications result from distortion, migration, and erosion of epicardial
INTRODUCTION: Since their introduction in 1980, implantable defibrillators into adjacent lung structures. Reported complications in-
cardioverter defibrillators (ICD) have played a significant role in the clude persistent atelectasis, progressive fibrosis of adjacent lung tissue,
management of malignant ventricular arrhythmias. The early ICD systems recurrent pneumonias, hemoptysis, hemothorax, bronchopleural and
utilized epicardial patches and extra-cardiac wires that were often placed
bronchopericardial fistulas, empyema, and chronic pleural effusions.
at the time of cardiac bypass surgeries using an anterior thoracotomy or
median sternotomy approach. Epicardial patches and lead systems have Proposed mechanisms for patch distortion and migration include peri-
given way to transvenous devices that can be implanted by surgeons or patch infection, inflammation, and breakdown of the securing sutures.
cardiologists without the need for major surgery. A rare but important CONCLUSION: Erosions of epicardial defibrillator hardware into
complication of epicardial defibrillator patches is the potential for erosion adjacent pulmonary structures are rare and unpredictable events with
into adjacent lung structures resulting in catastrophic outcomes. potentially fatal outcomes. This complication should be considered in the
CASE PRESENTATION: A 59 year old male with non-ischemic differential diagnoses of recurrent hemoptysis, cough, and non-resolving
dilated cardiomyopathy had four ICDs implanted over a period of nine pulmonary infections in patients with ICDs.
years for sustained ventricular tachycardia. His first ICD unit utilized the REFERENCES:
epicardial patch/lead system and was implanted 15 years ago after he 1 Verheyden CN, et al. Implantable Cardioverter Defibrillator Patch
experienced an episode of sudden cardiac death. Over the next several Erosion Presenting as Hemoptysis. J Cardiovasc Electrophysiol
years, he had three additional units implanted secondary to malfunction of 1994; 5:961-963.
various defibrillator components. His most recent ICD consisted of 2 Dasgupta A, et al. Erosion of Implantable Cardioverter Defibrillator
endocardial leads that were placed transvenously. The patient presented Patch Electrode into Airways: An Unu
with complaints of hemoptysis, dyspnea, and left side pleuritic chest pain. DISCLOSURE: Nguyen-Steve Vu, None.
His chest x-ray revealed opacities in the left upper lobe (LUL) and left
lower lobe (LLL). A CT of the thorax demonstrated a soft tissue density
in the lingula with partial atelectasis of the LLL. Bronchoscopy revealed
the presence of an endobronchial lesion involving the lingular bronchus as COMPLETE RESECTION OF ENDOBRONCHIAL B-CELL
well as the superior segment bronchus in the LLL. Biopsies were negative LYMPHOMA BY ARGON PLASMA ABLATION VIA FLEXIBLE
for malignancy. Due to his long history of tobacco smoking and concern VIDEOBRONCHOSCOPY
for lung cancer, the patient was referred to cardiothoracic surgery for Rehan A. Haque MD Jennifer Toth MD* Milos Tucakovic MD Penn
thoracotomy and possible lobectomy. A repeat bronchoscopy prior to the State University Medical Center, Hershey, PA
planned surgery revealed the presence of an ICD patch eroding into the
lingular bronchus and lead wire eroding into the superior segment INTRODUCTION: Endobronchial involvement by non-Hodgkin’s
bronchus in the LLL. Due to extensive fibrosis involving adjacent lymphoma (NHL) is uncommon and usually occurs in the presence of
structures, the patient was deemed not to be a candidate for surgical more generalized disease. Solitary endobronchial lymphoma in the ab-
removal of the patch and wire. A repeat CT of the thorax one year later sence of disease elsewhere is extremely rare. In this report we describe a
revealed further erosion of patch and wire into the previously involved patient with an obstructing endobronchial mass which was the initial
bronchi. Following this, the patient required admission for massive manifestation of NHL that was completely ablated by argon plasma
hemoptysis. Despite bronchial artery embolization and control of bleed- coagulation via flexible videobronchoscopy.
ing, he ultimately died of pneumonia and sepsis. CASE PRESENTATION: 78 years old lady was admitted into Inter-
mediate Care Unit with one months’ history of progressively worsening
dyspnea, dry cough and wheeze. She denied hemoptysis, fever, chest pain,
weight loss and recent travel. Her past medical history was significant for
hypertension and uterine cancer treated by total abdominal hysterectomy
seven years ago. She never smoked in her life. Her physical exam was
remarkable for diminished breath sounds in lower half of right lung zone.
A CT scan of chest showed an endobronchial lesion in right intermediate
bronchus, partially collapsing right lower lobe. There was no mediastinal
lymphadenopathy. This led to bronchoscopy, which revealed completely
CASE REPORTS
obstructing lesion in right intermediate bronchus, at a level of right upper
lobe take-off. This lesion was first biopsied and then completely ablated,
using argon plasma coagulation. Pathology of the resected lesion revealed
large B-cell lymphoma. Patient’s symptoms improved dramatically. Sub-
sequently, head, abdomen and pelvic CT scans were unremarkable. A
positron emission tomogram was also unremarkable. The patient was then
treated with external beam radiation therapy followed by three cycles of
CHOP chemotherapy. The patient remained tumor-free one year after
the initial diagnosis.
DISCUSSIONS: Pulmonary non-Hodgkin’s lymphoma is usually seen
in the presence of intra and/or extrathoracic disease. Solitary endobron-
chial mass with resultant atelectasis in the absence of systemic lymphoma
is uncommon. The patients present with dyspnea, cough, wheeze, hemop-
tysis, chest pain and constitutional symptoms. Chemotherapy with or
without radiotherapy is mainstem treatment. All patients should be
treated with curative intent unless concomitant intercurrent illness pre-
cludes combination chemotherapy. If a relief of rapidly deteriorating
dyspnea is desired, an airway obstruction may be relieved by a self-
expanding endobronchial stent or tumor ablation by laser or Argon plasma
coagulation.
DISCUSSIONS: Complications associated with ICDs utilizing epicar- CONCLUSION: Argon plasma coagulation is safe and effective addi-
dial patches and leads include early and delayed infection, constrictive tional treatment option for rapid resolution of symptoms associated with
pericarditis, malfunction of various defibrillator components, and erosion B-cell non-Hodgkin’s lymphoma presenting as an isolated endobronchial
of ICD components into adjacent structures including the heart, lung, and lesion.

Bronchology II, continued
the depression may not have been well-controlled. All three patients
presented with dyspnea, wheezing, cough, and right-sided FBA. Because
of the smoking history, all were mistakenly diagnosed with bronchitis. The
first two cases were diagnosed by chest imaging and the third by FOB. All
3 foreign bodies were retrieved by FOB. Symptomatic improvement was
reported by all patients after extraction of the foreign bodies.
CONCLUSION: In adult smokers, FBA can easily be mistaken for
chronic bronchitis, causing unnecessary delay in diagnosis and initiation of
therapy.
REFERENCE:
1 Baharloo F, Veyckemans F, Francis C et al. Tracheobronchial
foreign bodies: presentation and management in children and
adults. Chest 1999;115(5):1357-62.
DISCLOSURE: Jennifer Toth, None.
FOREIGN BODY ASPIRATION IN THREE ADULT SMOKERS

MIMICKING CHRONIC BRONCHITIS
Mohamed B. Bakry MD* Louis Voigt MD Gerard Lombardo MD Suhail
Raoof MD Stephen M. Pastores MD Neil Halpern MD Memorial Sloan
Kettering Cancer Center, New York, NY
INTRODUCTION: Foreign body aspiration (FBA) in adults is un-

common but may present as a diagnostic dilemma. The clinical picture
may vary from chronic nonspecific symptoms mimicking other lung
diseases to severe obstructive pneumonitis. We present a case series of
FBA occurring in three adults who shared similar symptomatology and
risk factors but were mistakenly treated for chronic bronchitis.
CASE PRESENTATION: Case #1: A 47-year-old man with a 40-pack-
year smoking history presented with a long history of multiple hospital-
izations for dyspnea, halitosis, productive cough, and wheezing. Ten
months earlier, he had undergone a right inguinal hernia repair performed
under general anesthesia, associated with a difficult intubation. Postoper-
atively, the respiratory symptoms persisted despite inhaled bronchodila-
tors, broad-spectrum antibiotics, corticosteroids, and smoking cessation.
Multiple chest radiographs were normal. On the current hospitalization,
chest CT revealed an intraluminal linear density involving the trachea and
right mainstem bronchus (RMB). Fiberoptic bronchoscopy (FOB) re-
trieved a 4.1 x 2.0 x 0.1 cm piece of wooden tongue depressor. The
patient’s symptoms subsequently resolved. Case #2: A 42-year-old man
with a 45-pack-year smoking history and several years of alcohol use was
evaluated at his physician’s office for a 4-month history of cough and
wheezing. He was treated with inhaled bronchodilators, oral antibiotics,
and advised to stop smoking and drinking alcohol; however, his symptoms
worsened. A chest radiograph revealed a coin shadow in the RMB. FOB
retrieved a penny from the RMB following which his symptoms subse-
quently resolved. Case #3: A 57-year-old man with a 100-pack-year
smoking history and major depression (well controlled with medication)
presented with a one-year history of dyspnea, cough, wheezing, dyspho-
nia, and a choking sensation. Chest radiograph demonstrated infiltrates in
the right middle and lower lobes. FOB retrieved a gauze-like object from
the right bronchus intermedius; pathologic exam confirmed the object to
be vegetable matter.
DISCUSSIONS: Although rare, tracheobronchial FBA in adults can DISCLOSURE: Mohamed Bakry, None.
occur in various clinical settings. The most common presenting feature is
a history of aspiration. When this history is absent, diagnosis is sometimes
delayed by hours or even years. Food products such as vegetable matter, PROLONGED ENTRAPMENT OF FOREIGN BODY IN THE
meat, and bones are the most common aspirated foreign bodies in adults. AIRWAY
Up to 80% of patients have normal radiographs. In our 3 patients, the Rubina Kerawala MD* Ross Reul MD James P. Herlihy MD Baylor
major risk factor was an altered mental state which precluded any recall of College of Medicine, Houston, TX
aspiration. In case #1, the aspiration occurred most likely during the
difficult intubation when a tongue depressor (used for better visualization INTRODUCTION: Foreign body aspiration (FBA) is a phenomenon
of the airways) may have broken into pieces. The second patient was commonly seen in infants and young children. Adult aspiration of foreign
involved in the so-called “drinking quarters” game in the weeks preceding bodies acutely presents with symptoms of upper airway obstruction. When
the initial symptoms. In this game, patrons at the bar drink glasses of aspirations into the distal airways are silent they can lead to prolonged
alcoholic beverages when coins bouncing on the bar table fall directly into entrapment of foreign materials and significant complications. Those
their drinking glasses. It is customary for the patrons to spit the coin out complications can lead to misdiagnosis and inadequate treatment.
of their mouths, but inebriety may have predisposed our second patient to CASE PRESENTATION: 32 year old African American female
aspiration. Finally, despite good compliance to the antidepressants, admitted to the hospital for worsening shortness of breath and cough for
aspiration may have occurred in the third patient at an earlier period when two weeks and left sided pleuritc chest pain. Past medical history was

notable for morbid obesity and asthma for 16 years treated with inhaled proximal granulation response was again noted; a 2.5-hour procedure using
bronchodilators, inhaled and systemic steroids. Physical exam revealed a heart electrocautery, cryotherapy and balloon dilation was used to clear the proximal
rate of 127, respiratory rate of 28, and blood pressure of 168/92. Temperature tissue. A 25% stenosis from granulation re-growth within the distal 3 cm of the left
was 98F.She had diffuse expiratory wheezes bilaterally. Laboratory evaluation main stem stent and LLL stent was observed but un-treated. Follow-up
revealed a WBC of 15,000. Chest radiograph revealed left lingular infiltrate bronchoscopy was performed 2 weeks later to plan therapy and surprisingly 100%
and left hemidiaphragm elevation. Patient was treated for asthma exacerba- of the left main stem and LLL stents were visible. Optical biopsies of skin and oral
tion and pneumonia and underwent ventilation/perfusion (V/Q) scan (sec- mucosa at the time of the follow-up bronchoscopy unexpectedly showed photo-
ondary to iodine allergy) to rule out pulmonary embolism (PE). V/Q scan was sensitizer at significant levels.
low probability for PE but remarkable for absence of ventilation to the left DISCUSSIONS: Photofrin® has 5 significant absorption peaks, clini-
lung. As a result, the patient underwent flexible FOB which revealed almost cally we employ the absorption peak at 632nm (red-light); the highest
completely occluded left main stem bronchus by an endobronchial mass 4 absorption peaks are in the blue-green spectrum (500-600nm). WLB has
cms from the carina. Pathology of the mass revealed granulation tissue.Patient a typical measured output of 3 mW in the visible light spectrum, with the
was taken to the operating room for FOB after intubation under general preponderance of the total energy delivered in the 500-600nm ranges.
anesthesia which revealed a left mainstem bronchial lesion and distal to it a The total bronchoscopy time was 9000 seconds in an area defined by a
FB, successfully removed by alligator forceps. Pathology confirmed the 1.4 x 4cm stent. The total power delivered was approximately 18-20 J/cm2,
presence of a 1.1cm long, 0.8 cm wide tip of a plastic drinking straw. Close in a frequency range that interacted with the highest absorption peak of
questioning revealed a history of aspiration while chewing a plastic straw at Photofrin®. Drug levels in the granulation tissue may have been signifi-
the age of 16 yrs. cant, based on the optical biopsies from skin and oral mucosa done later.
DISCUSSIONS: FBA in adults is limited to the larynx or proximal Drug-light dosimetry, done in retrospect, supports an ablative response.
trachea and usually occurs on the right. Common aspirated materials CONCLUSION: WLB may have the ability to activate a PDT response
include food, dental prosthesis, and bone, fragments of teeth, tracheos- in some patients. This potential should be taken into consideration when
tomy tubes and speech devices. The first case of an aspirated drinking treatment-planning post PDT salvage procedures. Point spectroscopy is a
straw obstructing an airway was reported in 1990.Common initial symp- vital tool for identification of clinically significant residual photosensitizer.
toms following an acute event include coughing and choking. Severe DISCLOSURE: Franklin McGuire, None.
symptoms can occur including hemoptysis and diffuse wheezing. Silent
aspirations can occur after major trauma, drug or alcohol intoxication, or
in patients with dysfunction of the oropharynx and neurological disorders. BRONCHOESOPHAGEAL FISTULA SECONDARY TO BRON-
Diagnosis is often delayed because of absence of early specific symptoms. CHOLITHIASIS
This leads to FBA misdiagnosed as other respiratory diseases including Wei Peng MD* Holly Carveth MD Mark Elstad MD Scott Woller MD
asthma, lung cancer and infectious causes. Aspirated foreign body in the University of Utah, Salt Lake City, UT
airway usually induces bronchial wall edema and inflammation. Eventu-
ally, foreign body is surrounded by granulation tissue leading to airway INTRODUCTION: We describe a case of bronchoesophageal fistula
stenosis. The granulation tissue response can be severe and can mimic secondary to boncholithiasis not detected by swallow study prior to
endobronchial carcinoma.Diagnosing a FB in the airway can be challeng- broncholith removal.
ing. The chest radiographic findings are inadequate to exclude the CASE PRESENTATION: This 43 year old advertising consultant, with
diagnosis and include atelectasis and bronchiectasis. Computed Tomog- a history of hypertension and tobacco addiction, presented with one year of
raphy is more accurate for detecting lung parenchymal manifestations of intermittent cough. The patient had approximately eight ounces of greenish
FBA and should be used as a tool for guiding retrieval of FB using FOB. sputum production per day with paroxysms of coughing and rare hemoptysis
CONCLUSION: Endobronchial FBA should be included in the worst upon awakening and upon drinking. His social history was remarkable
differential diagnosis for patient’s with atypical onset, adult onset, and for living in Minnesota and working with manure during past summer jobs.
difficult to control asthma. Patient’s with FBA can present with diffuse Initial work-up included spirometry and chest radiography were normal.
bilateral wheezes as a result of bronchial hyperresponsiveness prompted Empiric therapeutic interventions including successful smoking cessation, a
by the aspirate. Aspiration can be asymptomatic at first if the foreign body course of antibiotics, bronchodilators, corticosteroids, and acid suppression
is aligned in such a way that it does not obstruct the airway. However, did not alter his symptoms. A high resolution CT of the chest demonstrated
prolonged entrapment can lead to granulation tissue formation and airway small calcified mediastinal lymph nodes suggestive of remote granulomatous
obstruction. Pathologic finding of granulation tissue upon biopsy of an disease. The patients symptoms persisted and a barium esophagram showed
airway lesion should lead to the suspicion of FBA. Full recovery after a focal outpouching of the left lateral mid-esophageal wall with a tethered
prolonged entrapment of FB of 16 years as in our case is possible. appearance adjacent to a one centimeter calcified lymph node without
DISCLOSURE: Rubina Kerawala, None. evidence of barium extravasation. Rigid bronchoscopy confirmed the pres-
ence of bronchioliths, and three were removed from the left main bronchus
(Figure). The patient developed worsened cough after drinking. Repeat
CASE REPORTS
AIRWAY RESPONSE TO RESIDUAL PHOTOSENTIZER DUR- barium esophagram showed barium extending from the apex of the previ-
ING WHITE LIGHT BRONCHOSCOPY (WLB) ously-seen diverticula into the left lower lobe bronchus. The patient under-
Franklin R. McGuire MD* Carter Childs MD Ron Allison MD Rosa went thoracotomy and broncheosphageal repair with symptomatic resolution
Cuenca MD Claudio Sibata PhD Gordon Downie MD East Carolina and return to usual activities within 2 months.
University, Greenville, NC DISCUSSIONS: We report a case of acquired bronchoesophageal
fistula secondary to broncholithiasis. Such fistula is more commonly
INTRODUCTION: Photodynamic therapy (PDT) is FDA approved for the caused by infection, tumor, or trauma. Broncholithiasis is a rare disorder
treatment of both early stage lung cancer and the palliation of advance stage lung and usually the consequence of infection with tuberculosis, histoplasmo-
cancer. Off label lung applications have included augmentation of fluorescence sis, coccidiodides, actinomycosis or sarcoidosis with rare reports of silicosis
bronchoscopy, ablation of granulation tissue causing airway obstruction and as a as a cause[1,2]. We did not find any evidence of infection by tissue
radiosensitizer prior to brachytherapy. Photofrin® tissue levels have been docu- pathology or fungal serum titers and stains. However our patient came
mented to clear from mucosa and skin in 4-10 weeks. Drug activation within the from Minnesota, a histoplasma-predominated area, and calcified medias-
airway has been with laser light transmitted through diffusing fibers or micro-lens tinal lymphadenopathy on CT is highly suggestive of and consistent with
at red-light frequencies. histoplasmosis infection. The most common symptoms of broncholithiasis
CASE PRESENTATION: 47 yo WF w/o any significant PMHx was are nonproductive cough often associated with hemoptysis. Fever or
diagnosis with adenoid cystic lung cancer in 2000. Pneumonectomy was purulent sputum suggestive of respiratory infection is present 11-61% of
not offered because of extensive main carinal involvement. Treatments the time[2,3]. Broncholithasis arises most commonly from the erosion and
included XRT, neutron beam radiation, left main stem and left lower lobe extrusion of calcified material from bronchopulmonary lymph nodes. In
stents placed for palliative care. Stent function was complicated by this case, a large lymph node was found between the esophagus and
granulation tissue in-growth. She received APC, electrocautery, and PDT bronchus which eroded into the esophagus and caused the bonchoesopha-
to ablate the stent-granulation stenosis. PDT was performed on January geal fistula. Importantly, the diagnosis of bronchioesophageal fistula is
7th, 2005, 2 mg/kg IV was given, drug uptake into the granulation tissue sometimes overlooked when the broncholith occupies the space between
was documented by optical biopsies employing green and blue light point the bronchus and esophagus as occurred in our patient yielding the initial
spectroscopy. Initial complete response was seen with exposure of 90% of unremarkable gastrografin swallow study as described above. Bronchos-
the stent wires. At the 3-month surveillance WLB, a large left main stem copy for broncholith extraction has been characterized as safe and

effective for the removal of broncholiths, and the diagnosis of bronchoe- wheezes or crackles. Pertinent clinical findings included several 1-3 cm
sophageal fistula was made only after bronchoscopic removal of the firm and mobile subcutaneous nodules, mainly over the upper extremities,
broncholith in this case[4]. trunk, and abdomen. They were exquisitely tender but had no overlying
CONCLUSION: Bronchoesophageal fistula is a rare disease poten- skin discoloration or breakdown. The patient had no neurological defi-
tially caused by broncholithiasis. The diagnosis may be missed if the cits.Pertinent laboratory data included hemoglobin of 12.7 g/dL and room
broncholith occupies the space of the fistula. Clinical history can provide air arterial blood gas of pH 7.479, pCO2 33.8 mm Hg, pO2 48.0 mm Hg
some diagnostic clues. Patients benefit from thoracotomy with bronchoe- and SaO2 88 %. BUN, creatinine and LFT’s were within normal limits.
sophageal repair. Chest radiograph revealed a left hilar mass with patchy alveolar opacities
REFERENCES: involving the left upper lobe. CT scan of the chest demonstrated a large
1 Groves LK, Effler DB. Broncholithiasis: a review of twenty-seven mass with varying densities surrounding the lower trachea and left
cases. Am Rev Respir Dis 1956;73:19-302. Seo JB, Song KS, Lee SJ, mainstem bronchus, completely occluding the left pulmonary artery. A
et al. Broncholithiasis: Review of the Causes with Radiographic- brain MRI showed numerous lesions in the cerebellum, right thalamus
Pathologic Correlation. RadioGraphics 2002; 22:S199-S and right occiput. Bone scan showed widespread metastatic lesions.A
2 Seo JB, Song KS, Lee SJ, et al. Broncholithiasis: Review of the diagnosis of extensive stage small cell lung cancer with skin metastases was
Causes with Radiographic-Pathologic Correlation. RadioGraphics made following excisional biopsy of a subcutaneous nodule and broncho-
2002; 22:S199-S213. scopic needle aspirate of the mediastinal mass.
3 Dixon. GF, Donnerberg RL, Schonfeld SA, et al. Advances in the
diagnosis and treatment of broncholithiasis. Am Rev Respir Dis
1984; 129:1028-1030
4 Olson EJ, Utz JP, Prakash UB. Therapeutic bronchoscopy in
Broncholithiasis. Am J Respir Crit Care Med 1999; 160: 766-770.
DISCLOSURE: Wei Peng, None.
Cancer Cases III

2:00 PM - 3:30 PM
A 77-YEAR-OLD MAN WITH MULTIPLE SKIN NODULES
Sebastian Fernandez-Bussy MD* James Magill MD P.S. Sriram MD DISCUSSIONS: Although cutaneous metastasis from lung cancer is
University of Florida, Gainesville, FL rare, it may be the first clinical manifestation of occult cancer. Cancers
that metastasize to other organs, also involve the skin. While lung cancer
INTRODUCTION: Cutaneous metastases of internal neoplasms are is the commonest cause of skin metastasis in men, it is second only to
rare. They can often be the first clinical manifestation of an occult breast cancer in women. Other tumors involving the skin include cancers
malignancy and harbingers of advanced disease. Lung cancer, which often of the oral cavity, colon, ovary and stomach. The incidence varies from 3-7
metastasizes to brain, liver, bone and adrenal gland, is responsible for %. Most cutaneous metastases are firm, mobile, round, solitary or multiple
majority of skin metastasis in men and is second only to breast as a source and vary in size from 1-6 cm. While some are superficial, others may
in women. extend deep into the subcutaneous tissue. Skin discoloration or ulceration
CASE PRESENTATION: A 77-year old white man presented to may be present. Skin metastases in lung cancer are commonly found in
clinic, having developed several painful “lumps” all over his body during the chest, abdomen and back and rarely involve the scalp, face and
a 4 month period. In addition, he noted exertional dyspnea, dry cough and extremities. Of all the lung cancers, adenocarcinoma and large cell
a 50 lb weight loss. He denied fevers or night sweats but had a dull carcinoma most commonly metastasize to the skin compared to small cell
headache for several weeks. He was a 120 pack year smoker with an and squamous cell carcinoma. Skin metastases often herald a dismal
otherwise unremarkable past medical history. Physical examination re- outcome as they often are associated with widespread metastases with
vealed a cachectic male with pulse of 82 beats/min, BP 166/96 mm Hg, involvement of the liver, brain, bone and adrenal gland. Response to
temperature of 97.7 F, and respiratory rate of 18 breaths/min. He had no chemotherapy is poor. Median survival after diagnosis of skin metastases
clubbing or palpable lymphadenopathy. Chest examination revealed no is approximately 4 months. Poor prognostic factors include non-resect-

Cancer Cases III, continued
ability of primary tumor, small-cell lung cancer and multiple cutaneous or

extracutaneous lesions.
CONCLUSION: It is important to recognize that skin metastases may
be the first clinical manifestation of an occult malignancy. Biopsy speci-
mens must be taken from all unexplained skin nodules in patients who
smoke or who have a history of lung cancer. Survival after diagnosis of skin
metastases associated with lung cancer is dismal. Our patient died within
a week following diagnosis.
DISCLOSURE: Sebastian Fernandez-Bussy, None.
ANEMIA AND AN ANTERIOR MEDIASTINAL MASS

Philippe R. Bauer MD* James R. Jett MD Mayo Clinic, Rochester, MN
INTRODUCTION: Pure red cell aplasia (PRCA) is an auto-immune

mediated disorder with isolated hypoproliferation of the erythrocytes
precursors in the bone marrow. It can be associated with thymoma. We
present a case of this rare paraneoplastic syndrome and results of
treatment.
CASE PRESENTATION: An otherwise healthy 71 year-old gentle-
man developed dyspnea and was found to have a severe anemia as low as
6.2 g/dL that required blood transfusion. His bone marrow examination
showed marked erythroid hypoplasia, CD8-positive lymphocytosis, con-
sistent with PRCA. Clonal T-cell receptor gene rearrangement and PCR
for parvovirus B19 were negative. The patient was a previous smoker with
a history of ehrlichiosis, tonsillectomy and vasectomy. Physical examina-
tion was unremarkable. The chest radiogram (Figure 1) showed an
anterior mediastinal mass. The computerized chest CT (Figure 2)con-
firmed a 3 x 4 cm encapsulated mass. Pulmonary function tests were
normal. Striated muscle antibody was positive. The patient underwent
sternotomy and thymomectomy. Pathology showed a mixed subtype
thymoma invading into but not through the capsule. One month later, the
patient remained anemic, requiring repeated blood transfusions. He was
then given prednisone (1 mg/kg) for 8 weeks without improvement. He
was subsequently treated with antithymocyte globulin therapy (ATG) and
cyclosporine A. Two months later he was feeling better. His hemoglobin
remained low but stable and he has not required any further transfusion
since starting the cyclosporine A. Further follow up will be provided.
DISCUSSIONS: Acquired pure red cell aplasia is a rare condition of
profound anemia with no reticulocytes and no erythroid precursors in the
bone marrow. The association with thymoma is very rare. For example, in
one study (1), from 1980 to 1994, 47 adult patients with PRCA were
identified, of whom only 4 patients had thymoma. Up to forty percent of
patients with thymoma present with one or more paraneoplastic syn-
dromes, including myasthenia gravis, pure red cell aplasia, and hypoga-
DISCLOSURE: Philippe Bauer, None.
mmaglobulinemia (2). In case of PRCA associated with thymoma, the
mechanism involves suppression of erythropoiesis by nonclonal T cells.
PRCA can occur before or after the discovery of thymoma. PRCA may
CASE REPORTS
BRONCHIOLOALVEOLAR CARCINOMA PRESENTING AS A
disappear or persist after thymomectomy. In that latter case, prednisone FLU-LIKE ILLNESS WITH BILATERAL CAVITARY PNEUMO-
alone or in combination with cytotoxic agents has a response rate of about NIA AND A PSEUDO-ABSCESS
50%. Anti-thymocyte globulin therapy and tacrolimus seem promising. To Marcus P. Kennedy MD* D. N. Hayes MD M. P. Rivera MD University
date, the most effective agent, however, is cyclosporine A with a response of North Carolina, Chapel Hill, NC
rate of 82% in a series of 150 patients with PRCA (3). In the case reported,
anemia was the revealing feature with a new onset dyspnea. A mediastinal INTRODUCTION: Bronchioloalveolar carcinoma (BAC) accounts for
mass was found on the chest radiogram. Although the surgery was a 2.6-4.3% of all lung cancers with incidence rising especially in females and
complete resection, PRCA persisted and did not improve with prednisone non –smokers (1). We report an unusual presentation of BAC masquer-
alone but appeared stabilized when associated with ATG and cyclosporine ading as bilateral community acquired pneumonia (CAP) with pseudo-
A. Due to the paucity of cases reported, no prospective study can be abscess formation.
entertained to analyze accurately what the best therapeutic option would CASE PRESENTATION: A 30-year-old female, with a 12 one-pack-
be. In our case, although a 6-month follow up seem encouraging, the long year smoking and no prior medical history, presented with a history of
term prognosis remain uncertain since PRCA is a criterion of poor flu-like symptoms, cough and right-sided pleuritic chest pain. Chest
prognosis when associated with thymoma (4). radiograph (CXR) revealed a right lower lobe (RLL) infiltrate, and she
was treated with 10 days of levofloxacin. Repeat CXR demonstrated a
CONCLUSION: A case of PRCA associated with thymoma is re-
large RLL cavity. A chest CT scan confirmed a 2 cm cavity with an
ported. Although the surgical resection was complete, PRCA persisted air-fluid level and surrounding consolidation. A pigtail catheter was placed
and needed immunosuppressive therapy. The long term prognosis is and opaque fluid was drained from the cavity initially culture negative,
uncertain. with an unspeciated gram negative rod isolated two weeks later. She was
REFERENCES: then treated with a six-week course of amoxicillin-clavunalic acid, cipro-
1 Lacy MQ et al. Blood, 1996, 87: 3000-6. floxacin and metronidazole. Repeat CT revealed persistence of a fluid
2 Morgenthaler TI et al, Mayo Clin Proc 1993, 68: 1110-23. filled thick walled RLL cavity, progressive dense consolidation through
3 Mamiya S et al, Eur J Haematol 1997, 59: 199-205. out the RLL, as well as new thick walled small cavities and parenchymal
4 Nakayama H et al, Kyobu Geka. 1993, 46:13-20. infiltrate within the left lung. Standard therapy for lung abscess was

deemed a failure and she was referred to our center.Repeat CT GIANT CELL CARCINOMA OF THE LUNG: THE DOUBLING
revealed an increase in the diameter of the RLL cavity (4cms) with new TIME DILEMMA
right upper lobe involvement (figure 1). She was afebrile and chest Douglas R. Fiedler MD* Samir N. Bhakta MD Jay I. Peters MD
examination revealed reduced breath sounds at the right lung base University of Texas Health Science Center at San Antonio, San Antonio,
with fine rales in the RLL. Complete blood count was normal and TX
serum ANA, ANCA, rheumatoid factor and HIV antibody were
negative. Serum Immunoglobulins were normal. Fungal serologies
were negative. Bronchoscopy revealed no endobronchial lesions and
cultures and cytology were negative. A course of intravenous vanco- INTRODUCTION: It may be difficult to differentiate an infectious
mycin and ceftazidime was commenced and repeat CT was performed versus a malignant process when a mass is discovered on chest x-ray.
3 weeks later revealing stable appearance of RLL cavity with progres- History, physical examination, and laboratory are used in the decision-
sion of disease in the right middle and upper lobe. At that time, a left making process. If serial radiographic examinations are available, doubling
upper lobe thorascopic wedge excision was performed and pathology time of the lesion can be useful in determining the etiology. It is generally
revealed non-mucinous adenocarcinoma with a bronchioloalveolar
believed that a lesion which doubles faster than every 30 days represents
growth pattern throughout the specimen (figure 2). The tumor was
focally positive for TTF-1 and negative for estrogen receptor consistent a benign process (1).
with BAC. She was commenced on a course of erlotinib 150 mg/day. CASE PRESENTATION: A 49-year-old male was admitted for
Repeat CT scan after 4 weeks of therapy revealed stable disease, but evaluation of fevers and chills of two weeks’ duration. He reported
progression of disease was noted after 8 weeks. She was therefore recent onset of scant hemoptysis and a ten-pound weight loss. Physical
switched to systemic chemotherapy. examination was significant for a temperature of 102 degrees Fahren-
DISCUSSIONS: Patients with BAC can present with symptoms such as heit and expiratory wheezing. Laboratory revealed a white blood cell
cough, chest pain and sputum production making it difficult to distinguish count of 23,500/cm3. Chest x-ray and CT revealed an 8.5 cm mass-like
from pneumonia. BAC typically arises in the lung periphery and grows along density in the anterior segment of the right upper lobe. The patient
the alveolar wall without, by definition, destruction of the underlying was treated with amoxicillin/clavulanate and improved clinically within
parenchyma. The radiographic presentation of BAC is varied. The most 48 hours. He underwent bronchoscopy which revealed purulent
common presentation is the solitary pulmonary nodule, followed by an drainage from the right upper lobe. There were no endobronchial
infiltrate involving one lung with or without air bronchograms. Cavitation is
rare, occurring in about 10% of cases (2). BAC masquerading as a large lesions. Transbronchial biopsies revealed inflammatory tissue with
pseudo-abscess with cavitary pneumonia has not been previously described. fibrosis. Due to his clinical improvement, a decision was made to treat
CONCLUSION: BAC can present with a variety of clinical and the patient with a prolonged course of antibiotics for a lung abscess.
radiographic features and should be included in the differential diagnoses The patient was seen 4 weeks post-discharge and had inadvertently
of CAP with cavitation. Biopsy or cytological examination should always be received only one week of antibiotics. The mass had enlarged to 11cm
considered when patients with CAP do not respond to conventional and his white blood cell count had increased to 39,500/cm3. A pigtail
antibiotic therapy. catheter was placed and drained 10 cc of non-diagnostic material. His
REFERENCES: white count continued to rise and the patient underwent surgical
1 Dumont et al. Chest 1998, 113. resection. Pathology revealed giant cell carcinoma. A CT scan of the
2 Hill et al. Radiology 1998, 150. head (compared to a study obtained for trauma two months prior to
admission) revealed multiple new metastatic lesions.
DISCUSSIONS: Giant cell carcinoma of the lung is a malignant,
aggressive, and rare neoplasm. Radiographic features are peripheral,
round or oval, and without lobulation or cavitation. Histologically it is
classified as a subtype of large cell undifferentiated carcinoma by the
World Health Organization. The tumor contains highly pleomorphic,
often multinucleated, tumor giant cells. It must contain at least 40%
giant cells that are greater than 40␮m to make a diagnosis.We report
a case of giant cell carcinoma which was very aggressive. Initial
bronchoscopy and clinical features favored an infectious etiology;
however, his tumor doubled in size within 4 weeks and metastasized to
his brain while he received treatment for a presumed lung abscess. A
Japanese study showed giant cell carcinoma to have the fastest
doubling time of all lung cancers at 67.5 days (2). Rapid growth with
early hematogenous spread occurs frequently (3).Effective therapy for
this tumor is difficult to determine due to the lack of controlled trials.
Resection and radiation have been suggested to prolong survival time;
however, one series found the average survival time was 7.4 months
from the time of initial symptoms, and only 4.2 months from the time
of diagnosis (4).
CONCLUSION: Although fever, chills, leukocytosis, and rapid expan-
sion of a pulmonary lesion are suggestive of an infectious process, giant
cell carcinoma of the lung should be considered in the differential
diagnosis. Delay in diagnosis may result in a poor outcome with this
aggressive tumor.
REFERENCES:
1 Nathan MH. Management of Solitary Pulmonary Nodules. JAMA
1974; 227(10):1141-1144.
2 Arai T, Kuroishi T, et al. Tumor doubling time and prognosis in lung
cancer patients: evaluation from chest films and clinical follow-up
study. Jpn J Clin Oncol 1994; 24(4):199-204.
3 Patz EF. Imaging bronchogenic carcinoma. CHEST 2000; 117:90S-
95S.
4 Shin MS, Jackson LK, et al. Giant cell carcinoma of the lung.
CHEST 1986; 3:366-369.
DISCLOSURE: Marcus Kennedy, None. DISCLOSURE: Douglas Fiedler, None.

LUNG ADENOCARCINOMA WITH SOLITARY METASTASIS TO organs. In addition to pulmonary complications, there does appear to be
NASOPHARYNX: A LONG-TERM SURVIVOR AFTER RADIO- an association with an increased risk of developing malignant neoplasms.
THERAPY We present a case of a patient with PLCH with typical radiographic
Randolph H. Wong MB, ChB* Gary M. Tse MD Alan D. Sihoe MB, BCh findings who subsequently developed metastatic bronchogenic carcinoma
T. W. Lee MD Innes Y. Wan MB, ChB Ahmed A. Arifi MD Anthony P. originating in an area with nodules presumed to be secondary to PLCH.
Yim MD The Chinese University of Hong Kong, Shatin, Hong Kong PRC CASE PRESENTATION: A 32 year old white male was initially
diagnosed with LCH at age 4. He had several episodes of recurrent
INTRODUCTION: Adenocarcinoma of the lung seldom metastasizes bony involvement, which were treated with a combination of surgery,
to the nasopharynx and there are sparse reports in the literature concern- radiation therapy, and chemotherapy. At age 30, the patient presented
ing this issue. From the report available, nasopharyngeal metastasis with dyspnea. Pulmonary involvement of his LCH was suspected, with
usually accompanied by other distant metastases and there was no typical CT findings of nodules and upper lobe predominant cystic
long-term survivor reported thus far. In this report, we present a patient lesions. The patient also had an obstructive pattern on pulmonary
with adenocarcinoma of the lung with nasopharyngeal metastasis who was function studies. He entered into a smoking cessation program and was
able to achieve long-term survival after radiotherapy to the nasopharynx. able to reduce his use but was unable to quit. His pulmonary symptoms
CASE PRESENTATION: A 51-year-old male who was a chronic and lung function did stabilize without further interventions. He
smoker with an otherwise unremarkable past medical history, presented presented two years later with headaches and increasing dyspnea. A
with bloodstained sputum for 5 months. Subsequently, he was confirmed brain MRI was performed that revealed multiple ring enhancing
to have 5 cm adenocarcinoma over left upper lobe without radiological lesions. A chest x-ray and CT were obtained that showed a 3x4 cm left
evident of distant metastasis. The patient was treated with left upper lobe lower lobe mass. Tissue obtained by CT-guided fine needle aspiration
lobectomy and mediastinal lymph nodes sampling. The pathological showed a well differentiated adenocarcinoma consistent with a primary
staging was stage IB. The patient ran an uneventful post-operative course lung cancer. The patient began brain irradiation and palliative chemo-
and was discharged on the 5th post-operative day.He developed haemop- therapy with taxol and carboplatin.
tysis two months after the operation. Bronchoscopic examination revealed
a 1 cm nasopharyngeal pedunculated lesion with surface ulceration and
biopsy of that lesion confirmed to be adenocarinoma. The histomorpho-
logical pattern of the nasopharyngeal tumor (Fig 1) was similar to that of
the pulmonary tumor (Fig 2). Immunohistochemically, both tumors
showed the same profile of expressing cytokeratin 7 and thyroid transcrip-
tion factor I, but not cytokeratin 20. In situ hybridization for EBV RNA
was also negative. This profile demonstrated that both tumors were clonal,
and were of pulmonary origin, thus confirming a nasopharyngeal metas-
tasis of the lung adenocarcinoma.Metastatic work up including bone scan
and ultrasonography of the abdomen excluded other distant metastasis.
The patient was referred to oncologist and subsequently received radio-
therapy to the nasopharynx. Repeated nasopharyngoscope showed com-
plete regression of the lesion. The patient has been followed up for five
years since the initial operation and remains disease free. A repeated
position emission tomography does not reveal any suspicious focus of
uptake.
DISCUSSIONS: It was estimated that about 60% of non-small cell
carcinoma metastasize at the time of presentation. There are reports in
the literature that mentioned rare sites of metastases from lung cancer,
however, there is no report of adenocarcinoma of lung with solitary
nasopharyngeal metastasis. In our locality, malignancy occurring in the
nasopharynx is usually undifferentiated carcinoma (nasopharyngeal carci-
noma), which is associated with EBV genome within the tumor. In this
case, the histomorphologic pictures of both tumors were similar, and the
characteristic features for undifferentiated carcinoma of the nasopharynx
were absent. Both tumors showed similar antigenic profiles, which were
consistent with that of a pulmonary origin. Hence this case is highly
unusual in terms of a solitary metastasis to the nasopharynx, without
CASE REPORTS
involvement of other organs and the mechanism of such metastatic spread
remains a matter of speculation. In this particular case, there are two
learning points. First, for patient presented with haemoptysis after lung
resection, a surveillance of nasopharynx should be undertaken during
bronchoscopic evaluation. Second, long-term survival is still possible
despite confirmation of metastatic lung cancer, hence, nasopharyngeal
metastasis from lung primary may represent a different clinical entity
which responses to radiotherapy. DISCUSSIONS: Pulmonary nodules are typical findings in PLCH that
CONCLUSION: Nasopharyngeal metastasis from lung primary is a have been associated with early disease. Over time the nodules may
rare condition and often carries poor prognosis. There is a possible role of cavitate and result in thin-walled cavities that are often described in
radiotherapy in the treatment of solitary head and neck metastasis from PLCH. These nodules, however, may represent a diagnostic dilemma.
adenocarcinoma of lung. Though in most cases these nodules will represent the usual radiographic
DISCLOSURE: Randolph Wong, None. findings of PLCH, malignant lesions cannot be entirely excluded. There is
a clear association with LCH and malignancy. Lymphoma and leukemia
are most frequently seen, but solid organ malignancy, particularly lung
BRONCHOGENIC CARCINOMA IN A 32-YEAR-OLD WITH A cancer, has been reported. It appears that between 5 and 14% of patients
HISTORY OF PULMONARY LANGERHANS CELL HISTIOCY- with pulmonary Langerhans’ cell histiocytosis will be diagnosed with lung
TOSIS cancer.(1,2,3) There appears to be an increased tendency for malignancy
Bradley R. Harrold MD* Maria R. Lucarelli MD The Ohio State in all forms of LCH. Cumulative tobacco exposure and previous chemo-
University, Columbus, OH therapy and radiation therapy confer additional risk of developing cancer.
These factors suggest that more aggressive cancer screening may be
INTRODUCTION: Pulmonary nodules are a typical radiographic warranted in patients with LCH. This case is noteworthy because the lung
finding in patients with pulmonary Langerhans’ cell histiocytosis (PLCH). cancer occurred in an area of a nodule seen on the initial CT scan. Though
Langerhans’ cell histiocytosis (LCH) is a rare disorder that is character- pulmonary nodules are commonly seen in radiology studies in PLCH, this
ized by an uncontrolled accumulation of Langerhans’ cells in various case suggests that these nodules may need to be followed closely by serial

CT scans and may require histologic confirmation. The overall approach ment of ventilatory distribution to left lung following incentive spi-
to appropriate surveillance for lung cancer in these patients needs further rometry compared to distribution at baseline inspiration. (Figures 3 &
elucidation. 4).
CONCLUSION: Lung cancer screening is an essential component to DISCUSSIONS: The cases above demonstrate the potential for non-
the long-term management of patients with PLCH. invasive quantification of end expiratory flow and impact of incentive
REFERENCES: spirometry on distribution of flow.
1 Sadoun D, Vaylet F, Valeyre D, et al. Bronchogenic Carcinoma in CONCLUSION: VRI provides real time non-invasive acoustic lung
Patients with Pulmonary Histiocytosis X. Chest 1992; 101:1610-1613 imaging allowing documentation of both spatial distribution and quanti-
2 Tomashefski JF, Khiyami A, Kleinerman J. Neoplasms Associated fication of airflow. It may potentially offer significant diagnostic and
with Pulmonary Eosinophilic Granuloma. Archives of Pathology & treatment value to patient management in the ICU.
Laboratory Medicine 1991; 115:499-506
3 Vassallo R, Ryu JH, Schroeder DR, et al. Clinical Outcomes of
Pulmonary Langerhans’-Cell Histiocytosis in Adults. New England
Journal of Medicine 2002; 346:484-490
DISCLOSURE: Bradley Harrold, None.
Critical Illness and the Lung

2:00 PM - 3:30 PM
VIBRATION RESPONSE IMAGING IN CRITICALLY ILL PA-
TIENTS IN THE INTENSIVE CARE UNIT
Susmita Rajanala MD* Smith Jean PhD Igal Kushnir MD R. P. Dellinger
MD J E. Parrillo MD Cooper University Hospital, UMDNJ, Camden, NJ
INTRODUCTION: Vibration response imaging (VRI) measures

vibration response energy generated from airflow, to create a radiation
free, dynamic, real-time structural and functional image of the respi-
ration process. VRI technology records airflow generated vibrations
reaching the chest surface using a set of 42 ultra high-frequency DISCLOSURE: Susmita Rajanala, None.
microphones that are attached to the patient’s back (Figure 1). Using
custom-designed software the signals are fitted to a mathematical
function and a gray level frame is constructed. VRI is designed to RESPIRATORY FAILURE FROM HANTAVIRUS PULMONARY
capture images not attainable by other lung imaging technology. In the SYNDROME TREATED WITH HIGH FREQUENCY OSCILLA-
critically ill patient, VRI may facilitate diagnosis and management of TORY VENTILATION
critically ill patients such as intrathoracic assessment of flow as well as Nauman A. Chaudary MBBS* Melanie Fisher MD Luis Teba MD West
response to bedside interventions. Virginia University School of Medicine, Morgantown, WV
INTRODUCTION: Hantavirus Pulmonary Syndrome (HPS) may

lead to respiratory distress, hemodynamic compromise, and noncar-
diogenic pulmonary edema. High-Frequency Oscillatory Ventilation
(HFOV) is an alternative mode of ventilatory support for patients with
severe adult respiratory distress syndrome (ARDS). We present a
severe case of HPS who did not respond to conventional ventilation.
He was treated with HFOV, and recovered.
CASE PRESENTATION: A 41 year old man, presented with fever,
headache, myalgias, dyspnea, and hematuria. He removed rodent
droppings at a cabin in rural West Virginia two weeks earlier. He
developed respiratory distress, and was intubated. Chest radiographs
showed bilateral infiltrates (Figure 1). All cultures including bron-
choalveolar lavage fluid were negative. Hantavirus-specific IgM and
IgG antibodies were positive, and confirmed by the CDC. One day
after intubation, PaO2 was 62 mmHg on pressure control ventilation
(PCV), FIO2 1, and PEEP 14 cmH2O. Then, the ventilator was
changed to HFOV, and oxygenation improved, but his blood pressure
decreased, and he required fluids boluses, vasopressors, and a pulmo-
nary artery catheter. The initial HFOV settings were mean airway
pressure of 35 cmH2O, frequency 5 Hz, and amplitude 100 cmH2O.
On subsequent days, he remained hypotensive and with low cardiac
output. Pulmonary overdistension was suspected. HFOV was held for
a few seconds, and a sudden improvement in hemodynamics was
observed (table 1). Afterward, HFOV settings were adjusted. He
developed multiple organ failure. CVVHD and anticoagulation were
CASE PRESENTATION: Case 1 – A 55-year-old male patient started. During the 2nd week in ICU, his PaCO2 level continued to
admitted to the intensive care unit (ICU) following exploratory rise (pH 7.13, and PaCO2 71 mmHg), and oxygen saturation dropped
laparotomy was mechanically ventilated. He was found to have signif- to 88% on FIO2 1. Pneumothorax was ruled out, and an emergent
icant auto-PEEP. The VRI technology allowed assessment of intensity bronchoscopy was performed. A large amount of thick mucus obstruct-
of expiratory flow (Figure 2, arrow demonstrates continued end ing lobar and segmental divisions in both lungs was observed and
expiratory vibration signal extending into next inspiration). Case 2 – A removed. This resulted in a sudden increase in oxygen saturation to
51-year-old male patient was admitted to the ICU with respiratory 98%. At the end of the procedure pH was 7.21, PaCO2 51 mmHg, and
failure. Following extubation major left lung atelectasis was present. PaO2 87 mmHg. After 10 days of HFOV, his clinical course improved
VRI images during inspiration at maximal flow demonstrated improve- slowly, and he was eventually discharged.

Critical Illness and the Lung, continued
DISCUSSIONS: HPS carries a high mortality rate. In the USA, it is CASE PRESENTATION: A 41 year-old white male, previously healthy
usually caused by the Sin Nombre virus. It was initially reported in the smoker, presented with two weeks of increasing dyspnea, diffuse myalgias,
SW, but sporadic cases have also been observed in other states. It is and hemoptysis. On presentation the patient was profoundly hypoxemic with
transmitted by inhalation of contaminated aerosol from excreta of infected respiratory distress requiring intubation in the emergency room. On exam, he
rodents. Our patient apparently acquired it while cleaning the cabin. After was afebrile, tachycardic, and hypotensive. Lungs had diminished breath
a three-week incubation period, patients usually present with fever, sounds at bases and bilateral rhonchi. Laboratory studies were significant for
malaise, and myalgias. As in our patient, these symptoms may be followed elevated creatinine and blood urea nitrogen, leukocytosis and eosinophilia.
by the common clinical characteristics of HPS: fever, thrombocytopenia, Chest x-ray revealed bilateral pulmonary infiltrates. The patient developed
hemoconcentration, respiratory compromise, and ARDS. Positive Hanta- progressive renal failure needing dialysis, shock requiring pressor support,
virus-specific IgM or IgG antibodies confirm the diagnosis. Treatment of and persistent hypoxemia. A bronchoscopy was preformed with bronchoaveo-
HPS includes intensive care support, and initiation of mechanical venti- lar lavage demonstrating alveolar hemorrhage with 23% macrophages, 30%
lation if needed. In ARDS, HFOV has been been shown to be safe and neutrophils, and 46% eosinophils. Serum quantitative immunoglobulins were
effective in improving oxygenation. To our knowledge, the use of HFOV significant for IgE of 1043 mg/dl. A sinus CT showed right maxillary and
in patients with HPS has not been reported. Barotrauma and hemody- ethmoid sinusitis. Based on the clinical picture, elevated IgE, and peripheral
namic compromise are complications of HFOV. The hemodynamic / BAL eosinophilia a diagnosis of Churg-Strauss was made and the patient
changes result from HFOV induced increase in intrathoracic pressure. In initiated on prednisone therapy without improvement for five days. Subse-
our case, holding of HFOV for a few seconds resulted in a sudden increase quent serologic evaluation was positive for rheumatoid factor (1:80), C-ANCA
in blood pressure and cardiac output. In addition, mucus inspissation is (1:512), and anti-GBM at 106 EU/ml. The patient was then initiated on
another potential problem with HFOV. The presence of unexplained plasmapheresis for presumed Goodpasture’s disease. A renal biopsy displayed
refractory hypercapnea, as occurred in our patient, should alert the anti-glomerular basement membrane antibody and C-ANCA positive focal
physician for possible endotracheal tube or diffuse airway obstruction, and crescentric and necrotizing glomerulonephritis consistent with ANCA-posi-
the need for bronchoscopy. tive Goodpasture’s Overlap Syndrome. He was continued on plasma ex-
CONCLUSION: Intensive critical care support in patients with HPS change until his anti-GBM titers normalized, and treated with high dose
may lead to a complete recovery. HFOV appeared to be effective in our prednisone and cyclophosphamide. The patient was successfully liberated
patient. However, clinicians using this ventilator mode should be aware of from mechanical ventilation and hemodialysis. He was discharged home on
the potential deterioration in hemodynamics, and possible diffuse in- prednisone and mycophenolate mofetil. He continues to be dialysis indepen-
trapulmonary mucus plugging that HFOV can induce. dent at one year follow-up.
Table 1. Hemodynamic Profile During Conventional

Ventilation, High Frequency Oscillatory Ventilation
(HFOV) With Mean Airway Pressure (MAP) of 35
cmH2O, and HFOV With MAP 25 cmH2O
CVP PCWP CO CI BP
(mmHg) (mmHg) (L/min) (L/min/m2) (mmHg)
Conventional 15 14 6.3 3.3 110/70

Ventilation
HFOV (MAP 35 23 20 3.7 1.9 85/55
cmH2O )
CASE REPORTS
HFOV (MAP 25 23 24 5.2 2.7 150/80
cmH2O )
CVP ⫽ Central Venous Pulse
DISCUSSIONS: Pulmonary renal syndrome, initially described by
Goodpasture in 1919, is usually associated with either ANCA (60-70%) or
DISCLOSURE: Nauman Chaudary, None. anti-GBM (20%) antibodies. It has recently been recognized that patients
may present as both ANCA and anti-GBM antibody positive. Approxi-
mately 5% of ANCA positive sera is also anti-GBM positive and 32% of
PULMONARY RENAL SYNDROME SEROPOSITIVE FOR BOTH anti-GBM positive sera is ANCA positive. However there are only a few
ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES AND case series that correlate these serological findings with the clinical
ANTI-GLOMERULAR BASEMENT MEMBRANE AUTOANTI- progression of these patients. There is still debate on the prognosis of
BODIES these patients, in part due to a lack of clear diagnostic criteria and
Matthew C. Exline MD* John G. Mastronarde MD The Ohio State differences in treatment modalities. Some studies suggest these dually
University, Columbus, OH positive patients have a similar clinical course to ANCA vasculitis with
many (75%) recovering renal function. Others suggest near universal renal
INTRODUCTION: Pulmonary renal syndrome (PRS) confers a high failure in these patients similar to those with Goodpasture’s syndrome.
mortality in the critical care setting. Prompt diagnosis and appropriate CONCLUSION: It is vital to differentiate ANCA-positive anti-GBM
treatment are keys to preventing long term morbidity. However, there patients from other causes of PRS in order to deliver appropriate
may be considerable overlap both symptomatically and serologically treatment in a timely fashion. Plasma exchange, while a mainstay of
between the various etiologies of PRS, making initial treatment decisions therapy in Goodpasture’s syndrome, is not thought to be beneficial for
difficult. We present a case of a patient with the antineutrophil cytoplas- ANCA positive vasculitis and may not be routinely offered unless an
mic autoantibodies (ANCA)-positive Goodpasture’s Overlap Syndrome, diagnosis of Overlap Syndrome is considered. Prompt and aggressive
defined as PRS seropositive for both ANCA and anti-glomerular basement plasmapheresis for ANCA positive anti-GBM positive patients may por-
membrane autoantibodies (anti-GBM). tend a greater likelihood of renal recovery as in our patient.

REFERENCES: site⫽gt&id⫽8888891&key⫽143YUK-Kv-2.VeL&gry⫽&fcn⫽y&fw⫽-
1 Gallagher H et al. Pulmonary renal syndrome: a 4-year, single- JOs&filename⫽/profiles/tuberous-sclerosis/index.html Access date: April
center experience. Am J Kidney Dis 2002; 39(1):42-47. 28 2005
2 Levy JB et al. Clinical features and outcome of patients with both ANCA 3 Jost CJ, Gloviczki P, Edwards WD, et al. Aortic aneurysms in
and anti-GBM antibodies. Kidney Int 2004; 66(4):1535-1540. children and young adults with tuberous sclerosis: report of two
3 Hellmark T et al. Comparison of anti-GBM antibodies in sera with cases and review of the literature. J Vasc Surg. 2001; 33:639-642
or without ANCA. J Am Soc Nephrol 1997; 8(3):376-385.
DISCLOSURE: Matthew Exline, None.
NEW-ONSET WHEEZING: A RARE PRESENTATION FOR TU-

BEROUS SCLEROSIS COMPLEX
Melissa J. Gowans MD* Heather T. Keenan MD Susan L. Bratton MD
Constance Maves MD John Hawkins MD University of Utah, Salt Lake
City, UT
INTRODUCTION: Wheezing is a common symptom among in-

fants and toddlers most frequently due to asthma, viral infection/
bronchiolitis, or foreign body aspiration. We present a case of a 17
month old girl with wheezing refractory to bronchodilator treatments,
without a history suspicious for foreign body aspiration or recurrent
wheezing.
CASE PRESENTATION: The patient is a 17 month old female
who presented with a one day history of congestion, cough and tactile
fever. She rapidly progressed to respiratory failure and was intubated
prior to transport. During the patient’s transport and after her arrival
at our institution, she was difficult to ventilate, unless she was deeply
sedated. When sedated she had normal airway compliance. The
transport nurse noted a large “apple-sized” pulsatile midline mass in
her abdomen suspicious for an abdominal aortic aneurysm and she was
hypertensive. Initial chest x-ray demonstrated significant deviation of
the upper thoracic esophagus, prominence of the aortic shadow and
dysplastic ribs. Computed tomography with contrast of the thorax,
abdomen, and pelvis revealed dilatation of the descending thoracic
aorta, with a saccular protrusion off the medial aspect of the proximal
descending aorta which compressed the distal thoracic trachea. (Fig-
ure 1) The trachea at that level measured 2.5mm in the anterior to
posterior diameter. There were two other saccular aneurysms of the
thoracic aorta. Below the level of the superior mesenteric artery there
was again marked aortic dilatation that extended to the aortic bifurca-
tion and included the proximal-most portion of the right common iliac
artery. The right renal artery came off the aneurysm and was also
saccular in nature.The right kidney was small with irregular contour
containing multiple cysts. (Figure2) Five days after her initial presen-
tation the thoracic aorta was repaired due to tracheal compression. Ten
days after her admission she underwent repair of the large abdominal DISCLOSURE: Melissa Gowans, None.
aneurysm and right nephrectomy due to her hypertension and the
aneurysm size. Magnetic resonance imaging of her brain demonstrated
multiple cortical tubers, as well as nodules thought to be early giant FATAL TRANSFUSION-RELATED ACUTE LUNG INJURY IN A
cell tumors and multiple subependymal nodules. The diagnosis of PATIENT WITH CRYPTOGENIC CIRRHOSIS
tuberous sclerosis complex (TSC) was made based on these findings. Stephen R. Gorman DO* Timothy Wu MD Lahey Clinic Medical Center,
DISCUSSIONS: TSC was described in the 1880’s by a French Burlington, MA
physician name Bourneville. TSC, also known as Bourneville’s disease,
is an autosomal dominant condition with variable penetrance that INTRODUCTION: Transfusion-related acute lung injury (TRALI), a
occurs de novo in approximately 60% of cases.1 TSC typically involves primary cause of mortality associated with blood transfusions, is an
abnormalities of the skin, brain, kidney and heart with widespread underdiagnosed and underreported complication. In our fulminant and
development of hamartomas. The diagnosis of TSC is based on clinical ultimately fatal case, the diagnosis of TRALI was confirmed by the finding
findings and the criteria for diagnosis were revised in July 1998.2 The of passively transfused antibodies in the recipient. The hematologic
patient must have two major features or one major feature plus two investigation led to the identification of a sensitized donor who has been
minor features for a definitive diagnosis of TSC. Two causative genes, deferred from future donations, hopefully preventing additional transfu-
TSC1 and TSC2, have been identified. Molecular testing is available sion complications.
for confirmatory diagnostic testing, prenatal diagnosis, and for genetic CASE PRESENTATION: A 69 year-old man with a history of
counseling. Most children with TSC have brain involvement and Child-Pugh class C, cryptogenic cirrhosis was admitted because of ataxia,
seizures and developmental disabilities are common. Aneurysms are an confusion, and jaundice. Hyperbilirubinemia was discovered. The patient
uncommon presentation for TSC in children, Jost et. al. reported a underwent magnetic resonance cholangiopancreatography (MRCP),
review of the literature with the addition of two new cases n⫽15. Most which revealed choledocholithiasis. During an attempted endoscopic
of the patients had either a thoracic aortic aneurysm or abdominal retrograde cholangiopancreatography (ERCP), an actively bleeding arte-
aortic aneurysm, and only one patient had both. The series reported an riovenous malformation was found in the stomach and injected with
acute mortality rate of 40%.3. epinephrine. Two days later, fresh frozen plasma (FFP) was administered
CONCLUSION: Tuberous sclerosis complex affects approximately to correct a prolonged prothrombin time prior to a repeat ERCP. Ten
one in 8,000 adults and one in 6,000 newborns(ref). Although rare, aortic minutes after beginning infusion of the the second unit, acute dyspnea
aneurysms do occur. developed. A chest radiograph showed bilateral airspace disease consis-
REFERENCES: tent with pulmonary edema. The patient was intubated and mechanically
1 Tuberous Sclerosis Complex http://biology.kenyon.edu/slonc/bio38/ ventilated for hypoxemic respiratory failure. Brain natriuretic peptide
howell/tsc.htm Access date: April 29 2005 level and left ventricular function by echocardiography were normal.
2 Gene Reviews http://www.genetests.org/servlet/access?db⫽geneclinics& Severe metabolic acidosis, shock, and acute tubular necrosis ensued.

Broad spectrum antibiotics and vasopressor support were administered. DISCUSSIONS: Activated charcoal is often administered in cases of
No microbiologic etiology for possible sepsis was identified. The patient known or suspected toxic ingestion. Although occult aspiration of small
died on the third intensive care unit day. An antibody screen to human amounts of charcoal is not uncommon in mechanically ventilated patients
leukocyte antigens (HLA) I and II performed by solid phase ELISA on (1), large-volume aspiration is rarely reported (2-5), and usually leads to
the patient’s blood was positive for class I antibodies. HLA testing of the life-threatening hypoxemia. Charcoal-mediated increases in pulmonary
first unit of fresh frozen plasma administered to the patient was positive microvascular permeability have been suggested as a mechanism (6). In
by solid phase ELISA for both class I and class II antibodies. The donor, our patient, we believe that the primary cause of respiratory compromise
a multiparous female, was deferred from further donations. was charcoal impaction in the small airways, and that exogenous surfactant
DISCUSSIONS: TRALI is estimated to occur in 1 in 7,900 units of was beneficial more due to its ability to penetrate and loosen charcoal
FFP and should be considered when respiratory insufficiency occurs plugs than due to its effects on alveolar surface tension. The rapid
within six hours of transfusion of any plasma-containing blood product. A improvement in this patient’s oxygen saturation and concomitant increase
high index of clinical suspicion is necessary to confirm the diagnosis, in delivered volumes on pressure-control ventilation after surfactant
which may be mimicked by cardiogenic pulmonary edema and volume administration argue for an immediate mechanical benefit. To our
overload. Most cases are self-limited, but hemodynamic collapse may knowledge, this is the first reported use of surfactant for this purpose.
occur. Mortality occurs in 5 to 8% of cases. A double hit hypothesis has CONCLUSION: Mechanical small airway obstruction can complicate
been proposed to explain the pathophysiology of TRALI. Initially, an charcoal aspiration; the use of exogenous surfactant may aid in dislodging
underlying condition, such as sepsis, trauma, or surgery, causes priming charcoal plugs.
and adherence of neutrophils to the pulmonary endothelium. Leukocyte REFERENCES:
antibodies from donors, especially multiparous women, are passively 1 Roy TM, et al. Pulmonary complications after tricyclic antidepres-
transfused and activate targets on recipient neutrophils, especially HLA sant overdose. Chest 1989; 96:852-6.
class I and II antigens. This results in microvascular permeability and
2 Menzies DG, Busuttil A, Prescott LF. Fatal pulmonary aspiration of
pulmonary edema fluid. Biologically active lipids in blood products are
oral activated charcoal. BMJ 1988; 297:459-60.
also thought to initiate neutrophil priming. The latter is unlikely in our
3 Elliott CG, et al. Charcoal lung. Bronchiolitis obliterans after
case as antibodies were detected, and FFP does not contain these lipids,
which are found in cellular products. aspiration of activated charcoal. Chest 1989; 96:672-4.
CONCLUSION: Our case is noteworthy for several reasons. A high 4 Givens T, Holloway M, Wason S. Pulmonary aspiration of activated
index of suspicion of TRALI spurred a formal investigation by the blood charcoal: a complication of its misuse in overdose management.
bank. HLA antibody testing implicated a recently transfused unit, not the Pediatr Emerg Care 1992; 8:137-40.
one during which the patient’s symptoms began. By investigating the 5 Harris CR, Filandrinos D. Accidental administration of activated
possibility of TRALI, we were able to confirm the etiology of the patient’s charcoal into the lung: aspiration by proxy. Ann Emerg Med 1993;
respiratory failure and identify a sensitized donor who is deferred from 22:1470-3.
future blood product donations. 6 Arnold TC, et al. Aspiration of activated charcoal elicits an increase
REFERENCES: in lung microvascular permeability. J Toxicol Clin Toxicol 1999;
1 Looney MR, Gropper MA, Matthay MA. Transfusion-related acute 37:9-16.
lung injury: a review. Chest 2004; 126:249-258. DISCLOSURE: LeRoy Essig, None.
2 Toy P, Popovsky MA, Abraham E, et al. Transfusion-related acute
lung injury: definition and review. Crit Care Med 2005; 33:721-726.
DISCLOSURE: Stephen Gorman, None.
RESPIRATORY FAILURE AND REFRACTORY HYPOXEMIA Infectious Disease II

DUE TO ACTIVATED CHARCOAL ASPIRATION: TREATMENT
WITH BRONCHOSCOPIC SURFACTANT ADMINISTRATION 2:00 PM - 3:30 PM
LeRoy W. Essig MD* James N. Allen MD The Ohio State University,
Columbus, OH SEPSIS FROM NECROTIC TONGUE AS AN UNUSUAL COMPLI-
CATION OF SEISURE DISORDER
INTRODUCTION: Large-volume aspiration of activated charcoal is a Marina Dolina MD* Janette D. Foster MD Jon Isaacson MD Francis
rare complication of overdose management, and often leads to profound Ruggiero MB, BCh Margaret Wojnar MD Pennsylvania State University
respiratory failure and death. We present a case of severe, refractory College of Medicine, Hershey, PA
hypoxemia due to charcoal aspiration that was treated successfully with
exogenous surfactant administration. INTRODUCTION: Tongue bites and lacerations are well-recognized
CASE REPORTS
CASE PRESENTATION: A 19-year-old male was admitted to an
complications of seizure disorder. Because of the rich vascular supply,
outside facility after being found unresponsive at home; he required
most tongue lacerations heal rapidly without interventions. Only tongue
intubation upon arrival to the Emergency Department due to hypercarbic
lacerations in which poor healing may compromise tongue function
respiratory failure. Initial oxygen saturation was 100%. Toxicology screen
was positive for alcohol, marijuana, and benzodiazepines; there was also require more aggressive treatment. We present a case, in which the
concern the patient may have ingested a fentanyl patch. A nasogastric tube tongue injury led to severe complications, including sepsis.
was unknowing placed past the endotracheal tube cuff into the trachea, CASE PRESENTATION: An otherwise healthy 23-year old male with
and several hundred milliliters of activated charcoal were instilled. The severe mental retardation, autism and seizure disorder was brought to
patient became rapidly hypoxemic and difficult to ventilate, with high emergency department for evaluation of severe bleeding from a swollen
airway pressures and oxygen saturations in the 70’s. He was emergently tongue. According to patient’s mother, he had a seizures 5 days prior and
flown to our intensive care unit; his oxygen saturations remained in the was seen in outside facility with significant bleeding from tongue bite. By
70’s despite pressure-control ventilation with an FiO2 of 1.00 and high the time of initial evaluation bleeding stopped and patient was discharged.
levels of PEEP, neuromuscular paralysis, inhaled nitric oxide, and pron- Over next few day patient became progressively more combative with
ing. Bronchoscopy revealed charcoal plugs impacted in virtually all of the increasing tongue swelling, intermittent bleeding and became unable to
distal airways; attempts to remove them via aggressive bronchoscopic swallow pills or food. Mother noted him spitting out pieces of bloody
lavage and use of an external percussion vest were unsuccessful. Repeat tissue. In emergency department patient was intubated for combative
bronchoscopy was performed, with the instillation of 80 milliliters of behavior and airway protection. Hypotension required fluid resuscitation
beractant (Survanta (R), Ross USA) into each lobe. Oxygen saturations and vasopressors. A foul smell was noted as the examination revealed a
improved dramatically, increasing to the low 90’s by completion of the necrotic tongue. Patient was evaluated by the otolaryngology service and
procedure (Figure 1). Delivered tidal volumes also increased. Copious taken to operating room for debridement. Tissue cultures and blood
amounts of charcoal, suspended in surfactant, were expressed from the cultures grew positive for Beta Streptococcus group B. Patient was treated
endotracheal tube in the ensuing hour. The patient’s oxygen requirements with ceftriaxone and clindamycin for 6 days and then was switched to
decreased by 50% over the following 48 hours, and he was successfully ampicillin-sulbactam in accordance with culture and sensitivity report.
extubated on hospital day 8. The patient ultimately recovered, with Patient recovered with the assistance of speech therapy and was dis-
minimal residual pulmonary and neurologic sequelae. charged to his group home in stable condition.

Infectious Disease II, continued
DISCUSSIONS: The tongue is very vascular organ that usually Changing epidemiological factors may contribute to increased recognition
tolerated a great degree of trauma. Most injuries heal well. Because the of this pathogen in nosocomial infections.
tongue plays such a major role in speech and swallowing, surgical CASE PRESENTATION: Following a routine colonoscopy, a 69-
resection is usually withheld until other options are exhausted. The year old male with hypertension and peripheral vascular disease
recognition of the severity of our patient’s self-inflicted injury was delayed. presented to an outside hospital with pleuritic chest pain and shortness
Not until the necrosis led to infection and sepsis did the seriousness of the of breath following several episodes of nausea and vomiting. A Chest
trauma became undeniably obvious. CT scan showed a left sided pleural effusion. A chest tube was placed,
CONCLUSION: Even though complications from tongue lacerations and the patient was transferred to a tertiary care hospital with a
are rare due to very rich vasculature in the tongue, this case clearly diagnosis of an esophageal perforation. Upon arrival, he was emer-
demonstrate that the possibility of serious infection and sepsis can occur. gently taken to the OR. He underwent a bronchoscopy, left sided
REFERENCES: thoracotomy with primary repair of a distal esophageal perforation with
1 Lamell, CW, Fraone, G, Casamassima, PS, Wilson, S. Presenting intercostal muscle flap, and a pericardial window. Postoperatively, the
characteristics and treatment outcomes for tongue laceration in patient was intubated, maintained on vasopressors, and given broad-
children, Pediatr Dent 1999; 21:34 spectrum antibiotics. Within the next 16 hrs, the patient developed
2 Bailey, BJ. Management of soft tissue injuries. In: Oral and Maxil- severe sepsis, manifested by renal failure and worsening hemodynam-
lofascial Traum ics. He developed multisystem organ failure. His serum creatinine
kinase (CK) levels increased to 3,924, (baseline 259). Over the next 10
days, the BUN gradually increased to 99 (baseline 21), and his
creatinine increased to 4.9. CK levels progressively increased and
peaked at 143,000 (Fig. 1). He developed right lower extremity
compartment syndrome, requiring bedside fasciotomies and right
above knee amputation. Following the amputation, he developed frank
cellulitis of his right stump, buttock and flank. At this point, the family
decided against any more aggressive treatment. The patient died on
POD11. The muscle biopsy showed multi-drug resistant Acinetobacter
Calcoaceticus-Baumanii complex, with intermediate sensitivity to Ami-
kacin, Imipenem, Colistin, and Tobramycin. The only other positive
culture was a sputum culture growing the same organism on POD8.
DISCUSSIONS: Necrotizing fasciitis (NF) is a rapidly progressive
infection of the subcutaneous tissues that leads to destruction of fascia
and fat, characterized by fascial necrosis with thrombosis of the
subcutaneous blood vessels, leading to cutaneous gangrene. Type I NF
is a polymicrobial process, often seen in the postoperative setting and
associated with gas formation. Type II NF is usually due to infection
with group A beta-hemolytic streptococci and occasionally in conjunc-
tion with Staph Aureus. Other reported organisms implicated in the
pathogenesis include pseudomonas, Clostridia, Streptococcus pneu-
moniae, serratia, aeromonas, and vibrio. Acinetobacter is a free-living
gram-negative bacterium, commonly found in water and soil. It is an
inhabitant of the human skin. Acinetobacter baumannii is resistant to
all available antibacterials except sublactum1. It has been identified as
a difficult to eradicate nosocomial pathogen with a potential to cause
opportunistic infections, leading to fatal disease in intensive care units.
In the world’s literature, there are only three cases reporting soft tissue
infections caused by multi-drug resistant Acinetobacter1. Acineto-
bacter baumanni has been found to be an increasingly important cause
of nosocomial infections, particularly in ICUs during the period
between 1963-2003. This species has intrinsic resistance to certain
antimicrobial agents, has acquired resistance, and has been reported to
cause blood stream infections in patients at military medical facilities
treating service members injured in Afganistan, Iraq, and Kuwait1.
Since MDRA poses therapeutic difficulties, spread of infection could
be very hazardous.
CONCLUSION: Acinetobacter is an opportunistic pathogen in pa-
tients with severe co-morbidities. Although uncommon as a skin pathogen,
acinetobacter may become a menacing agent in patients with necrotizing
fasciitis. Given the potential of this multi-drug resistant organism to cause
deadly outbreaks, the appropriately compromised host in the infectious
cauldron of an ICU provides the setting for the perfect storm.
REFERENCE:
1 Scott PT et al, Acinetobacter baumannii Infections Among Patients
at Military Medical Facilities Treating Injured U.S. Service Mem-
bers, 2002-2004 Journal of American Medical Association 2004 Dec;
292(24):2964-66
DISCLOSURE: Namrata Patil, None.
DISCLOSURE: Marina Dolina, None.

A CASE OF PULMONARY TUBERCULOSIS AND MILIARY SAR-
COIDOSIS
MULTI-DRUG-RESISTANT ACINETOBACTER FASCIITIS IN A Marilyn Y. Kline MD* Eric M. Leibert MD NYU Medical Center, New
PATIENT WITH PERFORATED ESOPHAGUS York, NY
Namrata Patil MD* Selwyn O. Rogers, Jr MD Brigham and Women’s
Hospital, Boston, MA INTRODUCTION: A miliary pattern of pulmonary sarcoid is rare and
in fact, is difficult to distinguish from miliary tuberculosis. Similarly, bone
INTRODUCTION: Multi-drug resistant Acinetobacter (MDRA) is involvement in sarcoid is infrequent and can be overlooked. We present a
not normally considered a pathogen for postoperative necrotizing fasciitis. patient with known tuberculosis and disseminated sarcoid including

involvement of the spleen, liver, and bone, and presenting in the lung as DISCLOSURE: Marilyn Kline, None.
miliary disease.
CASE PRESENTATION: This is a case of a 53-year-old female with
an established diagnosis of tuberculosis on treatment. The patient is a PULMONARY DIROFILARIASIS PRESENTING AS MULTIPLE
citizen of Panama (who appears African-Carribean) and came to the U.S. PULMONARY NODULES IN A PATIENT WITH A HISTORY OF
in May 2004. She had no other medical history and had a recent normal LYMPHOMA
pap smear and mammogram. She is a never smoker, uses rare alcohol and Chandra K. Katikireddy MD* Ware G. Kuschner MD Stanford University
never used IV drugs. She works in a clothing factory as a clerk, has no Hospital, Palo alto, CA
known occupational exposures, and is HIV negative. In April she was
diagnosed with sputum culture positive pansensitive tuberculosis and was INTRODUCTION: Pulmonary Dirofilariasis (PD) is an unusual in-
started on INH, rifampin, pyrazinamide, and ethambutol. Chest radio- fection manifesting as a solitary or multiple pulmonary nodules mimicking
graph revealed a left basilar infiltrate and miliary disease. In May, the lung carcinoma. This is a case of PD detected incidentally in a patient with
patient developed hepatic failure thought secondary to the tuberculosis a history of stage 4 lymphoma in remission. We are aware of no prior
medications and was admitted to an outside hospital. The patient was reports describing an association between PD and lymphoma.
discharged on streptomycin, levaquin, and ethambutol; sputums were CASE PRESENTATION: A 59 year old man with history of stage 4
AFB culture negative. Six weeks after the initiation of this regimen, the diffuse large B cell lymphoma involving lungs and bone marrow currently
patient complained of dizziness. The patient was admitted to the Bellevue in remission, presented for routine follow-up. The patient was asymptom-
Hospital Chest Service in October for evaluation of her dizziness which atic from a pulmonary perspective. He denied fever, weight loss and other
had then been of 4 1⁄2 months duration. Tuberculosis medications were constitutional symptoms. Physical examination was normal. Surveillance
held. Neurologic exam was non-focal. Chest radiograph showed multiple chest CT showed bilateral pulmonary nodules. PET scan showed in-
small nodules unchanged from June, and resolution of the left infiltrate. A creased uptake in the corresponding areas. CBC showed mild eosinophilia
chest CT showed diffuse perilymphatic small (⬍1cm), well-defined, and metabolic profile was normal. Serological tests for various infections
non-calcified, mainly non-cavitating nodules bilaterally, left basilar scar- were negative. Bone marrow biopsy was negative for marrow relapse of
ring and was interpreted as likely metastatic cancer. No effusions or lymphoma. Lung biopsy showed extensive caseating granulomas with the
adenopathy was noted. An abdominal CT scan noted a lesion in the liver, necrotic areas ringed by palisaded histiocytes and scattered multinucle-
and diffuse granulomatous-type infiltration of the liver and spleen. Osseus ated giant cells. There were many scattered fragments of partially calcified
lucencies suggestive of bone metastases were noted. A bone scan revealed material, some of it with ill-defined cylindrical shapes. The inner structure
uptake in the right femoral neck, T8 vertebra, and the calvarium. A brain morphology was consistent with decaying fragments of Dirofilaria immitis.
MRI revealed multiple metastatic appearing lesions in the calvarium. A Elisa test for IgG4 antibody against filarial antigen was positive at 1: 16.
malignancy work-up ensued. The liver was biopsied with a cytopathology Based on the clinicoradiological and pathological features, the diagnosis of
needle revealing non-necrotizing granulomatous inflammation and no pulmonary dirofilariasis was made.
malignancy. AFB was negative. A bronchoscopy was similarly unrevealing
and AFB was negative. In January, rifabutin and isoniazid were restarted.
The patient refused open-lung biopsy. A calvarial biopsy was performed
revealing multiple non-caseating granulomas and no malignant cells.
Cultures, including AFB, were negative. An ACE level was elevated at
160.
DISCUSSIONS: We diagnosed the patient with sarcoidosis, steroids CASE REPORTS

have been started. There is some resolution of the patient’s pulmonary
nodules. The dizziness, which may be a cranial nerve dysfunction related
to sarcoid, has diminished slightly. Follow-up imaging of the bone, liver
and spleen involvment is planned.
CONCLUSION: We present an unusual case of concurrent tubercu-
losis and sarcoidosis, including a miliary pulmonary pattern without
adenopathy, bone, diffuse liver and spleen involvement and possibly
cranial nerve VIII dysfunction. This patient was initially misdiagnosed
with miliary TB and later worked up for cancer. Miliary TB typically
presents with multiple, small (1-5 mm) nodules that are randomly DISCUSSIONS: Pulmonary dirofilariasis (PD) is a rare zoonotic
positioned in the parenchyma, and along the pleura and fissures. Miliary disease in the United States. Most reported cases are from southeastern,
tuberculosis represents 1-3% of all tuberculosis cases. A miliary presen- eastern and south coastal states. Dashiell described first case of human
tation of sarcoidosis is rare and can be very difficult to distinguish from pulmonary dirofilariasis (PD) infection due to Dirofilaria immitis (DI) in
tuberculosis and malignancy. The overall frequency of bone involvement 1961 (1). PD is a rare entity caused by DI (the dog heart worm), a filarial
in sarcoid is about 3% and is generally asymptomatic. The short bones of nematode that is transmitted to humans by mosquitoes. From the
the hands and feet are usually involved while the long bones, and subcutaneous tissue the worm travels to the heart and then enters the
vertebrae are rarely involved. lungs. Humans are not suitable hosts for this worm, so the larva dies and
REFERENCE: antigens from the dead worm provoke endarteritis and a granulomatous
1 Fraser, Richard S, et al; Diagnosis of Diseases of the Chest, Fourth response. Patients with PD are typically asymptomatic though occasionally
Edition. Vol 3. 1551-1563. they may present with cough, chest pain and hemoptysis. The most

common presentation of PD is a solitary pulmonary nodule discovered on genetic susceptibility to the toxins combined with inability to clear them
a chest radiograph (2). Rarely, PD may present as multiple pulmonary in patients with impaired renal function, especially in those with under-
nodules simulating lung metastases. There are no characteristic clinical or lying immunodeficiency. Features of SSSS in adults include: erythema-
radiological features to distinguish PD from the solitary / multiple tous rash, fever, skin tenderness, positive Nikolsky’s sign, and positive
pulmonary nodule(s) secondary to other causes like primary lung neo- blood cultures. Treatment consists of antibiotics directed toward the
plasm, metastatic pulmonary lesions and various other granulmonatous staphylococcal focus of infection. IVIG has shown efficacy in treating TSS
lesions. Microscopically the nodules consist of coagulation necrosis and and was administered on that basis. However, TSS shares clinical features
caseating granulomas with worms at various stages of disintegration. The with SSSS, and although not studied yet, IVIG may provide some benefit
diagnosis is generally made based on the histopathology. Serological tests in SSSS as well. Toxin testing is ongoing.Our patient’s infectious source
may aid in the diagnosis. Serological titres by enzyme linked immunosor- was bacterial pyomyositis, which led to SSSS due to his chronic renal
bent assay and indirect hemagglutination tests have good sensitivity early insufficiency in conjunction with high dose corticosteroids.
in the course of infection but sensitivity declines as the time interval CONCLUSION: This case highlights two unusual sources of septic
between worm death and serological testing prolongs (3). As these shock, bacterial pyomyositis and SSSS. To our knowledge there have been
organisms are dead, no specific anti dirofilarial treatment is indicated. no previous case reports due to this combination and none in which IVIG
CONCLUSION: One should be aware of and consider pulmonary has been used.
dirofilariasis in the differential diagnosis of solitary/multiple pulmonary REFERENCE:
nodules and granulomatous pulmonary lesions in the appropriate clinical 1 Cribier B, et al: Staphylococcal scalded skin syndrome in adults.
and epidemiologic setting. No anti-helminthic treatment is required as J Am Acad Dermatol 1994; 30: 319-24.
this is the pathological response to the dead parasite.
REFERENCES:
1 Hiroshi Hirano; Tomoshiko Kizaki, Terumasa Sashikata. Pulmonary
Dirofilariasis-Clinicopathological Study Kobe J Med Sci. 2002 48(3):
79-86
2 Asimacopoulos PJ: Katras A; Christie B Pulmonary dirofilariasis.
The largest single-hospital experience. Chest 1992 Sep;102(3):851-5
3 Glickman LT; Grieve RB; Schantz PM Serological diagnosis of
zoonotic pulmonary dirofilariasis Am J Med 1986 Feb; 80(2):161-4.
DISCLOSURE: Chandra Katikireddy, None.
A SHOCKING PAIN IN THE NECK

Allen J. Blaivas DO* Lisa L. Dever MD Rebecca K. Connell MD
UMDNJ-New Jersey Medical School, Newark, NJ
INTRODUCTION: Staphylococcal scalded skin syndrome (SSSS) is

an exceedingly rare diagnosis in adults, with fewer than fifty cases
reported in the literature. We report a case of septic shock that occurred
with associated SSSS secondary to another uncommon diagnosis, bacterial
pyomyositis.
CASE PRESENTATION: A 64-year-old Caucasian male with a past
medical history of diabetes mellitus type 2 and chronic renal insufficiency
presented to the hospital complaining of loss of vision in the left eye that
occurred after the development of a painful vesicular rash on the left DISCLOSURE: Allen Blaivas, None.
forehead and temple. On exam, the patient had palsy of cranial nerves III,
IV, and VI. His laboratory studies were unremarkable except for a
creatinine of 2.7 mg/dl. He was admitted for treatment of herpes-zoster PSEUDOALLESCHERIA BOYDII BREAST IMPLANT INFEC-
ophthalmicus and optic neuritis with intravenous acyclovir and methyl- TION IN A LUNG TRANSPLANT RECIPIENT
prednisolone. On the fifth hospital day, the patient developed an exquis- Hina Sahi MD* Marie Budev DO Robin Avery MD Cleveland Clinic
itely painful and rapidly expanding left neck mass with surrounding Foundation, Cleveland, OH
erythema, fever of 102°F, and white blood cell count of 40 x 109/L (93%
segmented neutrophils). Despite vigorous hydration, the patient became INTRODUCTION: Pseudoallescheria boydii is a ubiquitous fungus
hypotensive and required norepinephrine, and was empirically treated present in soil, sewage and polluted waters. Although the clinical spec-
with vancomycin, clindamycin, and cefepime. His physical exam was trum is wide, the most frequent disease in immunocompetent individuals
significant for crusted lesions around the left eye and temple with a is a mycetoma. An increasing number of cases of disseminated infections
painful diffuse erythematous rash on the left lower face, neck, and chest are reported in immunocompromised patients yet a review of the lung
with areas of skin sloughing. Nikolsky’s sign was present. The left neck was transplant literature yields relatively few cases of disseminated infection.
markedly tender with diffuse swelling and a 4 cm area of induration on the The clinical and histopathological presentation of Psedoallescheria boydii
lateral aspect of sternocleidomastoid muscle (SCMM). A noncontrast CT infection is similar to Aspergillus spp and Fusarium spp; therefore culture
scan of the neck revealed multiple locules of air with extensive surround- is necessary to make the correct diagnosis. This is especially relevant since
ing phlegmonous changes of the left SCMM without mature abscess. The infection due to Psedoallescheria boydii is often resistant to antimycotic
patient was taken emergently to the operating room for incision and drugs such as Amphoterecin B and Flucytosine, which are commonly used
drainage of the left SCMM. Operative findings were significant for an to treat Aspergillus infections.
indurated left SCMM with extensive seropurulent discharge without CASE PRESENTATION: A 57-year-old female patient, status post a
discrete abscess. The infectious tract extended from the mastoid bone right single lung transplant 14 months ago for COPD, presented to the
down to the anterior chest wall and anterior to the level of the clavicle and hospital with complaints of severe left mastalgia. She had bilateral silicone
sternum. Blood and intraoperative wound cultures grew Staphylococcus breast implants placed twenty five years ago. She was maintained on
aureus, supporting the diagnosis of bacterial pyomyositis with SSSS. As routine triple drug therapy consisting of Tacrolimus, Mycophenolate
the patient exhibited some signs of toxic shock syndrome (TSS) and his mofetil and Prednisone. She was also on routine opportunistic infection
condition was critical, he was given intravenous immunoglobulin (IVIG). prophylaxis with Itraconazole, Valgancyclovir and Trimethoprim-Sulfa-
He improved initially and no longer required pressor-support, however, methaoxazole. Recently the patient was diagnosed with Grade A1B0 acute
the patient subsequently developed nosocomial infections that prolonged rejection on surveillance bronchoscopy which had not resolved inspite of
his hospital stay and led to ventilator dependence. two treatments with pulse steroids. The patient reported a one week
DISCUSSIONS: SSSS is caused by infection with an exfoliative history of severe, continuous left breast pain with concomitant right sided
exotoxin secreting S. aureus. SSSS is primarily a disease of children and pleuritic chest pain. In addition she had developed painful skin lesions on
neonates which carries a good prognosis. However, it is very rare in adults both thighs and blurring of vision. On examination the left breast was
and has a reported mortality of 60%. SSSS is believed to be due to a swollen, warm and exquisitely tender to touch around the implant capsule.

She had multiple skin lesions which were approximately one centimeter in interconnected vascular spaces. Focally, there was a prominent lymphop-
diameter, red, warm, tender and nonblanching. Neurological exam re- lasmacystic infiltrate with active fibrosis and cholesterol deposition. There
vealed ptosis of the left eyelid and third cranial nerve palsy. CT scan chest was no evidence of malignancy.
showed a loculated right pleural effusion suggestive of empyema and a
persistent right lower lobe parenchymal nodule. Skin biopsy from the
thigh lesion revealed a fungal abscess with pseudohyphae consistent with
Pseudoallescheria spp. An MRI breast revealed marked inflammatory
enhancement around the left silicone breast implant. Bilateral breast
implants were emergently surgically removed. Microscopic evaluation of
the left implant capsule demonstrated evidence of invasion by fungal
hyphae. MRI brain revealed multiple ring enhancing lesions in the
parenchyma suggestive of a hematogenously disseminated infection. The
patient was treated with high dose intravenous Voriconazole. Multiple
tissue cultures grew Pseudoallescheria boydii. We suspect that the initial
nidus was the pulmonary nodule, with hematogenous dissemination at a
time of increased immunosuppression. We were unable to identify a
definite exposure inspite of extensive questioning. This makes it difficult
to predict any future re-infection.
DISCUSSIONS: P.boydii may cause life-threatening illness in the lung
transplant population. Early diagnosis should be done by fungal culture so
that adequate therapy may be initiated. In the pre Voriconazlole era,
disseminated infection in the lung transplant population was uniformly
fatal. However in recent years there have been some treatment successes
with the use of Voriconazole. To our knowledge this is the first reported
case of breast implant infection with Psedoallescheria boydii in the lung
transplant population. This raises questions regarding the safety of these
foreign bodies in an immunosuppressed population prone to opportunistic
infections. There may be a case here for removal of such foreign bodies
prior to transplant. We reviewed our lung transplant database but could
not identify any other case of breast implant infection.
CONCLUSION: Cosmetic foreign bodies in a lung transplant recipi-
ent have the potential to be infected, due to the immune suppressed
nature of the patient. There should be a low threshold to remove such
implants during the pre-transplant work up, if agreeable to the patient.
DISCLOSURE: Hina Sahi, None.
Surprising Surgical Successes

2:00 PM - 3:30 PM
ANTERIOR MEDIASTINAL CAVERNOUS HEMANGIOMA:
CASE PRESENTATION AND REVIEW OF LITERATURE
Luis M. Argote-Greene MD* Michael T. Jaklitsch MD Lambros Zellos
MD David J. Sugarbaker MD Brigham and Women’s Hospital, Harvard
Medical School, Boston, MA
CASE REPORTS
INTRODUCTION: Anterior mediastinal masses deserve prompt di-
agnosis to rule out malignant conditions. We review the literature and
present a rare case of benign cavernous hemangioma of the anterior
mediastinum.
CASE PRESENTATION: The patient was a 57-year-old male who DISCUSSIONS: Hemangiomas are rare mediastinal tumors. They
was referred for evaluation of an anterior mediastinal mass. His past account for 0.5% of mediastinal masses. Anatomically they may arise in
medical history was significant for colon cancer treated with adjuvant the anterior or posterior mediastinum. They are categorized as capillary,
chemotherapy two years earlier, hypertension, paroxysmal atrial fibrilla- cavernous, and venous. Microscopically they are formed by dilated vessels,
tion refractory to multiple cardioversion attempts, for which he was on lined by endothelial cells, with fibrous septae. They might contain fat,
Coumadin. He was a former 16-pack year smoker with an allergy to fibrous tissue, localized areas of thrombus, cholesterol deposition, and
contrast dye.A chest CT revealed a 6.3 x 4.10 cm mass in the anterior calcification. Dr. Goldstraw reports that patients may present with
mediastinum with marked circumferential calcification. Hounsfield units symptoms suggestive of advanced malignancy, like hoarseness, recurrent
were consistent with a soft tissue mass. Physical exam revealed an pleural effusions, and superior vena cava obstruction. These lesions have
irregular cardiac rate and rhythm but was otherwise normal. A cardiac not been found to be prone to malignant degeneration. VATS resection
stress test showed no signs of ischemia and no evidence of hemodynam- was not possible in this case since the osseous shell mandated a portal
ically significant coronary artery disease. The radiological report con- incision at least the diameter of the tumor. Symtoms from these tumors
cluded that the lesion was most likely consistent with thymoma, with arise from compression of adjacent structures. These vascular tumors have
possible consideration of a calcified thymic cyst. The mass was resected been treated in children with alpha-2a interferon. These might be used to
through a left parasternal approach with resection of the 3rd cartilage. The reduce the volume of a bulky tumor and increase the safety in patients
mass adhered to the left thymic pole , left pleura and underlying with vital structure encasement.Differential diagnosis of calcified medi-
pericardium, all of which was resected en bloc. . The chest tube was astinal masses are calcified thymic cysts, recurring tracheal leiomyoma,
removed on postoperative day #1 and the patient was discharged home on mediastinal teratoma, and calcified lymph nodes usually due to fungal
postoperative day #2. Pathology revealed a calcified cavernous hemangi- disease.
oma with associated secondary changes. It measured 7.9 x 5.4 x 4.2 cm. CONCLUSION: Most anterior mediastinal masses are malignant, and
(68.2 g). Microscopic examination revealed multilocular cysts representing should be biopsied. Incisional biopsy was precluded in this case due to the

Surprising Surgical Successes, continued
calcified shell. Excisional biopsy through a limited incision allowed PULMONARY MUCINOUS CYSTADENOCARCINOMA: A CASE
complete removal of the tumor. Allows resolution of associated compres- REPORT AND REVIEW OF THE LITERATURE
sion symptoms, and establishes the diagnosis with certainty. Melhem A. Imad MD* Vicki Schnadig MD Adegboyega Patrick MD
DISCLOSURE: Luis Argote-Greene, None. Gulshan Sharma MBBS University of Texas Medical Branch, Galveston,
TX
INTRODUCTION: Mucinous Cystadenocarcinoma (MCA) is a rare

primary tumor of the lung.
USE OF ABIOMED BVS-5000 AS ‘BRIDGE’ IN THE REPAIR OF CASE PRESENTATION: We present a case of a 64-year-old previ-
POST-INFARCT LEFT VENTRICULAR RUPTURE ously healthy female evaluated for 6-month history of non-productive
Rammohan Marla MD* Robert C. Gallagher MD Fred G. Rubin MD cough. She is a life long non-smoker. Her physical exam revealed mildly
Kevin P. Keating MD University of Connecticut, Hartford Hospital, decreased breath sounds over the left upper hemithorax. A chest X-Ray
Hartford, CT revealed a left upper lobe mass confirmed by a CT scan of the thorax. An
attempt to make tissue diagnosis by CT guided transthoracic biopsy
INTRODUCTION: Incidence of left ventricular free wall rupture is yielded abundant mucin with rare atypical epithelial cells. Inconclusive
about 7%-24% following acute myocardial infarction. Mortality is up to core biopsies and patient’s persistent symptoms required surgical resec-
90% without surgery. Hemodynamic status of the patient prior to surgery tion. During surgery, significant adhesions were noticed and a left
and the extent of myocardial necrosis are the major factors influencing the pneumonectomy was performed for complete removal. Pathologic exam-
surgical outcome. Ventricular assist devices (VADs) have been success- ination revealed a well-circumscribed spherical mass measuring 8x8x6 cm
fully used as a “bridge to recovery” after myocardial infarction. We report in the posterior superior segment of the right upper lobe. Microscopically,
a case in which the Abiomed BVS-5000 left ventricular assist device there was large cysts filled with mucin and lined by an atypical columnar
(VAD) was used as ‘bridge’ to stabilize the patient following post- epithelium with areas of invasion of lung parenchyma consistent with the
infarction left ventricular rupture. diagnosis of mucinous cystadenocarcinoma (MCA). Work up for a primary
CASE PRESENTATION: A 66-year old male was emergently oper- site other than the lung was unrevealing. The patient had a favorable post
ated due to left ventricular rupture secondary to acute posterolateral operative course and had no signs of recurrence at 6-month post resection.
myocardial infarction. Extensive necrosis was noted in the posterolateral
wall of the left ventricle extending to the apical region. No attempt was
made to perform definitive repair at that time due to the presence of
extensive necrosis. Abiomed BVS-500 LVAD was placed using left atrium
and aorta for the inflow and outflow conduits respectively. The patient was
successfully weaned off cardiopulmonary bypass (CPB) on moderate
pressor support. Postoperative anticoagulant regimen included heparin
and clopidogrel. He was extubated on 3rd postoperative day. Cardiac
catheterization on 6th postoperative day showed subtotal occlusion of two
marginal arteries. Plan was made to explant LVAD and repair LV on 10th
postoperative day. On 9th postoperative day, moderate decrease in LVAD
flows was noted. Patient was taken to surgery. Left ventricular wall
appeared to have reasonably well healed. LVAD was explanted and left
ventricle was repaired using an endocardial pericardial patch and but-
tressed with epicardial teflon felt. Subsequent postoperative recovery was
uneventful. He was discharged home on 14th postoperative day . Echo-
cardiography at the time of discharge showed an ejection fraction of
40-45%. He returned back to full work 3 months after surgery.
DISCUSSIONS: : Mortality rates following repair of ruptured ventri-
cle vary depending on the hemodynamic status of the patient at the time
of repair. Various approaches have been described1,2. These included
definitive immediate repair with various patch techniques, both sutured
and sutureless3. Definitive primary surgical repair of post-infarction
myocardial rupture is always the first option if feasible. However our
patient was in severe cardiogenic shock at the time of initial surgery. We
postulated that LVAD support would allow time for organ recovery and
would greatly diminish left ventricular diastolic and systolic pressures,
therebylessening the chance for further bleeding, and would allow time
for better demarcation of necrotic myocardium and formation of adequate
scar tissue. Definitive repair could therefore be performed under more
controlled conditions with the myocardium holding the repair better. The
ideal time period for adequate myocardial scar tissue formation following
extensive necrosis is not known, although we chose to wait 10 days. To the
best of our knowledge, this is the first such repair using the Abiomed
LVAD BVS 5000 as a ‘bridge’ to recovery.
CONCLUSION: LVAD can be used in selected cases of post-
infarction myocardial rupture in the presence of extensive myocardial
necrosis as a ‘bridge’ to recovery. Definitive repair can be undertaken
once adequate myocardial scar has formed and the patient has recovered DISCUSSIONS: Mucinous cystadenocarcinoma, a variant of adeno-
from shock. carcinoma, is a rare primary pulmonary tumor. MCA belongs to a group
REFERENCES: of tumors characterized by copious mucin production usually occurring in
1 Iemura J, Oku H, Otaki M et al. Surgical strategy for left ventricular the ovary, breast, pancreas or the gastro intestinal tract. First described by
free wall rupture after myocardial infarction. Ann Thorac Surg 2001; Gowar in 1978, it wasn’t until 1999 that this group of tumors was divided
71(1): 201-204 into three different entities: Mucinous adenoma, mucinous carcinoma and
2 Reardon MJ, Carr CL, Diamond A et al. Ischemic left ventricular mucinous cystadenocarcinoma. The exact prevalence of primary pulmo-
free wall rupture: prediction, diagnosis, and treatment. Ann Thorac nary MCA is difficult to estimate due to the confusion in classification of
Surg 1997; 64(5): 1509-1513 these tumors in the past and the inconsistencies in the terminology. When
3 Lachapelle K, deVarennes B, Ergina PL et al. 3.Sutureless patch symptomatic, pulmonary MCA presents as cough, chest pain or weight
technique for post infarction left ventricular rupture. Ann Thorac loss. Radiographically, MCA appears as a benign cyst with low attenuation
Surg 2002; 74:96-101 and characteristic enhancing septa on CT scan. Grossly MCA is a
DISCLOSURE: Rammohan Marla, None. peripherally located well-demarcated tumor ranging from 1 to 13 cm in

diameter. Pathologically, MCA is composed of cysts filled with copious CONCLUSION: The benefits of LASER(NdYAG) ablation include
amounts of extracellular mucus lined by a small number of columnar the fact that it is minimally invasive, can be repeated if tumor regrows,
mucin producing neoplastic cells showing various degrees of atypia. A reduces hospital stay and cost. This patient has lived up to 44 months since
distinct cytopathologic feature of MCA is having invasive adenocarcinoma diagnosis of Anaplastic thyroid cancer and seven months since interven-
foci, or groups of atypical cells suspended in pools of mucin. Although tion without any recurrence of airway symptoms.
MCA cytologically resembles mucinous broncho-alveolar carcinoma, the REFERENCES:
former seems to have a more favorable prognosis. Surgical resection is the 1 Jia,B.Advances in Diagnosis and Management of Thyroid Neo-
treatment of choice with a favorable long-term survival. Finally, in plasms:Current opinions in Oncology 2000;12;54-59.
considering the diagnosis of this rare neoplasm, the most important task 2 Pasieka.Anaplastic Thyroid Cancer.Current opinions in Oncology
for the clinician is to exclude an extrapulmonary site of origin. 2003;15;78-83.
CONCLUSION: Although rare in the lung, MCA should be included 3 Mathur,P,Mehta,A.Seminars in Respiratory and Critical Care Med-
as part of the differential of a benign appearing lung mass. Aspiration of icine.25;4;AUG.2004
mucinous material by CT guided or transbronchial biopsy should further
raise the suspicion for this tumor. When presenting as a pulmonary mass,
an extrapulmonary site of origin should be ruled out. Surgical resection
offers long-term cure.
DISCLOSURE: Melhem Imad, None.
ENBLOC RESECTION OF SUBGLOTTIC ENDOTRACHEAL

TUMOR IN A PATIENT WITH KNOWN ANAPLASTIC THYROID
CANCER
Abdur R. Shad MD* Fariborz Ashtyani MD Hackensack University
Medical Center UMDNJ, Hackensack, NJ
INTRODUCTION: Anaplastic thyroid carcinoma is a highly aggres-

sive tumor with very poor survival. Despite chemotherapy and radiation,
survival is only a matter of months. Recurrence with tumor invasion of
vital cervical structures and airway obstruction leads to death.
CASE PRESENTATION: A 53 year old gentleman with history of
benign thyroid goiter confirmed by thyroid scan about 20 years ago. He
noted a rather rapid increase in his goiter about 4 years ago. An incisional
biopsy revealed anaplastic thyroid carcinoma (spindle cell). Patient un-
derwent tumor debulking 3 months after diagnosis. He had a prophylactic
Tracheostomy done prior to receiving chemotherapy and radiation. He
had a complete remission and was decannulated. Patient had moderate
tracheal stenosis secondary to tracheostomy and extensive mediastinal
fibrosis at the site of radiation causing chronic stridor in the past 4 years.
Initially paient was followed by CT scan but he stopped follow up for the
past 2 years. Four weeks prior to the current admission, he developed
severe dyspnea on exertion. CT scans revealed no distant metastases, but
a flow volume loop showed fixed obstruction and the patient underwent
flexible bronchoscopy. Bronchoscopy revealed a 7 mm subglottic endo-
tracheal mass approximately 10 mm below the vocal cords (see photo) and
a subglottic stenosis (5 mm lumen) below the mass at the level of the
previous tracheostomy. Virtual bronchoscopy was performed and the
patient underwent several surgical evaluations and was deemed inopera-
ble secondary to the extent of tumor invasion. The patient agreed to rigid
bronchoscopy with Laser ablation of endotracheal tumor, with capability
for emergent cardiopulmonary bypass if necessary. ND:Yag LASER
coagulation was performed of the endotracheal lesion which was highly
CASE REPORTS
friable and bled easily. A coagulating snare was then introduced for
further debulking. Pathologic evaluation revealed fibrovascular tumor
with no malignant cells. The patient had immediate relief of symptoms
post-operatively with and improvement of FEV1 from 53% to 79%
predicted.
DISCUSSIONS: Anaplastic thyroid carcinoma is a lethal tumor with a
14% survival rate at 10 years, and a mean survival of six months. The
tumor causes external compression or with endotracheal growth, obstruc-
tion with asphyxiation. Options for treating advanced disease are limited.
Radiation, while initially leads to regression of tumor, can also stimulate
future recurrence. Indium ablation, external-beam or intraoperative
radiation therapy can be useful in controlling symptoms related to local
tumor recurrences. Tracheal resection and re-anastomosis is a technically
difficult or virtually impossible undertaking due to local tissue invasion. DISCLOSURE: Abdur Shad, None.
The patients usually die of respiratoy obstruction or local invasion of the
tumor. LASER has been used to remove central airway obstruction and
improve survival. This case is unique because the patient did not have REMISSION OF IDIOPATHIC PULMONARY ARTERY HYPER-
recurrence of the Anaplastic tumor or distant metastases. The discovery of TENSION DISCOVERED AFTER REJECTION OF A SINGLE
a tumor in his trachea was suggestive of recurrence of the Anaplastic LUNG TRANSPLANT
Thyroid Ca. Surgery was not an option due to post radiation changes. Joon S. Yun MD* Subrato Deb MD Edward Omron MD Stephen Nathan
When tumors are causing airway obstruction LASER ablation is the MD National Naval Medical Center, Bethesda, MD
treatment of choice. We were able to not only relieve his symptoms,
but also diagnose a benign lesion. There are reports of malignant INTRODUCTION: Idiopathic Pulmonary Artery Hypertension
tumor recurrences after radiation therapy of Thyroid tumors, but there (IPAH) is a fatal disease in which progressive pulmonary arterial hyper-
is no report of a benign fibrovascular tumor in the literature. tension leads to right heart failure and death. Recent advances in the

medical management of this disease has successfully delayed the necessity supporting laboratory/radiographic evidence the working diagnosis is
of lung transplant but has not resulted in remissions. This case report still IPAH.
details an unusual remission of IPAH. CONCLUSION: This case report details the remission of IPAH in
CASE PRESENTATION: A 40 year-old African-American female a long term survivor of a single lung transplant. Many patients have
with a prior history of IPAH s/p single lung transplant 10 years ago benefited from new therapies for PAH such as epoprostenol and
presents to her new pulmonologist after discharge from her transplant bosentan limiting the number of patients that progress on to surgical
center with a diagnosis of chronic rejection of her lung. She was intervention. The option of lung transplant still has a role and in this
initially diagnosed with IPAH 12 years earlier after presenting with intriguing patient may have assisted in a remission.
recurrent syncope and progressive dyspnea. Elevated pulmonary artery
systolic pressure (PASP) 80mmHg on echocardiogram was confirmed
with right heart catheterization (RHC). After developing profound
fatigue with overt right heart failure she underwent successful left
single lung transplant with normalization of right heart function and
was maintained on tacrolimus and mycophenolate mofetil. The next
ten years were complicated by episodes of acute rejection, pneumonia
and development of severe hypertension with chronic kidney disease.
Renal biopsy showed Focal Segmental Glomerulosclerosis (FSGS)
presumed to be secondary to tacrolimus. Spring 2004 she developed a
persistent non-productive cough with mild but progressive dyspnea
and eventually presented to her pulmonologist. Her chest radiograph
revealed complete atelectasis of her left lung with proximal air-
bronchograms and was immediately referred to her transplant center
for further evaluation. Bronchoscopy was performed with unsuccessful
transbronchial biopsies due to obliteration of her small airways. Laser
ablation of her left mainstem anastomosis site with wire stent place-
ment was performed without improvement. She was discharged with
presumed chronic rejection of her transplanted lung and tapering of
her immunosuppressive regimen. Complete functional loss of her
transplanted lung was confirmed by V/Q scan and an echocardiogram
with follow-up RHC revealing only mild pulmonary hypertension with
PASP 44mmHg. An evaluation for secondary pulmonary artery hyper-
tension was unremarkable. Persistent fatigue and malaise with wors-
ening chronic cough and no clinical utility of preserving her rejected
lung prompted the decision to recommend resecting the source of her
symptoms. She underwent a successful resection of her transplanted
lung January 2005 after discontinuing her tacrolimus with immediate
resolution of her symptoms. Follow-up echocardiography 2 months
after resection revealed stable mild pulmonary hypertension without
treatment.
DISCLOSURE: Joon Yun, None.
DIAGNOSIS OF A PULMONARY ARTERY SARCOMA BY ENDO-

VASCULAR BIOPSY
Rajashekar Adurty MD* Brian Carlin MD Sanjay Godara MD Gustav
Eles MD Donald Fisher MD Division of Pulmonary and Critical Care
Medicine, Allegheny General Hospital, Pittsburgh, PA
INTRODUCTION: Pulmonary artery sarcoma is a rare vascular

tumor. The diagnosis is rarely made antemortem. We present an
unusual case of a pulmonary artery mass in a patient with a horseshoe
kidney that was diagnosed via an endovascular biopsy procedure.
CASE PRESENTATION: A 37-year-old healthy man developed
chest discomfort, worsening shortness of breath, and palpitations over
a ten month period. He was treated for recurrent episodes of
bronchitis. With progressive dyspnea on exertion to the point he was
unable to walk across the room without significant shortness of breath,
DISCUSSIONS: This unique case of IPAH that resolved after an a repeat echocardiogram now showed a large solid mass (5 cm by 3 cm)
astounding 10 year survival of a single lung transplant presents a in the main pulmonary artery and right ventricular dysfunction. A
glimpse into the role of pressure and the abnormal response of the computed tomography scan revealed a large filling defect in the main
pulmonary artery endothelium in the pathogenesis of this disease. Our pulmonary artery and was felt to be a saddle embolus. Additionally, a
patient was near death when her right pulmonary vasculature was horseshoe kidney was noted. Upon transfer to our institution, a cardiac
unloaded by the shunted flow through the transplanted lung. 10 years
MRI scan confirmed the irregularly shaped mass in the pulmonary
later as my patient lost her transplanted lung to bronchiolitis obliter-
ans, a sentinel event for most transplant recipients, resulting in only artery. Due to the chronicity of his symptoms, a tumor of the
modest symptoms. Whether the remodeling of her native pulmonary pulmonary artery was suspected. A pulmonary artery angiogram
vasculature was directly due to hemodynamic offloading, an unknown revealed the mass obstructing 80% of the lumen of the main pulmo-
process or an inadvertently treated occult secondary pulmonary artery nary artery. An endovascular biopsy was performed then using myo-
hypertension (SPAH) has yet to be discovered. The SPAH candidates cardial biopsy forceps. Surgical pathology and cytology revealed a high
that may have responded to her immunosuppressive regimen include grade sarcoma. No metastatic disease was detected. He underwent
vasculitides such as Takayasu Arteritis, collagen vascular diseases as surgical excision with reconstruction of the pulmonary artery two
well as Sarcoidosis. Without a clinical suspicion for these entities or weeks later and is alive one year later.

diagnosis prior to the intervention allowing for appropriate therapy to be

afforded.
REFERENCES:
1 JM Parish et al. Pulmonary artery sarcoma:Clinical features Chest
1996 110: 1480-1488.
2 Joseph E. Cox et al. Pulmonary artery sarcomas: A review of clinical
and radiological features. Journal of Computer Assisted Tomogra-
phy 1997 21(5):750-755
DISCLOSURE: Rajashekar Adurty, None.
Unusual Cases of Hemoptysis and

Pneumothorax
2:00 PM - 3:30 PM
PULMONARY HEMATOMAS COMPLICATING PNEUMOTHO-
RAX TREATMENT WITH TUBE THORACOSTOMY
Jason C. Graff MD* Jason M. Huddleston DO Gary A. Salzman MD
University of Missouri-Kansas City, Kansas City, MO
INTRODUCTION: The authors present a patient with emphysema

who developed pulmonary hematomas as a consequence of anticoagula-
tion and rapid lung re-expansion after tube thoracostomy.
CASE PRESENTATION: An 80-year-old African American male with
emphysema was admitted to the hospital for a one week history of shortness
of breath. On admission, a CT pulmonary angiogram revealed chronic
emphysematous changes without bullae, as well as a filling defect within the
left main pulmonary artery consistent with pulmonary embolism. Intravenous
unfractionated heparin was administered. On hospital day four, the patient
developed sudden respiratory distress and hypotension, and a chest X-ray
demonstrated a large right-sided secondary spontaneous pneumothorax. A
chest tube was placed emergently and immediately connected to a pleural
drainage system and 20 cm of wall suction. At the time, the protime and
platelet count were normal, and the partial thromboplastin time was 86.4
seconds. A chest X-ray done after tube thoracostomy showed complete
expansion of the right lung and scattered right-sided infiltrates thought to
represent re-expansion pulmonary edema. Three hours later, another chest
film showed two well-demarcated masses within the right lung (Figure 1). A
repeat CT scan revealed two large intraparenchymal pulmonary hematomas
measuring 10.5 x 6.6 cm and 9.1 x 6.1 cm, and the chest tube positioned
within the pleural space (Figure 2). The patient’s hemoglobin subsequently
dropped from 12.7 to 9.6 g/dl over 48 hours. Anticoagulation was reversed,
and an inferior vena cava filter was placed. The size of the hematomas
remained stable, and the patient was able to be discharged home after chest
DISCUSSIONS: Antemorem diagnosis of a pulmonary artery sarcoma tube removal.
is unusual. In such instances, the diagnosis is usually suspected and DISCUSSIONS: Pulmonary hematomas commonly occur due to blunt
confirmed only at the time of surgical excision. We describe a case in or penetrating thoracic trauma or after thoracic surgery1. These hemato-
which the diagnosis was suspected and confirmed via an endovascular mas have been infrequently reported as a complication of systemic
biopsy.Pulmonary artery sarcomas are rare malignant tumors with about anticoagulation2,3, thrombocytopenia4, and subclavian vein catheteriza-
138 cases reported in the literature. The clinical presentation may include tion. Bullous emphysema is often a concomitant factor. To our knowledge,
CASE REPORTS
chest pain, cough, hemoptysis, or dyspnea. Various laboratory studies will pulmonary hematomas occurring after tube thoracostomy and rapid lung
assist with the diagnosis. An echocardiogram may show the lesion in the re-expansion have not yet been reported. The most common presenting
pulmonary artery and right ventricular strain and a computed tomography symptoms of pulmonary hematoma include hemoptysis, chest pain, and
scan may also show a filling defect in the pulmonary artery. In many transitory dyspnea. Radiographically they commonly present as discrete
instances a pulmonary embolism is suspected. Cardiac MRI has rarely bizarrely shaped masses5 or “coin lesions.” The pathogenesis of pulmonary
been used in the diagnosis of pulmonary artery tumors but characteristics hematomas depends upon the underlying cause. In blunt thoracic trauma,
showing signals consistent with tissue compared to thromboembolism are parenchymal tearing due to transmitted sheer forces results in rupture of
noted. Pulmonary angiography will help to define the extent of the lesion. small vessels, capillaries, and alveoli, leading to interstitial hemorrhage1.
Unfortunately none of the imaging studies can confirm the diagnosis. A We propose that similar sheer forces can be generated when a lung is
biopsy can be difficult to obtain unless obtained by surgical intervention. rapidly re-expanded following treatment of pneumothorax with tube
While chronic thromboembolism was considered, the progressive nature thoracostomy. Underlying emphysema and anticoagulation were contrib-
of the dyspnea over a ten month period in conjunction with the MRI uting factors in this case. Pulmonary hematomas usually resolve within
findings led us to suspect a pulmonary artery tumor as the cause for the two to four weeks, but some may remain for an extended period of time.
patient’s symptoms. During the pulmonary angiography, a sample of the These blood collections can become infected, presenting as an abscess
mass was obtained using myocardial biopsy forceps using fluoroscopic requiring antimicrobials and drainage1.
guidance without complications. Interestingly, a horseshoe kidney was CONCLUSION: Underlying emphysema, anticoagulation, and exces-
found in this patient. Various anomalies of other body systems (e.g. Wilms’ sive sheer forces induced by rapid lung expansion after chest tube
tumor, Renal cell carcinoma, Renal sarcoma, and Carcinoid tumor) have placement contributed to the development of pulmonary hematomas in
been reported and in this young patient, a relationship between these two this patient. Avoiding the immediate use of suction in treating pneumo-
diseases could be suspected. thorax may prevent this complication, especially in anticoagulated emphy-
CONCLUSION: We present an unusual case of a pulmonary artery sema patients.
sarcoma (associated with a horseshoe kidney) that was diagnosed prior to REFERENCES:
surgery via an endovascular biopsy procedure. This procedure was 1 Errion AR, Houk VN, Ketterling DL. Pulmonary hematoma due to
performed safely and afforded the diagnosis with minimal morbidity. blunt non-penetrating thoracic trauma. Am Rev Respir Dis 1963;
Surgical intervention could then be performed with knowledge of the 88: 384-92.

Pneumothorax, continued
2 Charkraborty AK, Dreisen RB. Pulmonary hematoma secondary to menses. Bronchial lavage obtained from these areas did not show
anticoagulant therapy. Ann Intern Med 1982; 96:67-69. endometrial cells. Biopsies were not performed. Given the strong clinical
3 Kaira K, Takei Y, Matsuura M, et al. Pulmonary hematoma resulting suspicion of TES, she was started empirically on danazol and became
from anticoagulant therapy. Am J Roentgenol 2003; 180:1740. amenorrheic. She experienced another episode of life-threatening hemop-
4 Luna MA, Leary WV, Jing BS. Pulmonary hematoma associated tysis approximately 3 months after starting danazol therapy, coincident
with thrombocytopenia. Chest 1970; 57:487-89. with an infectious exacerbation of CF, and subsequently underwent
5 Williams JR. The vanishing lung tumor: Pulmonary hematoma. Am J double lung transplantation. Histological evaluation of her native lungs did
Roentgenol 1959; 81:296. not demonstrate the presence of endometrial tissue. Three months
post-transplant, she has had no further episodes of hemoptysis.
DISCUSSIONS: Although a histopathologic confirmation was not
obtained in this patient, the clinical history, as well as the dynamic
radiographic and bronchoscopic appearance, strongly suggest a diagnosis
of TES. In previous reports, the volume of blood expectorated in the
setting of catamenial hemoptysis is usually less than 200 cc, and we
propose that the massive nature of the hemoptysis experienced by this
patient may be a consequence of the bronchial arterial collateral circula-
tion that had developed due to underlying bronchiectasis. The optimal
treatment of TES is uncertain, with surgical resection and ovarian
suppression being the most widely reported3; danazol is reported to be
effective, although treatment failures are described1. In this case, trans-
plantation would be expected to be curative, although the risk of
recurrence of pulmonary endometriosis in the lung allograft is unknown.
CONCLUSION: This is the first report of catamenial hemoptysis and
pneumothoraces occurring in a patient with CF. The clinical presentation
is consistent with TES.
REFERENCES:
1 Joseph J, Sahn SA. Thoracic endometriosis syndrome: New obser-
vations from an analysis of 110 cases. Am J Med 1996; 100: 164-170
2 Wood DJ, Krishna K, Stocks P et al. Catamenial hemoptysis: a rare
cause. Thorax 1993; 48: 1048-1049
3 Ziedalski TM, Sankaranarayanan V, Chitkara RJ. Thoracic endome-
triosis: A case report and literature review. J Thorac Cardiovasc Surg
2004; 127: 1513-1514
DISCLOSURE: Jason Graff, None.
CATAMENIAL HEMOPTYSIS AND PNEUMOTHORACES IN A

PATIENT WITH CYSTIC FIBROSIS
Chris M. Parker MD* Robert Nolan MD M. D. Lougheed MD Queen’s
University, Kingston, ON, Canada
INTRODUCTION: Catamenial hemoptysis and pneumothorax are

rare conditions that occur as part of the thoracic endometriosis syndrome
(TES). Fewer than 30 cases of catamenial hemoptysis are described in the
literature1. Diagnosis of TES is often made on the basis of the clinical
history of symptoms that recur with menses; a histopathologic diagnosis
was only established in approximately one-third of reported cases2. We
report a case of a patient with cystic fibrosis (CF) and a history of
catamenial pneumothoraces who presented with life-threatening catame-
nial hemoptysis.
CASE PRESENTATION: Over several months, a 32-year-old female
with CF (⌬F508, ⌬I507 phenotype) had experienced several episodes of DISCLOSURE: Chris Parker, None.
mild, streaky hemoptysis, and two episodes of spontaneous pneumothorax,
which coincided with the onset of menses. In September 2004, she was
assessed following an episode of sudden massive hemoptysis (⬃500 cc), BENIGN METASTASIZING LEIOMYOMA PRESENTING WITH
occurring on the second day of menses, during which she collapsed and SPONTANEOUS PNEUMOTHORAX
required resuscitation. Prior to this event, she had felt relatively well, her Deepa G. Lazarous MBBS* Edward Tsou MD Eric Anderson MD Anne
baseline lung function had remained stable (FEV1 30-40% predicted), E. O’Donnell MD Georgetown University Medical Center, Bethesda,
and there were no symptoms to suggest an antecedent infectious exacer- MD
bation. She was nonetheless treated with antibiotics and recovered. With
the onset of her next menstrual cycle she experienced recurrent streaky INTRODUCTION: Benign metastasizing leiomyoma (BML) is a rare
hemoptysis, and subsequently underwent bronchial angiography and disorder in which myomatous tissue thought to be of uterine etiology is
embolization of bilateral ectatic bronchial arteries. A CT scan obtained at found in other organs, most commonly lungs and lymphoid tissue. Usually
the end of menses demonstrated the presence of pulmonary parenchymal BML is seen after myomectomy and hysterectomy. To our knowledge it
lesions, consistent with hemorrhage, that resolved completely on a has never been reported to present with spontaneous pneumothorax and
follow-up CT obtained midway through her menstrual cycle (Figure 1). has not been described after uterine artery embolization (UAE). We
Thoracic T2-weighted MRI, obtained at the end of menses, demonstrated report a case of BML presenting as spontaneous pneumothorax 1 year
hyperintense lesions (consistent with hemorrhage or endometrial tissue) after UAE.
localized to the left apical pleura, and pulmonary parenchymal lesions CASE PRESENTATION: 32 yr old African American female who
were also seen corresponding to those visualized on thoracic CT. Bron- presented to the emergency room with progressive dyspnea and chest
choscopy, performed premenstrually, showed at least two friable purplish tightness of 2 days duration. Past medical history was significant for
lesions in her segmental bronchi, which were no longer evident after uterine fibroids for which she had underwent a myomectomy in 1998 and

UAE in 2003.A chest x-ray upon presentation showed complete pneumo- theories exist, but vascular dissemination and hematogenous spread
thorax on the left side and multiple small nodules on the right. A CT scan during surgical manipulation seems to be the most accepted one. How-
confirmed multiple small nodules, some of which were cavitating. Tho- ever, there are case reports in which pulmonary nodules have been seen
racoscopic biopsies and pleurodesis were performed. Pathology revealed prior to any surgical intervention of the uterine fibroids. Multi-focal origin
multifocal nodular proliferation of immature smooth muscle cells with of the tumor has been advocated to account for this3.Treatment of BML
formation of subpleural and intraparenchymal cyst-like structures. Our is based on hormonal manipulation as these tumors express estrogen and/
differential diagnosis had included lymphangioleiomyomatosis (LAM) or progesterone receptors. Progesterone treatment has been shown to be
because of the cystic nature of some of the nodules, however stains for successful in regression and prophylaxis against recurrence. Bilateral
HMB 45 were negative, making the diagnosis of LAM unlikely. oopherectomy and parenchymal sparing surgical resection of these lesions
are also advocated as treatment modalities. The prognosis of this disease
seems to depend on the hormonal receptor status. There are case reports
describing regression of tumor during pregnancy and in post menopausal
women. We are postulating that the dissemination of myomatous tissue
occurred during uterine artery embolization. It is possible that this could
have happened during her prior myomectomy. However we do not have
any radiographic evidence of this as the patient did not have a chest X-ray
performed in the interval.
CONCLUSION: Dissemination of myomatous tissue may occur with
any invasive uterine procedure. UAE is a relatively new modality for
treatment of fibroids. Physicians should be aware that BML can present
after UAE.
DISCLOSURE: Deepa Lazarous, None.
MASSIVE HEMOPTYSIS: A CASE OF METASTATIC CHORIO-

CARCINOMA
K. M. Dinesh Chandra MD* Marshall Tanner MD Douglas Farman MD
Jerome Tift MD University of Alabama at Birmingham, Birmingham, AL
INTRODUCTION: Choriocarcinoma is a rare tumor in men, com-

prising less than 5% of germ cell tumors and frequently associated with an
elevated serum beta-human chorionic gonadotropin (B-HCG). Markedly
elevated B-HCG (⬎ 50,000 miu/ml) is a marker of poor prognosis.
Choriocarcinoma has high propensity for hemorrhage, and complications
from the bleeding likely contribute to higher mortality.
CASE PRESENTATION: 21 year old Spanish-speaking male pre-
sented to the hospital with one week history of nausea and vomiting. He
also had a mild epigastric pain and loose stools averaging 3/day for the past
3-4 days. His friends reported that patient had worked on a farm and was
exposed to herbicides and pesticides. Additional history noted for patient
becoming ill after eating a hamburger. No prior history of medical
problems. Pertinent review of systems: Cough with yellowish white
sputum of 10 days duration with occasional specks of blood. No history of
weight loss, hemetemesis, fever, chills, night sweats, masses, or lymph
node enlargement. Physical examination: T 97.7, BP 111/64, HR 117, RR
18, oxygen saturation 94% on room air. Well built and nourished Hispanic
male in no acute distress. Lung exam revealed bilateral crackles at the
bases. Abdominal exam showed mild epigastric and RUQ tenderness on
deep palpation. Rest of the examination was unremarkable. Pertinent lab
data: WBC 13000, hematocrit 38.8%. Chest radiograph showed bilateral
diffuse air space/ interstitial pattern predominantly in the lower zones with
CASE REPORTS
possible small effusion. Patient was admitted and initially treated for acute
gastroenteritis and community acquired / atypical pneumonia. Next day
patient started coughing up small amount of blood and he was noted to
have increasing respiratory distress. Patient was transferred to the inten-
sive care unit and subsequently intubated for the worsening respiratory
failure. Following day patient had significant amount of blood from his
endotracheal tube with his hematocrit dropping to 22%. Several differ-
ential diagnoses were considered including mycobacterial infection, HIV,
DISCUSSIONS: BML refers to histologically benign smooth muscle opportunistic fungal infections, legionnaires, Good Pasteur’s /autoimmune
tumors which originate from uterine fibroids. It is a rare disorder with and connective tissue disorders. Following day patient continued to have
about 100 cases reported in the literature. It is seen exclusively in females. massive hemoptysis. Despite aggressive resuscitative efforts, patient de-
It is associated with a history of uterine fibroids and is more commonly veloped profound hypotension leading to cardiac arrest and death.
seen in patients who have undergone some form of invasive intervention DISCUSSIONS: Autopsy revealed massively enlarged and hemor-
for their fibroids. Though it has been described in various organs, it is rhagic lungs with multiple metastases of choriocarcinoma. A single
most frequently seen in the pulmonary parenchyma and lymph nodes . metastasis was present in the left kidney. Testes were grossly normal, but
BML lesions appear anywhere from 3 months to 20 years after manipu- the microscopic examination of the left testis showed a small focus of
lation of the uterine lesion. It is usually an incidental finding as most malignant intratubular germ cells without differentiating features. No
patients are asymptomatic. However, patients may present with dyspnea, invasive carcinoma was identified but there was a small scar with
cough or chest pain. Our patient is the first reported case of spontaneous calcifications. Premortem serum was examined for B-HCG, which was
pneumothorax in BML.Radiographically these lesions are well circum- found to be present in high concentration (331,819 miu/ml). Death in this
scribed solitary or multiple pulmonary nodules ranging in size from a few case was the result of pulmonary hemorrhage and respiratory failure
millimeters to several centimeters in diameter. Typically these nodules are secondary to metastatic choriocarcinoma of testicular origin. Germ cell
non-calcified and do not enhance with intravenous contrast administra- tumors can arise in normal testes, and this is particularly true of
tion. They may cavitate.Usually there is bronchial and pleural spar- choriocarcinoma, which can metastasize widely, and yet leave minimal
ring.The exact pathogenesis of BML remains controversial. Multiple tumor or scar in the testis of origin.

CONCLUSION: Metastatic choriocarcinoma is a rare cause of massive inflammatory process that may be due to a defect in the processing of
hemoptysis. Radiographic features of pulmonary hemorrhage may ob- bacteria by macrophages. Treatment consists of a total nephrectomy,
scure metastatic nodules of choriocarcinoma. Even with a normal testic- removal of all involved tissue and closure of all fistulas. Prognosis is
ular exam, germ cell tumors should be considered in young males with excellent following surgery. There are over 300 reported cases of XGP but
massive hemoptysis. only a few reported cases of it involving the lung.
REFERENCES: CONCLUSION: We present an unusual case of hemoptysis due to
1 Peckham MJ, Oliver RTD, et al. Prognostic factors in advanced xanthogranulomatous pyelonephritis involving the lung.
non-seminomatous germ-cell testicular tumors: results of a multi-
center study. Lancet. 1985;1:8-11
2 Benditt JO, Farber HW, et al. Pulmonary hemorrhage with diffuse
alveolar infiltrates in men with high volume choriocarcinoma. Ann
Intern Med. 1988 Oct 15; 109(8):674-5
3 Duggard G, von der Masse H, et al. Carcinoma-in-situ in patients
with assumed extragonadal germ-cell tumors. Lancet 1987; Sept
5:528-530
DISCLOSURE: K Dinesh Chandra, None.
XANTHOGRANULOMATOUS PYLONEPHRITIS PRESENTING

AS HEMOPTYSIS
Michael R. Baydarian MD* Bruce Ludwig MD National Naval Medical
Center, Bethesda, MD
INTRODUCTION: The term hemoptysis comes from the Greek

words haima meaning blood, and ptysis meaning spitting. Though this is
a simple definition, discerning the source of the blood is not always
simple. The differential is very broad and includes the categories of
infection, vascular, pulmonary and, neoplastic etiologies. The work up
involves many modalities such as history and physical, radiography, lab
studies and bronchoscopy. This case is an example of an uncommon
etiology with an interesting diagnostic work up.
CASE PRESENTATION: Patient is a 32 year-old female with 2-3
weeks of blood-streaked sputum, which occurred daily. She had no fevers,
chills, cough, epistaxis, night sweats, weight loss, chest pain, or gastro-
intestinal complaints though she had chronic intermittent left sided chest
pain. Her history is significant for Iron deficiency anemia and recurrent
urinary tract infections (UTI’s), which she gets 3-4 times per year. She
emigrated from Poland 10 years ago. She has a 12-pack year tobacco
history and no sick contacts or recent travel. On physical exam she had left
costo-phrenic tenderness, her lungs were clear to auscultation, she had no
adenopathy and her abdominal exam was benign. Labs revealed a 20
millimeter PPD, a white blood cell count of eleven and a microcytic
anemia with a hematocrit of thirty-five. Her Acid Fast Bacillus (AFB) DISCLOSURE: Michael Baydarian, None.
smears and cultures were negative times nine. A chest x-ray revealed a left
lower lung mass and a subsequent CT scan revealed a renal mass that
extended through the diaphragm into the lower lobe of her left lung. CASE OF HEMOPTYSIS ONE MONTH AFTER ENDOBRON-
Bronchoscopy showed normal airways without evidence of bleeding. CHIAL VALVE PLACEMENT FOR LUNG VOLUME REDUC-
Bronchoalveolar lavage, transbronchial biopsies and cytobrush were non- TION
diagnostic including negative AFB smears and cultures. The differential Simrit Bhular DO* Sabrina Haney RN Rolando Berger MD Michael
included xanthogranulomatous pyelonephritis (XGP), tuberculosis, and Zgoda MD University of Kentucky, Lexington, KY
malignancy. Based on history and CT findings the decision was made to
proceed with surgery, which included a left radical nephrectomy, partial INTRODUCTION: Bronchoscopic valve placement is currently under
diaphragmatic resection, diaphragmatic fistula repair, partial gastric re- investigation as a non-surgical means of lung volume reduction for the
section and partial left lower lobectomy. The pathology revealed XGP with treatment of emphysema. This is a case of hemoptysis one month after 5
lipid-laden macrophages (xanthoma cells), giant cells and plasma cells. valves were placed to exclude the right lower lobe.
The culture from the tissue grew out Proteus mirabilis. She recovered CASE PRESENTATION: Sixty-four year old female with emphysema
uneventfully without recurrence of hemoptysis. was randomized to valve placement and treated according to the research
DISCUSSIONS: XGP, first described in 1916, is the sequelae of protocol. The lung parenchyma was scored by CT scan and the right lower
chronic, severe obstructive parenchymal inflammation. There is destruc- lobe was selected as the optimal lobe for lung volume reduction. Five
tion of the renal medulla and cortex, which is the result of normal cells valves were needed to exclude the right lower lobe and were placed in
being replaced by sheets of lipid-laden macrophages (xanthomas). XGP B6-B10 respectively. All valves were placed with their most proximal
has three stages: Stage I (nephric) granuloma tissue is confined to the portion being centered within the airway as shown in the figure below.
kidney; Stage II (perinephric) granuloma invades the kidney and Gerota’s The patient was discharged home on day two without complication. One
fat; Stage III (paranephric) granuloma invades the kidney, fat and month later, the patient presented with intermittent, scant, bright red
retroperitoneal structures which include gastro-intestinal structures, the hemoptysis for 2 days without dyspnea, epistaxis, fevers, lower extremity
diaphragm and rarely extension into the lungs. Patients present with fever, swelling, or chest pain. Oxygenation on room air was improved compared
flank pain, recurrent UTI’s from gram-negative organisms and, anemia. to her pre-valve evaluation. CT scan with intravenous contrast showed
The differential includes renal carcinoma, malakoplakia, megalocystic partial lower lobe collapse but was otherwise non-diagnostic. Bronchos-
interstitial nephritis and inflammatory processes such as pyonephrosis, copy revealed that the proximal portion of the valve placed in B7 (medial
tuberculosis and abscess secondary to renal calculi. The preferred diag- basal segment) had eroded into the opposite endobronchial wall, as shown
nostic tool is the CT scan, which can exclude renal cancer, detect renal in the figure below, causing hemoptysis . Some airway inflammation
stones and determine the extent of granuloma involvement. Macroscopic localized to the area of the valve was appreciated. No intervention was
pathology reveals necrotic yellow material and layers of orange-colored employed as some epithelialization of the valve was evident and in our
tissue. Microscopic findings include necrosis with lymphocytes and mac- opinion the risks of bleeding and damage to the airway outweighed the
rophages, vascularized granulation tissue, giant cells and cholesterol clefts. benefits of removal. A first generation cephalosporin was given in
The pathogenesis and etiology of XGP are unknown but it is an conjunction with 40 mg of prednisone daily for ten days. No active

bleeding was evident at bronchoscopy and she has not had any hemoptysis Furthermore the patient had been briefly exposed to welding fumes.
or worsening dyspnea two months after the described episode. Based on There were no risk factors for HIV and he denied a history of intravenous
our experience with metal stents, a follow up bronchoscopy was planned drug abuse. His family history was unremarkable. He was using no
for 90 days post-hemoptysis in hopes that epithelialization would be medications. Examination revealed normal vital signs, a prolonged expi-
complete as well as resolution of the inflammation preventing the valve ratory phase without wheezing, no chest wall tenderness, pleural friction
from coming into contact with the opposite bronchial wall. rub or dullness to percussion. There was no lymphadenopathy and the
remainder of his physical examination was unremarkable. Pulmonary
function studies disclosed the following: FEV1 2.09L(51%) with 28%
improvement following administration of albuterol, RV 3.06L (177%),
DLCO 20.8(65%). There was no oxygen desaturation. Complete blood
count, electrolytes, HIV serology and alpha-1-AT were unremarkable. The
patient underwent wedge biopsy of middle lobe. Histologic analysis
indicated pulmonary MALT-lymphoma, non-necrotizing granulomatous
inflammation, and light chain accumulation without amyloid deposition on
congo red staining. Other findings included paraseptal emphysema in
close proximity to areas of light chain deposition (Fig. 1C-E). Cultures
from lung specimens showed no evidence of infectious organisms.CT
DISCUSSIONS: The endobronchial valves used in this patient are
scans of abdomen and pelvis, PET scan, and bone marrow biopsy
essentially specialized silicone covered nitinol stents and will likely have
confirmed localized pulmonary disease. Initially the patient received one
the same issues that other nitinol stents possess including bleeding,
cycle of rituximab, cyclophosphamide, doxorubicin, vincristine and pred-
granulation tissue formation, and airway stenosis. Endobronchial valve
nisone(R-CHOP). Due to localized disease and side effects associated
placement is a safe procedure however, this case suggests that a combi-
with his chemotherapeutic regimen he subsequently received three cycles
nation of airway inflammation and any lobar collapse could potentially
of rituximab alone. His disease remained stable throughout the last 12
change the anatomy in such a way as to allow a valve to erode into the
months.
opposite wall of the bronchous causing hemoptysis. This could possibly be
DISCUSSIONS: Pulmonary MALT-lymphomas are commonly discov-
prevented by placing the valves more distal within the bronchus having
ered incidentally by chest imaging in clinically asymptomatic patients[1].
thier opening flush to the bronchial opening.
The most frequent radiographic findings include solitary (17%), multifocal
CONCLUSION: Endobronchial valve placement for lung volume
(79%) or bilateral 60%) nodules and infiltrates[2,3]. Air-bronchograms
reduction has the potential to provide palliation to a population that have
(90%) and bubble-like radiolucencies(50%) and cavitating nodules have
few options for the treatment of their dyspnea however, hemoptysis
been described [2]. Our patient had bilateral focal infiltrates and nodular
caused by the endobronchial valves will require long term follow up to
consolidations as well as large cystic structures with a well defined wall.
determine if this is a reproducible phenomena.
Similar cystic changes have been reported in lymphoid interstitial pneu-
REFERENCES:
monia and other benign disorders associated with pulmonary lymphocytic
1 Yim AP, Hwong TM, Lee TW et al. Early results of endoscopic lung
infiltration[4]. These cysts are frequently lined with respiratory epithe-
volume reduction for emphysema. J Thorac Cardiovasc Surg 2004;
lium. It hasbeen proposed that the cysts are caused by airtrapping
127(6):1564-1573.
secondary to a ball valve mechanism. Alternatively amyloid deposition in
2 Lim LH, Cotton RT, Azizkhan RG, Wood RE, Cohen AP, Rutter
the setting of lymphocytic infiltration has been implicated in three
MJ. Complications of metallic stents in the pediatric airway. Oto-
radiographically identical cases[4]. In our patient no amyloid was identi-
laryngol Head Neck Surg. 2004; 131(4):355-61.
fied but light chain deposition was present. We surmise that light chain
3 Cynthia P. Saad, Sudish Murthy, Georgiann Krizmanich, and Atul
deposits may also be associated with cyst formation in the setting of
C. Mehta. Self-Expandable Metallic Airway Stents and Flexible
pulmonary lymphocytic infiltration. The associated non-necrotizing gran-
Bronchoscopy: Long-term Outcomes Analysis. Chest, Nov 2003;
ulomatous inflammation was felt to be related to the MALT-lymphoma
124:1993-1999.
[1].
DISCLOSURE: Simrit Bhular, Grant monies (from industry related
CONCLUSION: MALT-lymphoma can present with the unusual
sources) Emphasys Medical, Inc., Redwood City, CA
feature of bilateral cystic changes in the lungs. The development of these
abnormalities is potentially linked to associated light chain deposition.
REFERENCES:
1 Kurtin PJ. Am J Surg, Path 2001; 25:997-1008
2 Lee DK. J Comput Assist Tomogr, 2000; 24:30-34
3 King LJ. Eur Radiol, 2000; 10:1932-1938
Zebras and Unicorns 4 Desai RS. J Thoracic Imaging, 1997; 12:215-220
CASE REPORTS
2:00 PM - 3:30 PM
CYSTIC LUNG DISEASE ASSOCIATED WITH PULMONARY
MUCOSA ASSOCIATED LYMPHOID TISSUE LYMPHOMA
Tobias Peikert MD* Jeffrey L. Myers MD Udaya B. Prakash MD Mayo
Clinic College of Medicine, Rochester, MN
INTRODUCTION: Extranodal marginal zone B-cell lymphoma of the

mucosa associated lymphoid tissue (MALT-lymphoma) comprises ⬍ 1%
of all non-Hodgkin’s lymphomas but represents the most common form of
primary pulmonary lymphoma[1] It is considered part of the spectrum of
pulmonary lymphoid lesions. MALT-lymphomas are frequently associated
with rheumatologic disorders, monoclonal gammopathies, immunodefi-
ciencies and chronic infections[1]. Here, we present a case of pulmonary
MALT-lymphoma with unusual presenting features.
CASE PRESENTATION: A previously healthy 43 year-old man
presented with left-sided chest pain. Chest radiograph showed bilateral
bullous changes and nodular opacities(Fig. 1A). Chest CT scan revealed
bilateral cystic changes with lower lung predominance, bilateral areas of
nodular consolidation and patchy infiltrates. No pneumothorax, pleural
effusion or lymphadenopathy was visualized (Fig. 1B). The patient was a
cigarette smoker (30 pack years). In addition he had used marijuana on an
almost daily basis during the past 20 years. He worked for a company
installing floors and reported significant exposure to various glues. DISCLOSURE: Tobias Peikert, None.

Zebras and Unicorns, continued
GIANT CELL INTERSTITIAL PNEUMONIA ASSOCIATED

WITH NITROFURANTOIN
Charles W. Hargett MD* Thomas A. Sporn MD Victor Roggli MD John
W. Hollingsworth MD Duke University Medical Center, Durham, NC
INTRODUCTION: Giant cell interstitial pneumonia (GIP) is a

distinct and uncommon form of chronic interstitial pneumonia most
frequently found in workers exposed to hard metals such as tungsten
and cobalt. Nitrofurantoin-induced lung disease demonstrates a wide
spectrum of histopathologic findings and, of these patterns, GIP is an
exceedingly rare manifestation. To our knowledge, we report the
second case of GIP associated with nitrofurantoin (1).
CASE PRESENTATION: A 77 year-old female presented with a six
month history of progressive dyspnea and exercise intolerance. She had
received daily nitrofurantoin therapy for the previous nine months for
recurrent urinary infections. The patient had no other cardiopulmo-
nary or constitutional symptoms. On physical exam, basilar “velcro”
crackles were auscultated, and her room air oxygen saturation fell from
95% to 89% with minimal exertion. Hematologic, biochemical, and
rheumatologic serologies were normal. The patient was taking no other
medications and there was no evidence of active infection. She was a
retired school librarian and had no history of heavy metal or other
environmental exposures. Pulmonary function tests demonstrated pat-
terns of mild obstruction and restriction with a severely decreased
DLCO. CT of the chest showed nodular ground glass opacities within
areas of peripheral intralobular septal thickening in both lungs with no
lobar predominance (Figure 1). Flexible bronchoscopy was performed.
The airway exam was normal and cultures were negative. Bronchoal-
veolar lavage fluid (BALF) showed a predominance of lymphocytes
(71%), a mild eosinophilia (10%), and a CD4/CD8 ratio decreased at
0.25. BALF cytology demonstrated bizarre appearing multinucleated
giant cells (MGC), and transbronchial biopsies demonstrated cellular
interstitial infiltrates and scattered MGC consistent with GIP (Figure
2). Nitrofurantoin was discontinued; therapy with prednisone 1 mg/
kg/day was initiated and tapered over six months. Performance status
and pulmonary function tests were dramatically improved after two
months of therapy. DISCLOSURE: Charles Hargett, None.
DISCUSSIONS: Although GIP has become nearly synonymous
with hard metal pneumoconiosis, we report the second case of GIP
associated with chronic nitrofurantoin therapy (1). The majority of PLEUROPARENCHYMAL FIBROELASTOSIS PRESENTING AS
cases of nitrofurantoin-induced pulmonary toxicity represent acute USUAL INTERSTITIAL PNEUMONITIS
lung injury which resolves rapidly following cessation of nitrofurantoin. D. A. Sams DO* David A. Solomon MD University of South Florida,
Chronic toxicity is more insidious and carries a worse prognosis. Both Tampa, FL
reported cases of GIP associated with nitrofurantoin occurred with
chronic therapy and presented with a subtle onset of symptoms. Unlike INTRODUCTION: A 62 year-old man presented with decreased
some other forms of chronic nitrofurantoin toxicity and other forms of exercise capacity and chronic cough over the last year. High resolution
interstitial lung disease, rapid and appropriate diagnosis of GIP has computed tomography(HRCT) of the chest, pulmonary function testing,
been associated with a favorable outcome. Careful analysis of the and cardiopulmonary exercise testing were performed with non-specific
results, eventually followed by video assissted thorascopic surgical(VATS)
BALF can support the diagnosis of GIP. While the presence of
lung biopsy that led to a diagnosis of pleuroparenchymal fibroelastosis.
multinucleated giant cells in the BALF can be seen in many lung This is a rare, relatively new clinicopathologic diagnosis. The clinical
diseases, the finding of bizarre or cannibalistic MCG in the BALF may presentation is similar to other chronic interstitial pneumonias, with
be more suggestive of GIP (2-4). After the observation of bizarre pathologic findings that do not fit any other currently defined conditions.
MCG, the diagnosis of GIP can be further supported by increased CASE PRESENTATION: The patient is a 62 year-old male who
numbers of T-lymphocytes with an inverted T-cell helper/suppressor presented with complaints of decreased exercise tolerance as well as
ratio in the BALF as seen in other cases of GIP as well as our patient chronic cough over the past year. He continued to walk 3-4 miles several
(3,4). Ultimately, we feel that the diagnosis of GIP requires histopatho- times per week, play golf, and lift weights, although he noted he could not
logic confirmation. be as vigorous as before. His cough occurred throughout the day, and was
CONCLUSION: It is important to recognize GIP as a rare manifes- occasionally productive of clear sputum. He denied any hemoptysis,
tation of chronic nitrofurantoin toxicity as appropriate therapy can be wheezing, or dyspnea at rest or with minimal exertion. His other review of
associated with a favorable outcome. systems was only positive for persistent hoarseness. He has no significant
REFERENCES: past medical or surgical history. He is a lifelong non-smoker and has no
1 Magee F, Wright JL, Chan N, et al. Two unusual pathological exposure history. His medications included inhaled steroids. His mother
reactions to nitrofurantoin: case reports. Histopathology 1986; 10: died at the age of 47 of an unknown pulmonary disease. His physical exam
701-706 was unremarkable, with lungs clear to auscultation and percussion.
2 Kern I, Kecelj P, Kosnik M, et al. Multinucleated giant cells in Several prior computed tomography scans were reviewed that revealed
bilateral patchy infiltrates that had been stable back to 2001. Spirometry
bronchoalveolar lavage. Acta Cytol 2003; 47:426-430
showed a decrease in volumes without evidence of airflow obstruction and
3 Kinoshita M, Sueyasu Y, Watanabe H, et al. Giant cell interstitial no significant change post bronchodilator. Lung volume measurements
pneumonia in two hard metal workers: the role of bronchoalveolar indicated a moderately severe reduction in residual volume and a mild
lavage in diagnosis. Respirology 1999; 4:263-266 reduction in total lung capacity. Diffusing capacity for carbon monoxide
4 Forni A. Bronchoalveolar lavage in the diagnosis of hard metal was also mildly reduced. Arterial blood gas on room air revealed a pH of
disease. Sci Total Environ 1994; 150:69-76 7.45, PaCO2 of 40 mmHg and a PaO2 of 96 mmHg. His 6-minute walk

test was normal. A HRCT of the chest showed multiple pleural based opacities in a peripheral pattern involving the lower lung fields. Labora-
abnormalities and bronchiectasis in the superior segment of the lower tory data revealed a normal white blood cell count and differential. Serum
lobes. Pleural based opacities were noted in both upper lobes, as well as chemistry was significant for an elevated lactic dehydrongenase of 1054.
extensive interstitial opacities throughout both lungs. VATS biopsy sub- Arterial blood gas on room air was pH 7.42, pCO2 39, PaO2 73, and 94%
sequently revealed fibrosing interstitial pneumonia with marked pleural saturation. Cardiac enzymes were normal and rheumatologic work-up was
fibrosis and subpleural accentuation. It also showed abundant chronic negative. A spiral CT chest was negative for pulmonary embolism,
inflammation with small lymphocytes present in association with the however there was bilateral bulky axillary and mediastinal adenopathy
pleura, predominantly in the upper lobes. with diffuse pleural thickening and scattered interstitial infiltrates. Lower
DISCUSSIONS: Idiopathic pleuroparenchymal fibroelastosis is a clin- extremity doppler ultrasonogram was negative. Upon further questioning,
icopathologic diagnosis that has been recently reported. The clinical the patient revealed she had been massaging her breasts secondary to
presentation is similar to other chronic idiopathic interstitial pneumonias increasing pain in them. We proceeded to bronchoscopy with transbron-
with symptoms of exertional dyspnea and chronic cough. Radiographs chial-biopsy which revealed lung parenchyma showing chronic inflamma-
show pleural and parenchymal involvement with upper lobe predomi- tion and giant cell reaction to foreign particles consistent with what would
nance. Pathologic findings include intense fibrosis of the visceral pleura, be seen in silicone pneumonitis. The BAL was negative for any infectious
prominent, homogenous subpleural fibroelastosis, sparing of the paren- process. At that time a surgical consult was placed and the patient was
chyma distant from the pleura, mild, patchy lymphoplasmacytic infiltrates, advised to have a radical bilateral mastectomy.
and small numbers of fibroblastic foci present at the leading edge of the DISCUSSIONS: Medical problems may develop immediately, years,
fibrosis. Other entities that are characterized by both pleural and paren- or even decades later after subcutaneous silicone injections. Typically,
chymal fibrosis include asbestos-related disease, connective tissue-associ- “black-market” silicone is mixed with paraffin, oil, and other non-sterile
ated disease, and lung disease related to radiation injury or drugs. Our materials. Massive subcutaneous injections of this highly viscous prepara-
patient did not have evidence of any of the above. tion is especially dangerous when introduced into the breast. Pulmonary
CONCLUSION: This report highlights the clinical presentation, ra- complications are due to hematogenous spread and include acute or latent
diographic findings, and pathology of a relatively new entity that is not pneumonitis, pulmonary edema, adult respiratory distress syndrome,
classifiable as one of the currently defined chronic interstitial pneumonias. diffuse alveolar hemorrhage, and pulmonary embolism. Axillary and
It has been termed idiopathic pleuroparenchymal fibroelastosis. mediastinal lymphadenopathy may also occur. The demonstration of
silicone in cells by a trans-bronchial biopsy, or BAL with atomic absorp-
tion and infrared spectrometry is useful in establishing the diagnosis. BAL
may also be characterized by increased cellularity, or alveolar macro-
phages with large pleomorphic cytoplasmic inclusions seen on electron
microscopy. Pulmonary function tests often reveal a mild restrictive
pattern. If available, MRI may be used to image the spread and
complications of injected silicone. Once diagnosed, diverse medical and
surgical interventions are recommended to treat the complications includ-
ing antibiotics, systemic corticosteroids, NSAID’s, local resection, and
mastectomy.
CONCLUSION: Since the 1960’s, the U.S. Food & Drug Administra-
tion has warned of the dangers of injected silicone, labeling it an illicit
practice. Despite the systemic dangers and risk of disfigurement caused
by silicone injection, the practice is still common, especially in the
transgendered population. Patients with silicone injections should be
followed carefully and warned that severe acute respiratory failure may be
induced by local tissue damage or breast massage.
DISCLOSURE: Sonali Sethi, None.
AN UNUSUAL COMPLICATION OF PULMONARY SILICOSIS

Theodossis Zacharis MD* Scott H. Beegle MD Alida Hayner-Buchan
DISCLOSURE: D Sams, None. MD Albany Medical College, Albany, NY
INTRODUCTION: Silica can trigger autoimmune diseases via pro-

THE RESPIRATORY RISK FROM “INSTANT CURVES” IN A duction of autoantibodies (1). We describe a case of pulmonary silicosis
CASE REPORTS
TRANSSEXUAL MALE-TO-FEMALE complicated by microscopic polyangiitis.
Sonali Sethi MD* Joseph Cicenia MD Patricia Tietjen MD Saint Vincents CASE PRESENTATION: A 42-year-old Caucasian male was admitted
Catholic Medical Center, New York, NY to the hospital with hematuria associated with fevers, night sweats and
fifteen pounds weight loss of three months duration. Simultaneously, he
INTRODUCTION: The injection of liquid silicone into human tissue had a non-productive cough. Past medical history was only significant for
has become a common cosmetic procedure that is often performed by cigarette smoking. He was not on any medications. He worked as a
individuals with no medical credentials. While one’s appearance may stonecutter, cutting blue sandstone in his backyard for 15 years without
initially be enhanced, most silicone injections are disfiguring as the using any protective equipment. He was afebrile, normotensive and
silicone migrates, changes shape, or hardens. Furthermore, it can spread non-distressed. Oxygen saturation was 96% on room air. Chest ausculta-
hematogenously resulting in a variety of medical and pulmonary compli- tion revealed crackles diffusely. Digital clubbing was present. Urinalysis
cations. showed red blood cells and red blood cell casts. Serum creatinine was 3.5
CASE PRESENTATION: A 25-year-old transsexual (male-to-female) mg/dL and hemoglobin was 8.6 g/dL. Erythrocyte sedimentation rate was
presented with progressive shortness of breath for 4 days, a non- 146 mm/h and antinuclear antibodies titer was 1:320. Anti double-
productive cough, and diffuse chest discomfort. She denied fever/chills, stranded DNA antibodies, anti-glomerular basement membrane antibod-
hemoptysis, weight loss, or night sweats. Her medical history was ies and antistreptolysin-O antibodies were all negative. Antineutrophil
significant for asthma and multiple silicone injections for “instant curves” cytoplasmic antibodies against myeloperoxidase (P-ANCA) titer was pos-
to her hip, thighs, face, and chest in 2001. Since that time, she had itive at 1:640. Chest radiograph showed diffuse micronodular infiltrates
experienced mild intermittent dyspnea on exertion. She was on no along with calcified hilar and mediastinal lymphadenopathy (figures 1 and
medications. She denied illicit drug use or alcohol abuse, but had a 2). Transbronchial biopsies revealed fibro-inflammatory changes involving
30-pack year smoking history. On physical exam she was not in acute the alveolar septae. Examination under polarized light demonstrated
respiratory distress. Her vital signs were significant for a temperature of refractile material (figure 3). Energy Dispersive X-ray Analysis indicated
100.2° F; her oxygen saturation was 94% on room air. She had decreased the presence of silica. A percutaneous kidney biopsy demonstrated
facial and body hair, and moderate breast and buttocks augmentation. necrotizing crescentic glomerulonephritis, a renal form of microscopic
There were clear breath sounds bilaterally. The rest of her physical exam polyangiitis (figure 4). Diagnosis: pulmonary silicosis with P-ANCA
was unremarkable. Chest radiograph showed areas of patchy parenchymal associated microscopic polyangiitis. The patient was treated with pred-

nisone and cyclophosphamide with resolution of his hematuria and 1978, its’ etiology and pathogenesis remains uncertain (1). In most cases,
stabilization of his renal function. this disorder is elusive until necroscopy. We describe a unique case with
a family history of pulmonary hypertension, in which two distinct sets of
pathology were attained and several therapeutic agents were adminis-
tered.
DISCUSSIONS: Silicosis is a disease produced by inhalation of

crystalline silica, most commonly quartz. Blue sandstone contains 50%
quartz (2). Silica containing compounds have an adjuvant effect on
immune responses and are potent stimulators of lymphocytes and mono- CASE PRESENTATION: A thirty-four year old previously healthy
cytes or macrophages. Silicosis has been associated with different connec- Caucasian man presents with severe dyspnea and chest pain a few days
tive tissue diseases. Branwell in 1914 reported a relation between after cleaning out his garage. A strong family history of pulmonary
scleroderma and silica exposure. Caplan in 1953 described an association hypertension (paternal grandmother, father, two paternal aunts) was
between rheumatoid arthritis and silicosis. Silica exposure has been noted. Clinical exam was significant only for an audible P2. High
associated with a high prevalence of autoantibodies such as antinuclear resolution computed tomography demonstrated bilateral diffuse reticu-
antibodies, rheumatoid factor and antineutrophil cytoplasmic antibodies lonodular infiltrates. Laboratory data was unremarkable aside from sig-
(ANCA) (1,3). The antigens targeted by ANCA have been identified as nificant hypoxemia with an elevated alveolar-arterial gradient. Cardiac
either myeloperoxidase (P-ANCA) or proteinase-3 (C-ANCA). Patients catherization showed preserved left ventricular function with patent
with ANCA-associated vasculitis have 4.4 times greater odds ratio for silica arteries, a pulmonary artery pressure of 59/22 with a wedge pressure of 8.
exposure compared with control subjects (4). Six cases of suspected Pulmonary function tests demonstrated reduced diffusion capacity(30%
microscopic polyangiitis have been described in patients with pulmonary predicted). Bronchoscopy with lavage revealed 69% histiocytes, 27%
silicosis before 1990 with unknown ANCA titers (5). Three cases of lymphocytes, 4% neutrophils and hemosiderin laden macrophages. An
P-ANCA associated microscopic polyangiitis have been reported in open lung biopsy displayed morphologic evidence of pulmonary hyper-
patients with pulmonary silicosis since 1994 (5,6,7). tension and prominent arterial thromboembolic changes with some
CONCLUSION: We report a case of pulmonary silicosis with P-ANCA venous involvement. Therapy included oxygen, steroids and anticoagula-
associated microscopic polyangiitis. Our case is unique in that both tion with initial clinical improvement. Persistent pulmonary hypertension
diagnoses are definitively proven histologically. Exposure to silica should prompted a vasoreactivity study and initiation of epoprostenol. Patient’s
be considered in the history of patients with autoimmune diseases. clinical course included several hospitalizations for infection, with a
Furthermore patients with pulmonary silicosis may develop ANCA- positive response to antibiotics and high dose steroids. Bilateral lung
associated vasculitis in extrapulmonary sites. transplantation was performed 11 months after initial presentation.
REFERENCES: Explant lung pathology was consistent with pulmonary capillary heman-
1 Rosenman KD, et al. Connective Tissue Disease and Silicosis. Am J giomatosis.
Ind Med 1999;35:375-381
2 Reginald Hardy Jr, H et al. A study of the physical properties of
Pennsylvania bluestone. Department of Mineral Engineering the
Pennsylvania State University. RML-IR/72-18.
3 Wichmann I, et al. Antimyeloperoxidase antibodies in individuals
with occupational exposure to silica. Ann Rheum Dis 1996;55:205-
207.
4 Hogan SL, et al. Silica Exposure in Anti-Neutrophil Cytoplasmic
Antibody-Associated Glomerulonephritis and Lupus Nephritis.
J Am Soc Nephrol 2001;12:134-142.
5 Tervaert JWC, et al. Silicon exposure and vasculitis. Curr Opin
Rheumatol 1998;10:12-17.
6 Baik JJ, et al. Two patients with microscopic polyangiitis and unusual
pulmonary manifestation. Respirology 2002;7:73-76.
7 Mulloy KB, et al. Silica Exposure and Systemic Vasculitis. Environ
Health Persp 2003;111:1933-1938.
DISCLOSURE: Theodossis Zacharis, None.
DON’T CLEAN OUT THE GARAGE?

Saadia A. Faiz MD* Dani S. Zander MD Bela Patel MD University of
Texas at Houston, Dept Pulmonary, Critical Care & Sleep Medicine,
Houston, TX
INTRODUCTION: Pulmonary capillary hemangiomatosis is a rare DISCUSSIONS: Pulmonary capillary hemangiomatosis is a rare cause
cause of pulmonary hypertension. First described by Wagenvoort et al. in of pulmonary hypertension. The reported age ranges from 5 to 71, with a

peak between 20 and 40 years. No gender prevalence is noted. Most cases responded positively to steroids and his course was ameliorated with
are sporadic; however, a hereditary form with possible autosomal-reces- epoprostenol. It is possible that his initial pathology and response to
sive inheritance was reported(2). Histologically, the disorder is character- therapy stems from an earlier presentation as compared to other reported
ized by proliferation of small capillaries within the pulmonary interstitum cases. Finally, the history of pulmonary hypertension in three generations
and invasion of vessel and airway walls. Patients usually present with suggests a hereditary form of the disease.
progressive dyspnea, cough, pulmonary hypertension, hemoptysis and CONCLUSION: Pulmonary capillary hemangiomatosis is a rare dis-
bilateral reticulonodular infiltrates. Typically, death ensues 1 to 5 years ease. Our case suggests that “early” pulmonary capillary hemangiomatosis
after onset of symptoms. Although presentation, imaging, and other may only display features of small vessel thromboembolic pulmonary
studies may be suggestive, a strong clinical suspicion and tissue is needed hypertension. Although an objective and subjective improvement with
for diagnosis. Successful treatment includes lung transplantation or
moderate-high dose steroids was noted initially, a rapidly progressive
pneumonectomy. Interferon-alpha and doxycycline have provided positive
results in case reports; however, glucocorticoids have no proven role. course ensued. Interestingly, the cardiopulmonary status did not worsen
Vasodilators such as calcium channel blockers and epoprostenol have on epoprosternol. Although major aspects of the disease have been
resulted in lethal results and are “contraindicated”(3).In comparison, our characterized, a complete definition of the disease process continues to
case presents several unique aspects. Two individual open lung biopsies evolve.
(obtained eleven months apart) demonstrated distinct histologies. The REFERENCES:
first was suggestive of chronic small vessel thromboembolic pulmonary 1 Wagenvoort, et.al. Histopathology, 1978;2:401-406.
hypertension, and the second clearly showed pulmonary capillary heman- 2 Langleben, et al. Annals of Internal Medicine, 1988;109:106-9.
giomatosis. One may propose that the initial pathology may be an early 3 Almagro, et al. Medicine, 2002;81(6):417-24.
presentation of the disease. In terms of therapy, our patient initially DISCLOSURE: Saadia Faiz, None.
CASE REPORTS

Lung Adenocarcinoma With Solitary Metast

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Monday, October 31, 2005

408S CHEST 2005—Case Reports

PHOTODYNAMIC THERAPY (PDT) FOR GRANULATION TIS-

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 409S

UPPER AIRWAY OBSTRUCTION AND RESPIRATORY FAILURE

410S CHEST 2005—Case Reports

PRIMARY ADRENOCORTICAL CARCINOMA WITH ENDO-

INTRODUCTION: Pulmonary metastases to the adrenal gland are

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 411S

412S CHEST 2005—Case Reports

PULMONARY ARTERIAL HYPERTENSION: AN UNUSUAL PRE-

INTRODUCTION: Pulmonary hypertension is a known complication

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 413S

DISCUSSIONS: Iatrogenic air embolism requires that two conditions

414S CHEST 2005—Case Reports

CASE PRESENTATION: A 62-year-old male investigated for an

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 415S

SUCCESFULL SURGICAL REPAIR OF AN ACCUTE TYPE A

INTRODUCTION: Acute Type A dissecting Aortic Aneurysm associ-

416S CHEST 2005—Case Reports

DISCLOSURE: Jun Chiong, None.

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 417S

DISCLOSURE: Svetolik Djurkovic, None.

RIGHT CORONARY ARTERY ANEURYSM: THE LARGEST AN-

INTRODUCTION: Coronary artery aneurysm is dilatation greater

418S CHEST 2005—Case Reports

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 419S

DISCUSSIONS: This case report is notable for several reasons. First,

420S CHEST 2005—Case Reports

DISCLOSURE: Sam Parsia, None.

ACUTE CARDIAC TAMPONADE AFTER REMOVAL FROM

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 421S

INTRODUCTION: Nocardiosisis a bacterial infection seen predomi-

422S CHEST 2005—Case Reports

DISCLOSURE: Mihwa Pak, None.

PROGRESSIVE CAVITARY DESTRUCTION OF THE RIGHT

INTRODUCTION: Numerous malignancies and infectious etiologies

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 423S

424S CHEST 2005—Case Reports

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 425S

single or multiple lesions, or involve the lung parenchyma as one or more

426S CHEST 2005—Case Reports

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 427S

pathic or occur in association with systemic disorders such as connective tissue

ROTAVIRAL RNA IN TYPE II PNEUMOCYTES AND ALVEOLAR

428S CHEST 2005—Case Reports

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 429S

CONCLUSION: Myelomatous pleural effusion with mediastinal in-

TENSION PNEUMOTHORAX FOLLOWING COLONSCOPY

430S CHEST 2005—Case Reports

A CASE OF A PLEURAL EFFUSION, CALCIFIED PLEURAL

INTRODUCTION: The annual incidence of malignant mesothelio-

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 431S

A UNIQUE CASE OF EXOPHTHALMOS IN A PATIENT WITH

INTRODUCTION: Erdheim-Chester disease is a rare histiocytosis,

432S CHEST 2005—Case Reports

CHEST / 128 / 4 / OCTOBER, 2005 SUPPLEMENT 433S

aggressive diuresis, PA pressures and oxygen requirements improved.

PULMONARY HYPERTENSION (PHTN) AND ALVEOLAR HEM-