Article / Clinical Case Report

Artigo / Relato de Caso Clínico

Bilateral mandibular osteomyelitis mimicking periapical cysts in a

patient with sickle cell anemia
Juliane Pirágine Araújoa, Ana Maria Hoyos Cadavida, Celso Augusto Lemosa,
Marilia Trierveilerb, Fabio Abreu Alvesa,c
Araújo JP, Cadavid AMH, Lemos CA, Trierveiler M, Alves FA. Bilateral mandibular osteomyelitis mimicking periapical cysts in
a patient with sickle cell anemia. Autopsy Case Rep [Internet]. 2015;5(3):55-60. http://dx.doi.org/10.4322/acr.2015.013

ABSTRACT

Sickle cell anemia (SCA) is a hemoglobin disorder that occurs more commonly among Afro-descendants. The authors
report the case of a 28-year-old Afro-descendent male patient with the diagnosis of homozygotic sickle cell disease
(SCD) referred for evaluation of mandibular lesions. The patient’s main complaints included pain and bilateral teeth
mobility. An intraoral examination revealed gingiva recession affecting the lower molars with extensive root exposure.
A panoramic x-ray showed two radiolucent symmetrical periapical lesions evolving both the first and the second lower
molars, bilaterally. The diagnostic hypotheses comprised odontogenic infection, among others. Besides antimicrobial
therapy, the two molars of both sides were extracted and bone was collected for histopathological and microbiological
analyses. Osteomyelitis was diagnosed, and Streptococcus viridans was recovered from the culture media. Mandibular
osteomyelitis should be considered as a diagnosis in patients with SCD. The present case offers an alert to clinicians about
the importance of knowing jaw lesions related to SCA.

Keywords
Osteomyelitis; Anemia, Sickle Cell; Mandible.

INTRODUCTION

Sickle cell anemia (SCA) is a common genetic and renal failure. Blood transfusion and iron-chelation
condition characterized by the presence of abnormal therapy may be necessary in severe cases.1
hemoglobin, designated hemoglobin S (HbS), and is Osteomyelitis, due to altered blood supply
particularly common among people whose ancestors associated with sickle cell disease (SCD), is more
come from sub-Saharan Africa, India, Saudi Arabia, and commonly observed in long bones. In the jaw bones,
Mediterranean countries. The red blood cells become the posterior region of the mandible is the area
sickle-shaped, blocking capillaries. Consequently, a low most frequently affected, and oral microorganisms
level of oxygen reaches the tissues and the patients will may reach this area via periodontal ligaments or by
present recurrent painful crises, bone or joint necrosis, hematogenous spread.2-4 In addition, vaso-occlusive

a
Stomatology Department - School of Dentistry - Universidade de São Paulo, São Paulo/SP – Brazil.
b
Oral Pathology Department - School of Dentistry - Universidade de São Paulo, São Paulo/SP – Brazil.
c
Stomatology Department - AC Camargo Cancer Center, São Paulo/SP – Brazil.

Autopsy and Case Reports. ISSN 2236-1960. Copyright © 2014. This is an Open Access article distributed
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Bilateral mandibular osteomyelitis mimicking periapical cysts in a patient with sickle cell anemia

radiopaque lesions can be observed in the mandible teeth (Figure 1A-1D). The panoramic radiography
and can represent mandibular infarcts whose onset showed two radiolucent symmetrical periapical lesions
coincides with severe facial pain in sickling crises. evolving both the first and the second lower molars,
As the initial radiographic appearance of infarction may bilaterally (Figure 2).
be identical to that seen in osteomyelitis, clinical and The main differential diagnoses were odontogenic
laboratory features can help to distinguish infarction infection, periapical cyst and keratocystic odontogenic
and osteomyelitis, which involve different therapeutic tumor related to nevoid basal cell carcinoma syndrome
aproaches.5-7 (KOCT-NBCCS). Some hematological exams were
The aim of this report is to describe a case of requested, and the results were: 3.09 million/mm3 red
bilateral mandibular osteomyelitis related to SCD, cells, 8.9 g/dL of hemoglobin, red cell distribution width
which could be misdiagnosed as periapical cyst. 22%, anisocytosis (+++), 14,400 /mm 3 leukocytes,
320,000 /mm 3 platelets, the presence of sickle
erythrocytes, 1:30 min bleeding time, and prothrombin
CASE REPORT
time was normal. The clinical management consisted
A 28-year-old Afro-descendent male was referred of an antimicrobial prescription followed by extraction
to our institution for evaluation of painful mandibular of the first and the second lower molars (on both sides
lesions. His medical history included homozygotic SCA of the mandible). During the procedure, a sequestrum
diagnosis and some previous hospitalizations due to of alveolar bone was removed. In addition, tissue
chest and femoral pain. (His most recent hospitalization fragments for histopathology and microorganism
occurred 1-month prior, when antibiotics and a blood culture were sampled. The histopathological findings
transfusion were prescribed.) Intraoral examination consisted of granulation tissue with lymphoplasmacytic
showed extensive root exposure affecting the lingual inflammatory infiltrate and areas with bacterial colonies
side of the right and the left lower molars, localized and microabscesses. A large fragment of necrotic bone
gingival purulent drainage, mobility, and caries in those was also observed (Figure 3A and 3B).

Figure 1. A and C – Right molars presenting caries and lingual root exposure; B and D – Left molars with extensive
lingual root exposure.

56 Autopsy and Case Reports 2015;5(3):55-60

 Araújo JP, Cadavid AMH, Lemos CA, Trierveiler M, Alves FA

The culture of the lesion showed the growth in its ability to deform.8 Thus, the red blood cell lifespan
of Streptococcus viridans, which was sensitive to is decreased from 120 days to 10-12 days, resulting
penicillin, clindamycin, erythromycin, and vancomycin. in hemolytic anemia.2,9 The increased susceptibility of
Anaerobic bacteria were not recovered in the culture SCD patients to infections, including osteomyelitis, has
media. The patient was followed-up for 3 months and long been recognized by several mechanisms, including
has been asymptomatic ever since (Figure 4A-4C). hyposplenism and the presence of infarcted or necrotic
bone. 3 A sickle cell crisis may be spontaneously
DISCUSSION triggered, but patients may describe specific events
preceding the pain crisis, such as cold temperature,
SCA is characterized by the synthesis of an emotional stress, anxiety, or other painful states, such
abnormal hemoglobin molecule (HbS), which is a result as tooth pain.2
of point mutation in the hemoglobin gene. There is Aseptic necrosis has been more frequently
the substitution of the valine for glutamic acid as observed in long bones than in the mandible.
the sixth amino acid of the beta chain, which results Interestingly, Salmonella sp is more related to the
in abnormal reactions between molecules of HbS former, and Streptococcus sp and Staphylococcus
when oxygen tension is lowered. The polymerization aureus to the latter. 3,8 Olaitan et al. 10 evaluated
of deoxygenated HbS is the primary event in the 16 patients with mandibular osteomyelitis associated
molecular pathogenesis of SCD, resulting in a distortion with SCA: 13 were men (81%) and the majority of them
of the shape of the erythrocyte and a marked decrease
were in the third decade of life (mean age = 23 years).
All lesions were located in the mandible (angle and
ascending ramus). Mixed flora and S. aureus infection
was the predominant agents and no Salmonella sp was
isolated in that study.
Increased radiolucency of bone is more commonly
observed in the area between the apices of the lower
teeth. In addition, suppurative osteomyelitis usually
presents as indistinct radiolucency and painful osseous
resorption.8 In the present case, two symmetrical and
Figure 2. Panoramic x-ray showing two symmetrical
radiolucent lesions involving the roots of the lower well-defined radiolucent lesions were observed in
molars. the body of the mandible (the lesions involved the
roots of the first and second molars, bilaterally). Such

Figure 3. A – Intense lymphoplasmacytic inflammatory infiltrate (HE, 400X); B – Necrotic bone presenting bacterial
colonies (HE, 100X).

Autopsy and Case Reports 2015;5(3):55-60 57

Bilateral mandibular osteomyelitis mimicking periapical cysts in a patient with sickle cell anemia

Figure 4. A-C – Clinical and radiographic control 3 months after the surgeries.

lesions had radiographic features similar to that of necrosis of those teeth (Figure 1A-1D). In our opinion,
periapical cysts. Extensive caries and restorations this information was not essential in the present case,
also contributed to cyst diagnosis. Furthermore, since these patients usually present asymptomatic
keratocystic odontogenic tumors related to nevoid pulpal necrosis and increased incidence of dental
basal cell carcinoma syndrome can be a differential caries, mandibular osteomyelitis, mandibular nerve
diagnosis mainly because the lesions were synchronic. anesthesia, and the aforementioned radiographic
findings.3,4 In addition, Guzeldemir et al. 11 reported
However, a thin sclerotic halo, which is commonly
that plaque and gingival indices were significantly
observed in cystic lesions, was not present in our
higher in patients with SCD than in healthy individuals.
case. Another interesting radiographic feature is a
The treatment of odontogenic infections
small radiopaque area around the apices of the left
usually includes the removal of the causative tooth,
first lower molar suggesting bone sequestrum, which
followed by drainage and antibiotictherapy. 12
was confirmed during surgery. Antibiotic prophylaxis for oral surgical procedures
Although we did not perform the dental pulp is recommended, not only because of the patient’s
sensibility test, the extensive caries of the right first increased risk for infection, but also because
and second molars and the advanced periodontal infection can potentially induce hemolytic crisis.
diseases in the left molars likely caused the pulp Penicillin V, amoxicillin, and clindamycin are

58 Autopsy and Case Reports 2015;5(3):55-60

 Araújo JP, Cadavid AMH, Lemos CA, Trierveiler M, Alves FA

acceptable preoperative oral antibiotics and should 3. Almeida A, Roberts I. Bone involvement in sickle
be administered approximately 1 hour before the cell disease. Br J Haematol. 2005;129(4):482-90.
procedure. 2 Treatment of SCA osteomyelitis in http://dx.doi.org/10.1111/j.1365-2141.2005.05476.x.
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prescription. However, antibiotics alone remain the report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod.
mainstay of bone infection treatment in SCA. Surgery 1996;82(2):225-8. http://dx.doi.org/10.1016/S1079-
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drainage of an obvious periosteal abscess or a 5. Sanger RG, Greer RO Jr, Averbach RE. Differential
sequestrectomy.6 The treatment of our case included diagnosis of some simple osseous lesions associated
exodontia of the lower first and second molars, with sickle-cell anemia. Oral Surg Oral Med Oral Pathol.
administration of amoxicillin 500 mg three times 1977;43(4):538-45. http://dx.doi.org/10.1016/0030-
daily for 2 weeks, curettage of the lesion, and wound 4220(77)90106-2. PMid:265481.
closure. The patient had a good postoperative period
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without pain or bleeding. The wound showed a current therapy. J Oral Maxillofac Surg. 1999;57(2):171-8.
mild healing delay on the left side compared with http://dx.doi.org/10.1016/S0278-2391(99)90234-3.
the right side.
7. Kavadia-Tsatala S, Kolokytha O, Kaklamanos EG,
Antoniades K, Chasapopoulou E. Mandibular lesions
CONCLUSION of vasoocclusive origin in sickle cell hemoglobinopathy.
Odontology. 2004; 92( 1) : 68- 72. http://dx.doi.
This case illustrates that patients with SCA can org/10.1007/s10266-004-0036-3. PMid:15490308.
present oral abnormalities with challenging diagnosis
8. Sansevere JJ, Milles M. Management of the oral and
and management. Furthermore, the osteomyelitis is
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ACKNOWLEDGEMENTS 9. Shroyer JV 3rd, Lew D, Abreo F, Unhold GP. Osteomyelitis

of the mandible as a result of sickle cell disease: report
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Federal Agency for the Support and Evaluation 1991;72(1):25-8. http://dx.doi.org/10.1016/0030-
4220(91)90184-E. PMid:1891241.
of Graduate Education—CAPES—and the
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Bilateral mandibular osteomyelitis mimicking periapical cysts in a patient with sickle cell anemia

Conflict of interest: None

Submitted on: March 7, 2015
Accepted on: June 20, 2015
Correspondence
Fabio Abreu Alves
Rua Prof Antonio Prudente, 211 – Liberdade
CEP: 01509-900 – São Paulo/SP – Brazil
Phone: +55 (11) 2189-5129
E-mail: [email protected]

60 Autopsy and Case Reports 2015;5(3):55-60