BDP in Children—TR Sai Prasad et al 905

Case Report

Bile Duct Perforation in Children: Is it Truly Spontaneous?

TR Sai Prasad,1MRCS, MCh, Chan Hon Chui,1FRCS, FAMS, Yee Low,1FRCS, FAMS, Chia Li Chong,2FRCR, FAMS,
Anette Sundfor Jacobsen,1FRCS, FAMS

Abstract
Introduction: Bile duct perforation (BDP) with resultant biliary ascites in children is a rare
clinical condition. The aetiopathogenesis is still an enigma, with increasing evidence suggesting
anomalous union of pancreaticobiliary ductal (AUPBD) system as the prime causative factor.
Clinical Picture: We report 2 cases of spontaneous perforation of the bile duct confirmed on
histopathological examination as choledochal cyst, in a 6-month-old female child and a 4-year-
old boy who presented with subtle clinical symptoms. Treatment and Outcome: Both patients
were successfully managed by excision of the gall bladder and common bile duct and Roux-en-
Y hepaticojejunostomy. This procedure was performed following initial cholecystostomy drainage
in the second case. Conclusions: From the available literature and experience with our patients,
BDP is not merely spontaneous but may be related to AUPBD and choledochal cyst.
Ann Acad Med Singapore 2006;35:905-8

Key words: Aetiology, Biliary ascites, Choledochal cyst, Treatment

Introduction was admitted to the paediatric ward with complaints of

Bile duct perforation (BDP) and biliary ascites in children poor feeding and lethargy of 2 days’ duration and 1 episode
is a rare clinical entity. The aetiopathogenesis is still of non-bilious vomiting. Over the next 2 days, the child
elusive. Growing evidence suggests that BDP and developed fever and seemed to draw up her legs when
choledochal cyst (CC) are interrelated entities with a abdominal examination was performed. The child could
common pathogenetic factor, the anomalous union of the tolerate oral feeds but had clay-coloured stools.
pancreaticobiliary ductal system (AUPBD).1-4 Virtually all The preliminary investigations were unremarkable except
fully investigated cases of BDP in oriental patients have for raised total leukocyte count (23 x 109/L). The septic
demonstrated AUPBD, which is also an aetiopathogenetic screen was also unremarkable. The blood investigations
factor for genesis of choledochal cyst.1-4 The differentiation showed total bilirubin 55 µmol/L (≤14), direct bilirubin
between BDP and rupture of the choledochal cyst is 31 µmol/L (0-7), total protein 47 g/L (53-78), albumin 28
debatable and this has bearing on the final management g/L (38-53), alkaline phosphatase 110 U/L (157-363 U/L),
plan. Simple drainage with or without suture closure, alanine transaminase 8 U/L (9-48 U/L), aspartate trans-
T-tube or cholecystostomy has been proposed as the aminase 29 U/L (16-54 U/L) and gamma glutamyl
treatment of choice. Definitive surgery in the form of transferase 269 U/L (5-26 U/L) (Figures in brackets indicate
excision of the CC, cholecystectomy and Roux-en-Y normal range). Serial abdominal radiographs showed
hepatico/portoenterostomy seems to be the ideal option. stagnant bowel loops. Ultrasonography revealed fairly
We present 2 cases of bile duct perforation in children and large amount of ascites with low level internal echoes,
discuss the dilemmas in the aetiopathogenesis and septations and loculations on the right side of the abdomen,
management in the light of available literature. likely inflammatory in nature. The gall bladder contained
sludge and had a thickened wall (26 mm). The intra-hepatic
Case Report biliary radicles were normal and the common bile duct was
Case 1 obscured by bowel gas. A few bowel loops in the right
A 6-month-old girl with no previous medical problems lower quadrant were thickened and oedematous. The

1
Department of Paediatric Surgery
2
Department of Diagnostic Imaging
KK Women’s and Children’s Hospital, Singapore
Address for Reprints: Dr Sai Prasad TR, Department of Paediatric Surgery, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899.
Email: [email protected]

December 2006, Vol. 35 No. 12

906 BDP in Children—TR Sai Prasad et al

Fig. 1. CT scan of the abdomen showing dilated CBD (arrow) at the Fig. 2. CT scan of the abdomen showing thickened gall bladder with
pancreatic head with periductal fluid collection. pericholecystic collection (1) and dilated CBD (2).

Case 2
A 4-year-old male child with no previous medical history,
presented with complaints of abdominal pain and non-
bilious vomiting of a day’s duration. The right upper
quadrant of the abdomen was tender on palpation. The
haemogram showed leukocytosis (19.6 x 109/L) and
neutrophilia (89%). The liver function test revealed
transaminitis and elevated gamma glutamyl tranferase (total
bilirubin 17 µmol/L (≤14) direct bilirubin 5 µmol/L (0-7),
alanine transaminase 227 U/L (8-29), aspartate transaminase
132 U/L (16-54), gamma glutamyl tranferase 75 U/L (9-41)
alkaline phosphatase 256 U/L (110-345) and amylase 123
U/L (39-123).
Fig. 3. Cholangiogram showing dilated CBD suggestive of a fusiform
choledochal cyst (1) with reflux into the pancreatic duct (2) and a long bilio-
Ultrasonography of the abdomen revealed a grossly
pancreatic channel (3). distended gall bladder with pericholecystic fluid collection
and a dilated CBD extending to the intrapancreatic portion.
findings were suggestive of intraabdominal sepsis although Intravenous cefazolin was administered with a provisional
the primary source could not be ascertained. Computed diagnosis of acute acalculus cholecystitis. CT scan of the
tomography (CT) scan of the abdomen confirmed the same abdomen was done as the symptoms worsened. This revealed
findings and also revealed a dilated common bile duct significant ascites as well as dilated hepatic and CBD (8
(CBD) with its largest diameter (1 cm) at the level of the mm) and thickening of the gall bladder wall with
pancreatic head (Fig. 1). pericholecystic fluid collection (Fig. 2).
The child was subjected to diagnostic laparoscopy with Laparoscopy was performed with a provisional diagnosis
provisional diagnosis of infective peritonitis. The findings of biliary ascites due to spontaneous BDP or rupture of the
revealed bile in the peritoneum, which was found later to be CC. At surgery, significant biliary ascites was noted, but
sterile with total bilirubin of 383 mmol/L. Conversion to definite site of perforation could not be seen. The antrum
laparotomy was done and extensive bile staining of of the stomach was adherent to the undersurface of the
abdominal viscerae was noted with a perforation on the liver. Cholecystostomy was performed and drains were
posterior wall of the CBD, which appeared to be mildly placed in the subhepatic space and pelvis after peritoneal
dilated. The liver was unremarkable and the gall bladder lavage.
was distended. As the condition of the baby was stable, Cholangiography performed on the eighth postoperative
excision of the common bile duct and gall bladder with day through the cholecystostomy tube revealed a fusiform
Roux-en-Y hepaticojejunostomy was done. The diagnosis Type 1 CC with a long common bilio-pancreatic channel
of perforated Type 1 CC with bile duct measuring 7 mm in (Fig. 3). The patient was discharged and readmitted 3
diameter was confirmed on histopathology. The post- weeks postoperatively for the excision of the choledochal
operative recovery was uneventful and the child is well at cyst. At laparotomy, a Type 1 fusiform CC was noticed
2 years after surgery. with a long common bilio-pancreatic channel. The CC was

Annals Academy of Medicine

 BDP in Children—TR Sai Prasad et al 907

excised and Roux-en-Y hepaticojejunostomy was retrospective review of the CT scan in our first case, the
performed. The postoperative recovery was uneventful and CBD had a crinkled appearance and there appeared to be a
the child is well 1 year after the surgery. defect in its posteromedial aspect. The crinkled appearance
was postulated to be due to the release of tension when the
Discussion cyst ruptured. Abdominal paracentesis can be diagnostic
Biliary ascites in children due to BDP is a rare clinical with ascitic bilirubin levels ranging from 7.7 mg% to 420
condition with fewer than 100 cases reported in the literature mg%, but carries the risk of infecting the sterile bile.8 The
worldwide.5-7 The peak age of occurrence is around 6 presence of bile in the peritoneal cavity associated with
months with age ranging from 25 weeks gestation to 7 obstructive jaundice in the absence of liver derangement is
years.8 The aetiopathogenesis is yet to be elucidated as considered pathognomonic for BDP.12 Hepatobiliary
various theories have been proposed such as congenital scintigraphy with Tc 99m imidodiacetic acid derivatives
weakness of the bile duct, pancreaticobiliary anomalies may demonstrate the leak of radionuclide tracer and aid in
including AUPBD, pancreatitis, distal obstruction of the the definitive diagnosis of BDP.13 Magnetic resonance
bile duct due to inspissated bile, stones or stenoses, presence cholangiopancreatography (MRCP) provides a non-
of a diverticulum or abnormal gland of the bile duct wall, invasive method of evaluating the biliary tree using heavily
viral infection of the bile duct, tuberculosis, necrotising T2-weighted sequences.14 The signals from either static or
enterocolitis and birth trauma.2,6,9 Obstruction of the bile slowly moving liquids are augmented, and liquid-filled
duct by protein plugs, inspissated bile or stone is considered compartments like bile ducts or pancreatic ducts and the
as a consequence of BDP, slow bile transit and biliary stasis contrast between ducts and base are made evident without
rather than the cause of perforation.8 Filling defects in the using a paramagnetic contrast media. Owing to its excellent
common channel seen on cholangiograms may be anatomical and contrast resolutions, MRCP can delineate
misleading, as stenoses, calculi, protein plugs and biliary BDP. MRCP also evaluates the pancreatobiliary junction
sludge have resolved spontaneously with adequate anomalies such as AUPBD, which would aid in discerning
drainage.6,7 the aetiopathology and management plans for BDP.
Although BDP and CC are considered as different entities Recently, the effectiveness of dynamic MRCP with secretin
there is growing evidence that both may be interrelated stimulation has been reported in the evaluation of pancreato-
problems with a common pathogenesis. AUPBD has been biliary maljunction anomalies.15 However, subtle clinical
demonstrated in fully investigated cases of BDP1-4,6 but signs, cost and availability limit the use of MRCP in
many former reports have not taken into consideration the evaluation of children presenting with features of acute
AUPBD.1,2 The difficulty in ascribing the cause of abdomen. Endoscopic retrograde cholangiopancreato-
perforation secondary to CC is due to the first time graphy aids in the delineation of BDP and AUPBD, but the
presentation, small size of the cyst and collapse of the cyst invasiveness and risk of complications such as pancreatitis,
wall following perforation. This was true with our first cholangitis, sepsis, adverse reactions to contrast media and
case. Yamaguchi et al9 reported 26 cases of perforation bile duct or duodenal injury limits its utilisation in evaluation
(1.8%) in 1433 patients with CC. The incidence of of suspected BDP.16 Laparoscopy is useful in ascertaining
perforation was 2.1% as reported by the American Academy the diagnosis and also to drain the perforation.8 Due to
of Pediatrics in a review of 188 patients with CC.10 worsening abdominal distension, our patients were
BDP is frequently diagnosed only at laparotomy as the subjected to diagnostic laparoscopy with a provisional
condition is rare and the clinical presentation is usually diagnosis of appendicular perforation or primary peritonitis
insidious with subtle symptoms.7,8,11 The symptomatology in the first case and BDP in the second case.
includes mild jaundice, progressive abdominal distension, The perforation is usually seen as a punched-out hole in
ascites, vomiting and acholic stools. Acute onset of the anterior aspect of BD at the junction with the cystic duct
symptoms in the form of abdominal distension, fever, as it has been suggested that this may be prone to mural
vomiting and signs of fulminant bile peritonitis is malformation during embryogenesis.6 On the contrary,
exceptional.7,8 there are reports stating that the usual location of perforation
The investigations usually reveal cholestatic jaundice is on the posterior wall of the CC as was seen with our
with near normal liver function tests, which differentiate patient. Operative cholangiogram confirms the diagnosis,
BDP from biliary atresia and neonatal hepatitis. 7 sites the perforation, evaluates the status of the distal BD
Ultrasonography shows ascites, localised fluid collection including AUPBD and provides a road map for surgery.7,8
around the porta and non-dilated bile duct.8 CT scan of the It was not done in our first case as the perforation was
abdomen reveals the same findings as ultrasound and it is evident but retrospectively, we believe that it would aid as
difficult to diagnose the perforation preoperatively. On described.

December 2006, Vol. 35 No. 12

908 BDP in Children—TR Sai Prasad et al

Management options vary from simple drainage to REFERENCES

definitive correction in the form of excision and bilioenteric 1. Ohkawa H, Takahashi H, Maie M. A malformation of the pancreatico-
drainage.7,8 Operative modalities suggested include simple biliary system as a cause of perforation of the biliary tract in childhood.
drainage with or without suture closure,5,10 T tube drainage J Pediatr Surg 1977;12:541-6.

and/or cholecystostomy.8 Other options like cholecystec- 2. Yano H, Matsumoto H. Choledochal cyst following operation for
idiopathic perforation of the biliary tract in childhood. Jpn J Surg
tomy, hepaticocholedochostomy, hepaticoduodenostomy, 1983;13:441-5.
hepaticojejunostomy, choledochocholedochostomy, 3. Hasegawa T, Udatsu Y, Kamiyama M, Kimura T, Sasaki T, Okada A, et
choledochojejunostomy or resection of biliary pseudocyst al. Does pancreatico-biliary maljunction play a role in spontaneous
are considered to be unnecessary and carry a high morbidity perforation of the bile duct in children? Pediatr Surg Int 2000;16:550-3.
and mortality risk.8 Simple drainage with or without suture 4. Ng WT, Cheung CH, Chan S. Is spontaneous perforation of the bile duct
closure entails less morbidity and may cure the condition or in children due solely to pancreatico-biliary maljunction? Pediatr Surg
Int 2002;18:565-6.
stabilise the patient for a second look definitive surgery.
5. Kasat LS, Borwankar SS, Jain M, Naregal A. Spontaneous perforation of
Presence of T tube/cholecystostomy facilitates biliary the extrahepatic bile duct in an infant. Pediatr Surg Int 2001;17:463-4.
drainage and postoperative assessment of the BD anatomy.7
6. Ando H, Ito T, Watanabe Y, Seo T, Kaneko K, Nagaya M. Spontaneous
AUPBD has been claimed to be responsible for the perforation of choledochal cyst. J Am Coll Surg 1995;181:125-8.
development of CC and BDP in infancy and may be the 7. Spigland N, Greco R, Rosenfeld D. Spontaneous biliary perforation:
cause of cancer of BD in later life.8 The risk of malignant does external drainage constitute adequate therapy? J Pediatr Surg
1996;31:782-4.
degeneration of CC is well documented in the literature.
8. Banani SA, Bahador A, Nezakatgoo N. Idiopathic perforation of the
This risk appears to be age related with less than 1% risk in
extrahepatic bile duct in infancy: pathogenesis, diagnosis, and
patients less than 10 years of age and about a 15% risk in management. J Pediatr Surg 1993;28:950-2.
patients over 20 years of age. Since there is potential risk 9. Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients
of subsequent malignant degeneration, we believe that in the Japanese literature. Am J Surg 1980;140:653-7.
these patients with BDP should undergo excision of the 10. Kim SH. Choledochal cyst: survey by the surgical section of the
BD/CC and bilioenteric drainage. There are few case American Academy of Pediatrics. J Pediatr Surg 1981;16:402-7.
reports of successful treatment of the condition by definitive 11. Moss RL, Musemeche CA. Successful management of ruptured
surgery, as most believe that extensive procedures bear choledochal cyst by primary cyst excision and biliary reconstruction. J
Pediatr Surg 1997;32:1490-1.
high risk of morbidity and mortality.11 We were prompted
12. Ford WD, Sen S, Morris L, LeQuesne G. Spontaneous perforation of the
towards cholecystostomy drainage with peritoneal lavage common bile-duct in the neonate: imaging and treatment. Aust Paediatr
for the second case, based on the reports in the literature.5,7,8,10 J 1988;24:306-8.
The postoperative cholangiogram delineated the 13. Fawcett HD, Hayden CK, Swischuk LE, Lobe TE. Spontaneous
choledochal cyst with a long common bilio-pancreatic extrahepatic biliary duct perforation in infancy. Can Assoc Radiol J
channel.17 1986;37:206-7.
14. Sugiyama M, Baba M, Atomi Y, Hanaoka H, Mizutani Y, Hachiya J.
Although around 100 cases of BDP have been described Diagnosis of anomalous pancreaticobiliary junction: value of magnetic
and the incident is spontaneous, the etiopathogenesis is still resonance cholangiopancreatography. Surgery 1998;123:391-7.
an enigma. In the light of available literature evidence and 15. Fukukura Y, Fujiyoshi F, Sasaki M, Nakajo M. Pancreatic duct:
experience with our patients, there are reasons to deem that morphologic evaluation with MR cholangiopancreatography after secretin
BDP is not merely spontaneous and may be related to stimulation. Radiology 2002;222:674-80.
AUPBD and choledochal cyst. 1-4,6 Hence, we believe that 16. Farrell RJ, Noonan N, Mahmud N, Morrin MM, Kelleher D, Keeling PW.
Potential impact of magnetic resonance cholangiopancreatography on
if the condition of the baby were stable, definitive surgery
endoscopic retrograde cholangiopancreatography workload and
as described would be an ideal option, reserving simple complication rate in patients referred because of abdominal pain.
drainage procedure with or without T tube/cholecystostomy Endoscopy 2001;33:668-75.
for patients with poor general condition.3,6,11 Proper meta 17. Wagholikar GD, Chetri K, Yachha SK, Sikora SS. Spontaneous
analysis of all the cases reported with long-term follow-up perforation – a rare complication of choledochal cyst. Indian J
Gastroenterol 2004;23:111-2.
may provide an answer.

Annals Academy of Medicine