Spontaneous perforation of the extrahepatic bile duct in infancy: report of two cases and literature review

Sahnoun, L.; Belghith, Mohsen; Jallouli, Mohamed; Maazoun, K.; Mekki, M.; Brahim, M. Ben; Nouri, Anass

doi:10.1007/s00431-006-0167-3

Cited by 11 publications

(10 citation statements)

References 5 publications

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“…SBDP, however, has never previously been linked to cystic fibrosis or meconium ileus [4,7]. While patients with long standing CF may have biliary complications associated with their disease including cholestasis and strictures leading to cirrhosis and portal hypertension [9], SBDP has not been reported.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous bile duct perforation with cystic fibrosis and meconium ileus

Hooft

Notrica

Bae

2015

Journal of Pediatric Surgery Case Reports

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a b s t r a c tSpontaneous bile duct perforation in the newborn is rare, with less than 150 cases reported. A term male newborn presented with abdominal distention and respiratory distress on the first day of life. Paracentesis revealed 420 mL of bile without succus or meconium, and laparoscopic washout and drainage was performed. A follow-up contrast enema several days later revealed a microcolon. The second operation confirmed the spontaneous bile duct perforation, but also revealed meconium ileus with ileal perforation and thick meconium. A sweat chloride study was abnormal and the patient was homozygous for the delta-F508 mutation. This is the first reported case of spontaneous bile duct perforation in association with meconium ileus or cystic fibrosis. Ó 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Case reportA newborn 37 6/7weeks gestation Caucasian male infant weighing 3075 g presented at birth with abdominal distention. He was born via cesarean section with no significant antenatal history and a normal newborn screening panel to a G2P2 mother. Shortly after birth he developed apnea and respiratory distress associated with worsening abdominal distention requiring intubation and chest compressions. He was subsequently transferred for a higher level of care. On exam the patient had severe abdominal distention noted with thin skin and visible veins. He had not passed any meconium. An abdominal radiograph demonstrated non-dilated, centralized lops of bowel with no gas in the expected location of the colon (Fig. 1). Abdominal sonography demonstrated fluid with calcifications.An urgent bedside paracentesis was done for respiratory compromise and 420 mL of viscous, clear goldenegreen bile, with no succus or meconium, was drained. The excessive quantity of clear bile lead us to make the presumptive diagnosis of spontaneous bile perforation. He was then taken to the operating room for laparoscopic washout and drain placement.At laparoscopy, chronic bile peritonitis was encountered as evidenced by bile-stained fibrinous exudate completely covering the bowel and liver which caused the bowel to be completely adherent to itself. As there was no meconium spillage or identification of small bowel perforation, the loops of bowel were not completely separated during this operation. A laparoscopic-guided intraoperative cholangiogram was preformed via the gallbladder with a micro-puncture needle. This study was technically difficult due to the bile peritonitis and a discrete site of leakage was not identified. The puncture site on the gallbladder was closed and the abdomen was irrigated at the end of the procedure. A closed suction drain was left in the right upper quadrant and the patient was transferred back to the neonatal intensive care unit.Over the next few days there was no bilious drain output and no re-accumulation of fluid by ultrasound. The patient continued to be distended without any clinical signs o...

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Section: Discussionmentioning

confidence: 99%

Spontaneous bile duct perforation with cystic fibrosis and meconium ileus

Hooft

Notrica

Bae

2015

Journal of Pediatric Surgery Case Reports

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“…Hence, it is very important to make a prompt diagnosis for optimal surgical management. 1,3 Clinical features, such as jaundice, abdominal distension, ascites, normal to acholic stool, dark urine, anorexia, vomiting, irritability, mild fever, failure to thrive, peritonitis, and shock, may occur in spontaneous perforation of the bile duct. 1e3 If there has been a healthy interval from birth to clinical presentation, the probability of spontaneous perforation of the bile duct is increased.…”

Section: Discussionmentioning

confidence: 99%

“…Drainage often leads to spontaneous closure of a perforated bile duct, but surgical treatment should be individualized. 1,3,4 Bile duct stenosis is the most common complication after simple drainage and may progress to biliary cirrhosis and portal hypertension. Intraoperative cholangiography is necessary to confirm the potential anatomical anomaly or distal obstruction.…”

Section: Discussionmentioning

confidence: 99%

“…However, clinical features and laboratory evaluations are not pathognomonic, and diagnosis is mostly confirmed by laparotomy. 1,3 Surgical methods include simple drainage with or without repair of perforation, cholecystectomy, external biliary diversion, or biliary reconstruction. Drainage often leads to spontaneous closure of a perforated bile duct, but surgical treatment should be individualized.…”

Section: Discussionmentioning

confidence: 99%

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Spontaneous perforation of the bile duct in a neonate: Drainage or resection?

Chen¹,

Chen²,

Chou³

2012

Journal of the Chinese Medical Association

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Spontaneous perforation of the bile duct is a rare disease, and delayed diagnosis without optimal treatment can be fatal. Abdominal drainage with or without repair of perforation seems to be adequate in most case series. We report on a 10-day-old female neonate with spontaneous perforation of the bile duct over the junction of cystic duct and common hepatic duct, who recovered uneventfully with follow-up for 3 years after receiving a single-stage operation of cholecystectomy and biliary reconstruction. Drainage only or resection of the gall bladder or bile duct should depend on the patient's clinical conditions and intraoperative findings.

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“…Spontaneous perforation of the biliary tract in the absence of gallstones is mostly reported in (young) children. Mechanisms of perforation of the biliary tract are biliary tract anomalies (especially cysts), ascariasis and cholecystitis [ 6 - 8 ]. A possible mechanism of spontaneous perforation of the cystic duct in our patient is local necrosis due to microcirculatory failure as a result of hypoperfusion and microthrombosis.…”

Section: Discussionmentioning

confidence: 99%