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Spontaneous perforation of the biliary tract in infancy: a series of 11 cases
C Chardot1, F Iskandarani, O De Dreuzy
1Surgical Unit, Department of Paediatrics of Bicêtre Hôpital, Le Kremlin-Bicêtre, France.
Insights
Spontaneous biliary tract perforation is a rare condition in infants. Prompt diagnosis and surgical intervention are crucial for improving outcomes and reducing long-term complications in affected children.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Biliary Tract Diseases
Background:
- Spontaneous perforation of the biliary tract is a rare neonatal surgical emergency.
- It often presents with cholestatic jaundice after a symptom-free interval.
- Presentation patterns include generalized biliary peritonitis, localized biliary peritonitis, and secondary biliary stenosis.
Purpose of the Study:
- To review the clinical presentation, management, and outcomes of spontaneous biliary tract perforation in infants.
- To identify factors influencing prognosis and long-term sequelae.
Main Methods:
- Retrospective case series of eleven patients treated between 1971 and 1993.
- Analysis of clinical data, surgical procedures, complications, and long-term follow-up.
- Classification of patients based on local presentation patterns.
Main Results:
- Ten patients underwent surgery with varying procedures including cholecystectomy, biliary drainage, and reconstruction.
- Postoperative complications included bile leak, cholangitis, and portal vein thrombosis.
- Four patients had late sequelae, including portal hypertension and liver fibrosis.
Conclusions:
- Spontaneous biliary tract perforation requires prompt diagnosis and appropriate surgical management.
- Early intervention can improve prognosis and minimize long-term complications.
- This rare condition necessitates a high index of suspicion in neonates with cholestatic jaundice.
Abstract:
Eleven patients presenting with spontaneous perforation of the biliary tract were treated at Bicêtre Hospital between 1971 and 1993. Three groups were individualised, each with a different pattern of local presentation: generalised biliary peritonitis (n = 2), localised biliary peritonitis (n = 4), secondary biliary stenosis (n = 5). In each case, cholestatic jaundice developed after a postnatal symptom-free interval. Ten patients were operated on. Perforation was located in the cystic duct (n = 2), at the junction of the cystic and hepatic ducts (n = 4), in the common hepatic duct (n = 1) or common bile duct (n = 1). The site of perforation was no longer identifiable in two cases with stenosis. A cholecystectomy was performed in the 2 cases with cystic duct perforation; in the cases of lesions of the main duct, either simple external biliary drainage (n = 3) or biliary reconstruction (n = 5) was carried out. Postoperative complications included bile leak (n = 2), ascending cholangitis (n = 1), portal vein thrombosis (n = 2). Five patients were submitted to further surgery including biliary revision (n = 3), porto-systemic shunt (n = 1), and other procedures (n = 2). One infant died from postoperative sepsis; 2 were lost to follow-up, one of which probably did not survive; 4 are alive and well. Late sequelae are present in 4 children: portal hypertension (n = 1), mild residual bile duct dilatation without cholestasis (n = 1), and mild to moderate liver fibrosis (n = 2). Prompt diagnosis and appropriate treatment should improve the prognosis of this rare condition.