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Spontaneous perforation of choledochal cyst: a study of 13 cases
1Department of Pediatric Surgery, Juntendo University, School of Medicine, Shizuoka Children's Hospital Tokyo, Japan.
Insights
Spontaneous perforation of infantile choledochal cysts is not rare, occurring in 13 of 187 cases. Pancreatico-biliary malunion and infancy-related mural immaturity are likely causes.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Hepatobiliary Surgery
Background:
- Infantile choledochal cyst (CC) is a congenital biliary anomaly.
- Spontaneous perforation is a rare but serious complication.
Purpose of the Study:
- To investigate the incidence and etiology of spontaneous perforation in infantile choledochal cysts.
Main Methods:
- Retrospective review of 187 infantile choledochal cyst cases.
- Analysis of perforation characteristics, clinical presentation, and associated factors.
Main Results:
- 13 cases (7%) of spontaneous perforation were identified in patients under 4 years old.
- Perforations presented as biliary peritonitis (8 cases) or sealed perforations (5 cases).
- Etiology linked to pancreatico-biliary malunion and mural immaturity, not solely ductal pressure or weakness.
Conclusions:
- Spontaneous perforation of infantile choledochal cysts is an underrecognized complication.
- Refluxed pancreatic juice due to malunion and infantile mural immaturity are primary etiological factors.
Abstract:
Of the 187 cases of infantile choledochal cyst treated at our hospitals, we encountered 13 with spontaneous perforation. All cases were under 4 years old. Eight cases were found to have biliary peritonitis and 5 had a sealed perforation. The shape of the extrahepatic bile duct was cystic in 8 and fusiform in 5. The cyst wall around the perforation was filmy and bile was found to be oozing through the thinned wall. Nine perforations were single while 4 cases had multiple perforations. Four of 17 perforations occurred in the posterior part of the cyst wall. Only 1 case of perforation was associated with protein plugs in a common channel, while 7 of the 10 cases of choledochal cyst requiring percutaneous biliary drainage due to signs of raised intrabiliary pressure were found to have protein plugs. We consider that spontaneous perforation of a choledochal cyst is not rare in infancy. The etiology of a perforation must be epithelial irritation of the biliary tract due to refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity due to infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point.