Spontaneous perforation of the extrahepatic biliary tract in infancy and childhood |
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Authors: | Thomas C. Moore Robert B. Cameron |
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Affiliation: | (1) Department of Surgery UCLA School of Medicine and the Pediatric Surgical Service, Harbor-UCLA Medical Center, 90509 Torrance, CA, USA;(2) Present address: Pediatric Surgical Service, Harbor-UCLA Medical Center, 90509 Torrance, CA, USA |
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Abstract: | Spontaneous perforation of the extrahepatic biliary tract in infancy and early childhood is a poorly understood and infrequently reported disorder of unknown etiology. The reported experience with 77 operative cases is reviwed. The onset is generally insidious, but may be acute in 25% of cases. The major symptoms are abdominal distention and jaundice. The younger the patient at the time of onset, the more likely is jaundice to be found. Drainage-type operations were most commonly used in the early reported cases while more recently anastomosis/bypass procedures have predominated due to the relatively frequent occurrence of distal biliary tract obstruction. This disorder appears to be a part of the biliary atresia — neonatal hepatitis — choledochal cyst complex which occurs in the neonatal period and early infancy and appears to be acquired rather than congenital. Because of the theoretical potential for late malignancy, lifelong follow-up with periodic evaluation of the intra- and extrahepatic biliary tracts is recommended. The advisability of liver transplantation (and accompanying immunosuppression) as the primary surgical treatment for biliary atresia may be questioned. |
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Keywords: | Spontaneous perforation Bile ducts Infancy Literature review |
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