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An association of multiple intestinal atresia and biliary atresia: a case report
J Yanagihara1, K Nakamura, T Shimotake
1Division of Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine.
Insights
This report details a rare case of a newborn with both biliary atresia and multiple intestinal atresia. The infant experienced severe gastrointestinal issues and jaundice, ultimately succumbing to complications.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatal Medicine
Background:
- Biliary atresia and intestinal atresia are serious conditions affecting newborns.
- Simultaneous occurrence of these atresias is exceptionally rare.
Observation:
- A 2-day-old female infant presented with intractable vomiting and signs of jaundice.
- Diagnostic imaging revealed jejunal and rectal atresia.
- Initial laparotomy confirmed multiple intestinal atresias (10 small bowel, 3 colon).
Findings:
- Postoperative persistence of acholic stools and jaundice indicated biliary atresia.
- A second laparotomy confirmed Type 3 biliary atresia.
- Kasai procedure with jejunostomy was performed, but complications arose.
Implications:
- This case highlights the critical need for early diagnosis of combined biliary and intestinal atresias.
- Management challenges include short bowel syndrome and postoperative complications like cholangitis.
- Further research into the etiology and optimal surgical strategies for associated atresias is warranted.
Abstract:
This is the first report of a 2-day-old girl having an association of biliary atresia and multiple intestinal atresia. She developed intractable vomiting in the first hours of life. An upper gastrointestinal series and barium enema showed jejunal atresia and rectal atresia. A laparotomy was performed on the fourth day of life. The operative findings were multiple sites of atresia of the intestine, 10 atretic sites in the small bowel, and 3 in the colon. The gall bladder was normal in size and position. After the operation, the patient's stool remained acholic and her jaundice did not resolve. On the 58th day of life, another laparotomy was performed with the presumptive diagnosis of biliary atresia. The operative finding was biliary atresia, type 3; the Kasai operation with external jejunostomy was performed. The patient suffered from postoperative cholangitis and enterocolitis because of her short bowel and subsequently died at 9 months of age. This is the first case of biliary atresia associated with multiple intestinal atresia reported in the literature.

