A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome

A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest X-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the r...

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Bibliographic Details
Published in:Pediatric surgery international 2000-11, Vol.16 (8), p.599-601
Main Authors: SHIONO, Satoshi, YAMAGIWA, Iwao, OBATA, Kazuya, OKUYAMA, Naoki, OUCHI, Takayuki, SHIMAZAKI, Yasuhisa
Format: Article
Language:English
Subjects:
Bile Duct Diseases - complications
Bile Ducts, Extrahepatic - pathology
Biliary Atresia - complications
Biological and medical sciences
Cardiology. Vascular system
Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava
Dilatation, Pathologic
Female
Gastroenterology. Liver. Pancreas. Abdomen
Heart
Humans
Infant
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Malformations
Medical sciences
Spleen - abnormalities
Syndrome
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Summary:A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest X-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the right side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia syndrome, a hepatic portojejunostomy was performed at 80 days of age. The postoperative course was uneventful, and the jaundice cleared. Although the occurrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. Patients with BA and polysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice.
ISSN:0179-0358
1437-9813
DOI:10.1007/s003830000351