EN
TÜRKÇE
Poster - 16
A rare subtype of biliary atresia: report of two cases with cystic biliary atresia
İ Çiftçi*, M Sarıkaya*, F Özcan Sıkı*, M Gündüz*, T Sekmenli*, İ Yağmurlu*, M Öztürk**, M Konak***, S Sündüs Uygun***, M Gümüş****, HH Emiroğlu****
*Selcuk University Faculty of Medicine, Department of Pediatric Surgery
**Selcuk University Faculty of Medicine Department of Radiology
***Selcuk University Medical Faculty Department of Neonatology
****Selcuk University Faculty of Medicine Department of Pediatric Gastroenterology
Introduction
Cystic biliary atresia (CBA) is a rare but an important type of biliary atresia (BA). It has been determined that this accounts for approximately 5-10% of cases of biliary atresia. It is very important to differentiate this from choledochal cyst in infants with cholestasis and cystic lesions. The objective of this study was to present two cases of CBA that were operated on.
Case Presentation
Case 1
A 37-week-old male patient was examined on postnatal day 10 due to the presence of hyperbilirubinemia and acholic fecal matter. On postnatal day 25, Kasai portoenterostomy was performed upon the detection of CBA. Liver biopsy revealed congenital hepatic fibrosis. Liver function tests, direct bilirubin value, growth, and development were found to be within normal limits during the 7th postnatal month.
Case 2
A 38-week-old female patient was examined for hyperbilirubinemia and acholic stool during postnatal follow-up. The patient's total bilirubin level was 9.27 mg/dL, and the imaging findings were indicative of CBA. On postnatal day 30, Kasai portoenterostomy was performed. Liver biopsy revealed congenital hepatic fibrosis. Liver function tests, direct bilirubin value, growth and development were found to be normal in the 8th postnatal month.
Conclusion
CBA, a rare subtype of BA, is frequently misdiagnosed as a choledochal cyst. The preferred treatment is to perform the kasai operation as soon as possible. This disadvantageous situation, as evidenced by the cases presented, results in earlier diagnosis and decreased postoperative mortality and morbidity of BA disease, for which early operation (within the first 60 days) is imperative.