Treatment of biliary atresia and analysis of other cholestases: a 10-year single-center retrospective study

Abstract

Objective To analyze the clinical data and to summarize the clinical features of biliary atresia (BA) and other causes of cholestasis. Methods For this retrospective study between January 2003 and March 2014, a total of 1321 cholestatic infants with a strong suspicion of BA were recruited and assigned into BA/non-BA group depending on the findings of intraoperative cholangiography (IOC). Preoperative demographics (age & gender), types of obstruction, rate of Kasai’s operation and associated anomalies were documented. And the specific diagnoses of suspected BA cases were recorded. Results Among them, 1202 were diagnosed as BA while 119 excluded of BA by IOC. While 1108(92.1%) cases of BA underwent Kasai’s procedure while 94(7.8%) received IOC only. There were 611 boys (50.8%) in BA group and 101 boys (84.9%) in non-BA group (P<0.001). The mean ages of both groups was comparable [(73.4±24.6) vs. (74.1±24.7) days, P=0.93]. Around 100 (8.3%) of them had associated anomalies. Intraabdominal anomalies included intestinal malrotation (n=5), intestinal atresia (n=3), Meckel’s diverticulum (n=1), congenital hypertrophic pyloris (n=1), rectovestibular fistula (n=1), situs inversus (n=6), polysplenia (n=1) and hepatic arterial anomaly (n=2). And cardiac malformations included atrial septal defect (n=55), patent ductus arteriosus (n=13), ventricular septal defect (n=11) and pulmonary artery stenosis (n=5). One patient had hydronephrosis. Other associated anomalies included pulmonary cyst (n=1), diaphragmatic eventeration (n=1) and polydactyly (n=1). Seven BA cases had multiple malformations. The suspected cases of BA were idiopathic neonatal hepatitis, cytomegalovirus hepatitis, Alagille’s syndrome, neonatal intrahepatic cholestasis due to citrin deficiency (NICCD) and biliary hypoplasia. Conclusions At our center, age at diagnosis has not dramatically decreased. An early diagnosis and proper treatment of BA remain challenging. Cardiac malformations and intestinal malrotations are common associated anomalies in BA. To avoid unnecessary surgery, a meticulous preoperative workup should be performed to exclude other causes of cholestasis. And highly suspected cases of BA may be caused by idiopathic neonatal hepatitis and cytomegalovirus hepatitis. Key words: Biliary atresia; Abnormalities, multiple; Cholestasis; Infant, newborn