title: Biliary atresia with a "cyst at porta": management and outcome as per the cholangiographic anatomy.
abstract: The purpose of this study is to classify biliary atresia (BA) with a "cyst at porta" according to the cholangiographic anatomy and to define management strategy and outcome in each group. A cyst at porta was identified in 13 of 58 babies (22.4%) with BA at first presentation. The cholangiographic anatomy was classified as; Group A (n = 7), type III BA with extrahepatic cyst; Group B (n = 2), type I or II BA with extrahepatic biliary cyst; and Group C (n = 4), type I or II BA with both extrahepatic and intrahepatic biliary cysts. The remaining 45 patients were comprised of type III BA without a cyst. A Kasai's portoenterostomy (PE) was performed for all Group A patients. Groups B and C were treated by hepaticojejunostomy (n = 5) or portoenterostomy (n = 1). All 45 patients with type III BA without a cyst were treated by a Kasai's PE. The median age at surgery was 92 days (ranges 28-342 days). There were three early post-operative deaths, all in patients with type III BA without cyst. Overall 18/55 (32.7%) patients achieved a jaundice free state. In Group A, 5/7 (71.4%) patients had bile flow, 2/7 (28.6%) are anicteric and 2/7(28.6%) had 1-2 episodes of post-operative cholangitis. In Group B, both patients are anicteric and none had post-operative cholangitis. In Group C, all four babies had bile flow but, significant morbidity because of recurrent severe cholangitis. Only one patient reached a jaundice free state. Of the remaining 42 patients with type III BA without a cyst, 27 (64.3%) had bile flow, 13 (31%) became jaundice free and 14 (33.3%) have had 1-2 episodes of post-operative cholangitis. In conclusion, thirteen of 58 (22.4%) babies with BA had a "cyst at porta" at first presentation in this series. The outcome was most satisfactory in type I BA without intrahepatic cystic dilatation (Group B) in terms of achieving a jaundice free state and freedom from recurrent cholangitis. However, intrahepatic biliary cysts (Group C) were associated with recurrent severe cholangitis and a poor eventual outcome despite a good initial bile flow. The outcome in type III BA with extrahepatic cyst was comparable to type III BA without cyst.
authors.affiliation: Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, 226014 UP, India. richalal@sgpgi.ac.in
authors.forename: Richa
authors.initials: R
authors.lastname: Lal
authors.forename: D K V
authors.initials: DK
authors.lastname: Prasad
authors.forename: Phani
authors.initials: P
authors.lastname: Krishna
authors.forename: Sadiq S
authors.initials: SS
authors.lastname: Sikora
authors.forename: Ujjal
authors.initials: U
authors.lastname: Poddar
authors.forename: S K
authors.initials: SK
authors.lastname: Yachha
authors.forename: Niraj
authors.initials: N
authors.lastname: Kumari
mesh_terms: D001656:Biliary Atresia / Q000145:classification* / Q000000981:diagnostic imaging / Q000601:surgery*; D002758:Cholangiography; D003560:Cysts / Q000000981:diagnostic imaging* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000379:methods*