Biliary dilatation and strictures after composite liver-small bowel transplantation in children: defining a newly recognized complication.

Background: Biliary dilatation and strictures (BDS) are well recognized after liver transplantation but not reported after composite liver-small bowel transplantation (CLSBT). We aimed to describe and propose a classification of BDS in children undergoing CLSBT and analyze the potential risk factors.

Methods: Biliary complications of 47 consecutive children undergoing CLSBT were reviewed and classified according to presentation, location, and intervention required. The following variables were studied: small recipient (weight, <10 kg), donor-recipient weight ratio, liver/bowel graft reduction/not, partial/full pancreas, liver/bowel rejection, and median cold ischemia time (>454 min).

Results: Twenty-one (45%) children developed BDS at median 190 days (22 [7-138] months follow-up). Five distinct biliary lesions were identified. Most of the BDS (14/21; 67%) consisted of sphincter dysfunction-related bile duct dilatation (type I), whereas others (7/21; 33%) comprised extrahepatic bile duct (type II; n=3), hilar (type III; n=1), segmental (type IV; n=1), and diffuse (type V; n=2) intrahepatic strictures. None of the graft reduction strategies or other variables studied demonstrated a significant association with BDS. Therapeutic intervention was required in 1 of 14 type I and four of seven type II to V BDS in the form of percutaneous biliary dilatation with or without drainage.

Conclusion: This article identifies BDS after CLSBT as a frequent late complication after CLSBT, which has a benign outcome in most cases. The natural history of children with extrahepatic and intrahepatic strictures is not yet clear and will need multicenter prospective studies.