S2367 Push Enteroscopy-Assisted ERCP for Treatment of Biliary Obstruction and Cholangitis in a Patient With Kasai Porto-Enterostomy

Abstract

INTRODUCTION: Biliary obstruction and acute cholangitis are late complications in patients with Kasai porto-enterostomy performed for biliary atresia. Patients typically present with fevers and jaundice and can progress to liver failure. Only a few cases have reported successful endoscopic treatment for biliary obstruction in adults with a previous Kasai procedure. Here we describe the case of a 34-year-old female with Kasai porto-enterostomy with recurrent cholangitis. A successful enteroscopy-assisted ERCP was performed with resolution of symptoms. CASE DESCRIPTION/METHODS: A 34-year-old female with a history of biliary atresia status post Kasai procedure done shortly after birth presented with intermittent fevers, refractory pruritus, and recent hospitalization for acute cholangitis. Her workup revealed elevated liver tests and serum bile acids. MRCP showed right intrahepatic bile duct (RHD) dilatation with peribiliary cysts, trace left intrahepatic bile duct (LHD) dilatation and focal stricture at the porto-enterostomy anastomosis. We performed an enteroscopy-assisted ERCP with the patient in supine position. A pediatric colonoscope with a clear cap was used to access the biliary limb. After applying external abdominal pressure, the porto-enterostomy was reached and showed a severely stenosed opening of the bile ducts (Figure 1). The RHD was deeply cannulated using a 0.025” hydrophilic-tipped guidewire. Cholangiogram showed dilated RHD up to 10mm with severe stricture at the porto-enterostomy (Figure 2). The stricture was dilated with a balloon dilator first to 3mm then 4mm. A 5Fr x 4cm plastic stent with a single internal flange and single external pigtail was placed into the RHD. Cannulation of the LHD was unsuccessful due to complete obstruction of the ducts. A submucosal ink injection was used to mark the biliary limb. The patient was discharged home the same day. Both her fever and pruritus resolved and she has not had further episodes of cholangitis. Her liver tests improved markedly (Table 1). On repeat ERCP, the prior stent was exchanged for a 7Fr x 5cm stent. Liver enzymes normalized. No late complications occurred. DISCUSSION: Enteroscopy-assisted ERCP is a feasible treatment for biliary obstruction in patients with Kasai porto-enterostomy. Altered anatomy and limited choice of tools make interventions challenging. This procedure should be considered as an alternative to more invasive percutaneous and surgical techniques and as part of a multidisciplinary approach.Table 1.: Serum Bile Acids, Hepatic Panel, Complete blood count, C-reactive Protein prior to ERCP vs. 4 and 12 weeks post ERCP with stent placementFigure 1.: Severely stenosed bile ducts (black arrow).Figure 2.: Cholangiogram with dilated right intrahepatic bile duct.