Thesis (Ph.D.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Background: Progressive familial intrahepatic cholestasis (PFIC) and Allagille syndrome (AGS) are hereditary cholestatic diseases that cause jaundice, severe debilitating life-limiting pruritus refractory to maximal medical treatment, and progressive liver damage. Surgical interruption of the enterohepatic circulation is considered a mainstay of alleviating distressing symptoms and delay progression to cirrhosis in cholestatic diseases including AGS and PFIC disease. Several strategies for diverting bile flow, including Partial external biliary diversion (PEBD), have been demonstrated. PEBD is considered an effective procedure with low morbidity and mortality. Objectives: Evaluate the effect of PEBD on pruritus, liver disease progression, growth, and life quality in children with chronic intrahepatic cholestasis. Methods: A prospective case series in which all patients with PFIC and AGS who underwent PEBD from July 2019 to July 2021 were included. Patients received medical treatment prior to biliary diversion and showed no improvement. The follow-up was conducted with pruritus assessment, bile acid level, and liver function tests. To determine the short-term effects of PEBD, clinical and laboratory workups, including growth data and quality of life, were reviewed. Partial internal drainage was offered for the patients who refused external diversion. Results: Seven patients underwent PEBD (5 with PFIC and 2 with AGS). The pre and post-PEBD clinical and laboratory assessments were compared. A remarkable statistical improvement was detected in pruritus score, bile acid level, and health-related quality of life assessment score. Amelioration of existing preoperative growth failure.The downscaling in the bilirubin level was not statistically significant. Moreover, there was no influential difference in the other liver function tests

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