Outcome of partial internal biliary diversion for intractable pruritus in children with cholestatic liver disease

Abstract

To our knowledge, this is the largest reported series of children with PIBD. PIBD is a safe, well-tolerated and effective alternative to liver transplant in children with PFIC and AS who have intractable pruritus in the absence of synthetic liver failure.

Progressive Familial Intrahepatic Cholestasis (PFIC) in Indian Children: Clinical Spectrum and Outcome

“…There is paucity of literature from our country addressing the management options employed in PFIC and their outcome. In a series of 7 cases from India, only one responded to medical therapy, 2 required BD (1 underwent PEBD and 1 PIBD) and three of the four subjects with decompensated cirrhosis underwent LT. 13 In another study, 10 pruritus resolved in 8 out of 10 PFIC (9 PFIC 1 and 1 PFIC 2) cases that underwent PIBD, with significant reduction of serum bile acids. The reported complications of PIBD including intestinal obstruction, osmotic diarrhoea and ascending cholangitis were not seen in the present series.…”

“…More recently biliary diversion (BD) procedures have been considered for relief of pruritus in children with preserved synthetic liver function. 9,10 The published data on PFIC from India is in the form of case reports 11,12 and small case series. 13 Understanding the disease spectrum in Indian children is limited by lack of diagnostic facilities (genetic analysis and immunostaining).…”

Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis

“…The indications for and outcomes of nontransplant surgical interventions for intrahepatic cholestasis are not well delineated. Most of the published literature is single centered and subject to potential bias toward positive results . The current study was designed to be cross‐sectional, multicentered, and unbiased toward outcomes with an intent to give a real‐world view of this complicated area of surgical therapy.…”

“…B), which internally bypasses ∼15% of the terminal ileum, and an internal biliary diversion, which entails connecting the gallbladder either directly to the colon or utilizing a segment of jejunum as a conduit (gallbladder to colon [GBC]; Fig. C) . Each operation provides a variable degree of symptom relief.…”

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

“…Although promising follow-up results of PIBD have recently been described, thereby avoiding a permanent stoma, PEBD by cholecystojejunostomy is still widely used, resulting in partial external drainage of bile by creating a conduit between the gallbladder and the skin using a segment of jejunum [13,14].…”