Abstract

This study was performed to assess the effect of portosystemic shunt surgery (PSS) on portal cavernoma cholangiopathy (PCC) in children with extrahepatic portal veinobstruction (EHPVO). Children with EHPVO and PCC (unfit for Meso-Rex shunt) underwent magnetic resonance cholangiogram (MRC) and magnetic resonance portovenogram (MRPV) before non-selective PSS. PCC was graded by modified Llop classification. Those with patent shunt were re-evaluated at least 6months after surgery with MRC, MRPV and compared with pre-shunt images. Twenty-five children underwent PSS (central end to side splenorenal shunt with splenectomy [n=24], mesocaval shunt [n=1]). Pre-surgery MRC showed PCC grade I in 11, grade II in 1 and grade III in 13. MRPV showed superior mesenteric vein (SMV)block in 20. Re-assessment for PCC 18 (6 to 54) months after surgery showed grade I in 6 and grade III in 19. Thus, PCC was progressive in 6 and static in 19. Density of peribiliary collaterals decreased in 5 (SMV patent, static PCC), increased in 3 and remained unchanged in 17. Splenomegaly-related problems, gastroesophageal varices and other intra-abdominal (esophageal, perisplenic and perigastric) collaterals ameliorated in all. Non-selective PSS decompresses esophago-gastro-splenic venous circuit effectively but fails to ameliorate cholangiopathy and peribiliary collaterals. Persistence of cholangiopathy is attributable to SMV block.

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