PORTOSYSTEMIC SHUNTING FOR PEDIATRIC PORTAL-HYPERTENSION

The records of 22 patients who received portosystemic shunting for por tal hypertension from 1985 to 1995 inclusive at the Royal Alexandra Ho spital for Children (RAHC) were retrospectively reviewed. There were 1 1 girls and 11 boys. The average age at operation was 8 years, 3 month s (range, 2 years, 3 months to 16 years, 7 months). The aetiology was idiopathic portal cavernomatous transformation (n = 9), biliary atresi a (n = 4), cystic fibrosis (n = 3), documented neonatal portal vein th rombosis (n = 3), congenital hepatic fibrosis (n = 2), and portal vein obstruction after liver transplant (n = 1). The major presenting prob lem was upper gastrointestinal haemorrhage. Two patients had recurrent melaena from Roux-en-Y jejunal loop and caecal varices, respectively. Before receiving shunts, 12 patients had endoscopic sclerotherapy, 1 had gastric transection, and 2 had gastric varices oversewn. Portal pr essure at preoperative splenoportogram averaged 28 mm Hg (range, 20 to 41). Urgent shunts were performed on 13 patients. Two disadvantaged p atients had prophylactic shunts for severe hypersplenism. The types of shunts used were reversed splenorenal (n = 13), splenoadrenal (n = 6) , inferior mesenteric renal (n = 1), portocaval (n = 1), inferior mese nteric caval (n = 1), and superior and inferior mesenteric caval (n = 1), In all, 22 patients had 23 shunts. The patency rate was 96% on 6 m onths to 10 years follow-up (average, 5.8 years), No spleen was lost. There were 2 late deaths. Two cystic fibrosis patients and one child w ith extrahepatic portal hypertension experienced post-shunt encephalop athy. Three patients rebled in the early postoperative period despite a patent shunt. Two patients subsequently received liver transplantati on without any additional difficulties. Thus, portosystemic shunting u sing a method appropriate for the patient is a reliable option for tre ating children with portal hypertension in whom variceal sclerotherapy is inappropriate or has failed, Copyright (C) 1997 by W.B. Saunders C ompany.