Abstract
Purpose. Review the safety and long-term success with portosystemic shunts in children at a single institution. Methods. An IRB-approved, retrospective chart review of all children ages 19 and undergoing surgical portosystemic shunt from January 1990–September 2008. Results. Ten patients were identified, 8 females and 2 males, with a mean age of 15 years (range 5–19 years). Primary diagnoses were congenital hepatic fibrosis (5), hepatic vein thrombosis (2), portal vein thrombosis (2), and cystic fibrosis (1). Primary indications were repeated variceal bleeding (6), symptomatic hypersplenism (2), and significant liver dysfunction (2). Procedures performed were distal splenorenal bypass (4), side-to-side portocaval shunt (3), proximal splenorenal shunt (2), and an interposition H-graft portocaval shunt (1). There was no perioperative mortality and only minor morbidity. Seventy percent of patients had improvement of their symptoms. Eighty percent of shunts remained patent. Two were occluded at a median follow-up of 50 months (range 0.5–13.16 years). Two patients underwent subsequent liver transplantation. Two patients died at 0.5 and 12.8 years postoperatively, one from multisystem failure with cystic fibrosis and one from post-operative transplant complications. Conclusions. The need for portosystemic shunts in children is rare. However, in the era of liver transplantation, portosystemic shunts in selected patients with well-preserved liver function remains important. We conclude that portosystemic shunts are safe and efficacious in the control of variceal hemorrhage and symptoms related to hypersplenism.
Highlights
The approach to portal hypertension (PH) in children has evolved significantly over the past half-century
PH in children can be divided into two main categories: (i) extrahepatic portal hypertension (EHPH) and (ii) intrahepatic portal hypertension (IHPH)
Those patients with portal vein thrombosis were categorized as EHPH, and all other patients with congenital hepatic fibrosis, hepatic vein thrombosis, and cystic fibrosis with subsequent fibrotic liver disease were categorized as IHPH
Summary
The approach to portal hypertension (PH) in children has evolved significantly over the past half-century. With improved endoscopic treatments for variceal bleeding, liver transplantation and transjugular intrahepatic portosystemic shunts (TIPSs) , the use of portal systemic shunts (PSSs) has been relegated to a few selected patients in the pediatric population. PH in children can be divided into two main categories: (i) extrahepatic portal hypertension (EHPH) and (ii) intrahepatic portal hypertension (IHPH). EHPH in children is commonly due to main portal vein thrombosis, which may variably extend into the splenic, mesenteric, or intrahepatic portal veins. Hepatic decompensation is rare and liver transplantation is seldom indicated. In patients with IHPH, hepatic decompensation often occurs over time as evident clinically by ascites, coagulopathy, and encephalopathy. IHPH in children can result from a number of distinct causes including congenital hepatic fibrosis, hepatic vein thrombosis, inborn errors of metabolism, biliary atresia, and cystic fibrosis
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