Surgical treatment of massive splenomegaly and severe hypersplenism secondary to extrahepatic portal venous obstruction in children.

PURPOSE: Massive splenomegaly with severe hypersplenism can occur as a late complication of portal hypertension (PH) caused by extrahepatic portal venous obstruction (EHPVO) in children. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy (EST) and shunt surgery. We report our experience of managing this disorder surgically.

METHODS: We performed splenectomy and esophagogastric devascularization via laparotomy in 14 children with an average age of 9.7 years. Upper gastrointestinal endoscopy had shown esophageal varices of varying grade, and EST had been done for patients with a history of bleeding. The indications for surgery were pain and discomfort caused by a large spleen greater than 15 cm below the costal margin, and intractable symptomatic hypersplenism with a total leukocyte count <2500/mm3 and a platelet count <50,000/mm3, or both.

RESULTS: Postoperative recovery was uneventful and the leukocyte and platelet counts reverted to normal. After follow-up for 1-5 years, all 14 children were asymptomatic, with improved growth and nutrition and no reported episodes of gastrointestinal bleeding, sepsis, or encephalopathy.

CONCLUSION: Splenectomy with devascularization is effective for children with massive splenomegaly and severe hypersplenism secondary to EHPVO.