Management of extrahepatic portal hypertension in children

Among 69 patients with PVT, 338 variceal bleeding episodes occurred. Only two patients died from bleeding, and both lived in remote communities and were inaccessible to medical care. Fifty-three children underwent 164 operations for the management of PVT. Once operative management was undertaken, subsequent operations frequently were necessary. Nonoperative measures controlled acute variceal hemorrhage in most instances during the past 10 years. Almost all patients who underwent splenectomy alone, variceal ligation, gastric division, splenic transposition, or makeshift shunts subsequently rebled. These operations are rarely indicated in the current management of children with PVT. Portal venography is essential to define the portal venous circulation before a shunt operation is attempted. Cavomesenteric or central splenorenal shunts prevented further bleeding in eight of 15 patients and are the most reliable operations to control bleeding in patients with PVT. Emergency operation is rarely necessary to control bleeding. Sixteen patients (average age 14.6 years) with PVT did not undergo any operations, and are alive. Each of the six patients with PVT who died from complications of portal hypertension did so within nine months of an operation. Four of these patients had previous splenectomy and died with sepsis as one of the major factors. Bleeding episodes became less frequent as the patients increased in age. Patients who underwent shunts under unfavorable circumstances or who received various other operations to treat portal hypertension appeared to have a higher risk of morbidity and mortality than those managed nonoperatively.