Clinical characteristics of nonneoplastic cavernomatous transformation of the portal vein at a Gastroenterology Service in Spain.

OBJECTIVE: To identify predisposing factors, clinical characteristics and effective treatment in patients with nonneoplastic cavernomatous transformation of the portal vein in our gastroenterology service.

METHODS: We retrospectively reviewed the clinical records of 2,201 patients diagnosed as having portal hypertension (2,165 with cirrhosis and 36 with noncirrhotic portal vein hypertension) during the period from 1977 to 1998. The diagnosis of cavernomatous transformation was confirmed with angiographic or Doppler echographic studies, or both.

RESULTS: Thirteen patients (6 males, 7 females, age range 8 to 69 years) with cavernomatous transformation were found. Predisposing factors were omphalitis (1), echinococcal cyst (1), major abdominal surgery (3), liver cirrhosis (3), Sjögren syndrome (1), and no apparent cause (4). Eleven of the 13 patients had upper digestive tract bleeding from varices, 9 had splenomegaly, and 2 had cirrhotic decompensation. Splenectomy was done in 3 patients on admission, and in 5 patient shunts were used (splenorenal in 4, mesenteroatrial in 1) because of repeated bleeding.

CONCLUSIONS: Of the patients with noncirrhotic portal hypertension, 27.7% had nontumoral cavernomatous transformation of the portal vein. Previous abdominal surgery was the most frequent predisposing factor; the 2 cases of echinococcal liver disease and Sjögren disease were exceptional. Age younger than 30 years, bleeding esophageal varices and splenomegaly were the most frequent clinical features. Portosystemic shunt was the only effective treatment alternative in these patients.