Aim: To evaluate the outcome of non-malignant and non-cirrhotic patients with portal cavernoma and to determine the predictors for survival.
Methods: Between July 2002 and June 2010, we retrospectively enrolled all consecutive patients admitted to our department with a diagnosis of portal cavernoma without abdominal malignancy or liver cirrhosis. The primary endpoint of this observational study was death and cause of death. Independent predictors of survival were identified using the Cox regression model.
Results: A total of 64 patients were enrolled in the study. During a mean follow-up period of 18 ± 2.41 mo, 7 patients died. Causes of death were pulmonary embolism (n = 1), acute leukemia (n = 1), massive esophageal variceal hemorrhage (n = 1), progressive liver failure (n = 2), severe systemic infection secondary to multiple liver abscesses (n = 1) and accident (n = 1). The cumulative 6-, 12- and 36-mo survival rates were 94.9%, 86% and 86%, respectively. Multivariate Cox regression analysis demonstrated that the presence of ascites (HR = 10.729, 95%CI: 1.209-95.183, P = 0.033) and elevated white blood cell count (HR = 1.072, 95%CI: 1.014-1.133, P = 0.015) were independent prognostic factors of non-malignant and non-cirrhotic patients with portal cavernoma. The cumulative 6-, 12- and 36-mo survival rates were significantly different between patients with and without ascites (90%, 61.5% and 61.5% vs 97.3%, 97.3% and 97.3%, respectively, P = 0.0008).
Conclusion: The presence of ascites and elevated white blood cell count were significantly associated with poor prognosis in non-malignant and non-cirrhotic patients with portal cavernoma.
Keywords: Ascites; Extrahepatic portal vein obstruction; Portal cavernoma; Prognostic factors; Survival.