Spontaneous Hemoperitoneum in Non Cirrhotic Portal Hypertension: A Case Report: 1051

Purpose: We report a rare case of non -irrhotic portal hypertension with spontaneous intraperitoneal hemorrhage. This report details patient's presentation, management and literature review. Case Report: 29-year-old Ethiopian male with history of splenomegaly and thrombocytopenia presented with sudden onset of severe diffuse abdominal pain, associated with generalized weakness. Examination was significant for mild tachycardia, diffuse abdominal tenderness without guarding and splenomegaly. Lab work was significant for Hemoglobin 9.3 (previous hemoglobin 14.7), platelets 27, WBC 2.1, normal Bun/Cr, total protein 6.0, albumin 3.4, AST 30, ALT 23, Alkaline phosphatase 62 and total bilirubin 2.3. CT abdomen with contrast was remarkable for massive splenomegaly, associated with extensive varices as well as spontaneous splenorenal shunt. Portal and splenic vein appeared to be intact. There was fluid of high density adjacent to the liver as well as within the bilateral pericolic gutters. Density was compatible with hemorrhagic fluid in nature. It was decided for initial conservative management. Upper GI endoscopy showed very large esophageal varices that were banded along with gastric varix and portal HTN gastropathy. Paracentesis was done that showed 3 million red blood cells confirming hemoperitoneum and a low albumin with a high SAAG, consistent with portal hypertension. A comprehensive evaluation for causes of liver disease including hepatitis serology, autoimmune causes, metabolic and schistosoma antibodies was done and was found negative. Liver biopsy was consistent with hepatoportal sclerosis and no evidence of cirrhosis. He remained hemodynamically stable, so conservative management was continued and he was discharged after five days of monitoring. Conclusion: Non-cirrhotic portal hypertension is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction. The most common clinical presentation is variceal bleeding, which (in contrast to variceal bleeding in cirrhosis) is often relatively well tolerated due to the preserved liver function. Intraperitoneal rupture of ectopic varices is a rare complication of portal hypertension. Very Few case reports have been published in the literature and little is known about the best management strategy for these patients. The management is individualized according to the condition of the patient and the resources. Objectives of management include suspicion for diagnosis, aggressive resuscitation, and correction of coagulopathy, reduction of portal hypertension and if required, surgical interventions for direct control of the bleeding vessel.