Portal Hypertension

Abstract

Splenomegaly and thrombocytopenia in a child, with or without a history of upper gastrointestinal bleeding, should suggest the diagnosis of portal hypertension. Various conditions are associated with this and tend to vary according to age. Biliary atresia is one of the most common causes in young infants. Diagnosis of the underlying cause can be made through genetic testing, laboratory studies, and imaging studies. A liver biopsy will also contribute to the diagnostic process and will confirm the presence of cirrhosis. In an asymptomatic patient, focus should be on preventing a first bleeding episode through primary prophylaxis, using both endoscopic and medical measures. Patients who have experienced a first bleed from esophageal or gastric varices will be managed according to their clinical presentation. In the rare event that the bleeding is not controlled by conservative measures, emergency surgery or a transjugular intrahepatic portosystemic shunt might be required. If a patient suffers from recurrent bleeding despite maximal medical and endoscopic measures, surgical shunt procedures (preferably selective) are indicated. Idiopathic portal vein thrombosis requires another set of diagnostic and therapeutic maneuvers that are unique to this set of patients. Splenic preservation should be primary in any surgical option, as a splenectomy can preclude those patients from benefiting from other shunt procedures in the future. In some select cases, liver transplant is indicated.