Degeneración cavernomatosa de la vena porta

Background. The cavernous degeneration of the portal vein is one of the main causes of extrahepatic portal hypertension in children. The main initial clinical manifestation in this disease is bleeding from the upper gastrointestinal tract. Therapeutic measures include two fundamental aspects, pharmacological medical treatment and endoscopic treatment of varicose veins. Objective. To describe the clinical characteristics, the medical and endoscopic treatment of pediatric patients diagnosed with cavernomatous degeneration of the portal vein treated at the Centro Medico Nacional 20 de Noviembre. Material and methods. A case series that included pediatric patients with confirmed diagnosis of cavernomatous degeneration of the vein with the Doppler sonography. Results. Twelve patients were found. Male dominance in 9 patients (75%). Median age at diagnosis 49 months. Initial clinical manifestation: upper gastrointestinal bleeding (83.3%). 100% of patients received medical treatment with omeprazole, propranolol and sucralfate. Initial endoscopy: Esophageal varices in 100% of patients, of these 9 cases with gastric varices (75%). Secondary prophylaxis was performed in 11 of them; Ligation of esophageal varices in 2 cases and obliteration of gastric varices with cyanoacrylate in 9 patients with gastric varices. Three patients presented with bleeding, 2 had esophageal varices ligation and 1 of those treated with obliteration of gastric varices with cyanoacrylate.