A 14 YEARS OLD BOY WITH BANTI’S SYNDROME AND ATRIAL SEPTAL DEFECT ( A CASE REPORT )
Hamid Pramusyahid,Bambang Soebagyo,Sri Lilijanti
Department of Child Health Faculty of Medicine Sebelas Maret University
Background : Banti’s syndrome is clinically characterized by idiopathic portal hypertension, splenomegaly, gastrointestinal bleeding and anemia with the absence of hematological disease. The etiology is unknown. Atrial septal defect (ASD) is a hole in the wall between two upper chambers. About 8% of congenital heart disease is ASD. This case report aimed to management and investigate the relation between Banti’s syndrome and ASD.
Case Presentation Summary : A 14 y.o boy admitted to ER with bloody vomit. He experienced the same complaint at the age of 13 y.o. Physical examination releaved pale conjunctiva, systolic murmur, splenomegaly, no ascites, no spider nevi, no increased jugular pressure and no edema. The laboratory findings obtained normocytic normocromic anemia. Coagulation profile and liver function test were normal with negative hepatitis B and C serology. Sonography showed dilated porta vein in the portal and spleen accompanied by mid portal hypertension and splenomegaly. Echocardiography demonstrated secundum ASD with the diameter of 0.9 cm and mild TR with PG 34.8 mmHg. Endoscopy showed 2nd grade esophageal varices. Abdominal MSCT revealed splenomegaly, dilatation portal veins, mesenterica veins, lienalis veins without thrombus in porta veins. We treated this patient with partial splenic embolization for portal hypertension, ligation vein for esophageal varices and ASD closure used occluder.
Learning Points/Discussion : Banti’s syndrome has no correlation with ASD and it has good prognosis.