A case report: a six years old girl with bantis syndrome

Iqbal Alamsah Hardigaluh, Bambang Soebagyo, Evi Rokhayati

Departement of Child Health, Faculty of Medicine, Sebelas Maret University, Dr. Moewardi
General Hospital, Surakarta

Background : Bantis syndrome is a disorder characterized by splenomegaly and

hypersplenism, resulting in portal hypertension and anemia in the absence of hematological
diseases. The progress of Splenomegaly is slow and it can last up to twenty years or more
Objective : This case presentation aims to discuss the clinical features ,diagnosis, and
prognosis of Bantis syndrome disease
Case : A 6 years old girl admitted to dr.Moewardi hospital with a chief complaint abdominal
enlargement for 4 years without abdominal pain. On physical examination her general
appearance looked healty, there was no Cooley face. Abdominal circumference was 54 cm
with flat surface V Schuffner splenomagaly, without tenderness. Laboratory findings revealed
Hb 9.1gr / dl. Peripheral blood smear showed erythrocyte, leucocytes, and platelets number
decreased with normocytic normochromic, elliptical cells and cell ovalocyte, neutrophils
hypergranulation, atypical lymphocytes (-), blast cells (-), giant platelets (-). Hb
electroforesys was normal. USG abdomen showed dilated hepatic portal system and
prominent hepatic vein branches, no lesion, with the impression of chronic congestive
splenomegaly in portal hypertension due to Banti's Syndrome. Contrasted abdomen MSCT
demonstrated normal liver, and spleen size of 211.8 mm with normal parenchymeal density,
no mass, nodules or cysts. This patient underwent splenectomy and she had good prognosis.
Keywords: idiopathic portal hypertension, Non-cirrhotic portal fibrosis, splenomegaly,
bantis syndrome,