All issues > Volume 43(2); 2000

Case Report

J Korean Pediatr Soc. 2000;43(2):294-299. Published online February 15, 2000.

A Case of Tuberous Sclerosis with Hydrocephalus and Multiple Cardiac Tumors in Neonatal Period

Young Jun YJ Song¹, Won Seop WS Kim¹, Beom Soo BS Park¹, Heon Seok HS Han¹

¹Department of Pediatrics, College of Medicine, Chungbuk National University, Cheongju, Korea

Abstract

Several different tumors in different tissues(including the central nervous system, heart, kidney, liver, bone, and soft tissues) are found to be associated with tuberous sclerosis. In the brain, this tumor characteristically arises from the floor of the lateral ventricle and often occluded the foramen of Monro. Cardiac tumor is among the earliest signs of tuberous sclerosis and occurs in over half of affected patients, but symptomatic primary heart tumors are rare in newborn. Thus, early detections are rare in asymptomatic cases. We experienced a case of tuberous sclerosis, in a 2-day-old female newborn who presented with dilatation of lateral ventricle detected by fetal ultrasonography. Diagnosis was confirmed by brain MRI and echocardiogram demonstrated multiple cardiac tumor. Brief review and related literatures were also presented.

Keywords :Tuberous sclerosis, Hydrocephalus, Cardiac tumor, Newborn