[Bourneville's tuberous sclerosis. Prenatal diagnosis of a case]

Abstract

The discovery of a tumour of the heart on fetal ultrasound is a rare event. 60% of heart tumours in the fetus are rhabdomyomas. 50% of rhabdomyomas are associated with Bourneville's tuberous sclerosis. The inherited element of the effect of the gene is variable but often severe (75% of patients die before the age of 25 and 60-80% are disabled). Tuberous sclerosis Bourneville is a multifocal pathological condition of which only the presence of rhabdomyomata in the heart make it possible to diagnose the condition antenatally. Monitoring the fetus consists of looking carefully for the complications that can occur and of planning the health care of the newborn including the need to consider surgical intervention at birth.