Tuberous Sclerosis Revealed by Syncope due to a Rhabdomyoma in a 1-year Old Infant

Rhabdomyoma represent a rare cardiac tumor often associated with tuberous sclerosis however is often remains asymptomatic with a regressive pattern of evolution throughout childhood. We report a rare case of an infant of 1 year old from a uneventful pregnancy and delivery,with normal developmental milestones, whose mother noticed two episodes of brief period of loss of consciousness and muscle tone upon pediatric consultation clinical examination reveal a conscious child able to stand without support with hypopigmented skin lesion a CT scan revealed periventricular calcifications with subcortical intraparenchymal hypodense plaque an EEG without signs of epileptic pattern EKG didn’t reveal signs of arrythmias and transthoracic echogardiogram showing multiple hyperechogenic homogenous tumors one attached to the interventricular septum with intermittant left ventricular outflow tract obstruction due to motion second near to the tricuspid valve with no signs of obstruction and a third attached to the free wall of the right ventricle. Tuberous sclerosis was retained as a diagnosis with rhabdomyoma and the management was based on regular cardiac monitoring with marked regression of tumor size and no recurrence of syncope episodes.