Abstract

Objective: Cardiac rhabdomyoma is the most common,benign,primary cardiac tumor of infancy.Diagnosis is often made with echocardiography during fetal or early infantile period.Tuberous sclerosis,sebaceous adenomas and renal angiomyolipomas usually accompany the disease.The correlation rate with tuberous sclerosis is 51-86%.Therefore cardiac imaging is essential for all cases diagnosed with tuberous sclerosis and cranial imaging is necessary for cases with cardiac rhabdomyoma.

Case report: A female infant born at term from the first pregnancy of a 21-year-old woman was admitted on her postnatal 12th day because of a palpable sacral mass.On physical examination a 3x4cm palpable,soft,sacral mass which was defined as a hamartomateous mass on ultrasonography was identified.Echocardiogrphic examination performed because of a 1-2°/6 systolic murmur revealed a 2.2x1.8cm mass compatible with cardiac rhabdomyoma on the midmuscular region of the interventricular septum.No arrhythmia was observed on cardiac monitorization or Holter electrocardiography.Cranial magnetic resonance imaging performed with the suspicion of tuberous sclerosis revealed multiple,cortical tubers and periventricular subependymal hamartomas.Sacral mass was removed with total excision on postnatal day 22 and histopathological examination demonstrated a hamartoma with lipomatous differentiation.On follow-up she didn't develop arrhythmia or neuropathological findings.She was discharged on day 45 to be followed by the pediatric cardiology,neurology and oncology departments.

Conclusion: Rhabdomyomas are often asymptomatic cardiac tumors among newborns.However they may cause serious arrhythmias ending up with sudden infant death.Besides epileptic seizures,mental retardation and sensorial defects may develop due to associated tuberous sclerosis.It may be easily diagnosed with fetal echocardiography and poor clinical course may be prevented with appropriate medical approach in the early postnatal period.