Radiology

Case of the Month

This is a case of a one-day-old neonate delivered at full term by Caeserain section.

The baby was antenatally diagnosed to have cardiac masses. On delivery, the baby had a heart rate of more than 200/min and was in shock.

The ECG showed supraventricular tachycardia.

The chest radiograph showed no cardiomegaly and both lung fields were normal.

The baby was admitted in the NICU and on stabilisation referred for ultrasonography of the chest and abdomen. The chest ultrasound examination revealed multiple, well defined, globular, isoechoeic masses arising from the right and left ventricular walls and the interventricular septum (Figs 1,2,3,4). The atria and the cardiac valves were unaffected. There was no evidence of pericardial effusion.

The ultrasound examination of the abdomen was normal.

A diagnosis of cardiac rhabdomyoma was made.

A CT Scan of the brain was advised for to rule out tuberous sclerosis. This revealed multiple periventricular hyperdensities suggestive of tuberous sclerosis (Fig 5).

Fig.1	Fig.2

Fig.3

Fig.4	Fig.5

DISCUSSION:

Cardiac rhabdomyoma are also called as myocardial hamartoma. They are benign smooth muscle tumours of the myocardium, consisting of immature myocytes.

Rhabdomyomas are the most common primary pediatric cardiac tumour accounting for upto 90% of cardiac tumour in infants and children. They are not true neoplasms but hamartomas with a few mitotic figures. Metastases do not occur.

They are associated with tuberous sclerosis in 50% of the patients.

Most commonly, the tumour arises from the ventricular wall and interventricular septum. The tumours are frequently multiple and bilateral.

The tumors can cause cardiac arrythmias, pericardial effusion, hydrops and ventricular outflow tract obstruction.

The differential diagnosis includes cardiac fibroma, myxoma, teratoma and sarcoma. Spontaneous tumour regression may occur particularly in patients less than 4yrs of age.

Prognosis is good and management is conservative in absence of life threatening symptoms.