OBGY Radiology


Case 21
Thirty four year old female presented for antenatal sonography at 33 weeks of gestation.
Ultrasound image(four chamber view) through fetal heart and plain CT brain after birth at the level of lateral ventricles is shown below
What is the diagnosis.
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Imaging Finding

Ultrasound Image showed two echogenic masses one each in the interventricular septum and the left atrium and the diagnosis of tuberous sclerosis was suggested.
The CT scan of head shows subependymal calcific foci s/o tuberous sclerosis.



Diagnosis

Tuberous sclerosis with Cardiac Rhabdomyomas

Discussion

The incidence of cardiac tumors is 1 to 2 cases per 10,000 live births; 90% of these are benign out of which rhabdomyomas are the most common [58%]. Other rare cardiac tumors are teratoma [19%], myxomas, hemangiomas and mesotheliomas .
Rhabdomyomas also known as myocardial hamartoma are benign smooth muscle tumors of the myocardium consisting of immature myocytes. Histopathology reveals the typical spider cell. They occur with equal frequency in the left and right ventricle and the interventricular septum almost always these tumours are multiple.Patients may present with cardiac dysrhythymias and non immune hydrops as in the present case. There is a strong association between cardiac rhabdomyoma and tuberous sclerosis [50-86%]
The prognosis depends on the site, size, and number of tumours. The presence of cardiac dysrhthymias or non immune hydrops indicates a poor prognosis. Various studies have shown that rhabdomyomas are slow growing tumours in utero and show no or minimal postnatal growth. Some tumours are known to show regression after birth.
The prenatal diagnosis of tuberous sclerosis was suggested on the basis of the cardiac findings of rhabdomyomas.
The finding of cortical tubers at such an early gestational age has not previously been possible. Use of the ultrafast MR technique of HASTE allows prenatal visualization of cortical tubers without maternal or fetal sedation. Because cortical tubers are more common than cardiac rhabdomyomas and because cardiac rhabdomyomas are not frequently visualized on sonography until the third trimester, it is possible that MR imaging combined with sonography would allow improved prenatal diagnosis of tuberous sclerosis complex.



Dr Paresh Desai, Goa