|
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
|
