Rhabdomyomatose cardiaque et sclérose tubéreuse de Bourneville chez le foetus. A propos de 2 cas [Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. A propos of 2 cases].

Details

Serval ID
serval:BIB_C117416D1E8B
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Rhabdomyomatose cardiaque et sclérose tubéreuse de Bourneville chez le foetus. A propos de 2 cas [Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. A propos of 2 cases].
Journal
Archives des Maladies du Coeur et des Vaisseaux
Author(s)
Aggoun Y., Hunkeler N., Destephen M., Vial Y., Gudinchet F., Calame A., Payot M.
ISSN
0003-9683 (Print)
ISSN-L
0003-9683
Publication state
Published
Issued date
1992
Peer-reviewed
Oui
Volume
85
Number
5
Pages
609-613
Language
french
Notes
Publication types: Case Reports ; English Abstract ; Journal Article ; Review
Publication Status: ppublish
Abstract
Two cases of cardiac rhabdomyoma discovered fortuitously at foetal ultrasonography gave rise to no obstructive cardiac signs or arrhythmias either in the antenatal or postnatal periods. These multiple tumours often observed in Bourneville's tuberous sclerosis orientated the clinical investigations to the diagnosis of this disease from the outset. MRI demonstrated cortical tubers and subependymal nodules in both cases. A retinal hamartoma was present in one case. No renal involvement could be detected by ultrasonic examination. No neurological or cardiovascular symptoms appeared during follow-up (20 and 4 months after birth). Investigations in the parents were negative, these two cases being sporadic forms of Bourneville's tuberous sclerosis. The functional prognosis is related to the neurological outcome. Doppler echocardiography would appear to be the best method of following up cardiac rhabdomyomas, and enabled the demonstration of partial regression of the largest tumour in one of these two cases. Cardiac MRI is also an excellent diagnostic tool. As it is usually performed at the same time as cerebral MRI, essential in the follow-up of Bourneville's tuberous sclerosis, it does not represent additional discomfort to the patient.
Keywords
Adult, Echocardiography, Female, Fetal Diseases/diagnosis, Follow-Up Studies, Heart Neoplasms/complications, Heart Neoplasms/congenital, Humans, Infant, Newborn, Magnetic Resonance Imaging, Pregnancy, Rhabdomyoma/complications, Rhabdomyoma/congenital, Tuberous Sclerosis/complications, Tuberous Sclerosis/congenital, Ultrasonography, Prenatal
Pubmed
Web of science
Create date
08/04/2008 14:38
Last modification date
20/08/2019 15:35
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