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[Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. Apropos of 2 cases].
Arch Mal Coeur Vaiss. 1992 May; 85(5):609-13.AM

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.

Authors+Show Affiliations

Clinique cardiovasculaire pédiatrique, Bou Ismaïl, Tipasa, Algérie.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

fre

PubMed ID

1530402

Citation

Aggoun, Y, et al. "[Cardiac Rhabdomyomatosis and Bourneville's Tuberous Sclerosis in the Fetus. Apropos of 2 Cases]." Archives Des Maladies Du Coeur Et Des Vaisseaux, vol. 85, no. 5, 1992, pp. 609-13.
Aggoun Y, Hunkeler N, Destephen M, et al. [Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. Apropos of 2 cases]. Arch Mal Coeur Vaiss. 1992;85(5):609-13.
Aggoun, Y., Hunkeler, N., Destephen, M., Vial, Y., Gudinchet, F., Calame, A., & Payot, M. (1992). [Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. Apropos of 2 cases]. Archives Des Maladies Du Coeur Et Des Vaisseaux, 85(5), 609-13.
Aggoun Y, et al. [Cardiac Rhabdomyomatosis and Bourneville's Tuberous Sclerosis in the Fetus. Apropos of 2 Cases]. Arch Mal Coeur Vaiss. 1992;85(5):609-13. PubMed PMID: 1530402.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. Apropos of 2 cases]. AU - Aggoun,Y, AU - Hunkeler,N, AU - Destephen,M, AU - Vial,Y, AU - Gudinchet,F, AU - Calame,A, AU - Payot,M, PY - 1992/5/1/pubmed PY - 1992/5/1/medline PY - 1992/5/1/entrez SP - 609 EP - 13 JF - Archives des maladies du coeur et des vaisseaux JO - Arch Mal Coeur Vaiss VL - 85 IS - 5 N2 - 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. SN - 0003-9683 UR - https://www.unboundmedicine.com/medline/citation/1530402/[Cardiac_rhabdomyomatosis_and_Bourneville's_tuberous_sclerosis_in_the_fetus__Apropos_of_2_cases]_ L2 - http://www.diseaseinfosearch.org/result/7255 DB - PRIME DP - Unbound Medicine ER -