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Archives de pédiatrie
Volume 11, n° 4
pages 344-346 (avril 2004)
Doi : 10.1016/j.arcped.2004.01.014
Received : 15 May 2003 ;  accepted : 19 January 2004
Anévrisme sous-clavier associé à un syndrome de Turner
Aneurism of the subclavian artery associated with Turner's syndrome

M.  Lacombe a * ,  B.  Esteva b ,  I.  Tillous-Borde c ,  G.  Lesèche a
aService de chirurgie vasculaire, hôpital Beaujon, AP-HP, 92118 Clichy cedex, France 
bService d'explorations fonctionnelles endocriniennes, hôpital Armand-Trousseau, AP-HP, 75571 Paris cedex 12, France 
cConsultation de cardiologie pédiatrique, hôpital Armand-Trousseau, AP-HP, 75571 Paris cedex 12, France 

*Auteur correspondant.
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Les anévrismes artériels périphériques associés au syndrome de Turner sont rares.

Observation. - Nous rapportons un cas d'anévrisme sous-clavier chez une jeune fille de 16 ans porteuse d'un syndrome de Turner. Le traitement chirurgical a comporté la résection de l'anévrisme à partir de la crosse aortique, la suture de celle-ci et la réimplantation de la sous-clavière dans la carotide primitive gauche. L'évolution avec trois ans de recul est favorable.

Commentaires et conclusions. - Des anomalies cardiovasculaires existent chez 50 % des sujets atteints de syndrome de Turner ce qui justifie un bilan cardiaque complet chez ces patients. Les malformations congénitales (bicuspidie aortique, coarctation de l'aorte, communications intracardiaques, atteintes valvulaires) ou les anomalies acquises (hypertension artérielle, dissection aortique) sont fréquentes. Une seule observation comparable d'anévrisme sous-clavier a été publiée jusqu'à présent. Le risque potentiel de rupture justifie le traitement chirurgical.

Mots clés  : Syndrome de Turner ; Anévrisme artériel.

Abstract

Arterial aneurysms associated with Turner's syndrome are rare

Case report. - We report a case of aneurysm of the left subclavian artery in a 16-year-old girl with Turner's syndrome. This patient was operated on: resection of the aneurysm, suture of the aortic arch and reimplantation of the subclavian artery in the left common carotid were performed. At 3-year follow-up, the evolution is favourable.

Comments. - Cardiovascular anomalies are observed in 50% of subjects with Turner's syndrome. This justifies complementary cardiac investigations in these patients. Congenital malformations (bicuspid aortic valve, aortic coarctation, intracardiac communications, valvular lesions) or acquired anomalies (arterial hypertension, aortic dissection) are frequent. Only one similar case of subclavian artery aneurysm has been reported until now. The risk of rupture justifies the surgical treatment.

Mots clés  : Turner syndrome ; Aneurism ; Subclavian artery ; Child.




© 2004  Elsevier SAS. All Rights Reserved.

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