[The Apert's syndrome hand: therapeutic management].
Apert's syndrome is the most common among acrocephalosyndactylies with complex malformations of the hands. Treatment of the Apert hand is complex and numerous procedures are required. The aim of this study is to propose a strategy for hand management. Sixteen Apert syndrome hands were submitted to early surgery which included opening of the first web, separation of the fingers, realignment of the thumb and correction of the clinodactylies. We performed an average of six operations per child. Treatment of the first web depended on the classification of Upton: for the severe stages, we used a dorsal hand flap. Radical clinodactyly was treated by osteotomy of the delta phalanx and Z-plasty. We treated nine hands with four fingers and seven hands with five fingers. There was always bilateral opposition and symphalangism, excluding the fifth finger. All of the children have a rudimentary but functional pinch grip. Revision of the webs was necessary in 16% of the cases. The Apert hand requires early and specialised treatment that aims to provide a functional hand before two or three years, with the least surgical complications. The functional prognosis is darkened by symphalangism.
Institut de la Main, clinique Monticelli, 88, rue du Commandant-Rolland, 13008 Marseille, France. docteur.salazard@orange.fr
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Salazard
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Casanova
D000168:Acrocephalosyndactylia / Q000145:classification / Q000601:surgery*; D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D004954:Esthetics; D005260:Female; D005500:Follow-Up Studies; D006225:Hand / Q000601:surgery*; D018737:Hand Strength; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010027:Osteotomy; D011379:Prognosis; D013524:Surgical Flaps; D013997:Time Factors; D016896:Treatment Outcome
La main du syndrome d'Apert: stratégie thérapeutique.