Acta ortopedica mexicana · 2007
[Freeman-Sheldon's syndrome. Report of five cases].
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Fernández Henry Juver Vergara, Capdevila Leonori Román
Аннотация
INTRODUCTION: Freeman-Sheldon syndrome, described in 1938, is an infrequent form of arthrogryposis.
MATERIAL AND METHODS: We reviewed the files of the patients with the diagnosis of Freeman-Sheldon syndrome from 1990 to 2003, and carried out an evaluation of the clinical data, family history and malformations of extremities.
RESULTS: 5 patients were included, 4 male and 1 female. Upper extremities: All patients presented adducted thumbs, 3 patients were treated with a first web release and dorsal graft with improvement of grip on larger objects. Feet: Four patients had severe bilateral rigid clubfeet, they were operated on with a posteromedial release and bilateral talectomy, satisfactory results were obtained in 4 of 6 feet according to Niki criteria. Other non orthopedic anomalies: In three patients unilateral right undescended testis; 1 right inguinal hernia.
CONCLUSIONS: The patients have as common factor the whistler appearance, accompanied with mill crosses hand deformity, adducted thumbs, accompanied or not with inferior extremities deformities, intellectual capacity was normal.
MATERIAL AND METHODS: We reviewed the files of the patients with the diagnosis of Freeman-Sheldon syndrome from 1990 to 2003, and carried out an evaluation of the clinical data, family history and malformations of extremities.
RESULTS: 5 patients were included, 4 male and 1 female. Upper extremities: All patients presented adducted thumbs, 3 patients were treated with a first web release and dorsal graft with improvement of grip on larger objects. Feet: Four patients had severe bilateral rigid clubfeet, they were operated on with a posteromedial release and bilateral talectomy, satisfactory results were obtained in 4 of 6 feet according to Niki criteria. Other non orthopedic anomalies: In three patients unilateral right undescended testis; 1 right inguinal hernia.
CONCLUSIONS: The patients have as common factor the whistler appearance, accompanied with mill crosses hand deformity, adducted thumbs, accompanied or not with inferior extremities deformities, intellectual capacity was normal.