Acta Ortopédica Mexicana

Vergara FHJ, Román CL
Freeman-Sheldon´s syndrome. Report of five cases
Acta Ortop Mex 2007; 21 (6)

Language: Español
References: 13
Page: 338-343
PDF: 201.15 Kb.

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ABSTRACT

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.


Key words: arthrogryposis, thumb, lower extremity, upper extremity, face, mental retardation.


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