Surgical management of the hand in Freeman-Sheldon syndrome

Loree K. Kalliainen, David B. Drake, Milton T. Edgerton, Joseph L. Grzeskiewicz, Raymond F. Morgan

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


Freeman-Sheldon syndrome is a rare form of distal arthrogryposis characterized by craniofacial anomalies, a rheumatoid-appearing hand, and pedal deformities. The hand deformities include ulnar deviation of the fingers, camptodactyly, first web space contracture, and hypoplasia of the thumb. Because of clinical variability and rarity, there is no standard management protocol. The authors have developed a systematic method of management of the hand in Freeman-Sheldon syndrome using principles commonly applied to other complex hand problems. In 17 years, the authors have evaluated nine patients. Of 18 hands evaluated, the authors have operated on 15. Good results were achieved in five hands and fair results in 10. There were no cases of useless hands. The authors have been most pleased with the results after crossed intrinsic transfers, centralization of extensor tendons, and intrinsic release of the thumb. Although physical deformities remain, functional adaptation is generally good.

Original languageEnglish
Pages (from-to)456-462
Number of pages7
JournalAnnals of Plastic Surgery
Issue number5
StatePublished - May 1 2003

ASJC Scopus subject areas

  • Surgery


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