%0 Case Reports
%T A Rare Presentation of Postaxial Polydactyly in a 2-Year-Old Female with Ellis-van Creveld Syndrome.
%A Anthony T
%A Nguyen ET
%A Moyer B
%A Meter J
%A Williams R
%A Williams CN
%J J Hand Surg Glob Online
%V 5
%N 6
%D 2023 Nov
%M 38106942
暂无%R 10.1016/j.jhsg.2023.08.007
%X Postaxial or ulnar polydactyly is the most common form of polydactyly that may present with the duplication of soft-tissue structures only or with additional bony involvement. Surgical excision is the only viable treatment option for postaxial polydactyly with bony involvement, and psychological or cosmetic reasons are the main rationale for treatment. Ellis-van Creveld syndrome (EVC) is a rare congenital disorder characterized by chondral and ectodermal dysplasia, particularly postaxial polydactyly. The exact prevalence of EVC is unknown, and fewer than 300 cases have been reported. We present a case of a 2-year-old Hispanic female with EVC who presented with bilateral postaxial polydactyly and complete duplication of the metacarpal and phalanges. We describe the presentation and treatment of this patient, who ultimately underwent staged resection of the duplicated digits with reconstruction of the abductor muscle.