PDF(Pubmed)
Abstract：
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.
摘要：
轴后或尺骨多指是多指最常见的形式，可能仅伴有软组织结构的重复或伴有额外的骨累及。手术切除是骨累及后轴多指的唯一可行治疗选择，心理或美容原因是治疗的主要理由。Ellis-vanCreveld综合征（EVC）是一种罕见的先天性疾病，以软骨和外胚层发育不良为特征，特别是后轴多指。EVC的确切患病率未知，报告的病例不到300例。我们介绍了一例2岁的西班牙裔女性患者，患有EVC，其表现为双侧轴后多指，掌骨和指骨完全重复。我们描述了这个病人的表现和治疗，他最终接受了分阶段切除重复的手指并重建了外展肌。
