Mohan Monusha, Sabapathy S Raja
Department of Plastic, Hand and Reconstructive Microsurgery, Ganga Hospital, Coimbatore, Tamil Nadu, India.
Indian J Plast Surg. 2024 Nov 8;57(Suppl 1):S106-S110. doi: 10.1055/s-0044-1793845. eCollection 2024 Dec.
Postaxial polydactyly (PAP) in the form of rudimentary soft tissue masses is quite common. Management involves ligation or surgical excision. Rarely do literature discussions cover complex variants in which the extra finger is fully developed. Ellis-van Creveld syndrome (EVC) or chondroectodermal dysplasia is a rare disorder characterized by PAP. When chondral dysplasia encompasses PAP, dwarfism, and genu valgum, ectodermal dysplasia involves nails and teeth. We describe two EVC cases. When one had cardiac malformations, the other had dental anomalies. One of them underwent genu valgum correction and removal of the additional finger. The hand surgery proved complex due to fusion of its metacarpal with the fifth metacarpal. Pediatric Outcomes Data Collection Instrument (PODCI) scores at 1.5 and 2.5 years after hand and knee surgeries were excellent. A multidisciplinary approach is essential to achieve comprehensive care. Additional embryological research is necessary to elucidate the clinical manifestations described in this report.
以残留软组织肿块形式存在的轴后多指畸形(PAP)相当常见。治疗方法包括结扎或手术切除。文献讨论很少涉及多指完全发育的复杂变异情况。埃利斯-范克里弗德综合征(EVC)或软骨外胚层发育不良是一种以PAP为特征的罕见疾病。当软骨发育不良包括PAP、侏儒症和膝外翻时,外胚层发育不良则涉及指甲和牙齿。我们描述了两例EVC病例。其中一例有心脏畸形,另一例有牙齿异常。其中一人接受了膝外翻矫正和多指切除。由于其掌骨与第五掌骨融合,手部手术证明很复杂。手部和膝部手术后1.5年和2.5年的儿童结局数据收集工具(PODCI)评分优异。多学科方法对于实现全面护理至关重要。需要进行更多的胚胎学研究来阐明本报告中描述的临床表现。