Nudelman Hermann, Lőrincz Aba, Lamberti Anna Gabriella, Kassai Tamás, Józsa Gergő
Department of Paediatrics, Clinical Complex, Division of Surgery, Traumatology and Otorhinolaryngology, University of Pécs, 7 József Attila Street, 7623 Pécs, Hungary.
Department of Thermophysiology, Institute for Translational Medicine, Medical School, University of Pécs, 12 Szigeti Street, 7624 Pécs, Hungary.
Life (Basel). 2023 Sep 21;13(9):1943. doi: 10.3390/life13091943.
Syndactyly is the most common congenital malformation of the hand, leading to the fusion of the digits and frequently affecting the ring and middle fingers. The incidence is 1 out of 2500 children, predominantly occurring in boys and Caucasians. Clinically, the malformation may present as a soft tissue or bony fusion, resulting in the union of the fingers characterised as complete or incomplete. This fusion may involve the phalanges but may also extend to the carpal/tarsal bones, even to the metacarpal or metatarsal level, rarely to the distal end of the forearm and lower leg. The malformation is mostly isolated but may occur together with other disorders or malformations such as synostosis, acro-syndactyly, cleft hand, clinodactyly, or polydactyly. Syndromic syndactyly can be observed in cases of Apert syndrome, Poland's syndrome, Pfeiffer syndrome, and many others. A girl born in June of 2019 was diagnosed with congenital malformation of the right hand at birth-affecting the right middle, ring, and little fingers, respectively. After X-ray imaging, the fusion of the third and fourth proximal phalanges to a common metacarpal was identified, forming a unique diagnosis of clino-syndactyly with metacarpal aplasia. Surgical intervention was advocated for, including a wedge osteotomy to correct the synchondrosis at the phalangeal base and a dorsal flap to close the interdigital space created during the correction of the III and IV. fingers. A trapezoid flap for the release of the syndactyly of the IV and V. fingers was applied. The paper aims to present this surgical correction and its results regarding an atypical case of syndactyly with clinodactyly and metacarpal aplasia.
并指畸形是手部最常见的先天性畸形，导致手指融合，且常累及环指和中指。发病率为每2500名儿童中有1例，主要发生在男孩和白种人中。临床上，这种畸形可能表现为软组织或骨性融合，导致手指联合，其特征为完全或不完全融合。这种融合可能累及指骨，但也可能延伸至腕骨/跗骨，甚至到掌骨或跖骨水平，很少累及前臂和小腿远端。该畸形大多为孤立性，但可能与其他疾病或畸形同时出现，如骨融合、尖头并指畸形、裂手畸形、指侧弯畸形或多指畸形。综合征性并指畸形可见于Apert综合征、波兰综合征、Pfeiffer综合征等多种情况。一名2019年6月出生的女孩出生时被诊断为右手先天性畸形，分别影响右手的中指、环指和小指。经X线成像检查，发现第三和第四近端指骨融合至同一掌骨，形成了伴有掌骨发育不全的斜指并指畸形的独特诊断。建议进行手术干预，包括楔形截骨术以矫正指骨基部的软骨结合，以及背侧皮瓣以闭合在矫正第三和第四指时形成的指间隙。应用梯形皮瓣松解第四和第五指的并指畸形。本文旨在介绍这种手术矫正方法及其对一例伴有指侧弯畸形和掌骨发育不全的非典型并指畸形病例的治疗结果。
