A Complex Case of Clino-Syndactyly with Fourth Metacarpal Aplasia

Author:

Nudelman Hermann1,Lőrincz Aba2ORCID,Lamberti Anna Gabriella1ORCID,Kassai Tamás3,Józsa Gergő124ORCID

Affiliation:

1. Department of Paediatrics, Clinical Complex, Division of Surgery, Traumatology and Otorhinolaryngology, University of Pécs, 7 József Attila Street, 7623 Pécs, Hungary

2. Department of Thermophysiology, Institute for Translational Medicine, Medical School, University of Pécs, 12 Szigeti Street, 7624 Pécs, Hungary

3. Department of Pediatric Traumatology, Péterfy Hospital, Manninger Jenő National Trauma Center, 17 Fiumei Street, 1081 Budapest, Hungary

4. Department of Anatomy, Medical School, University of Pécs, 12 Szigeti Street, 7624 Pécs, Hungary

Abstract

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.

Funder

Department of Anatomy, Medical School, University of Pécs

Publisher

MDPI AG

Subject

Paleontology,Space and Planetary Science,General Biochemistry, Genetics and Molecular Biology,Ecology, Evolution, Behavior and Systematics

Reference20 articles.

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2. Syndactyly: Phenotypes, Genetics and Current Classification;Malik;Eur. J. Hum. Genet.,2012

3. Zaib, T., Rashid, H., Khan, H., Zhou, X., and Sun, P. (2022). Recent Advances in Syndactyly: Basis, Current Status and Future Perspectives. Genes, 13.

4. Genetic Determinants of Syndactyly: Perspectives on Pathogenesis and Diagnosis;Cassim;Orphanet J. Rare Dis.,2022

5. A case-control study on genetic and environmental factors regarding polydactyly and syndactyly;Luo;Zhonghua Liu Xing Bing Xue Za Zhi,2009

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