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A Complex Case of Clino-Syndactyly with Fourth Metacarpal Aplasia
Hermann Nudelman1, Aba Lőrincz2, Anna Gabriella Lamberti1
1Department of Paediatrics, Clinical Complex, Division of Surgery, Traumatology and Otorhinolaryngology, University of Pécs, 7 József Attila Street, 7623 Pécs, Hungary.
Insights
Syndactyly, a common congenital hand fusion, can present atypically. This case details surgical correction of syndactyly with clinodactyly and metacarpal aplasia in a young girl.
Area of Science:
- Orthopedics
- Pediatric Surgery
- Clinical Genetics
Background:
- Syndactyly is the most common congenital hand malformation, affecting 1 in 2500 children, often involving the middle and ring fingers.
- It presents as soft tissue or bony fusion, potentially involving phalanges, carpal/metacarpal bones, and can be isolated or part of syndromic conditions.
- Associated conditions include synostosis, acro-syndactyly, cleft hand, clinodactyly, polydactyly, and syndromes like Apert and Poland's.
Observation:
- A girl diagnosed at birth with congenital malformation of her right hand affecting the middle, ring, and little fingers.
- X-ray imaging revealed fusion of the third and fourth proximal phalanges to a common metacarpal.
- This resulted in a unique diagnosis of clino-syndactyly with metacarpal aplasia.
Findings:
- Surgical intervention involved a wedge osteotomy to correct phalangeal base synchondrosis and a dorsal flap for the III and IV interdigital space.
- A trapezoid flap was used for syndactyly release of the IV and V fingers.
- The study presents the surgical correction and outcomes for this atypical presentation.
Implications:
- Highlights the importance of individualized surgical approaches for complex syndactyly cases.
- Contributes to understanding and managing rare congenital hand anomalies.
- Provides insights into reconstructive techniques for syndactyly with associated metacarpal aplasia.
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.
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