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Cleft hands with six metacarpals
1UCLA Hand Center, Department of Orthopaedic Surgery and Division of Plastic and Reconstructive Surgery, University of California, Los Angeles 90095-9607, USA.
Insights
Children with cleft hands and 6 metacarpals demonstrate polydactyly and syndactyly, suggesting a shared developmental origin rather than a failure of formation. This finding supports a central polydactyly and osseous syndactyly hypothesis for cleft hand development.
Area of Science:
- Developmental biology
- Orthopedic surgery
- Pediatric radiology
Background:
- Cleft hand is a congenital limb malformation.
- The etiology of cleft hand remains debated, with theories including failure of formation and polydactyly/syndactyly processes.
Purpose of the Study:
- To investigate the radiographic features of cleft hands with 6 metacarpals.
- To support the hypothesis that cleft hand arises from central polydactyly and osseous syndactyly, not longitudinal failure of formation.
Main Methods:
- Screening of hand radiographs from children with upper limb deficiencies.
- Identification of 8 children with cleft hands exhibiting 6 metacarpals.
Main Results:
- Six cases presented with a missing middle finger, featuring a thumb and index finger separated by a V-shaped cleft from the ring and small fingers.
- Two severe cases showed absence of index and middle fingers, yet possessed 6 metacarpals.
Conclusions:
- The presence of polydactyly and osseous syndactyly in these cleft hands contradicts failure of formation.
- Findings support a unified teratogenic mechanism for cleft hand, polydactyly, and syndactyly.
- Severe cleft hand manifestations with 6 metacarpal polydactyly confirm a common failure of induction mechanism.
Purpose:
The purpose of this study was to identify children with cleft hands whose radiographs showed metacarpal polydactyly with 6 metacarpals to support the hypothesis that cleft hand develops not as a result of a longitudinal failure of formation but through a process of central polydactyly and osseous syndactyly.
Methods:
We screened the hand radiographs of all children with a transverse or longitudinal deficiency of the upper limb and identified 8 children with cleft hands containing 6 metacarpals.
Results:
Six cleft hands had a missing middle finger and consisted of a thumb and index finger separated from the ring and small fingers by a V-shaped central cleft. Two children had a more severe form of cleft hand with absence of both the index and middle fingers but presence of 6 metacarpals.
Conclusions:
These 8 cleft hands containing 6 metacarpals showed progression of polydactyly of the middle finger and osseous syndactyly between the 2 middle finger metacarpals and the adjacent index and ring finger metacarpals. This contradicts a longitudinal failure of formation mechanism and supports the alternative hypothesis that cleft hand, polydactyly, and syndactyly develop through a similar teratogenic mechanism. The 2 cleft hands that had more severe suppression of the index and middle fingers yet had 6 metacarpal polydactyly provided confirmatory evidence that both typical cleft hands and the more severe manifestations of cleft hand with absence of multiple digits develop through a similar failure of induction mechanism.
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