Syndactyly
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- AD, 50% bilateral
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- Apert’s – premature closure of physes of skull - carniosynostosis
- Poland Syndrome – Sprengel’s deformity, unilateral chest wall hypoplasia
- Carpenter – craniofascial malfunction, obesity
- Arthrogryposis
- Constriction band syndrome
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- Complete if it involves nail folds
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- Simple = skin only
- Complex = involves bones
- Complicated = involves bones, ligaments,tendons
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- 1. Timing – 1-2yo
- Balance between GA and size of structures
- Do earlier if border/ peripheral digit as they are different lengths and will result in more deformity)
- 2. Commisure reconstruction - with zig zag incision
- 3. Skin grafting - needed in complete syndactyly
- 4. Single vs multiple stage
- Multiple fingers ➔ staged due to risk of ischemia
- Do border digits first due to marked difference in lengths to prevent tethering of longer digits ➔ flexion contractures and rotational deformity.
- Border digits
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- Create web commissure with Volar and dorsal zig zag incision
- Preserve and protect digital NVB
- Local flap to reconstruct web space; may need graft from groin
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- Web creep, contractures, instability
- Nail abnormalities
Symbrachydactyly
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- Syndactyly + Short fingers
- Can be distinguished from constriction band syndrome
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- Type 1 = Short fingers
- 2 = Oligodactylic = atypical cleft hand. Central portion aplastic, border digits less affected
- 3 = Monodactylic = all fingers gone except thumb aplasia
- 4 = Peromelic = resembles a trasverse amputation at the MCPJ level.
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- Adactylous = no fingers (type 3)
- Monodactylous = only thumb (type 3)
- Bidactylous = thumb + little finger (type 2)
- Multidigits (Type 1)
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- Ranges from removal of nubbins, to toe transfers
- If do op, do before 15 months to allow child to adapt