Congenital Thumb Deformities
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1. Thumb Hypoplasia 2. Clasped Thumb 3. Triphalangeal Thumb
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Congenital Thumb Deformities
- 1. Congenital Thumb Deformities Dr Mujtuba Pervez Khan Resident Plastic Surgery DUHS/CHK
- 2. • Apical Ectodermal Ridge = Growth in proximal to distal direction • Zone of Polarizing Activity = Antero-posterior growth • Non ridge ectoderm = Dorso-ventral growth
- 5. • Normal thumb is just proximal to the PIPJ of the index finger • Isolated • Part of radial deficiency with normal forearm
- 7. workup • CBC • echo • X-ray spine • U/S KUB • X-ray Radius/Ulna • X-ray tibia/fibula • Chromosome challenge test
- 9. Type 1 Hypoplasia • Generalized hypoplasia usually involves thenar muscles • functional deficit -> tendon transfer Type 2 hypoplasia • Absence of muscles innervated by recurrent branch median nerve (APB, OPB, superficial head of FPB) • Deep head of FPB provides MCPJ flexion • MCPJ flexion is unstable due to UCL insufficiency, Both UCL and RCL may be involved • Narrow 1st web space, appears less narrow due to valgus position of MCPJ
- 10. Type 2 hypoplasia • 1st web space narrowing -> z-plasty, • extremely tight web may require partial release of Adductor/1st dorsal interosseous • UCL or/and RCL reconstruction by tendon graft • Chondrodesis for stable MCPJ • Release of pollex abductus for pollex abductus • Tendon transfer for opposition • FDS middle/ring finger, EIP, ECU, Adm
- 11. Type 3 hypoplasia • Type 2 + absence or hypoplasia of EPL, EPB, FPL • Stable CMC (3A) -> reconstruction • Trapezio-metacarpal joint ossify at 6 years. X-rays before this age will be unreliable • MRI • Wide index and long finger web space Unstable CMC (3B), Type 4, Type 5 -> Policization
- 15. Clasped Thumb
- 16. • Type 1 : Extensor mechanism absent/hypoplastic • Type 2 : Type 1 + Joint contracture, Collateral ligament abnormality, 1st web space contracture, Thenar muscle abnormality • Type 3 : Mild or no extensor abnormality, Arthrogryposis, associated syndromes MASA (mental retardation, aphasia, shuffling gait, adducted thumb)
- 17. C/F : • Thumb in flexion • Extension lag at MCPJ, may also be present at IPJ • EPB most commonly involved • +/- APL, EPL
- 18. Treatment • No treatment for mild MCPJ extension lag • Splintage 2-6 months • Surgery for failed splinting or if present after 2 years • 1st web space contracture -> z plasty (4 flap plasty) / dorsal rotation advancement flap • Tendon transfer (EIP, EDM, FDS) for extensors • Thumb spika for 6 weeks, night splintage for the next 6 weeks
- 20. • Autosomal dominant, can also be sporadic • May be isolated or associated with 1. Holt Oram Syndrome 2. Fanconi’s anemia 3. Wassel type VII thumb duplication 4. Thumb hypoplasia 5. Polydactyly 6. Syndactyly 7. Central deficiency
- 21. Type 1: Relatively normal appearing thumb with an extra phalanx of variable size Triangular, trapezoidal, rectangular shaped Type 2: Fully developed phalanx
- 22. Management Between 12-18 months – oppositional pinch Length and angulation • extra bone may show no growth and require no treatment • excess growth and angulation in ulnar direction -> Surgery • extra phalanx excision and collateral ligament reconstruction • Joint stiffness, instability, insufficient motion are common problems Extra interphalangeal joint • excision of phalanx should not be done due to instability • Fusion of abnormal phalanx with distal OR proximal phalanx, bone shortening • Joint with the greatest motion is preserved
- 23. Management Thumb index web space • z plasty / rotational advancement flap • Type 2 has no web space -> policization Opposition • Tendon transfer (FDS, ADM)
- 24. Thanks.