Updated: 10/12/2016

Cleft Hand

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Introduction
  • Definition
    • typical (central) cleft hand is characterized by absence of 1 or more central digits of the hand or foot
      • also known as lobster-claw deformity
    • Swanson type I failure of formation (longitudinal arrest) of central ray, leaving V-shaped cleft in the center of the hand
    • types
      • unilateral vs bilateral
      • isolated vs syndromic
  • Epidemiology
    • incidence
      • rare (1:10,000 to 1:90,000)
    • demographics
      • male:female ratio is 5:1 (more common in male)
    • location
      • hands, usually bilateral
        • associated with absent metacarpals (helps differentiate from symbrachydactyly)
        • missing middle finger
        • on the ulnar side, small finger is always present
      • often involves feet as well
  • Pathogenesis 
    • theory is wedge-shaped degeneration of central part of apical ectodermal ridge (AER) because of loss of function of certain genes expressed in that part of the AER
  • Genetics
    • inheritance pattern
      • autosomal dominant with reduced penetrance (70%) 
      • inherited forms become more severe with each generation
    • mutations
      • deletions, inversions, translocations of 7q
        • split hand-split foot syndrome
    • affected families should undergo genetic counseling
  • Associated conditions
    • Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome
    • sensorineural hearing loss
    • syndactyly and polydactyly
  • Prognosis
    • functional limitation dependent on involvement of 1st webspace
    • aesthetically displeasing, but not functionally limiting
Classification
 
Manske and Halikis Classification 
Type  Description Characteristics Images
I Normal web
Thumb space not narrowed  
IIA Mildly narrowed web Thumb space mildly narrowed  
IIB Severely narrowed web Thumb space severely narrowed   
III
Syndactylized web
Thumb and index rays syndactylized, web space obliterated  
IV Merged web Index ray suppressed, thumb web space merged with cleft  
V Absent web Thumb elements suppressed, ulnar rays remain, thumb web space no longer present  
 
Presentation
  • History
    • may have family history 
  • Symptoms
    • aesthetic limitation
    • functional limitation
  • Physical exam
    • absent or shortened central (third) ray
    • may have absent radial digits
    • may have syndactyly of ulnar digits
      • may involve feet
Imaging
  •  Radiographs
    • recommended views
      • AP, lateral, oblique views of bilateral hands
      • foot radiographs if involved
Treatment
  • Nonoperative
    • observation
      • indications
        • types I (normal web) and IV (merged web), no functional impairment
  • Operative
    • thumb web space, thumb, and central cleft reconstruction
      • indications
        • types IIA, IIB, III and V webs
Technique
  • Thumb, thumb web space reconstruction
    • web space deepening, tendon transfer, rotational osteotomy, toe-hand transfer
    • thumb web reconstruction has greater priority over correction of central cleft
    • thumb reconstruction should not precede cleft closure as it might compromise skin flaps
  • Central cleft reconstruction
    • depends on characteristic of thumb web space
    • close the cleft proper with local tissues from the cleft and stabilize and close intermetacarpal space
 

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