Updated: 5/22/2021

Cleft Hand

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  • summary
    • Cleft Hand is a congenital hand deformity caused by a defect in the central part of the apical ectodermal ridge during embryological development, that is characterized by the absence of 1 or more central digits of the hand or foot.
    • Diagnosis is made clinically with the absence or shortening of the central (third) ray with the absence of radial digits and/or syndactyly of the ulnar digits.
    • Treatment is observation versus reconstruction depending on the degree of functional limitation. 
  • Epidemiology
    • Incidence
      • rare (1:10,000 to 1:90,000)
    • Demographics
      • male:female ratio is 5:1 (more common in male)
    • Anatomic 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
  • Etiology
    • 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 
    • Types
      • unilateral vs bilateral
      • isolated vs syndromic
    • Forms 
      • typical (central) cleft hand is characterized by
        • 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
    • 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
  • Classification
    • Manske and Halikis Classification
      Type
      Description
      Characteristics
      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
  • Prognosis
    • Functional limitation dependent on involvement of 1st webspace
    • Aesthetically displeasing, but not functionally limiting
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