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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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