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Updated: May 22 2021

Congenital Clasped Thumb

  • Summary
    • Congenital Clasped Thumb is a congenital pediatric condition that results in a flexion-adduction deformity of the thumb that persists beyond the 3rd or 4th month of life.
    • Diagnosis is made clinically with the presence of a flexion-adduction deformity of the thumb, that is often bilateral.  
    • Treatment is usually a trial of nonoperative management with serial splinting and stretching for 3-6 months. Surgical management is indicated for persistent deformity, significant soft tissue defects or, MCP contractures. 
  • Epidemiology
    • Demographics
      • male-to-female ratio is approx 2.5:1
    • Risk factors
      • exact causative factors are not well known
      • possible pre-disposing factors include
        • consanguinity
        • family history
  • Etiology
    • Pathophysiology
      • genetics
        • autosomal dominance inheritance of variable expressivity
        • may be sporadic
      • pathoanatomy
        • attenuation or deficiency of EPB (more common, in mild cases) or EPL, or both
        • associated with 1st web contracture
        • contracture of adductor pollicis or first dorsal interosseous muscle
        • global instability of first MP joint
        • abnormal articular cartilage of first MP joint
    • Orthopedic considerations
      • common manifestations associated with disease
        • lower limb anomalies
          • congenital vertical talus
          • congenital talipes equinovarus (bilateral)
        • upper limb anomalies
          • flexion deformities of the four fingers
    • Associated conditions
      • arthrogryphosis (congenital joint contractures affect two or more areas in the body)
      • digitotalar dysmorphisms
      • Freeman-Sheldon syndrome
      • X-linked MASA syndrome
  • Classification
      • Tsuyuguchi Classification of Clasped Thumb
      • Type I (Supple clasped thumb)
      • Thumb can be passively abducted and extended against resistance of thumb flexors. No other digital anomaly present.
      • Type II (Clasped thumb with contracture)
      • Thumb cannot be passively extended and abducted. This may occur with or without other digital anomalies.
      • Type III (Rigid clasped thumb)
      • Clasped thumb that is associated with arthrogryposis and marked soft-tissue deficits.
  • Presentation
    • History
      • persistent flexion-adduction deformity beyond 3rd or 4th month of life, usually bilateral
      • family history
      • pre-natal history
    • Symptoms
      • pain usually with a contracture
      • associated with other musculo-skeletal deformities
    • Examination
      • type of clasped thumb
      • associated anomalies
  • Treatment
    • Nonoperative
      • serial splinting and stretching for 3-6 months
        • indications
          • first-line treatment for all types
          • begin treatment around the age of 6 month old
        • outcome
          • good definitive results with Type I congenital deformities when one of the EPL or EPB tendons are present
          • poor results with Type I deformities when both EPL/EPB tendons are absent
          • poor results with Type II or III deformities
    • Operative
      • EIP tendon transfer to EPL
        • indications
          • Type I or II with residual deficiency in active extension
      • thumb reconstruction
        • indications
          • failed conservative treatment
          • soft-tissue deficiency in the thumb-index finger webspace (Type III)
          • Type II or III deformity with significant MCP joint contractures
      • arthrodesis
        • indications
          • severe deformities when skin release and tendon transfer cannot overcome joint deformity.
  • Techniques
    • Thumb reconstruction
      • delayed until the age of 3 to 5 years old
      • procedure based on amount of contracture and may include
        • 1st web widening
          • transposition flap of skin (dorsal rotational advancement flap)
          • four-flap or five-flap Z plasty
        • deepening the first webspace by releasing soft-tissue
          • releasing origins of thenar musculature from transverse carpal ligament
          • releasing joint capsule of first MP joint
        • tendon transfer
          • FPL Z-lengthening in the forearm
          • EPB and EPL absence is best reconstructed with tendon transfer
            • isolated EPB absence will not usually require tendon transfer
  • Complications
    • Cosmetic appearance
    • Instability of the MP joint
    • Reduced thumb function
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