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Updated: May 25 2021

Radial Clubhand (radial deficiency)

Images
https://upload.orthobullets.com/topic/6067/images/TAR - xray - colorado_moved.jpg
https://upload.orthobullets.com/topic/6067/images/Case A - xray - Kosin_moved.jpg
https://upload.orthobullets.com/topic/6067/images/clinical photo - colorado_moved.jpg
https://upload.orthobullets.com/topic/6067/images/classification def.jpg
https://upload.orthobullets.com/topic/6067/images/Case A - clinical photo - Kosin_moved.jpg
https://upload.orthobullets.com/topic/6067/images/xray - colorado_moved.jpg
  • Summary
    • Radial Clubhand is a rare birth defect characterized by a deformity of hand, with perpendicular relationship between forearm and wrist, and an absent thumb. 
    • Diagnosis is made on physical exam.
    • Treatment may be observation or surgical reconstruction when the child is around 6 months of age. 
  • EPIDEMIOLOGY
    • Incidence
      • rare
        • 1 per 100,000
      • anatomic location
        • bilateral in 50-72%
        • thumb usually deficient as well
  • Etiology
    • Genetics
      • likely related to sonic hedgehog gene
    • Associated with
      • TAR
        • autosomal recessive condition with thrombocytopenia and absent radius
        • different in that thumb is typically present
      • Fanconi's anemia
        • autosomal recessive condition with aplastic anemia
        • Fanconi screen and chromosomal breakage test to screen
        • treatment is bone marrow transplant
      • Holt-Oram syndrome
        • autosomal dominant condition characterized by cardiac defects
      • VACTERL Syndrome
        • vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula, renal agenesis, and limb defects)
      • VATER Syndrome
        • vertebral anomalies, anal atresia, tracheoesophageal fistula, esophageal atresia, renal agenesis)
  • Classification
      • Bayne and Klug Classification
      • Type I
      • Deficient distal radial epiphysis
      • Type II
      • Deficient distal and proximal radial epiphyses
      • Type III
      • Present proximally (partial aplasia)
      • Type IV
      • Completely absent (total aplasia - most common)
  • Presentation
    • Physical exam
      • deformity of hand with perpendicular relationship between forearm and wrist
      • absent thumb
      • perform careful elbow examination
  • Imaging
    • Radiographs
      • entire radius and often thumb is absent
    • Laboratory
      • must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions
  • Treatment
    • Nonoperative
      • passive stretching
        • target tight radial-sided structures
      • observation
        • indicated if absent elbow motion or biceps deficiency
          • hand deformity allows for extra reach to mouth in presence of a stiff elbow
    • Operative
      • hand centralization
        • indications
          • good elbow motion and biceps function intact
          • done at 6-12 months of age
          • followed by tendon transfers
        • contraindications
          • older patient with good function
          • patients with elbow extension contracture who rely on radial deviation
          • proximate terminal condition
        • technique
          • involves resection of varying amount of carpus, shortening of ECU, and, if needed, an angular osteotomy of the ulna (be sure to spare ulnar distal physis)
          • may do as two stage procedure in combination with a distraction external fixator
          • if thumb deformity then combine with thumb reconstruction at 18 months of age
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