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Polydactyly of Hand

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Topic updated on 01/28/15 10:31pm
Introduction
  • A congential malformation of the hand
  • Three forms exist
    • preaxial polydactlyly 
      • thumb duplication
    • postaxial polydactlyly
      • small finger duplication
    • centrall polydactlyly
Preaxial Polydactyly (Thumb Duplication)
  • Epidemiology
    • incidence
      • 1 per 1,000 to 10,000 live births 
      • Type IV most common (43%) 
      • Type II second most common (15%)
    • demographics
      • M>F
      • caucasian > African Americans
  • Genetics
    • inheritance pattern
      • usually unilateral and sporadic 
      • except for Type VII which is associated with several syndrome including
        • Holt-Oram syndrome
        • Fanconi's anemia
        • Blackfan-Diamond anemia
        • imperforate anus
        • cleft palate
        • tibial defects
  • Associated conditions
    • pollex abductus
      • abnormal connection between EPL and FPL tendons, seen in approximately 20% of hypoplastic and duplicated thumbs
      • suggested by abduction of affected digit + absence of IP joint crease
  • Classification
 Wassel Classification of Preaxial Polydactyly 
Type I Bifid distal phalanx   
Type II Duplicated distal phalanx                            
Type III Bifid proximal phalanx                                
Type IV Duplicated proximal phalanx (most common)  
Type V Bifid metacarpal  
Type VI Duplicated metacarpal  
Type VII Triphalangia  

  • Treatment
    • operative
      • goals of treatment
        • to construct a thumb that is 80% of the size of the contralateral thumb
        • resect smaller thumb (usually radial component)
        • preserve / reconstruct medial collateral structures in order to preserve pinch function
        • reconstruction of all components typically done in one procedure
      • type 1 combination procedure (Bilhaut-Cloquet)   
        • indications
          • type I, II, or III
        • technique
          • involves removing central tissue and combining both digits into one
        • outcomes
          • approximately 20% have late deformity
          • problems include stiffness, angular and size deformity, growth arrest, and nail deformities
      • type 2 combination procedure
        • indications
          • usually favored approach for type III and IV
          • type V and VI usually require more complex transfer of intrinsics and collateral ligaments
        • technique
          • preserve skeleton and nail of one component and augment with soft tissue from other digit and ablation of lesser digit (radial digit most commonly)
      • type 3 combination procedure
        • indications
          • when one digit has superior proximal component and one digit has superior distal fragment (type V, VI, and VII)
        • technique
          • a segmental distal transfer (on-top plasty)
Postaxial Polydactyly (Small Finger Duplication)
  • Epidemiology
    • demographics
      • 10X more common in African Americans
  • Genetics
    • inherited as autosomal dominant (AD) in African Americans
    • more complex genetics in caucasians and a thorough genetic workup should be performed  
  • Classification
    • Type A - well formed digit
    • Type B - rudimentary skin tag (vestigial digits)
  • Treatment
    • operative
      • formal reconstruction with a Type 2 combination
        • indications
          • Type A
        • technique
          • preserve radial digit
          • preserve or reconstruct collateral ligaments from ulnar digit remnant
          • preserve muscles
      • tie off in nursery or amputate before 1 year of age
        • indications
          • Type B
Central Polydactyly
  • Epidemiology
    • commonly associated with syndactyly 
      • extra digit may lead to angular deformity or impaired motion
  • Treatment
    • osteotomy and ligament reconstructions
      • indications
        • perform early to prevent angular growth deformities

 

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Qbank (3 Questions)

TAG
(OBQ10.151) Which of the following congenital hand deformities displayed in figures A-E is more prevalent in patients of African-American ancestry? Topic Review Topic
FIGURES: A   B   C   D   E  

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶
TAG
(OBQ09.54) The Bilhaut-Cloquet procedure for thumb duplication, where the central portions of bone and nail are removed and the radial half of one thumb is combined with the ulnar half of the other to create one thumb, is most appropriate in which Wassel Type shown in Figure A? Topic Review Topic
FIGURES: A          

1. Type 2
2. Type 4
3. Type 5
4. Type 6
5. Type 7

PREFERRED RESPONSE ▶
TAG
(OBQ09.146) Which of the following upper extremity congenital anomalies, represented in the figures below, if found in an individual of Caucasian descent requires a work-up for chondroectodermal dysplasia or Ellis-van Creveld syndrome? Topic Review Topic
FIGURES: A   B   C   D   E  

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶



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