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Polydactyly of Hand

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Topic updated on 08/17/14 3:21pm
Introduction
  • A congential malformation of the hand.
  • Three forms exist
    • preaxial polydactlyly (see below)
      • thumb duplication
    • postaxial polydactlyly
      • small finger duplication
    • centrall polydactlyly
Preaxial Polydactyly (Thumb Duplication)
  • 1 per 1,000 to 10,000 live births
  • Type 4 most common (43%)
  • Type 2 second most common (15%)
  • Genetics
    • 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
  • 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
    • 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 (Bilhaut Cloquet)   
      • indications
        • Type 1, 2, or 3
      • 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 3 and 4
        • Type 5 and 6 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)
  • 10X more common in African Americans  
    • inherited as autosomal dominant
    • more complex genetic in whites and a thorough genetic workup should be performed  
  • Classification
    • Type A - well formed digit
    • Type B - rudimentary skin tag (vestigial digits)
  • Treatment
    • 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 amputated before 1 year of age
      • indications
        • Type B
Central Polydactyly
  • 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 ▶



Posts

post
Ogino T, Ishii S, Takahata S, Kato H
J Hand Surg Am. 1996 May;21(3):478-86. PMID: 8724483 (Link to Pubmed)
3 weeks ago
21 responses
0
post
Baek GH, Gong HS, Chung MS, Oh JH, Lee YH, Lee SK
J Bone Joint Surg Am. 2007 Mar;89(3):534-41. PMID: 17332102 (Link to Pubmed)
3 weeks ago
0 responses
0
post
Baek GH, Gong HS, Chung MS, Oh JH, Lee YH, Lee SK
J Bone Joint Surg Am. 2007 Mar;89(3):534-41. PMID: 17332102 (Link to Pubmed)
8/12/2013
36 responses
3
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