Polydactyly Hand

Author: Michael Hughes

Topic updated on 03/12/13 10:40am

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-Celoquet)
  - 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
- early osteotomy and ligament reconstructions 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

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

PREFERRED RESPONSE ▶

DISCUSSION: Image B is consistent for postaxial polydactyly, which is more prevalent in patients of African-American ancestry.

The cohort study by Woolf found the incidence of postaxial polydactyly in African americans is 12.42 per 1,000 (1.2%) compared to the Caucasian incidence of 0.91 per 1,000 (0.09%). If postaxial polydactyly is found in a patient of Caucasian ancestry then further workup for underlying syndromes (chondroectodermal dysplasia or Ellis-van Creveld syndrome) is needed.

The article by Orioli is a case-control study that hypothesizes that a sex-linked recessive modifier gene occurs more frequently in African americans and this gene then promotes the autosomal dominant polydactyly gene.

Incorrect Answers: Constriction band syndrome or amniotic band syndrome is a type of pseudosyndactyly (Figure A) and is not the result of failure of differentiation during embryogenesis, but a result of injury by bands after the fingers are formed. Preaxial polydactyly (Figure C) is more common in caucasians and is usually sporadic except for triphalangism which is associated with Holt-Oram and Fanconi's Anemia. Syndactyly (Figure D) is defined as an abnormal interconnection between adjacent digits and syndactyly variations are associated with Apert syndrome and Poland syndrome. Macrodactyly (Figure E) represents overgrowth of all structures of the involved digit and is associated with neurofibromatosis and Klippel-Trenaunay-Weber syndrome.

REFERENCES:

1. Ezaki M, Kay SPJ, Light TR, Tonkin MA, Wood VE, Dobyns JH. Congenital hand deformities, In: Green DP, Hotchkiss RN, Pederson WC, eds. Green's Operative Hand Surgery, 4th ed. New York, NY: Churchill Livingstone; 1999:325-551

2. Orioli IM. Segregation distortion in the offspring of Afro-American fathers with postaxial polydactyly. Am J Hum Genet. 1995 May;56(5):1207-11. PMID:7726178 (Link to Abstract)

3. Woolf CM, Myrianthopoulos NC. Polydactyly in American Negroes and Whites. Am J Hum Genet. 1973 Jul;25(4):397-404. PMID:4351996 (Link to Abstract)

Question Authors:

Michael Hughes MD, Sanj Kakar MD, Patrick McCulloch MD,

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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 ▶

DISCUSSION: The Bilhaut-Cloquet procedure has been shown to be successful in Wassel Type 1, 2, and 3 deformities. Asymmetrical Type I or II duplications with distinct components may also be treated with ablation of the smaller thumb with transfer of the collateral ligament and centralization of the extensor tendon. Types 3 and 4 duplications (Type 4= most common duplication) are often treated with selection of a dominant thumb and ablation of the lesser counterpart after preservation and transfer of intrinics and collateral ligaments. The article by Ogino states that the ablation of the radial thumb is most common and Miller's Review states radial thumb ablation is preferred. Treatment of Types 5 and 6 duplication utilizes similar principles as Type 4 deformities, with the added complexity of additional intrinsic reconstruction. Type 7 is a triphalangeal thumb and has variable treatment based on the presentation, and reconstruction includes excision versus fusion of the extra phalanx. Baek el al conducted a Level 4 review of 7 patients with Type 3 and Type 4 duplication treated with an extra-articular modified Bilhaut-Cloquet and found excellent IP joint range of motion, no nail deformities, and no episodes of growth arrest.

REFERENCES:

1. Ogino T, Ishii S, Takahata S, Kato H. Long-term results of surgical treatment of thumb polydactyly. J Hand Surg Am. 1996 May;21(3):478-86 PMID:8724483 (Link to Abstract)

2. Baek GH, Gong HS, Chung MS, Oh JH, Lee YH, Lee SK. Modified Bilhaut-Cloquet procedure for Wassel type-II and III polydactyly of the thumb. J Bone Joint Surg Am. 2007 Mar;89(3):534-41 PMID:17332102 (Link to Abstract)

Question Authors:

Michael Hughes MD, Bobby Chhabra M.D.,

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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

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

PREFERRED RESPONSE ▶

DISCUSSION: Postaxial polydactyly is rare in Caucasian individuals and deserves further workup for underlying syndromes. Postaxial polydactyly is ten times more common in African Americans and does not require further workup. Constriction band syndrome or amniotic band syndrome is a type of pseudosyndatyly(Figure A) and is not the result of failure of differentiation during embryogenesis, but a result of injury by bands after the fingers are formed. Preaxial polydactyly (Figure C) is more common in caucasians and is usually sporadic except for triphalangism which is associated with Holt-Oram and Fanconi's Anemia. Syndactyly (Figure D) is defined as an abnormal interconnection between adjacent digits and syndactyly variations are associated with Apert syndrome and Poland syndrome. Macrodactyly (Figure E) represents overgrowth of all structures of the involved digit and is associated with neurofibromatosis and Klippel-Trenaunay-Weber syndrome. The referenced article by Kozin is an excellent Current Concepts Review that summarizes upper limb embryogenesis and the most common anomalies.

REFERENCES:

1. Kozin SH. Upper-extremity congenital anomalies. J Bone Joint Surg Am. 2003 Aug;85-A(8):1564-76 PMID:12925640 (Link to Abstract)

Question Authors:

Michael Hughes MD,

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Groups

ABOS II: Polydactyly

General - Oral Boards Review

7/29/2011

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Evidence & References Show References

Level of Evidence 4 (Case Series)

Woolf CM, Myrianthopoulos NC. Polydactyly in American Negroes and Whites. Am J Hum Genet. 1973 Jul;25(4):397-404. PMID:4351996 (Link to Abstract)
Ogino T, Ishii S, Takahata S, Kato H. Long-term results of surgical treatment of thumb polydactyly. J Hand Surg Am. 1996 May;21(3):478-86 PMID:8724483 (Link to Abstract)
Baek GH, Gong HS, Chung MS, Oh JH, Lee YH, Lee SK. Modified Bilhaut-Cloquet procedure for Wassel type-II and III polydactyly of the thumb. J Bone Joint Surg Am. 2007 Mar;89(3):534-41 PMID:17332102 (Link to Abstract)

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

Orioli IM. Segregation distortion in the offspring of Afro-American fathers with postaxial polydactyly. Am J Hum Genet. 1995 May;56(5):1207-11. PMID:7726178 (Link to Abstract)
Kozin SH. Upper-extremity congenital anomalies. J Bone Joint Surg Am. 2003 Aug;85-A(8):1564-76 PMID:12925640 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Ezaki M, Kay SPJ, Light TR, Tonkin MA, Wood VE, Dobyns JH. Congenital hand deformities, In: Green DP, Hotchkiss RN, Pederson WC, eds. Green's Operative Hand Surgery, 4th ed. New York, NY: Churchill Livingstone; 1999:325-551

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Polydactyly Hand

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