Polydactyly Ppt

5/27/2018 Polydactyly Ppt

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By:Fauziah

( C 111 06 089)

ADVISORS:

Dr. A. SirfaDr. Nasrah

Department of Orthopedic and TraumatologyFaculty of Medicine Hasanuddin University


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Introduction Polydactyly is the most common congenital digital

anomaly of the hand and foot.

It may appear in isolation or in association with otherbirth defects.

Isolated polydactyly is often autosomal dominant

Syndromic polydactyly is commonly autosomal

recessive


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Pathophysiology

Polydactyly should not be considered as a singlemendelian trait but rather multifactorial.

Early theories for polydactyly concerned disorders inthe programmed cell death cycle of fetal limbdevelopment.

Current theories focus on mutations in specific genetic

locations that cause limb development to go awry.


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Mutations in the HOXD13gene . As limb growth inutero progresses along a preset time line, elongation ofthe limb, development of soft tissue, anddifferentiation of digits progresses.


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ClassificationPreaxial polydactyly (thumb duplication)

Preaxial (involving thumb or great toe, Wasselclassification ):

I: Bifid distal phalanx

II: Duplicated distal phalanx

III: Bifid proximal phalanx

IV: Duplicated proximal phalanxmost common

V: Bifid metacarpal

VI: Duplicated metacarpal

VII:Triphalangia


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Preoperative photograph of a 1-year-old child with preaxial polydactyly andsignificant varus of the duplicated toe.

Preaxial polydactyly of theright hand.


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Classification Central


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Postaxial (involving small finger or toe): Type A: Complete duplication with bony attachment to an

adjacent digit

Type B: Rudimentary, incomplete duplication of the

phalanges


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Epidemiology Preaxial form is more common in whites than in other

races and most cases are sporadic

No significant difference in reported occurrencebetween the genders

Postaxial hand polydactyly is a common isolateddisorder in African black and African American

children, and autosomal dominant transmission issuspected.

Central polydactyly is uncommon compared withborder polydactyly.


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Clinical ManifestationSigns and Symptoms Duplicated fingers or toes

Physical Exam Check for active and passive movement at each joint.

Assess the stability of the digit.

Look for an angular deformity at each joint.

Look at the skin coverage and webbing.All these factors are important in determining surgical

treatment.


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Imaging

Plain film radiography is indicated

Left foot with postaxial

polydactyly of 5th ray

Left hand with mid-ray duplicatio
http://en.wikipedia.org/wiki/File:Polydactyly_01_Lhand_AP.jpghttp://en.wikipedia.org/wiki/File:Polydactyly_01_Lhand_AP.jpghttp://en.wikipedia.org/wiki/File:Polydactyly_01_Lfoot_AP.jpghttp://en.wikipedia.org/wiki/File:Polydactyly_01_Lfoot_AP.jpg


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TREATMENT Preaxial polydactylyThe best possible thumb is

reconstructed from the available anatomic structures.When duplicate thumbs of equal size are present, theradial thumb is removed. Reconstruction of allcomponens is usually performed in one stage.

AxialThe tendons, nerves, and vessels may beshared to the point that only one finger from threeskeletons may be obtainable.


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TREATMENTPostaxial polydactyly

- Type ARequires operative ablation with transfer of

any important parts to the adjacent finger. Type B postaxial polydactylyin newborn tie off the

digit if it has no underlying skeletal connection. In thepresence of a skeletal connection, the extra digit

should be electively removed surgically after 6 monthsof age.


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Complications

Small skin tag at the site of the polydactyly removal(rare)

Angular deformity possible after complex polydactylyreconstructions; possible deformity after thumbreconstruction


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THANK YOU

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Polydactyly Ppt