By: Fauziah ( C 111 06 089) ADVISORS: Dr. A. Sirfa Dr.

Nasrah

Department of Orthopedic and Traumatology Faculty of Medicine Hasanuddin University

Introduction
Polydactyly is the most common congenital digital

anomaly of the hand and foot. It may appear in isolation or in association with other birth defects. Isolated polydactyly is often autosomal dominant Syndromic polydactyly is commonly autosomal recessive

Pathophysiology
Polydactyly should not be considered as a single

mendelian trait but rather multifactorial. Early theories for polydactyly concerned disorders in the programmed cell death cycle of fetal limb development. Current theories focus on mutations in specific genetic locations that cause limb development to go awry.

 Mutations in the HOXD13 gene . As limb growth in

utero progresses along a preset time line, elongation of the limb, development of soft tissue, and differentiation of digits progresses.

Classification
Preaxial polydactyly (thumb duplication)
Preaxial (involving thumb or great toe, Wassel

classification ):

I: Bifid distal phalanx II: Duplicated distal phalanx III: Bifid proximal phalanx IV: Duplicated proximal phalanx most common V: Bifid metacarpal VI: Duplicated metacarpal VII:Triphalangia

Preoperative photograph of a 1-yearold child with preaxial polydactyly and significant varus of the duplicated toe.

Preaxial polydactyly of the right hand.

Classification
Central

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

Epidemiology
Preaxial form is more common in whites than in other

races and most cases are sporadic No significant difference in reported occurrence between the genders Postaxial hand polydactyly is a common isolated disorder in African black and African American children, and autosomal dominant transmission is suspected. Central polydactyly is uncommon compared with border polydactyly.

Clinical Manifestation
Signs 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.

Imaging
Plain film radiography is indicated

Left foot with postaxial polydactyly of 5th ray

Left hand with mid-ray duplicatio

TREATMENT
Preaxial polydactyly The best possible thumb is

reconstructed from the available anatomic structures. When duplicate thumbs of equal size are present, the radial thumb is removed. Reconstruction of all componens is usually performed in one stage. Axial The tendons, nerves, and vessels may be shared to the point that only one finger from three skeletons may be obtainable.

TREATMENT
Postaxial polydactyly - Type A Requires operative ablation with transfer of any important parts to the adjacent finger. Type B postaxial polydactyly in newborn tie off the digit if it has no underlying skeletal connection. In the presence of a skeletal connection, the extra digit should be electively removed surgically after 6 months of age.

Complications
Small skin tag at the site of the polydactyly removal

(rare) Angular deformity possible after complex polydactyly reconstructions; possible deformity after thumb reconstruction

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