TYPE OF SURGERIES

Birth defects

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Swellings on any part of the body

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Orthopedic

Polydactyly

  • Polydactyly

Description of disability:

Polydactyly or  supernumerary digits 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, while syndromic polydactyly is often autosomal recessive

Polydactyly is associated with different mutations, either mutations in a gene itself or in a cis-regulatory element responsible for the expression of a specific gene. Mutations in Hoxa- or Hoxd clusters are reported leading to polydactyly.

In the specific case of preaxial polydactyly (Hemingway mutant), a cis-acting mutation approximately 1Mb upstream of Shh gene has been implicated. Normally Shh is expressed in an organiser region, called the zone of polarizing activity (ZPA) on the posterior limb side. From there it diffuses anteriorly, laterally to the growth direction of the limb. In the mutant, smaller ectopic expression in a new organiser region is seen on the posterior side of the limb. This ectopic expression causes cell proliferation delivering the raw material for one or more new digits.

Treatment:

The method for removing an extra digit depends on how it connects to the hand or foot. An extra digit may connect with only a narrow stalk of tissue, or it may connect more deeply and share bones, muscles and other tissues with the hand or foot.

If the digit is poorly formed and contains no bone, sometimes the treatment is as simple as attaching a vascular clip at the base during a clinic visit. The clip stops blood flow to the digit so it will fall off, like the stump of belly button does soon after birth. After attaching the clip, a bandage is put on the child's hand or foot. In a couple of weeks, the child comes back to the clinic to have the bandage removed.

If the digit is better formed, the surgeon removes it in the operating room when the child is about 1 year old. The child's surgery will be based on the exact condition. More complex cases may require complex surgery. The surgery may involve carefully cutting through or around bones, ligaments, muscles, tendons and other tissues to remove the extra digit. Then the surgeon may need to move or reconnect some structures before closing the skin so the whole hand or foot works well and looks normal.

Post operative care:

After surgery, the child may need to wear a cast or splint on the hand or foot while it heals. The child needs to come back for follow-up visits to make sure they are healing well. The children who need extensive surgery with cutting through many tissues may have occupational therapy to help with swelling, scarring and stiffness.


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