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Macrodactyly

What is macrodactyly?

It is a descriptive term used for a disproportionate enlargement of digit either present at birth or develops within the first few years of life.

It usually affects both the soft tissue and skeletal elements. This condition is also commonly called as gigantism.

What is the cause?

Though exact cause is not known proposed causes are abnormal nerve supply leading to unimpeded growth, or increase in blood supply or abnormal humoral mechanism stimulating growth.

Most commonly observed condition fatty infiltration and enlargement of the nerve associated with the digit. Other causes of macrodactyly are neurofibromatosis, other tumours, vascular malformation or lymph oedema.

How do we diagnose this condition?

Characteristic appearance is suggestive of this condition. The enlarged digit tends to deviate away from the involved interspace. Sometimes the growth may affect proximal parts of the hand or arm. Clinical examination would help is determining the function of affected part at individual joints. X rays are necessary to check for knowing the status of bones and joints.

Are there types/ classification for this deformity?

This condition is due to one of the four abnormalities.

  1. Lipofibromatosis of nerves: commonest and is also called as nerve territory oriented macrodactyly.
  2. Neurofibromatosis: secondary to neurofibromatosis.
  3. Hyperostosis: primarily involving bones
  4. Hemihypertrophy: enlargement of whole affected extremity. Proteus syndrome may affect hands feel and subcutaneous tissue elsewhere.

Most of these forms except hyperostosis run in families.

What is the treatment?

Macrodactyly is very difficult to treat. As it is progressive and diffuse in nature; complete correction is not possible. Sometimes surgery will require multiple stages.

Current options for surgical correction are procedures that limit ongoing growth, reduce the size of the digit, or correct the deviation, and amputation as last option.

Various procedures recommended are:

Limitation of growth

Digital nerve stripping/ Epiphysiodesis

Digit reduction

Soft tissue

Debulking

Skeletal

Terminalisation

Repositioning of the nail unit on a shortened skeleton

Resection of the distal portion of the nail and pulp

Correction of deviation

Closing wedge osteotomy

Amputation

Ray amputation

What are the complications of surgery?

Like any major procedure infection, bleeding and wound healing problems may happen. Rarely skin blood supply is affected and may need secondary procedure. The major problem in macrodactyly is recurrence of deformity with growth. This may need series of procedures for debulking of the affected part.

How long will be the stay in the hospital and postoperative downtime?

Normally the child stays in the hospital for 2-3 days. Patient goes home with dressings and is called periodically for dressing change. Wound healing normally takes around 2 weeks.

In special cases such as vascular malformation the hospital stay is longer as there is possibility of bleeding. In some cases, when skin blood supply is doubtful after the surgery we recommend hospital stay for dressings till the wound progresses to normal healing.

Before After
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Before After
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