What are the CDC recommendations for lower-limb contracture surgery in the early ambulatory phase of Duchenne muscular dystrophy (DMD)?

Updated: Aug 17, 2020
  • Author: Twee T Do, MD; Chief Editor: Jeffrey D Thomson, MD  more...
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Answer

The guidelines find no absolute situations in which lower-limb contracture surgery is invariably indicated. While surgical options exist, none could be recommended above any other. Options for surgery depend on individual circumstances, but can be utilized in both the ambulatory and non-ambulatory phases. Surgical options based on stage are provided below. [73]

Early ambulatory phase

Procedures include the following:

  • Heel cord (Achilles tendon) lengthening for equinus contractures
  • Hamstring tendon lengthening for knee-flexion contractures
  • Anterior hip-muscle releases for hip-flexion contractures
  • Excision of the iliotibial band for hip-abduction contractures

Middle ambulatory phase

Approaches to lower-extremity surgery to maintain walking include the following:

  • Bilateral multi-level (hip-knee-ankle or knee-ankle) procedures
  • Bilateral single-level (ankle) procedures
  • Unilateral single-level (ankle) procedures for asymmetric involvement (rarely used)

The surgeries may involve the following:

  • Tendon lengthening
  • Tendon transfer
  • Tenotomy (cutting the tendon)
  • Release of fibrotic joint contractures (ankle)
  • Removal of tight fibrous bands (iliotibial band at lateral thigh from hip to knee)

Equinus foot deformity (toe-walking) and varus foot deformities (severe inversion) can be corrected by heel-cord lengthening and tibialis posterior tendon transfer through the interosseous membrane onto the dorsolateral aspect of the foot to change plantarflexion-inversion activity of the tibialis posterior to dorsiflexion-eversion. Hamstring lengthening behind the knee is generally needed if the patient has a knee-flexion contracture of more than 15°.

After tendon lengthening and tendon transfer, postoperative bracing may be needed, which should be discussed preoperatively. Following tenotomy, bracing is always needed. When surgery is performed to maintain walking, the patient must be mobilized using a walker or crutches on the first or second postoperative day to prevent further disuse atrophy of lower-extremity muscles. Postoperative walking must continue throughout limb immobilization and post-cast rehabilitation. Close coordination between and an experienced team (eg, orthopedic surgeon, physical therapist, and orthoptist) is required.


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