What is the role of surgery in the treatment of arthrogryposis multiplex congenita (AMC)?

Updated: Jan 03, 2019
  • Author: Mithilesh K Lal, MD, MBBS, MRCP, FRCPCH, MRCPCH(UK); Chief Editor: Maria Descartes, MD  more...
  • Print
Answer

The perioperative management of the arthrogrypotic patient can be difficult and is managed by an experienced anesthesia and medical team. [35, 36, 7] The following issues are noted:

  • Difficult in venous access

  • Prone to develop intraoperative hyperthermia

  • Certain drugs influenced by decreased muscle mass

  • Postoperatively, increased difficulty with atelectasis and stridor with an increased risk of aspiration

  • Vertebral instability secondary to decreased muscle mass and high cervical hypoplasia

If surgery is contemplated, early (age 3-12 mo) one-stage (bone and tendon transfer) surgery should be performed. Fine-tuning procedures, such as opponensplasty, may improve function at a later stage.

Specific joint problems should be addressed with regard to treatment of other joints and the goals for the patient. Soft-tissue surgery should be performed early, with osteotomies performed when growth is completed. In soft-tissue release procedures, tenotomies should be accompanied by capsulotomies. Long-term bracing and assistive devices are usually needed.

  • Feet

    • The most common deformity is a rigid talipes equinovarus deformity. The goal of treatment is a plantigrade, braceable foot.

    • Casting in the first 3 months and attempting to stretch the skin often fail to correct the deformity. The patient eventually needs an extensive medial and lateral release, followed by prolonged casting and bracing.

    • Recurrence is common as the child grows, and the patient may need eventual treatment with bony procedures, such as lateral column shortenings (Lichtblau procedures) or talectomy.

    • In a skeletally mature deformed foot, triple arthrodesis often gives a satisfactory plantigrade foot. In an older child with no previous treatment, a combination of soft-tissue releases and bony procedures may be indicated.

  • Knees

    • The goal of treatment is an extended knee for ambulation. This is more easily accomplished in an extension or hyperextension deformity than in a flexion deformity.

    • Flexion knee deformities are more common than fixed knee deformities and are more resistant to treatment.

    • A mild contracture (< 20°) does not interfere with functional ambulation and can be treated with passive stretching and splinting.

    • Moderate contractures (20-60°) need soft-tissue releases, including posterior capsulotomy of the knee joint, followed by long-term bracing.

    • Severe contractures (>60°) may need femoral shortening, in addition to soft-tissue releases, to decrease tension on neurovascular structures behind the knee. In an older child with severe flexion deformity, a knee disarticulation may be indicated.

    • Extension deformities often present as recurvatum or even anterior dislocation, but they respond better to physical therapy than flexion deformities. Initial treatment for recurvatum is passive stretching and splinting. If unsuccessful, quadricepsplasty should be performed when the patient is younger than 6 months. The deformity should be corrected before treating a dislocated hip to facilitate hip reduction.

  • Hips

    • Hip surgery should follow foot and knee surgery, especially in the presence of knee extension deformities. Hip surgery should be performed when the patient is younger than 1 year to facilitate ambulation. In some patients with bilateral hip dislocations and extremely mobile hips, open reduction may be attempted.

    • Hip flexion contractures are more difficult to treat than dislocations. Mild hip flexion contracture is acceptable for ambulation. A flexion contracture greater than 35° requires soft-tissue releases.

    • A bilateral hip dislocation greater than 35° with flexion contracture should be treated with stretching and soft-tissue releases but not with reduction.

    • Unilateral hip dislocation requires reduction to avoid pelvic obliquity and scoliosis.

  • Upper extremities

    • Treatment involves development of self-help skills (eg, feeding and toileting) and mobility skills (eg, pushing out of chair and using crutches). In evaluating the upper extremities, overall function of the entire extremity should be considered rather than function of the individual joints.

    • Upper-extremity surgery should not be considered until the patient is older than 5-6 years. Shoulder function is usually satisfactory without a rotational osteotomy unless the shoulder is severely internally rotated.

  • Elbows

    • The goal of treatment is an elbow with passive or active flexion capability (feeding arm) and extension capability (toilet arm). Extension is corrected with either capsulotomy (ie, to allow passive flexion) or capsulotomy plus provision of a motor power, provided by a Steindler flexorplasty, a triceps transfer, or a pectoralis major transfer.

    • Because the elbow is crucial to hand function, the elbow must be mobile before a wrist deformity is corrected.

  • Wrists

    • The major wrist deformity is flexion with ulnar deviation. Treatment should begin with stretching and splinting.

    • A severe deformity requires proximal row carpectomy with or without fusion.

    • A trapezoid wedge excision improves dorsiflexion.

  • Fingers

    • Minimal to moderate flexion deformities require passive stretching and splinting.

    • More severe deformities require soft-tissue releases and often require proximal interphalangeal joint fusions.

    • Thumb-in-palm deformities need to be corrected to provide opposition-improved grasp.

  • Spine

    • The spine is affected in about one third of patients. Scoliosis begins early and progresses to become a long, severe, rigid, C-shaped curve. This curve responds poorly to orthoses because it is progressive.

    • Curves greater than 35° should be treated with spinal fusion and instrumentation.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!