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Related Experiment Videos

Anesthesia in 'stone man': myositis ossificans progressiva.

W H Stark1, S W Krechel, G W Eggers

  • 1Department of Anesthesiology, University of Missouri-Columbia School of Medicine.

Journal of Clinical Anesthesia
|September 1, 1990
PubMed
Summary

Myositis ossificans progressiva is a rare genetic disorder causing progressive muscle ossification. Anesthetic management requires careful consideration of respiratory compromise and potential intubation difficulties.

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Area of Science:

  • Anesthesiology
  • Genetics
  • Rare Diseases

Background:

  • Myositis ossificans progressiva (MOP) is a rare genetic disorder characterized by progressive ossification of the muscular system.
  • It follows an autosomal dominant inheritance pattern and is often misdiagnosed in childhood.
  • Anesthetic implications for MOP are sparsely documented in medical literature.

Observation:

  • Patients with MOP develop progressive ossification in striated muscle, ligaments, and fascia.
  • While cardiac and sphincter muscles are spared, chest wall muscles are affected, leading to deteriorating pulmonary function.
  • Common causes of mortality include pulmonary infections.

Findings:

  • Anesthetic challenges in MOP include positioning to prevent injury and potential need for advanced airway techniques like fiberoptic intubation or tracheostomy.

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  • Decreased thoracic compliance necessitates increased ventilatory pressures.
  • Disuse atrophy contraindicates the use of succinylcholine.

    • Implications:

    • Anesthetic management for MOP must be individualized based on disease severity.
    • Careful preoperative assessment and planning are crucial for safe anesthetic conduct.
    • Increased awareness of MOP's anesthetic implications can improve patient outcomes.