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Proper Positioning and Restraint of a Rat Hind Limb for Focused High Resolution Imaging of Bone Micro-architecture Using In Vivo Micro-computed Tomography

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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

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.
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.