Canadian Journal of Anesthesia, Vol 32, 543-547, Copyright © 1985 by Canadian Anesthesiologists' Society

Anaesthetic Implications of Nemaline Rod Myopathy

¹ Department of Anaesthesia, The Hospital for Sick Children and University of Toronto, Toronto, Ontario

Address correspondence to: Dr. F.A. Burrows, The Department of Anaesthesia, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8.

Nemaline rod myopathy is an inherited congenital myopathy first described in 1963. Affected patients characteristically present in infancy with a non-progressive hypotonia and symmetrical muscle weakness. The disease affects all skeletal muscles including the diaphragm with sparing of cardiac and other muscle. Facial dysmorphism is common as are skeletal deformities, including kyphosis, scolosis and pectus excavatum.

We present two sisters with nemaline rod myopathy and their anaesthetic management for scoliosis surgery. Facial dysmorphism was a feature of both cases. Preoperatively, both patients demonstrated poor respiratory function on pulmonary function testing. Both cases were successfully managed using controlled ventilation and inhalational anaesthetic agents, avoiding muscle relaxants. Postoperatively, there were no respiratory complications.

Although one case report describes the use of succinylcholine and pancuronium in a patient with nemaline rod myopathy, we feel that neuromuscular blocking agents should be avoided where possible and only used with careful monitoring.

Key Words: GENETIC FACTORS: nemaline rod myopathy • ANAESTHETIC TECHNIQUES: inhalational agents, muscle relaxants • COMPLICATIONS: pulmonary function, difficult intubation

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