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Canadian Journal of Anesthesia, Vol 33, 388-393, Copyright © 1986 by Canadian Anesthesiologists' Society

Freeman-Sheldon Syndrome: Report of three Cases and the Anaesthetic Implications

RICHARD S. LAISHLEY MB FFARCS1 and W. LAWRENCE ROY MD FRCPC1

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

Address correspondence to: Dr. W. L. Roy, Department of Anaesthesia, The Hospital for Sick Children, 555 University Avenue, Toronto, Ont. Canada, M5G 1X8.

The Freeman-Sheldon syndrome is a rare congenital myopathy and dysplasia. Fibrotic contractures of the facial muscles result in the characteristic "whistling face." Difficulties with intubation may be attributed in part to microstomia and micrognathia. In addition to other de-formities, limb myopathy results in ulnar flexion contractures of the hand and equinovarus/valgus deformities of the feet. Intravenous access may be difficult because of limb deformities and thickened subcutaneous tissues. Limbs may be encased in plaster casts or splints limiting the available sites for venepuncture. Three case reports of children with Freeman-Sheldon syndrome are presented. The pathophysiology and anaesthetic problems encountered are discussed.

Key Words: GENETIC FACTORS: Freeman-Sheldon syndrome, craniofacial dysostosis • ANAESTHESIA: complications






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Copyright © 1986 by the Canadian Anesthesiologists' Society.