Freeman-Sheldon syndrome: Report of three cases and the anaesthetic implications

  • Richard S. Laishley
  • W. Lawrence Roy
Clinical Reports


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 deformities, limb myopathy results in ulnar flexion contractures of the hand and equinovaruslvalgus 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 


Le syndrome de Freeman-Sheldon est une myopathie congénitale rare associé à une dysplasie. Les contractures fibrotiques des muscles faciaux aboutissent aux signes caractéristiques de la “face de siffleur” (whistling face). Les difficultés d’intubation seront attribuées en partie à la microstomie et la micrognatie. En plus d’autres déformités, la myopathie des membres provoque une contracture en flexion de la main el une déformité des pieds en equinovarus/valgus. L’accès intraveineux pent être difficile à cause des déformites des membres et de l’épaisseur des tissus sous-cutanés. Les membres peuvent être plâtrés imitant ainsi l’accès aux veines. On présente trois cas de patients atteint de ce syndrome. La pathophysiologie et les problèmes anesthésiques encourus sont discuteés.


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Copyright information

© Canadian Anesthesiologists 1986

Authors and Affiliations

  • Richard S. Laishley
    • 1
  • W. Lawrence Roy
    • 1
  1. 1.Department of AnaesthesiaThe Hospital for Sick ChildrenTorontoCanada

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