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

• Published in: Canadian Anaesthetists' Society journal Vol. 33; no. 3; pp. 388 - 393
• Main Authors: LAISHLEY, R. S, ROY, W. L
• Format: Journal Article
• Language: English
• Published: Toronto, ON Canadian Anaesthetists' Society 01-05-1986
• Subjects: Anesthesia; Anesthesia depending on patient's condition; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Bone and Bones - abnormalities; Bone and Bones - surgery; Child; Child, Preschool; Face - abnormalities; Face - surgery; Female; Humans; Infant; Male; Medical sciences; Muscular Diseases - congenital; Muscular Diseases - physiopathology; Muscular Diseases - surgery; Syndrome; Human; Stomatology; Diseases of the osteoarticular system; Lower limb; General anesthesia; Concomitant disease; Malformation; Skull; Upper limb; Craniotarsal dysplasia Freeman Sheldon; Intubation; Face; Child; Trachea
• ISSN: 0008-2856; 1496-8975
• DOI: 10.1007/BF03010755

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 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.