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- Title
Anesthesia Challenges in the Management of Freeman-Sheldon Syndrome: Report of Two Cases and Literature Review.
- Authors
Kamal, Geeta; Shah, Shreya Bharat; Gupta, Anju
- Abstract
Freeman-Sheldon syndrome is a congenital myopathy with a reported prevalence of less than 1 per 1 million. Also known as craniocarpotarsal dysplasia, this syndrome is characterized by muscle contractures and soft-tissue dysplasia of the face, hands, and feet. The resulting musculoskeletal deformities may require multiple orthopedic, ocular, and plastic reconstructive surgeries. The anesthetic challenges include a potentially difficult airway and intravenous access, suscep- tibility to malignant hyperthermia, and an unpredictable response to muscle relaxants. This report of the anesthetic management of 2 children with Freeman-Sheldon syndrome emphasizes the anesthetic considerations for the successful management of patients with this syndrome.
- Subjects
ACETAMINOPHEN; ANESTHESIA; ARTHROGRYPOSIS; BRONCHOSCOPY; EMLA (Anesthetics); FENTANYL; INTRAOPERATIVE monitoring; INTRAVENOUS therapy; LIDOCAINE; LOCAL anesthetics; MIDAZOLAM; ORAL drug administration; POSTOPERATIVE care; RESPIRATORY obstructions; DISCHARGE planning; PROPOFOL; BUPIVACAINE; LARYNGEAL masks
- Publication
AANA Journal, 2020, Vol 88, Issue 1, p35
- ISSN
0094-6354
- Publication type
Article