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Anesthesia Challenges in the Management of Freeman-Sheldon Syndrome: Report of Two Cases and Literature Review.
AANA J. 2020 Jan; 88(1):35-38.AJ

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, susceptibility 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.

Authors+Show Affiliations

is a senior specialist at Chacha Nehru Bal Chikitsalya, New Delhi, India.is an assistant professor at All India Institute of Medical Sciences, New Delhi, India.is an assistant professor at All India Institute of Medical Sciences, New Delhi, India.

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

32008616

Citation

Kamal, Geeta, et al. "Anesthesia Challenges in the Management of Freeman-Sheldon Syndrome: Report of Two Cases and Literature Review." AANA Journal, vol. 88, no. 1, 2020, pp. 35-38.
Kamal G, Shah SB, Gupta A. Anesthesia Challenges in the Management of Freeman-Sheldon Syndrome: Report of Two Cases and Literature Review. AANA J. 2020;88(1):35-38.
Kamal, G., Shah, S. B., & Gupta, A. (2020). Anesthesia Challenges in the Management of Freeman-Sheldon Syndrome: Report of Two Cases and Literature Review. AANA Journal, 88(1), 35-38.
Kamal G, Shah SB, Gupta A. Anesthesia Challenges in the Management of Freeman-Sheldon Syndrome: Report of Two Cases and Literature Review. AANA J. 2020;88(1):35-38. PubMed PMID: 32008616.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anesthesia Challenges in the Management of Freeman-Sheldon Syndrome: Report of Two Cases and Literature Review. AU - Kamal,Geeta, AU - Shah,Shreya Bharat, AU - Gupta,Anju, PY - 2020/2/4/entrez PY - 2020/2/6/pubmed PY - 2020/10/29/medline KW - Difficult airway KW - Freeman-Sheldon syndrome KW - laryngeal mask airway KW - nontriggering anesthesia SP - 35 EP - 38 JF - AANA journal JO - AANA J VL - 88 IS - 1 N2 - 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, susceptibility 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. SN - 2162-5239 UR - https://www.unboundmedicine.com/medline/citation/32008616/Anesthesia_Challenges_in_the_Management_of_Freeman_Sheldon_Syndrome:_Report_of_Two_Cases_and_Literature_Review_ L2 - http://www.diseaseinfosearch.org/result/2921 DB - PRIME DP - Unbound Medicine ER -