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[Anesthetic management of a patient with Freeman-Sheldon syndrome].
Masui. 1994 Nov; 43(11):1748-53.M

Abstract

The Freeman-Sheldon syndrome is a rare congenital myopathy and dysplasia that results in deformity of the face, hands, and feet. We describe here some problems in general anesthesia from the anesthetic management point of view. An 8-year-old girl with Freeman-Sheldon syndrome underwent surgery under general anesthesia for the correction of lip deformity and microstomia. Patients with this syndrome may present anesthetic problems involving difficulties in endotracheal intubation due to microstomia, micrognathia and neck rigidity, as well as postoperative respiratory complications and problems that relate to myogenetic morphology and myofunctional abnormalities. Preoperatively, we analyzed X-ray cephalograms to evaluate the difficulty of endotracheal intubation. Fiberscopic endotracheal intubation was performed; the time required was 55 minutes. On recovery from anesthesia and after becoming fully responsive, she was extubated in the operating room without any complications. Postoperative recovery was uneventful. Patients with Freeman-Sheldon syndrome should be managed by good preanesthetic preparation, and attention should be paid to postoperative respiratory complications due to the use of muscle relaxants and to securing intravenous access.

Authors+Show Affiliations

Second Department of Oral and Maxillofacial Surgery, Meikai University School of Dentistry, Saitoma.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

jpn

PubMed ID

7861610

Citation

Yamamoto, S, et al. "[Anesthetic Management of a Patient With Freeman-Sheldon Syndrome]." Masui. the Japanese Journal of Anesthesiology, vol. 43, no. 11, 1994, pp. 1748-53.
Yamamoto S, Osuga T, Okada M, et al. [Anesthetic management of a patient with Freeman-Sheldon syndrome]. Masui. 1994;43(11):1748-53.
Yamamoto, S., Osuga, T., Okada, M., Hashimoto, T., Shigematsu, H., Suzuki, S., Fujita, K., Matsumoto, N., & Hori, T. (1994). [Anesthetic management of a patient with Freeman-Sheldon syndrome]. Masui. the Japanese Journal of Anesthesiology, 43(11), 1748-53.
Yamamoto S, et al. [Anesthetic Management of a Patient With Freeman-Sheldon Syndrome]. Masui. 1994;43(11):1748-53. PubMed PMID: 7861610.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Anesthetic management of a patient with Freeman-Sheldon syndrome]. AU - Yamamoto,S, AU - Osuga,T, AU - Okada,M, AU - Hashimoto,T, AU - Shigematsu,H, AU - Suzuki,S, AU - Fujita,K, AU - Matsumoto,N, AU - Hori,T, PY - 1994/11/1/pubmed PY - 1994/11/1/medline PY - 1994/11/1/entrez SP - 1748 EP - 53 JF - Masui. The Japanese journal of anesthesiology JO - Masui VL - 43 IS - 11 N2 - The Freeman-Sheldon syndrome is a rare congenital myopathy and dysplasia that results in deformity of the face, hands, and feet. We describe here some problems in general anesthesia from the anesthetic management point of view. An 8-year-old girl with Freeman-Sheldon syndrome underwent surgery under general anesthesia for the correction of lip deformity and microstomia. Patients with this syndrome may present anesthetic problems involving difficulties in endotracheal intubation due to microstomia, micrognathia and neck rigidity, as well as postoperative respiratory complications and problems that relate to myogenetic morphology and myofunctional abnormalities. Preoperatively, we analyzed X-ray cephalograms to evaluate the difficulty of endotracheal intubation. Fiberscopic endotracheal intubation was performed; the time required was 55 minutes. On recovery from anesthesia and after becoming fully responsive, she was extubated in the operating room without any complications. Postoperative recovery was uneventful. Patients with Freeman-Sheldon syndrome should be managed by good preanesthetic preparation, and attention should be paid to postoperative respiratory complications due to the use of muscle relaxants and to securing intravenous access. SN - 0021-4892 UR - https://www.unboundmedicine.com/medline/citation/7861610/[Anesthetic_management_of_a_patient_with_Freeman_Sheldon_syndrome]_ L2 - http://www.diseaseinfosearch.org/result/2921 DB - PRIME DP - Unbound Medicine ER -