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[Anesthetic management of an infant with Freeman-Sheldon syndrome].
Masui 2002; 51(6):659-62M

Abstract

We report the anesthetic management of Freeman-Sheldon (whistling face) syndrome in a two-month-old boy scheduled for lateral canthoplasty. He had features of the syndrome including blepharophimosis, hypertelorism, a flat nose, microstomia with a limited opening, micrognathia, a very short webbed neck, scoliosis and multiple arthrogryposis. He was fed with a naso-gastric tube and suffered from several episodes of aspiration and oxygen desaturation. Difficult airway and intubation were anticipated. Anesthesia was induced via a mask with sevoflurane, although mask ventilation was difficult. Direct laryngoscopy and the insertion of a laryngeal mask airway were impossible due to microstomia with the limited opening as anticipated. A naso-tracheal intubation was achieved using a fiberoptic bronchoscope via a fiberoptic mask while ventilating the lungs. The operation and anesthesia afterwards were uneventful. In the ward, he was given supplemental oxygen but with occasional desaturation episodes. Thirteen days after the operation he was found cyanotic and resuscitation was attempted but failed. Autopsy demonstrated the hypoplasia of the lungs and thorax, atelectasis and bronchitis.

Authors+Show Affiliations

Department of Anesthesiology, Osaka Medical Center, Research Institute for Maternal and Child Health, Izumi 594-1101.No 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

12134659

Citation

Okawa, Megumi, et al. "[Anesthetic Management of an Infant With Freeman-Sheldon Syndrome]." Masui. the Japanese Journal of Anesthesiology, vol. 51, no. 6, 2002, pp. 659-62.
Okawa M, Kinouchi K, Kitamura S, et al. [Anesthetic management of an infant with Freeman-Sheldon syndrome]. Masui. 2002;51(6):659-62.
Okawa, M., Kinouchi, K., Kitamura, S., Taniguchi, A., Sasaoka, N., & Fukumitsu, K. (2002). [Anesthetic management of an infant with Freeman-Sheldon syndrome]. Masui. the Japanese Journal of Anesthesiology, 51(6), pp. 659-62.
Okawa M, et al. [Anesthetic Management of an Infant With Freeman-Sheldon Syndrome]. Masui. 2002;51(6):659-62. PubMed PMID: 12134659.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Anesthetic management of an infant with Freeman-Sheldon syndrome]. AU - Okawa,Megumi, AU - Kinouchi,Keiko, AU - Kitamura,Seiji, AU - Taniguchi,Akihiro, AU - Sasaoka,Noriyuki, AU - Fukumitsu,Kazuo, PY - 2002/7/24/pubmed PY - 2002/8/28/medline PY - 2002/7/24/entrez SP - 659 EP - 62 JF - Masui. The Japanese journal of anesthesiology JO - Masui VL - 51 IS - 6 N2 - We report the anesthetic management of Freeman-Sheldon (whistling face) syndrome in a two-month-old boy scheduled for lateral canthoplasty. He had features of the syndrome including blepharophimosis, hypertelorism, a flat nose, microstomia with a limited opening, micrognathia, a very short webbed neck, scoliosis and multiple arthrogryposis. He was fed with a naso-gastric tube and suffered from several episodes of aspiration and oxygen desaturation. Difficult airway and intubation were anticipated. Anesthesia was induced via a mask with sevoflurane, although mask ventilation was difficult. Direct laryngoscopy and the insertion of a laryngeal mask airway were impossible due to microstomia with the limited opening as anticipated. A naso-tracheal intubation was achieved using a fiberoptic bronchoscope via a fiberoptic mask while ventilating the lungs. The operation and anesthesia afterwards were uneventful. In the ward, he was given supplemental oxygen but with occasional desaturation episodes. Thirteen days after the operation he was found cyanotic and resuscitation was attempted but failed. Autopsy demonstrated the hypoplasia of the lungs and thorax, atelectasis and bronchitis. SN - 0021-4892 UR - https://www.unboundmedicine.com/medline/citation/12134659/[Anesthetic_management_of_an_infant_with_Freeman_Sheldon_syndrome]_ L2 - http://www.diseaseinfosearch.org/result/2921 DB - PRIME DP - Unbound Medicine ER -