{"title":"A Case of Freeman-Sheldon Syndrome: Anesthetic Challenges.","authors":"Thomas Hamilton, Madhankumar Sathyamoorthy","doi":"","DOIUrl":null,"url":null,"abstract":"<p><strong>Unlabelled: </strong>Patients with Freeman-Sheldon Syndrome (FSS) often need multiple surgical procedures. We present a case of FSS and discuss the anesthetic challenges associated with the case.</p><p><strong>Case presentation: </strong>A 10-week-old female with FSS presented for elective Nissen fundoplication and gastrostomy tube insertion. She had a history of difficult intubation at birth. General anesthesia with inhalational anesthetic and spontaneous respirations technique was used. Fiber optic bronchoscope (FOB)-assisted nasal intubation was successful after failed attempts with a Miller blade, GlideScope, and intubation through a laryngeal mask airway (LMA). She did not exhibit any signs of malignant hyperthermia (MH) during or immediately after the procedure.</p><p><strong>Discussion: </strong>Challenges to the anesthesiologist in a case with FSS include establishing IV access, intubating the trachea, risk of MH and MH-like symptoms, and postoperative pulmonary complications. Proper multidisciplinary preoperative planning is essential for optimum care of these patients, preferably in a tertiary care center.</p>","PeriodicalId":17380,"journal":{"name":"Journal of the Mississippi State Medical Association","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the Mississippi State Medical Association","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Unlabelled: Patients with Freeman-Sheldon Syndrome (FSS) often need multiple surgical procedures. We present a case of FSS and discuss the anesthetic challenges associated with the case.
Case presentation: A 10-week-old female with FSS presented for elective Nissen fundoplication and gastrostomy tube insertion. She had a history of difficult intubation at birth. General anesthesia with inhalational anesthetic and spontaneous respirations technique was used. Fiber optic bronchoscope (FOB)-assisted nasal intubation was successful after failed attempts with a Miller blade, GlideScope, and intubation through a laryngeal mask airway (LMA). She did not exhibit any signs of malignant hyperthermia (MH) during or immediately after the procedure.
Discussion: Challenges to the anesthesiologist in a case with FSS include establishing IV access, intubating the trachea, risk of MH and MH-like symptoms, and postoperative pulmonary complications. Proper multidisciplinary preoperative planning is essential for optimum care of these patients, preferably in a tertiary care center.
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