{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T22:16:03Z","timestamp":1740176163810,"version":"3.37.3"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2023,7,13]],"date-time":"2023-07-13T00:00:00Z","timestamp":1689206400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,7,13]],"date-time":"2023-07-13T00:00:00Z","timestamp":1689206400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["JA Clin Rep"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Freeman-Sheldon syndrome (FSS) is a rare disorder characterized by specific deformities of the extremities and face. There have been no reports of open-heart surgery in pediatric patients with FSS.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>We present the case of an 8-year-old girl with FSS who underwent atrial septal defect closure. Tracheal intubation was uncomplicated, although the patient had microstomia. Inhalational anesthetics and dopamine antagonists were avoided intraoperatively and perioperatively. We chose dexmedetomidine as an adjuvant for postoperative pain management contributing to adequate analgesia and early extubation without causing respiratory depression.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Anesthetic management of FSS requires consideration for airway management and prevention of malignant hyperthermia and respiratory complications. We successfully managed the case avoiding the use of malignant hyperthermia-triggering drugs.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s40981-023-00633-9","type":"journal-article","created":{"date-parts":[[2023,7,13]],"date-time":"2023-07-13T01:02:02Z","timestamp":1689210122000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Anesthetic management of a pediatric patient with Freeman-Sheldon syndrome undergoing atrial septal defect closure: a case report"],"prefix":"10.1186","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2246-4650","authenticated-orcid":false,"given":"Kyosuke","family":"Takahashi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kotaro","family":"Sakurai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Izumi","family":"Hamaya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,7,13]]},"reference":[{"key":"633_CR1","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1002\/ajmg.1320550406","volume":"55","author":"P Klemp","year":"1995","unstructured":"Klemp P, Hall JG. Dominant distal arthrogryposis in a Maori family with marked variability of expression. Am J Med Genet. 1995;55:414\u20139.","journal-title":"Am J Med Genet"},{"key":"633_CR2","first-page":"345","volume":"7","author":"HM Munro","year":"1997","unstructured":"Munro HM, Butler PJ, Washington EJ. Freeman-Sheldon (whistling face) syndrome. Anaesth Airway Manage Paediatr Anaesth. 1997;7:345\u20138.","journal-title":"Anaesth Airway Manage Paediatr Anaesth"},{"key":"633_CR3","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1097\/00000542-199209000-00031","volume":"77","author":"R Jones","year":"1992","unstructured":"Jones R, Dolcourt JL. Muscle rigidity following halothane anesthesia in two patients with Freeman-Sheldon syndrome. Anesthesiology. 1992;77:599\u2013600.","journal-title":"Anesthesiology"},{"key":"633_CR4","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1136\/adc.13.75.277","volume":"13","author":"EA Freeman","year":"1938","unstructured":"Freeman EA, Sheldon JH. Cranio-carpo-tarsal dystrophy. Arch Dis Child. 1938;13:277\u201383.","journal-title":"Arch Dis Child"},{"key":"633_CR5","doi-asserted-by":"publisher","first-page":"754","DOI":"10.1542\/peds.2005-1219","volume":"117","author":"DA Stevenson","year":"2006","unstructured":"Stevenson DA, Carey JC, Palumbos J, Rutherford A, Dolcourt J, Bamshad MJ. Clinical characteristics and natural history of Freeman-Sheldon syndrome. Pediatrics. 2006;117:754\u201362.","journal-title":"Pediatrics"},{"key":"633_CR6","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1155\/2018\/7862327","volume":"2018","author":"S Viehmeyer","year":"2018","unstructured":"Viehmeyer S, Gabriel P, Bauer K, Bauer S, Sodian R, Hilberath JN. Anesthetic considerations for an adult patient with Freeman-Sheldon syndrome undergoing open heart surgery. Case Rep Anesthesiol. 2018;2018:1\u20134.","journal-title":"Case Rep Anesthesiol"},{"key":"633_CR7","doi-asserted-by":"publisher","first-page":"790","DOI":"10.1111\/j.1460-9592.2004.01533.x","volume":"15","author":"JS Kim","year":"2005","unstructured":"Kim JS, Park SY, Min SK, Kim JH, Lee SY, Moon BK. Awake nasotracheal intubation using fiberoptic bronchoscope in a pediatric patient with Freeman-Sheldon syndrome. Paediatr Anaesth. 2005;15:790\u20132.","journal-title":"Paediatr Anaesth"},{"key":"633_CR8","doi-asserted-by":"publisher","first-page":"783","DOI":"10.1007\/BF03013916","volume":"46","author":"GF Cruickshanks","year":"1999","unstructured":"Cruickshanks GF, Brown S, Chitayat D. Anesthesia for Freeman-Sheldon syndrome using a laryngeal mask airway. 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The study was performed in accordance with the Declaration of Helsinki.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent for publication was obtained from the patient and her guardian.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"40"}}