{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T11:53:13Z","timestamp":1777377193888,"version":"3.51.4"},"reference-count":20,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2008,3,1]],"date-time":"2008-03-01T00:00:00Z","timestamp":1204329600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Anaesth Intensive Care"],"published-print":{"date-parts":[[2008,3]]},"abstract":"<jats:p>Freeman-Sheldon syndrome, or distal arthrogryposis type 2A, is a rare congenital myopathy and dysplasia characterised by multiple contractures, abnormalities of the head and face, defective development of the hands and feet and skeletal malformations. The facial muscle contracture produces the typical \u2018whistling face\u2019 appearance. Anaesthetic issues include difficult intravenous access, difficult airway and postoperative pulmonary complications. Although an association with malignant hyperthermia has been suggested, this has not been confirmed. We report the management of a seven-year-old girl with Freeman-Sheldon syndrome undergoing anterior and posterior spinal surgery and describe a successful anaesthetic regimen based on a total intravenous anaesthesia technique with remifentanil and propofol without neuromuscular blocking agents. The child had an uneventful anaesthetic and postoperative course. We believe the presence of the myopathy warranted the use of a \u2018non-triggering\u2019 anaesthetic, as suxamethonium and volatile agents may be associated with significant complications such as muscle rigidity and rhabdomyolysis in myopathic patients, even in the absence of malignant hyperthermia.<\/jats:p>","DOI":"10.1177\/0310057x0803600216","type":"journal-article","created":{"date-parts":[[2019,1,22]],"date-time":"2019-01-22T23:07:15Z","timestamp":1548198435000},"page":"249-253","source":"Crossref","is-referenced-by-count":7,"title":["Anaesthetic Management of a Child with Freeman-Sheldon Syndrome Undergoing Spinal Surgery"],"prefix":"10.1177","volume":"36","author":[{"given":"F. C.","family":"Richa","sequence":"first","affiliation":[{"name":"Department of Anaesthesia and Intensive Care, Saint-Joseph University, Hotel-Dieu de France Hospital, Beirut, Lebanon"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P. H.","family":"Yazbeck","sequence":"additional","affiliation":[{"name":"Department of Anaesthesia and Intensive Care, Saint-Joseph University, Hotel-Dieu de France Hospital, Beirut, Lebanon"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2008,3,1]]},"reference":[{"key":"bibr1-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1136\/adc.13.75.277"},{"key":"bibr2-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320110208"},{"key":"bibr3-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19961111)65:4<277::AID-AJMG6>3.0.CO;2-M"},{"key":"bibr4-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320550406"},{"key":"bibr5-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320250312"},{"key":"bibr6-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1007\/BF03010755"},{"key":"bibr7-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1046\/j.1460-9592.2003.01200.x"},{"key":"bibr8-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1046\/j.1460-9592.1998.00676.x"},{"key":"bibr9-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.23.3.231"},{"key":"bibr10-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2005-1219"},{"key":"bibr11-0310057X0803600216","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/S1695-4033(02)78951-0","volume":"56","author":"Alonso Calderon J.L.","year":"2002","journal-title":"An Esp Pediatr"},{"key":"bibr12-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1097\/00000542-198903000-00028"},{"key":"bibr13-0310057X0803600216","first-page":"1114","volume":"35","author":"Tateishi M.","year":"1986","journal-title":"Masui"},{"key":"bibr14-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.2004.01290.x"},{"key":"bibr15-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1097\/00000542-199209000-00031"},{"key":"bibr16-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1007\/BF03013916"},{"key":"bibr17-0310057X0803600216","doi-asserted-by":"publisher","DOI":"10.1046\/j.1460-9592.1997.d01-90.x"},{"key":"bibr18-0310057X0803600216","first-page":"659","volume":"51","author":"Okawa M.","year":"2002","journal-title":"Masui"},{"key":"bibr19-0310057X0803600216","first-page":"182","volume":"41","author":"Sobrado C.G.","year":"1994","journal-title":"Rev Esp Anestesiol Reanim"},{"key":"bibr20-0310057X0803600216","first-page":"1748","volume":"43","author":"Yamamoto S.","year":"1994","journal-title":"Masui"}],"container-title":["Anaesthesia and Intensive Care"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/0310057X0803600216","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/0310057X0803600216","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T09:06:56Z","timestamp":1777367216000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/0310057X0803600216"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,3]]},"references-count":20,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2008,3]]}},"alternative-id":["10.1177\/0310057X0803600216"],"URL":"https:\/\/doi.org\/10.1177\/0310057x0803600216","relation":{},"ISSN":["0310-057X","1448-0271"],"issn-type":[{"value":"0310-057X","type":"print"},{"value":"1448-0271","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,3]]}}}