{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T22:15:45Z","timestamp":1740176145618,"version":"3.37.3"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,6,11]],"date-time":"2020-06-11T00:00:00Z","timestamp":1591833600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,6,11]],"date-time":"2020-06-11T00:00:00Z","timestamp":1591833600000},"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"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Musculocontractural Ehlers-Danlos syndrome is a new and rare subtype of Ehlers-Danlos syndrome in which anesthetic considerations for airway and respiratory management, prevention of skin injuries and joint dislocations, and hemostatic management for severe perioperative bleeding are required.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>A 19-year-old woman with musculocontractural Ehlers-Danlos syndrome was scheduled to undergo posterior spinal fusion from the 4th thoracic to the 4th lumbar vertebrae under general anesthesia. Her trachea was easily intubated with a videolaryngoscope despite a small mouth and micrognathia. Pressure-controlled ventilation with limited peak inspiratory pressure was performed for prevention of pneumothorax. Skin damage and joint luxation were prevented by using a low rebounding mattress, terpolymer-based barrier film, and careful patient positioning. Blood transfusion was effectively performed on the basis of point-of-care viscoelastic hemostatic assay monitoring. She had an uneventful postoperative course without any complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We safely managed a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery.<\/jats:p><\/jats:sec>","DOI":"10.1186\/s40981-020-00352-5","type":"journal-article","created":{"date-parts":[[2020,6,11]],"date-time":"2020-06-11T14:03:03Z","timestamp":1591884183000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Anesthetic management of a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery"],"prefix":"10.1186","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6198-5806","authenticated-orcid":false,"given":"Ryo","family":"Wakabayashi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiko","family":"Tsuchiyama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsumi","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuki","family":"Maruyama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaori","family":"Numata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mikito","family":"Kawamata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,6,11]]},"reference":[{"key":"352_CR1","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1111\/ped.12878","volume":"58","author":"T Kosho","year":"2016","unstructured":"Kosho T. 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Spine (Phila Pa 1976). 2009;34:E153\u20137.","journal-title":"Spine (Phila Pa 1976)"},{"key":"352_CR4","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1007\/s00586-002-0507-6","volume":"12","author":"S Akpinar","year":"2003","unstructured":"Akpinar S, Gogus A, Talu U, Hamzaoglu A, Dikici F. Surgical management of the spinal deformity in Ehlers-Danlos syndrome type VI. Eur Spine J. 2003;12:135\u201340.","journal-title":"Eur Spine J"},{"key":"352_CR5","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1515\/folmed-2017-0100","volume":"60","author":"L Katranitsa","year":"2018","unstructured":"Katranitsa L, Gkantsinikoudis N, Kapetanakis S, Charitoudis G, Christodoulou A, Givissis P. Perioperative blood management in posterior instrumented fusion for adolescent idiopathic scoliosis: original study and short review of the literature. Folia Med (Plovdiv). 2018;60:200\u20137.","journal-title":"Folia Med (Plovdiv)"},{"key":"352_CR6","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1186\/s13023-014-0109-5","volume":"9","author":"T Wiesmann","year":"2014","unstructured":"Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis. 2014;9:109.","journal-title":"Orphanet J Rare Dis"},{"key":"352_CR7","doi-asserted-by":"publisher","first-page":"1366","DOI":"10.1213\/ane.0b013e318168b367","volume":"106","author":"MT Ganter","year":"2008","unstructured":"Ganter MT, Hofer CK. Coagulation monitoring: current techniques and clinical use of viscoelastic point-of-care coagulation devices. Anesth Analg. 2008;106:1366\u201375.","journal-title":"Anesth Analg"},{"key":"352_CR8","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3310\/hta19580","volume":"19","author":"P Whiting","year":"2015","unstructured":"Whiting P, Al M, Westwood M, Ramos IC, Ryder S, Armstrong N, et al. Viscoelastic point-of-care testing to assist with the diagnosis, management and monitoring of haemostasis: a systematic review and cost-effectiveness analysis. Health Technol Assess. 2015;19:1\u2013228 v\u2013vi.","journal-title":"Health Technol Assess"},{"key":"352_CR9","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1055\/s-0038-1661458","volume":"55","author":"M K\u00f6hler","year":"1986","unstructured":"K\u00f6hler M, Hellstern P, Miyashita C, von Blohn G, Wenzel E. Comparative study of intranasal, subcutaneous and intravenous administration of desamino-D-arginine vasopressin (DDAVP). Thromb Haemost. 1986;55:108\u201311.","journal-title":"Thromb Haemost"},{"key":"352_CR10","doi-asserted-by":"crossref","first-page":"1333","DOI":"10.1002\/ajmg.a.33498","volume":"152a","author":"T Kosho","year":"2010","unstructured":"Kosho T, Miyake N, Hatamochi A, Takahashi J, Kato H, Miyahara T, et al. A new Ehlers-Danlos syndrome with craniofacial characteristics, multiple congenital contractures, progressive joint and skin laxity, and multisystem fragility-related manifestations. Am J Med Genet A. 2010;152a:1333\u201346.","journal-title":"Am J Med Genet A"},{"key":"352_CR11","doi-asserted-by":"publisher","first-page":"332","DOI":"10.1097\/EJA.0000000000000630","volume":"34","author":"SA Kozek-Langenecker","year":"2017","unstructured":"Kozek-Langenecker SA, Ahmed AB, Afshari A, Albaladejo P, Aldecoa C, Barauskas G, et al. Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology: first update 2016. 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