{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T01:06:12Z","timestamp":1759971972790,"version":"build-2065373602"},"reference-count":9,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,1,9]],"date-time":"2023-01-09T00:00:00Z","timestamp":1673222400000},"content-version":"vor","delay-in-days":8,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["associationofanaesthetists-publications.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Anaesthesia Reports"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>We present the case of a 41\u2010year\u2010old man with Rapp\u2013Hodgkin syndrome who underwent nasal septum deviation surgery under general anaesthesia. This syndrome is rare, with approximately 70 cases reported worldwide. It is one of a group of ectodermal dysplasia syndromes and results from the aberrant development of ectoderm during fetal development. Some of the clinical features may affect anaesthetic management. The most important considerations are potentially difficult airway management, the need for meticulous temperature control, and the importance of skin protection. This case was uneventful, but as there are few case reports on the management of patients with ectodermal dysplasia syndromes undergoing anaesthesia this report contributes useful knowledge. The pathogenesis and clinical features of Rapp\u2013Hodgkin syndrome and the anaesthetic management for this patient are described.<\/jats:p>","DOI":"10.1002\/anr3.12210","type":"journal-article","created":{"date-parts":[[2023,1,10]],"date-time":"2023-01-10T00:49:21Z","timestamp":1673311761000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Peri\u2010operative management of a patient with an ectodermal dysplasia (Rapp\u2013Hodgkin) syndrome"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2289-2516","authenticated-orcid":false,"given":"J.","family":"Marialva","sequence":"first","affiliation":[{"name":"Department of Anesthesiology Centro Hospitalar T\u00e2mega e Sousa  Penafiel Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4849-0753","authenticated-orcid":false,"given":"L.","family":"Lopes","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Centro Hospitalar T\u00e2mega e Sousa  Penafiel Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8770-5270","authenticated-orcid":false,"given":"F.","family":"Moura","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Centro Hospitalar T\u00e2mega e Sousa  Penafiel Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8134-4857","authenticated-orcid":false,"given":"H.","family":"Cardoso","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Centro Hospitalar T\u00e2mega e Sousa  Penafiel Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4337-5761","authenticated-orcid":false,"given":"L.","family":"Cruz","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Centro Hospitalar T\u00e2mega e Sousa  Penafiel Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,1,9]]},"reference":[{"key":"e_1_2_5_2_1","unstructured":"AnsariA PillarisettyLS.Embryology ectoderm. StatPearls [Internet].2022.https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK539836\/(accessed November 27 2022)."},{"key":"e_1_2_5_3_1","first-page":"2372","volume-title":"Fitzpatrick's Dermatology","author":"Nieman EL","year":"2019"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2010.09859.x"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2005.05.038"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2020.01387"},{"key":"e_1_2_5_7_1","unstructured":"NganV WriterS.Ectodermal dysplasia. DermNet 2005.https:\/\/dermnetnz.org\/topics\/ectodermal\u2010dysplasia#:~:text=Ectodermal%20dysplasia%20is%20a%20large and%20the%20central%20nervous%20system(accessed December 16 2022)."},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/ALN.0000000000004002"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.atc.2006.09.001"},{"key":"e_1_2_5_10_1","unstructured":"National Institute for Health and Care Excellence.Hypothermia: prevention and management in adults having surgery (clinical guideline CG65). NICE Clinical Guidelines 2008.https:\/\/www.nice.org.uk\/guidance\/cg65\/chapter\/Recommendations#perioperative\u2010care(accessed October 30 2022)."}],"container-title":["Anaesthesia Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/anr3.12210","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/anr3.12210","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/associationofanaesthetists-publications.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/anr3.12210","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T18:04:11Z","timestamp":1759946651000},"score":1,"resource":{"primary":{"URL":"https:\/\/associationofanaesthetists-publications.onlinelibrary.wiley.com\/doi\/10.1002\/anr3.12210"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1]]},"references-count":9,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2023,1]]}},"alternative-id":["10.1002\/anr3.12210"],"URL":"https:\/\/doi.org\/10.1002\/anr3.12210","archive":["Portico"],"relation":{},"ISSN":["2637-3726","2637-3726"],"issn-type":[{"type":"print","value":"2637-3726"},{"type":"electronic","value":"2637-3726"}],"subject":[],"published":{"date-parts":[[2023,1]]},"assertion":[{"value":"2022-12-22","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-01-09","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e12210"}}