{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,25]],"date-time":"2026-07-25T03:24:02Z","timestamp":1784949842111,"version":"3.55.0"},"reference-count":40,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["JAMA Facial Plast Surg"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:sec>\n                    <jats:title>Importance<\/jats:title>\n                    <jats:p>Ehlers-Danlos syndromes (EDSs) are a group of heritable connective tissue disorders. Patients with EDSs can develop excessive facial rhytids, nasal deformities, and facial scarring, for which they may seek consultation with a facial plastic surgeon. Ehlers-Danlos syndromes can be associated with serious surgical complications and should be identified preoperatively to facilitate optimal treatment. To our knowledge, no management guidelines for patients with EDSs exist in the facial plastic surgery literature. We present a review of the literature and management recommendations for the facial plastic surgeon.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Observations<\/jats:title>\n                    <jats:p>We performed a PubMed\/MEDLINE search for all publications in the English language related to surgical experience in patients with EDSs. A total of 37 publications (including reviews and case series) were included. Ehlers-Danlos syndromes are more common than appreciated, with an overall point prevalence between 1 in 2500 and 1 in 5000 according to existing literature. There are 6 main subtypes of EDSs that have significant phenotypic heterogeneity. Patients with classic type and hypermobile EDS (the 2 most common subtypes) can typically undergo elective facial plastic surgery without major complications. However, specific preoperative, intraoperative, and postoperative issues, as outlined in this review, must be taken into consideration. Vascular EDS should serve as a contraindication to elective surgery given its potential for life-threatening complications.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions and Relevance<\/jats:title>\n                    <jats:p>Because of their skin laxity, patients with EDSs may present more frequently to facial plastic surgeons\u2019 offices. Simple screening questions can help the surgeon identify potential patients with EDSs. Treatment should be individualized and performed with extreme care to ensure optimal outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1001\/jamafacial.2017.0793","type":"journal-article","created":{"date-parts":[[2017,11,9]],"date-time":"2017-11-09T12:31:38Z","timestamp":1510230698000},"page":"70-75","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":43,"title":["Characteristics, Diagnosis, and Management of Ehlers-Danlos Syndromes"],"prefix":"10.1177","volume":"20","author":[{"given":"Andrew W.","family":"Joseph","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon S.","family":"Joseph","sequence":"additional","affiliation":[{"name":"Division of Oculoplastic Surgery, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clair A.","family":"Francomano","sequence":"additional","affiliation":[{"name":"Harvey Institute for Human Genetics, Greater Baltimore Medical Center, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theda C.","family":"Kontis","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland"},{"name":"Facial Plastic Surgicenter, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2018,1,18]]},"reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1002\/0471221929.ch9","volume-title":"Connective Tissue and Its Heritable Disorders","author":"Steinmann B","year":"2002","unstructured":"Steinmann B, , Royce PM, , Superti-Furga A. The Ehlers-Danlos syndrome. In: Royce PM, , Steinmann B, eds. Connective Tissue and Its Heritable Disorders. Hoboken, NJ: John Wiley & Sons, Inc; 2002:431-523, http:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/0471221929.ch9\/summary. Accessed November 2, 2016."},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/bjs.1800560404"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10029-010-0721-9"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.12968\/jowc.2012.21.5.223"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.prs.0000232417.05081.db"},{"issue":"6","key":"e_1_3_1_7_2","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1016\/S0196-0709(96)90081-2","article-title":"Ehlers-Danlos syndrome: aural manifestations and treatment","volume":"17","author":"Thomas DM","year":"1996","unstructured":"Thomas DM, , Wright JL, , Soucek S, , Shalom AS. Ehlers-Danlos syndrome: aural manifestations and treatment. 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