{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T09:28:50Z","timestamp":1776763730895,"version":"3.51.2"},"reference-count":10,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2022,5,4]],"date-time":"2022-05-04T00:00:00Z","timestamp":1651622400000},"content-version":"vor","delay-in-days":33,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Isolated small bowel perforation is low in the differential diagnosis of abdominal pain in the young, relatively healthy patient. It is, however, a rare manifestation of type IV (vascular) Ehlers-Danlos syndrome (EDS). In addition, there is no general consensus on the management of GI manifestations in patients with type IV EDS. We present the case of a 31-year-old male with history of type IV EDS, presenting with acute onset abdominal pain. Imaging was notable for intra-abdominal free air and thickened loops of small bowel in the pelvis. The patient underwent exploratory laparotomy with resection of the small bowel perforation with enteroenteric anastomosis. In our literature review, we evaluated gastrointestinal manifestations observed in patients with type IV EDS, management recommendations and potential complications to be mindful of in this population.<\/jats:p>","DOI":"10.1093\/jscr\/rjac192","type":"journal-article","created":{"date-parts":[[2022,4,14]],"date-time":"2022-04-14T11:13:12Z","timestamp":1649934792000},"source":"Crossref","is-referenced-by-count":4,"title":["Spontaneous small bowel perforation: a rare GI manifestation of type IV Ehlers-Danlos syndrome"],"prefix":"10.1093","volume":"2022","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4956-7353","authenticated-orcid":false,"given":"Alexandria M","family":"Gallagher","sequence":"first","affiliation":[{"name":"Department of General Surgery, Geisinger Wyoming Valley Hospital, Wilkes-Barre, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tara L","family":"McGraw","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Geisinger Wyoming Valley Hospital, Wilkes-Barre, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fredrick K","family":"Toy","sequence":"additional","affiliation":[{"name":"Department of Trauma and Emergency General Surgery, Geisinger Wyoming Valley Hospital, Wilkes-Barre, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,4,30]]},"reference":[{"key":"2022050520083211300_ref1","doi-asserted-by":"crossref","DOI":"10.1186\/1750-1172-2-32","article-title":"Ehlers-Danlos syndrome type IV","volume":"2","author":"Germain","year":"2007","journal-title":"Orphanet J Rare Dis"},{"key":"2022050520083211300_ref2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.anngen.2003.07.002","article-title":"Vascular Ehlers-Danlos syndrome","volume":"47","author":"Germain","year":"2004","journal-title":"Ann Genet"},{"key":"2022050520083211300_ref3","first-page":"173","article-title":"Cutis laxa, neigung zu haemorrhagien in der haut, lockerung mehrerer artikulationen","volume":"8","author":"Ehlers","year":"1901","journal-title":"Derm Zschr"},{"key":"2022050520083211300_ref4","first-page":"70","article-title":"Un cas de cutis laxa avec tumerurs par contusion chronique des coudes et des genoux (xanthome juvenile pseudo-diabetique de MM Hallopeau et Marc de Lepinay)","volume":"19","author":"Danlos","year":"1908","journal-title":"Bull Soc Franc Derm Syph"},{"key":"2022050520083211300_ref5","first-page":"1","article-title":"Ehlers-Danlos syndromes: revised nosology, Villefranche","volume":"1998","author":"Beighton","year":"1997","journal-title":"Am J Med Genet"},{"key":"2022050520083211300_ref6","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1056\/NEJM200003093421001","article-title":"Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type","volume":"342","author":"Pepin","year":"2000","journal-title":"N Engl J Med"},{"key":"2022050520083211300_ref7","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1159\/000343738","article-title":"Gastrointestinal surgery and related complications in patients with Ehlers-Danlos syndrome: a systematic review","volume":"29","author":"Buncharth","year":"2012","journal-title":"Dig Surg"},{"key":"2022050520083211300_ref8","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1016\/j.apmr.2011.01.016","article-title":"Medication, surgery, and physiotherapy among patients with the hypermobility type of Ehlers-Danlos syndrome","volume":"92","author":"Rombaut","year":"2011","journal-title":"Arch Phys Med Rehabil"},{"key":"2022050520083211300_ref9","first-page":"869","article-title":"The surgical complications of Ehlers-Danlos syndrome","volume":"62","author":"Freeman","year":"1996","journal-title":"Am Surg"},{"key":"2022050520083211300_ref10","doi-asserted-by":"crossref","first-page":"1038","DOI":"10.1007\/BF02049321","article-title":"Surgical pitfalls in a patient with type IV Ehlers-Danlos syndrome and spontaneous colonic rupture. Report of a case","volume":"37","author":"Berney","year":"1994","journal-title":"Dis Colon Rectum"}],"container-title":["Journal of Surgical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2022\/4\/rjac192\/43520912\/rjac192.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2022\/4\/rjac192\/43520912\/rjac192.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,5,5]],"date-time":"2022-05-05T20:40:05Z","timestamp":1651783205000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jscr\/article\/doi\/10.1093\/jscr\/rjac192\/6577154"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,1]]},"references-count":10,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,4,1]]}},"URL":"https:\/\/doi.org\/10.1093\/jscr\/rjac192","relation":{},"ISSN":["2042-8812"],"issn-type":[{"value":"2042-8812","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2022,4]]},"published":{"date-parts":[[2022,4,1]]},"article-number":"rjac192"}}