{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,29]],"date-time":"2025-08-29T09:43:12Z","timestamp":1756460592139,"version":"3.37.3"},"reference-count":16,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,12,8]],"date-time":"2020-12-08T00:00:00Z","timestamp":1607385600000},"content-version":"vor","delay-in-days":7,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["surg case rep"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Ehlers\u2013Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications.<\/jats:p>\n                <jats:p>Case presentation<\/jats:p>\n                <jats:p>Here, we present a case of a spontaneous sigmoid perforation in a 14-year-old boy. He was initially treated with laparotomy, oversew of the sigmoid perforation and a diverting ileostomy. He developed a complete wound dehiscence and enteroatmospheric fistulae. These were managed with a combination of negative pressure wound therapy and Eakin (TG Eakin\u2122) pouch changes. We discuss the clinical features and presentation of EDS type IV, the surgical implications of managing patients with the condition, and the challenges in management of enteroatmospheric fistulae in children.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Ehlers\u2013Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s40792-020-01051-0","type":"journal-article","created":{"date-parts":[[2020,12,8]],"date-time":"2020-12-08T10:03:43Z","timestamp":1607421823000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Ehlers\u2013Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child"],"prefix":"10.1186","volume":"6","author":[{"given":"Hemal","family":"Kodikara","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5396-0265","authenticated-orcid":false,"given":"Sebastian K.","family":"King","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth","family":"McLeod","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,12,8]]},"reference":[{"issue":"3","key":"1051_CR1","doi-asserted-by":"publisher","first-page":"193","DOI":"10.5223\/pghn.2016.19.3.193","volume":"19","author":"SH Kim","year":"2016","unstructured":"Kim SH, Cho YH, Kim HY. Spontaneous perforation of colon in previously healthy infants and children: its clinical implication. Pediatr Gastroenterol Hepatol Nutr. 2016;19(3):193\u20138.","journal-title":"Pediatr Gastroenterol Hepatol Nutr"},{"issue":"32","key":"1051_CR2","first-page":"1750","volume":"2","author":"D Germain","year":"2007","unstructured":"Germain D. Ehlers-Danlos syndrome type IV. Orphanet J Rare Dis. 2007;2(32):1750.","journal-title":"Orphanet J Rare Dis"},{"issue":"10","key":"1051_CR3","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1056\/NEJM200003093421001","volume":"342","author":"M Pepin","year":"2000","unstructured":"Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342(10):673\u201380.","journal-title":"N Engl J Med"},{"key":"1051_CR4","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1097\/00005373-200108000-00029","volume":"51","author":"SB Maltz","year":"2001","unstructured":"Maltz SB, Fantus RJ, Mellett MM, et al. Surgical complications of Ehlers-Danlos syndrome type IV: case report and review of the literature. J Trauma. 2001;51:387\u201390.","journal-title":"J Trauma"},{"key":"1051_CR5","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1002\/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O","volume":"77","author":"P Beighton","year":"1998","unstructured":"Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. 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The all too often missed diagnosis of Ehlers-Danlos syndrome. Am Surg. 2013;79:608\u201313.","journal-title":"Am Surg"},{"issue":"8","key":"1051_CR11","doi-asserted-by":"publisher","first-page":"1353","DOI":"10.1007\/s00423-012-0923-y","volume":"397","author":"IM Gutierrez","year":"2012","unstructured":"Gutierrez IM, Gollin G. Negative pressure wound therapy for children with an open abdomen. Langenbecks Arch Surg. 2012;397(8):1353\u20137.","journal-title":"Langenbecks Arch Surg"},{"issue":"8","key":"1051_CR12","doi-asserted-by":"publisher","first-page":"1555","DOI":"10.1016\/j.jpedsurg.2012.01.014","volume":"47","author":"AP Stoffan","year":"2012","unstructured":"Stoffan AP, Ricca R, Lien C, Quigley S, Linden BC. Use of negative pressure wound therapy for abdominal wounds in neonates and infants. 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