{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T16:55:59Z","timestamp":1791910559531,"version":"4.3.4"},"reference-count":36,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2024,7,12]],"date-time":"2024-07-12T00:00:00Z","timestamp":1720742400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background and Objectives: Spontaneous colonic perforations (SCPs) in teenagers and young adults are extremely rare. Common underlying conditions, such as colonic tumors and diverticulitis, are absent at that age. The vascular type of Ehlers\u2013Danlos Syndrome (vEDS) is one cause of SCP. Methods: A 23-year-old male presented with an acute abdomen. The abdominal CT showed pneumoperitoneum with a large amount of fluid in the pelvis and abdomen, indicating hollow viscus rupture. At the level of the sigmoid colon, a defect in the intestinal wall and gas bubbles were seen. Results: Exploratory laparotomy confirmed sigmoid colon perforation without underlying pathology. Loop sigmoid colostomy was performed. Revisional surgery was undertaken due to clinical deterioration and intra-abdominal free fluid with small-bowel distension and air-liquid levels on abdominal CT 6 days later. Ileal subserosal hematomas were found, and many had ruptured, leaving a \u201czebra\u201d pattern with lines of residual hematomas on the borders of subserosal hematomas. Genetic analysis confirmed vEDS. Conclusions: SCP in young adults or teenagers, in the absence of colonic disease, with clinical manifestations of connective tissue disorders should trigger genetic investigations for vEDS. SCP with a known vEDS could be treated with total colectomy to prevent further SCPs in the remaining colon. If segmental resections are performed, further SCP should be immediately excluded with any significant abdominal pain.<\/jats:p>","DOI":"10.3390\/jcm13144093","type":"journal-article","created":{"date-parts":[[2024,7,12]],"date-time":"2024-07-12T16:23:41Z","timestamp":1720801421000},"page":"4093","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Spontaneous Sigmoid Colon Perforation and Ruptured Subserosal (\u201cZebra\u201d Pattern) Small-Bowel Hematomas in Type IV Ehlers\u2013Danlos Syndrome: A Case Report and a Short Review"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0202-3324","authenticated-orcid":false,"given":"Goran","family":"Augustin","sequence":"first","affiliation":[{"name":"Department of Surgery, University Hospital Centre Zagreb, Ki\u0161pati\u0107eva 12, 10000 Zagreb, Croatia"},{"name":"School of Medicine, University of Zagreb, 10000 Zagreb, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iva","family":"Radin","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Zagreb, 10000 Zagreb, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomislav","family":"Bubalo","sequence":"additional","affiliation":[{"name":"Department of Surgery, University Hospital Centre Zagreb, Ki\u0161pati\u0107eva 12, 10000 Zagreb, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josip","family":"Mavrek","sequence":"additional","affiliation":[{"name":"Department of Surgery, University Hospital Centre Zagreb, Ki\u0161pati\u0107eva 12, 10000 Zagreb, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Goran","family":"Pavlek","sequence":"additional","affiliation":[{"name":"Department of Surgery, University Hospital Centre Zagreb, Ki\u0161pati\u0107eva 12, 10000 Zagreb, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,7,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1186\/s12957-017-1228-y","article-title":"Outcome of colon cancer initially presenting as colon perforation and obstruction","volume":"15","author":"Chen","year":"2017","journal-title":"World J. Surg. Oncol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1007\/BF02587541","article-title":"Diverticulitis of the colon","volume":"17","author":"Gennaro","year":"1974","journal-title":"Dis. Colon Rectum"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1097\/00000658-197701000-00010","article-title":"Toothpick Perforation of the Intestines","volume":"185","author":"Schwartz","year":"1977","journal-title":"Ann. Surg."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"e7862","DOI":"10.1002\/ccr3.7862","article-title":"Colon perforation due to collagenous colitis: A case report","volume":"11","author":"Ito","year":"2023","journal-title":"Clin. Case Rep."},{"key":"ref_5","first-page":"301","article-title":"Dilatation and rupture of the sigmoid flexure [short report]","volume":"1","author":"Berry","year":"1894","journal-title":"BMJ"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1002\/ajmg.c.31547","article-title":"The international consortium on the Ehlers\u2013Danlos syndromes","volume":"175","author":"Bloom","year":"2017","journal-title":"Am. J. Med. Genet. Part C Semin. Med. Genet."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1002\/ajmg.c.31553","article-title":"Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome","volume":"175","author":"Byers","year":"2017","journal-title":"Am. J. Med. Genet. Part C Semin. Med. Genet."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1016\/S0002-9343(01)01131-7","article-title":"Gastrointestinal involvement in polyarteritis nodosa (1986\u20132000): Presentation and outcomes in 24 patients","volume":"112","author":"Levine","year":"2002","journal-title":"Am. J. Med."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1097\/01.md.0000158825.87055.0b","article-title":"Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: Analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis","volume":"84","author":"Pagnoux","year":"2005","journal-title":"Medicine"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.jvs.2005.03.053","article-title":"The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: A 30-year experience","volume":"42","author":"Oderich","year":"2005","journal-title":"J. Vasc. Surg."},{"key":"ref_11","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":"ref_12","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1007\/s00595-004-2817-2","article-title":"Churg-Strauss syndrome manifesting as perforation of the small intestine: Report of a case","volume":"34","author":"Murakami","year":"2004","journal-title":"Surg. Today"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1097\/00005792-197509000-00004","article-title":"Colonic perforations in systemic lupus erythematosus","volume":"54","author":"Zizic","year":"1975","journal-title":"Medicine"},{"key":"ref_14","first-page":"A61","article-title":"FRI0110 Bowel perforation in systemic lupus erithematosus","volume":"60","author":"Pombo","year":"2001","journal-title":"Ann. Rheum. Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e34415","DOI":"10.1097\/MD.0000000000034415","article-title":"Intestinal perforation with systemic lupus erythematosus: A systematic review","volume":"102","author":"Chen","year":"2023","journal-title":"Medicine"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1002\/art.30107","article-title":"The incidence of gastrointestinal perforations among rheumatoid arthritis patients","volume":"63","author":"Curtis","year":"2011","journal-title":"Arthritis Rheum."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1770","DOI":"10.1093\/rheumatology\/keq147","article-title":"Assessment of gastrointestinal symptoms in patients with systemic sclerosis in a UK tertiary referral centre","volume":"49","author":"Thoua","year":"2010","journal-title":"Rheumatology"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1136\/pgmj.58.676.123","article-title":"Small intestinal perforation in Wegener\u2019s granulomatosis","volume":"58","author":"McNabb","year":"1982","journal-title":"Postgrad Med. J."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1136\/gut.27.4.450","article-title":"Intestinal perforation in Wegener\u2019s granulomatosis","volume":"27","author":"Geraghty","year":"1986","journal-title":"Gut"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1136\/pgmj.48.555.56","article-title":"Spontaneous bowel perforation in intestinal scleroderma: First report of a non-fatal case","volume":"48","author":"Jayson","year":"1972","journal-title":"Postgrad. Med. J."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1007\/BF01297244","article-title":"Spontaneous perforation of the small intestine due to scleroderma","volume":"24","author":"Battle","year":"1979","journal-title":"Dig Dis Sci."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1023\/A:1018847126143","article-title":"Intestinal perforation. A common complication of scleroderma","volume":"42","author":"Ebert","year":"1997","journal-title":"Dig. Dis. Sci."},{"key":"ref_23","first-page":"5124145","article-title":"When the Benign Pneumatosis Intestinalis Becomes No Longer Benign: A Rare Case of Bowel Perforation in a Patient with Systemic Sclerosis","volume":"2018","author":"Guan","year":"2018","journal-title":"Case Rep. Gastrointest Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"e233220","DOI":"10.1136\/bcr-2019-233220","article-title":"Spontaneous bowel perforation in the setting of colonic involvement with scleroderma","volume":"13","author":"Stupalkowska","year":"2020","journal-title":"BMJ Case Rep."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1097\/DCR.0000000000001383","article-title":"Natural History and Surgical Management of Colonic Perforations in Vascular Ehlers-Danlos Syndrome: A Retrospective Review","volume":"62","author":"Adham","year":"2019","journal-title":"Dis. Colon Rectum"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"102948","DOI":"10.1016\/j.amsu.2021.102948","article-title":"Total colectomy in Vascular Ehlers Danlos syndrome a case report and literature review","volume":"71","author":"Elhattabi","year":"2021","journal-title":"Ann. Med. Surg."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1186\/s40792-023-01643-6","article-title":"Repeated intestinal perforations in vascular Ehlers-Danlos syndrome: A case report of a novel mutation in the COL3A1 gene","volume":"9","author":"Horino","year":"2023","journal-title":"Surg. Case Rep."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1016\/0002-9343(62)90241-3","article-title":"Functional and morphologic alterations of the gastrointestinal tract in progressive systemic sclerosis (scleroderma)","volume":"33","author":"Hoskins","year":"1962","journal-title":"Am. J. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1002\/acr2.11387","article-title":"Disease Features and Gastrointestinal Microbial Composition in Patients with Systemic Sclerosis from Two Independent Cohorts","volume":"4","author":"Lee","year":"2022","journal-title":"ACR Open Rheumatol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/j.ejvs.2020.10.020","article-title":"Celiprolol Treatment in Patients with Vascular Ehlers-Danlos Syndrome","volume":"61","author":"Baderkhan","year":"2021","journal-title":"Eur. J. Vasc. Endovasc. Surg."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"494","DOI":"10.1097\/HCO.0b013e32834ad55a","article-title":"Contemporary management of vascular Ehlers\u2013Danlos syndrome","volume":"26","author":"Lum","year":"2011","journal-title":"Curr. Opin. Cardiol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1111\/codi.14749","article-title":"Management of colonic complications of type IV Ehlers\u2013Danlos syndrome: A systematic review and evidence-based management strategy","volume":"22","author":"Speake","year":"2020","journal-title":"Color. Dis."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1007\/s10151-018-1783-4","article-title":"Bowel perforation in type IV vascular Ehlers-Danlos syndrome. A systematic review","volume":"22","author":"Loong","year":"2018","journal-title":"Tech. Coloproctol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"5852438","DOI":"10.1155\/2019\/5852438","article-title":"Colorectal Perforation in Patients with Connective Tissue Disease","volume":"2019","author":"Sugimoto","year":"2019","journal-title":"Emerg. Med. Int."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1001\/archsurg.137.3.306","article-title":"Spontaneous intramural small-bowel hematoma: Clinical presentation and long-term outcome","volume":"137","author":"Abbas","year":"2002","journal-title":"Arch. Surg."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1530","DOI":"10.1161\/01.CIR.0000121741.50315.C2","article-title":"Increased Carotid Wall Stress in Vascular Ehlers-Danlos Syndrome","volume":"109","author":"Boutouyrie","year":"2004","journal-title":"Circulation"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/14\/4093\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T15:16:10Z","timestamp":1760109370000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/14\/4093"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,12]]},"references-count":36,"journal-issue":{"issue":"14","published-online":{"date-parts":[[2024,7]]}},"alternative-id":["jcm13144093"],"URL":"https:\/\/doi.org\/10.3390\/jcm13144093","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,7,12]]}}}