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Ehlers–Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child
Background Ehlers–Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications. Case presentation 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 d...
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| Published in: | Surgical case reports 2020-12, Vol.6 (1), p.312-312, Article 312 |
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| container_title | Surgical case reports |
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| creator | Kodikara, Hemal King, Sebastian K. McLeod, Elizabeth |
| description | Background
Ehlers–Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications.
Case presentation
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™) 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.
Conclusions
Ehlers–Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children. |
| doi_str_mv | 10.1186/s40792-020-01051-0 |
| format | article |
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Ehlers–Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications.
Case presentation
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™) 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.
Conclusions
Ehlers–Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children.</description><identifier>ISSN: 2198-7793</identifier><identifier>EISSN: 2198-7793</identifier><identifier>DOI: 10.1186/s40792-020-01051-0</identifier><identifier>PMID: 33289853</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Case Report ; Case reports ; Ehlers–Danlos syndrome type IV ; Enteroatmospheric fistula ; Medicine ; Medicine & Public Health ; Negative pressure wound therapy ; Ostomy ; Paediatric ; Sigmoid perforation ; Surgery</subject><ispartof>Surgical case reports, 2020-12, Vol.6 (1), p.312-312, Article 312</ispartof><rights>The Author(s) 2020</rights><rights>The Author(s) 2020. This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c533t-f18982910a2bbceb1ae01befc95d2733073dfedfd3bea56010f7bfbbbaf8e9d23</citedby><cites>FETCH-LOGICAL-c533t-f18982910a2bbceb1ae01befc95d2733073dfedfd3bea56010f7bfbbbaf8e9d23</cites><orcidid>0000-0001-5396-0265</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7724010/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.proquest.com/docview/2473377695?pq-origsite=primo$$EHTML$$P50$$Gproquest$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,881,25731,27901,27902,36989,36990,44566,53766,53768</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33289853$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kodikara, Hemal</creatorcontrib><creatorcontrib>King, Sebastian K.</creatorcontrib><creatorcontrib>McLeod, Elizabeth</creatorcontrib><title>Ehlers–Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child</title><title>Surgical case reports</title><addtitle>surg case rep</addtitle><addtitle>Surg Case Rep</addtitle><description>Background
Ehlers–Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications.
Case presentation
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™) 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.
Conclusions
Ehlers–Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children.</description><subject>Case Report</subject><subject>Case reports</subject><subject>Ehlers–Danlos syndrome type IV</subject><subject>Enteroatmospheric fistula</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Negative pressure wound therapy</subject><subject>Ostomy</subject><subject>Paediatric</subject><subject>Sigmoid perforation</subject><subject>Surgery</subject><issn>2198-7793</issn><issn>2198-7793</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><sourceid>PIMPY</sourceid><sourceid>DOA</sourceid><recordid>eNp9Ustu1DAUjRCIVqU_wAJZYsMm4EcydlggoVJgpEpsgK3lx_WMR4kd7KTS7PgAdvxhv6SeppSWBSvb5x6fe-7VqarnBL8mRKze5AbzjtaY4hoT3JIaP6qOKelEzXnHHt-7H1WnOe8wxqSlQnTkaXXEGBWdaNlx9et820PKVz9_f1ChjxnlfbApDoCm_Qho_f0tUsioDCjBGNOEoitAUgkKOhe4vPMYw6QCxLl895sheotGSC4mNfkYkAoWQZggRTUNMY9bSN4g5_M09wqQD4cWW9_bZ9UTp_oMp7fnSfXt4_nXs8_1xZdP67P3F7VpGZtqR4p52hGsqNYGNFGAiQZnutZSzhjmzDqwzjINql2V9TiundZaOQGdpeykWi-6NqqdHJMfVNrLqLy8AWLaSJUmb3qQ2qimxUIR4lyzElwAaFFW3zjGFDdN0Xq3aI2zHsCaMmhS_QPRh5Xgt3ITLyXntCnWisCrW4EUf8yQJzn4bKDvl41KWtoy1ja4K9SX_1B3cU6hrKqwyuCcr7q2sOjCMinmnMDdmSFYHrIjl-zIkh15kx15cPHi_hh3X_4kpRDYQsilFDaQ_vb-j-w1CanTxw</recordid><startdate>20201208</startdate><enddate>20201208</enddate><creator>Kodikara, Hemal</creator><creator>King, Sebastian K.</creator><creator>McLeod, Elizabeth</creator><general>Springer Berlin Heidelberg</general><general>Springer Nature B.V</general><general>SpringerOpen</general><scope>C6C</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope><orcidid>https://orcid.org/0000-0001-5396-0265</orcidid></search><sort><creationdate>20201208</creationdate><title>Ehlers–Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child</title><author>Kodikara, Hemal ; King, Sebastian K. ; McLeod, Elizabeth</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c533t-f18982910a2bbceb1ae01befc95d2733073dfedfd3bea56010f7bfbbbaf8e9d23</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Case Report</topic><topic>Case reports</topic><topic>Ehlers–Danlos syndrome type IV</topic><topic>Enteroatmospheric fistula</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Negative pressure wound therapy</topic><topic>Ostomy</topic><topic>Paediatric</topic><topic>Sigmoid perforation</topic><topic>Surgery</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kodikara, Hemal</creatorcontrib><creatorcontrib>King, Sebastian K.</creatorcontrib><creatorcontrib>McLeod, Elizabeth</creatorcontrib><collection>SpringerOpen</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Publicly Available Content Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>Surgical case reports</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kodikara, Hemal</au><au>King, Sebastian K.</au><au>McLeod, Elizabeth</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Ehlers–Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child</atitle><jtitle>Surgical case reports</jtitle><stitle>surg case rep</stitle><addtitle>Surg Case Rep</addtitle><date>2020-12-08</date><risdate>2020</risdate><volume>6</volume><issue>1</issue><spage>312</spage><epage>312</epage><pages>312-312</pages><artnum>312</artnum><issn>2198-7793</issn><eissn>2198-7793</eissn><abstract>Background
Ehlers–Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications.
Case presentation
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™) 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.
Conclusions
Ehlers–Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>33289853</pmid><doi>10.1186/s40792-020-01051-0</doi><tpages>1</tpages><orcidid>https://orcid.org/0000-0001-5396-0265</orcidid><oa>free_for_read</oa></addata></record> |
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| ispartof | Surgical case reports, 2020-12, Vol.6 (1), p.312-312, Article 312 |
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| source | Publicly Available Content Database; Springer Nature - SpringerLink Journals - Fully Open Access; PubMed Central |
| subjects | Case Report Case reports Ehlers–Danlos syndrome type IV Enteroatmospheric fistula Medicine Medicine & Public Health Negative pressure wound therapy Ostomy Paediatric Sigmoid perforation Surgery |
| title | Ehlers–Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child |
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