Techniques in Coloproctology

, Volume 22, Issue 5, pp 333–341 | Cite as

Bowel perforation in type IV vascular Ehlers–Danlos syndrome. A systematic review

  • H. El Masri
  • T.-H. Loong
  • G. Meurette
  • J. Podevin
  • F. Zinzindohoue
  • P.-A. Lehur


Spontaneous gastrointestinal (GI) perforation is a well-known complication occurring in patients suffering from Type IV vascular Ehlers–Danlos syndrome (EDS IV). The aim of the present study was to review the current literature on spontaneous GI perforation in EDS IV and illustrate the surgical management and outcome when possible. A systematic review of all the published data on EDS IV patients with spontaneous GI perforation between January 2000 and December 2015 was conducted using three major databases PUBMED, EMBASE, and Cochrane Central Register of Controlled Trails. References of the selected articles were screened to avoid missing main articles. Twenty-seven published case reports and four retrospective studies, including 31 and 527 cases, respectively, matched the search criteria. A case from our institution was added. Mean age was 26 years (range 6–64 years). The most frequent site of perforation was the colon, particularly the sigmoid, followed by small bowel, upper rectum, and finally stomach. The majority of cases were initially managed with Hartmann’s procedure. In recurrent perforations, total colectomy was performed. The reperforation rate was considerably higher in the “partial colectomy with anastomosis” group than in the Hartmann group. Colonic perforation is the most common spontaneous GI perforation in EDS IV patients. An unexpected fragility of the tissues should raise the possibility of a connective tissue disorder and prompt further investigation with eventual management of these high-risk patients with a multidisciplinary team approach in dedicated centres. In the emergency setting, a Hartmann procedure should be performed.


Intestinal perforation Colorectal perforation Peritonitis Ehlers–Danlos syndrome Complication 


Author contributions

HEM: acquisition, analysis, and interpretation of presented data; drafting of the article; final approval of the version to be published. THL: critical revision of the article for important intellectual content; final approval of the version to be published. GM: critical revision of the article for important intellectual content; final approval of the version to be published. JP: critical revision of the article for important intellectual content; final approval of the version to be published. FZ: critical revision of the article for important intellectual content; final approval of the version to be published. PAL: conception and design of the study; management of the patient presented; drafting of the article; critical revision of the article for important intellectual content; final approval of the version to be published.


Mr H. El-Masri was supported by a joint program between University hospital of Nantes (France) and St Charles hospital of Beyrouth (Lebanon). Mr T. H. Loong was supported by jointly funded program by the Ministry of Health, Singapore and the National Healthcare Group, Singapore.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical standard

According to French law, this study does not require approval from the local ethic committee.

Informed consent

Our patient was informed and consented to participation in this review.


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • H. El Masri
    • 1
  • T.-H. Loong
    • 1
  • G. Meurette
    • 1
  • J. Podevin
    • 1
  • F. Zinzindohoue
    • 2
  • P.-A. Lehur
    • 1
  1. 1.Digestive and Endocrine Surgery Clinic, Institute of Digestive DiseasesUniversity Hospital of Nantes, CHU Hôtel DieuNantesFrance
  2. 2.HEGP Paris, National Referral Centre for Rare Diseases “Ehlers-Danlos Syndrome”ParisFrance

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