SynthesisTechniques in coloproctology2018
Bowel perforation in type IV vascular Ehlers-Danlos syndrome. A systematic review.
Synthesis in Techniques in coloproctology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 33 citations in OpenAlex.
- Exploring the impact of collagen-I genetic variants on intestinal structure and gut microbiota in mouse models of osteogenesis imperfecta.Scientific reports · 2026Article
- Ehlers-Danlos syndrome and the risk of spontaneous colonic perforation: clinical insights and surgical implications.Journal of surgical case reports · 2025Article
- Perforation of the Sigmoid Colon due to Diverticular Rupture in a Woman WithClinical case reports · 2025Article
- Small bowel perforation caused by a fish bone in a patient with vascular Ehlers-Danlos syndrome: a case report.Journal of surgical case reports · 2025Article
- Investigating the association between osteopenia and bowel perforation through a multicenter radiologic analysis.Scientific reports · 2024Article
- Spontaneous Sigmoid Colon Perforation and Ruptured Subserosal ("Zebra" Pattern) Small-Bowel Hematomas in Type IV Ehlers-Danlos Syndrome: A Case Report and a Short Review.Journal of clinical medicine · 2024Article
- Bowel Perforation in Vascular Ehlers-Danlos Syndrome: Case Report and Comprehensive Review.Journal of personalized medicine · 2023Article
- A case of sigmoid colon perforation due to segmental absence of intestinal musculature (SAIM) accompanied by vascular Ehlers-Danlos syndrome: a case report.Surgical case reports · 2023Article
- Total colectomy in Vascular Ehlers Danlos syndrome a case report and literature review.Annals of medicine and surgery (2012) · 2021Article
- Gastric perforation leading to the diagnosis of classic Ehlers-Danlos syndrome: a case report.Journal of medical case reports · 2021Article
- Enteroatmospheric fistula repair in Ehlers-Danlos syndrome type IV: a novel management technique using ABRA device.BMJ case reports · 2020Article
- Gastrointestinal perforation: clinical and MDCT clues for identification of aetiology.Insights into imaging · 2020Article
- The Genetics of Thoracic Aortic Aneurysms and Dissection: A Clinical Perspective.Biomolecules · 2020Review
- Fragility of Life: Recurrent Intestinal Perforation Due to Vascular Ehlers-Danlos Syndrome.Digestive diseases and sciences · 2019Article
- Intestinal Perforation in Children as an Important Differential Diagnosis of Vascular Ehlers-Danlos Syndrome.The American journal of case reports · 2019Article
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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.
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