Surgical aspects of the Ehlers-Danlos syndrome a survey of 100 cases

Beighton, Peter; Horan, Frank

doi:10.1002/bjs.1800560404

Cited by 89 publications

(55 citation statements)

References 27 publications

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“…It has previously been postulated that the lack of perivascular structural integrity may predispose to increased bleeding from the gastric mucosa in patients with EDS [19]. Gastric diverticula are rare but have been reported frequently in patients with EDS [20,16]. Other commonly occurring diseases of the stomach in patients with EDS are delayed gastric emptying and gastric atony [17,21].…”

Section: Stomachmentioning

confidence: 99%

“…Gastric symptoms in patients with EDS extend from ordinary epigastric discomfort to severe bleeding, and in very rare cases, perforation of the stomach primarily in relation to trauma [17]. Peptic ulcers occur in patients with EDS [16], and complications of peptic ulceration have been described in patients with vascular-type EDS [18]. It has previously been postulated that the lack of perivascular structural integrity may predispose to increased bleeding from the gastric mucosa in patients with EDS [19].…”

Section: Stomachmentioning

confidence: 99%

“…However, there are few documented case reports of spontaneous liver rupture [26], splenic rupture [27] and gallbladder rupture [16], probably due to a reduced amount of structurally intact supportive tissue in these organs. Pancreatitis in patients with EDS has been described [28] and the cause is thought to be microvascular damage to the capillaries in the pancreas.…”

Section: Liver Spleen and Pancreasmentioning

confidence: 99%

“…The data on gastric symptoms in patients with EDS were derived from a single large clinical study including 100 patients [16] and case report data [17][18][19][20][21]. Gastric symptoms in patients with EDS extend from ordinary epigastric discomfort to severe bleeding, and in very rare cases, perforation of the stomach primarily in relation to trauma [17].…”

Section: Stomachmentioning

confidence: 99%

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Gastrointestinal Surgery and Related Complications in Patients with Ehlers-Danlos Syndrome: A Systematic Review

Burcharth

Rosenberg

2012

Dig Surg

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Introduction: Ehlers-Danlos syndrome (EDS) is a rare inherited group of connective tissue diseases characterized by joint hypermobility, skin hyperextensibility and bruising tendency. Common features of patients with EDS include vascular and gastrointestinal perforations. The purpose of this systematic review is to address gastrointestinal diseases and the complications associated with surgical treatment of diseases relating to the gastrointestinal system in patients with EDS. Methods: PubMed search including the Medical Subject Heading (MeSH) terms ‘Ehlers-Danlos Syndrome’ and ‘Gastrointestinal Diseases’, and an Embase search including the Map Term to Subject Heading ‘Ehlers-Danlos Syndrome’ with ‘AND’ function of the keyword ‘Gastrointestinal’. Results: The literature search resulted in inclusion of 53 articles after application of eligibility criteria. The primary results drawn from the literature was that spontaneous ruptures of vessels and spontaneous perforations of the sigmoid colon occur in patients with EDS. Conclusion: Surgery in patients with EDS is associated with a high risk of complications, which is why preoperative indications should be considered. Optimal therapy for these patients includes the awareness that EDS is a systemic disease involving fragility, bleeding and spontaneous perforations from almost all organ systems. Often, a nonsurgical approach can be the best choice for these patients, depending on the condition.

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Section: Stomachmentioning

confidence: 99%

Section: Stomachmentioning

confidence: 99%

Section: Liver Spleen and Pancreasmentioning

confidence: 99%

Section: Stomachmentioning

confidence: 99%

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Gastrointestinal Surgery and Related Complications in Patients with Ehlers-Danlos Syndrome: A Systematic Review

Burcharth

Rosenberg

2012

Dig Surg

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“…21 -3033 In only one reported instance could the fistula be safely occluded. 32 Surgeons have described the tissues of affected patients as resembling "wet blotting paper", 1 "cold porridge", 42 or "wet cotton", 25 and tissue friability was a particular problem in various operations in our patient 2. Arteries and veins tear easily due to the flimsiness of their walls, literally crumbling in the surgeon's hands.…”

Section: Discussionmentioning

confidence: 84%

Cerebrovascular disease in Ehlers-Danlos syndrome type IV.

Schievink

Limburg

Oorthuys

et al. 1990

Stroke

122

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We describe two patients with cerebrovascuiar complications of Ehiers-Danlos syndrome type IV. A 16-year-old girl with spontaneous internal carotid artery dissection and a 46-year-old woman with aneuiysmal subarachnoid hemorrhage and multiple aortic dissections were both deficient in collagen type III, analyzed in cultured skin fibroblasts. To our knowledge, spontaneous carotid artery dissection associated with collagen type III deficiency has not been reported previously. Early clinical recognition of this syndrome is of great importance in view of the hazards of angjography and surgery. Collagen type III deficiency plays a role in the pathogenesis of intracraniai saccular aneurysms and may also be involved in the pathogenesis of carotid cavernous fistulas and dissections of the cervical arteries. (Stroke 1990^1:626-632)

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