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

Abstract

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.

Surgical Recommendations in Ehlers-Danlos Syndrome(s) Need Patient Classification: The Example of Ehlers-Danlos Syndrome Hypermobility Type (a.k.a. Joint Hypermobility Syndrome)

“…Dear Sir, I read with great interest the work by Burcharth and Rosenberg [1] (Herlev, Denmark) on surgical aspects of Ehlers-Danlos syndrome(s) (EDSs). In this work, the authors review 53 previously published papers, summarize indications and complications by anatomic structure, and define some practical recommendations for improving outcomes of skin closure after surgical incisions.…”

Comparison of early complications using regular mesh versus Atramat Neoflex 25 mesh in inguinal, umbilical and post incisional hernias

“…[8][9][10][11] New techniques are proposed to reduce recurrence of inguinal hernias and to prevent early or chronic complications. 12 The two general therapeutic options are the inguinal plasty or herniorrhaphy, being the inguinal plasty the most common procedure nowadays with a lower recurrence rate than herniorrhaphy.…”

Acute Abdominal Surgery and Ehlers-Danlos Syndrome: Anesthesia Management

“…EDS her 5.000 doğumda 1 görülmektedir. 1 Ancak, hekimlerin farkındalığının artmasıyla bu insidans yükselmektedir. 2 Klinik bulguları, kalıtımsal geçişi, biyokimyasal ve moleküler bulgularına göre altı alt tipe ayrılmıştır.…”