A large abdominal intercostal hernia in a patient with vascular type Ehlers-Danlos syndrome: a surgical challenge

Weerd, Louis de; Kjæve, J.; Gurgia, Layth; Weum, Sven

doi:10.1007/s10029-010-0721-9

Cited by 19 publications

(15 citation statements)

References 11 publications

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“…Fifteen case reports describe surgical techniques (and their outcomes) for various GI problems in JHS/EDS‐HT (Table ). A definite diagnosis of JHS according to Brighton criteria or EDS‐HT according to Villefranche criteria was declared in seven instances [de Weerd et al, ; Reinstein et al, ; Dordoni et al, ; Fogel, ; Sardeli et al, 2013; Plackett et al, ]. In six patients, the diagnosis of EDS remained unclassified in the original report, but JHS/EDS‐HT was considered, retrospectively, the most likely based on the description of extra‐GI features [Douglas and Douglas, ; Shaikh and Turner, ; Leung, ; Defuentes et al, ; Levine and Adler, 2005; Chen and Jao, ].…”

Section: Resultsmentioning

confidence: 99%

“…In JHS/EDS‐HT, surgery appeared repeatedly successful for treating diaphragmatic defects leading to a variety of clinical presentations [Phadke, 1978; Shaikh and Turner, ; Leung, ; Levine and Adler, 2005]. In turn, surgery was uneffective multiple times for the correction of visceroptosis and pelvic organ prolapse [de Weerd et al, ; Dordoni et al, ; Pelizzo et al, ]. Laparoscopic subtotal colectomy for bowel ptosis had positive results in one instance [Reinstein et al, ], as well as the repair of a recto‐vaginal fascia with porcine small intestinal submucosa mesh in a woman with pelvic organ discomfort for multiple prolapses [Sardeli et al, 2013].…”

Section: Resultsmentioning

confidence: 99%

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Gastrointestinal and nutritional issues in joint hypermobility syndrome/ehlers–danlos syndrome, hypermobility type

Castori¹,

Morlino²,

Pascolini³

et al. 2015

American J of Med Genetics Pt C

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Gastrointestinal involvement is a well known complication of Ehlers-Danlos syndromes (EDSs), mainly in form of abdominal emergencies due to intestinal/abdominal vessels rupture in vascular EDS. In the last decade, a growing number of works investigated the relationship between a wide spectrum of chronic gastrointestinal complaints and various EDS forms, among which the hypermobility type (a.k.a. joint hypermobility syndrome; JHS/EDS-HT) was the most studied. The emerging findings depict a major role for gastrointestinal involvement in the health status and, consequently, management of JHS/EDS-HT patients. Nevertheless, fragmentation of knowledge limits its impact on practice within the boundaries of highly specialized clinics. In this paper, literature review on gastrointestinal manifestations in JHS/EDS-HT was carried out and identified papers categorized as (i) case-control/cohort studies associating (apparently non-syndromic) joint hypermobility and gastrointestinal involvement, (ii) case-control/cohort studies associating JHS/EDS-HT and gastrointestinal involvement, (iii) case reports/series on various gastrointestinal complications in (presumed) JHS/EDS-HT, and (iv) studies reporting gastrointestinal features in heterogeneous EDS patients' cohorts. Gastrointestinal manifestations of JHS/EDS-HT were organized and discussed in two categories, including structural anomalies (i.e., abdominal/diaphragmatic hernias, internal organ/pelvic prolapses, intestinal intussusceptions) and functional features (i.e., dysphagia, gastro-esophageal reflux, dyspepsia, recurrent abdominal pain, constipation/diarrhea), with emphasis on practice and future implications. In the second part of this paper, a summary of possible nutritional interventions in JHS/EDS-HT was presented. Supplementation strategies were borrowed from data available for general population with minor modifications in the light of recent discoveries in the pathogenesis of selected JHS/EDS-HT features.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Gastrointestinal and nutritional issues in joint hypermobility syndrome/ehlers–danlos syndrome, hypermobility type

Castori¹,

Morlino²,

Pascolini³

et al. 2015

American J of Med Genetics Pt C

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“…7 A more recent retrospective chart review of patients presenting for recurrent abdominal wall herniation at a tertiary institution found a 10% incidence rate, which was much higher than what the authors expected. 37 The literature is otherwise peppered with case reports of large and difficult hernias, 38 but does not allow for the evaluation of the case prevalence within the context of this patient population.…”

Section: Hernias and Collagen Vascular Diseasementioning

confidence: 99%

Collagenopathies—Implications for Abdominal Wall Reconstruction: A Systematic Review

Harrison

Sanniec

Janis

2016

Plastic and Reconstructive Surgery - Global Open

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Background:The etiology of hernia formation is strongly debated and includes mechanical strain, prior surgical intervention, abnormal embryologic development, and increased intraabdominal pressure. Although the most common inciting cause in ventral hernias is previous abdominal surgery, many other factors contribute. We explore this etiology through an examination of the current literature and existing evidence on patients with collagen vascular diseases, such as Ehlers–Danlos syndrome.Methods:A systematic review of the published literature was performed of all available Spanish and English language PubMed and Cochrane articles containing the key words “collagenopathies,” “collagenopathy,” “Ehlers-Danlos,” “ventral hernia,” and “hernia.”Results:Three hundred fifty-two articles were identified in the preliminary search. After review, 61 articles were included in the final review.Conclusions:Multiple authors suggest a qualitative or quantitative defect in collagen formation as a common factor in hernia formation. High-level clinical data clearly linking collagenopathies and hernia formation are lacking. However, a trend in pathologic studies suggests a link between abnormal collagen production and/or processing that is likely associated with hernia development.

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“…Patients with EDS may also be prone to ventral herniation [40]. A single case documented a large intercostal hernia after coughing [41], a hernia type which is not seen in other patients. In particular, patients with vascular-type EDS have poor wound healing, which causes a high frequency of incisional hernias after abdominal incisions [36].…”

Section: Herniamentioning

confidence: 99%

“…Data on occurrence of hernia among patients with EDS rely primarily on a single clinical study of 130 patients [39] as well as case reports [36,[40][41][42]. In all age groups, patients with EDS have an overall increased relative risk of 2.5 for development of inguinal hernias compared with the general population [39].…”

Section: Herniamentioning

confidence: 99%

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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A large abdominal intercostal hernia in a patient with vascular type Ehlers-Danlos syndrome: a surgical challenge

Cited by 19 publications

References 11 publications

Gastrointestinal and nutritional issues in joint hypermobility syndrome/ehlers–danlos syndrome, hypermobility type

Gastrointestinal and nutritional issues in joint hypermobility syndrome/ehlers–danlos syndrome, hypermobility type

Collagenopathies—Implications for Abdominal Wall Reconstruction: A Systematic Review

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

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