Gynecologic symptoms and the influence on reproductive life in 386 women with hypermobility type ehlers-danlos syndrome: a cohort study.

Justine Hugon-Rodin,Géraldine Lebègue,Anne Gompel,Stéphanie Becourt,Claude Hamonet

doi:10.1186/s13023-016-0511-2

Abstract

BackgroundHypermobile Ehlers-Danlos syndrome (hEDS), is probably the most common disease among heritable connective tissue disorders. It affects women more than men and causes symptoms in multiple organs. It is associated with chronic pain, skin fragility and abnormal bleeding. These characteristics may hamper reproductive life. We conducted a study to evaluate the gynecologic and obstetric outcomes in women with hEDS. We also explored a possible hormonal modulation of the hEDS symptoms. The gynecologic and obstetric history of 386 consecutive women diagnosed with hEDS was collected by a standardized questionnaire and a medical consultation performed by a senior gynecologist in an expert centre for hEDS between May 2012 and December 2014.ResultsWe observed a high frequency of gynecologic complaints, specifically: menorrhagia (76 %), dysmenorrhea (72 %) and dyspareunia (43 %). Endometriosis was not highly prevalent in this population. The obstetric outcomes were similar to those of the general French population for deliveries by cesarean section (14.6 %) and premature births (6.2 %) but the incidence of multiple spontaneous abortion (13 %) and spontaneous abortion (28 %) were significantly higher. A subset of women were sensitive to hormonal fluctuations with more severe symptoms occurring during puberty, prior to menstruation, during the postpartum period as well as on oral contraception.ConclusionsIncreased awareness of the gynecological symptomatology in women with hEDS can help discriminate between endometriosis and thus prevent useless, and potentially dangerous, surgery. This study also suggests that hormonal modulation may be an appropriate treatment for a subset of women with hEDS.

Highlights

Hypermobile Ehlers-Danlos syndrome, is probably the most common disease among heritable connective tissue disorders
We explored whether Hypermobile Ehlers-Danlos syndrome (hEDS) symptoms were sensitive to the hormonal environment
In cases of a clinical suspicion of endometriosis or severe menorrhagia/ dyspareunia, a pelvic sonogram and, if necessary, magnetic resonance imaging (MRI) were performed by an experienced radiologist

Summary

Introduction

Hypermobile Ehlers-Danlos syndrome (hEDS), is probably the most common disease among heritable connective tissue disorders It affects women more than men and causes symptoms in multiple organs. Four studies have reported on the gynaecologic and obstetric outcomes in women with both JHS, hEDS and other EDS types with some discrepancies on the prevalence of gynecologic complaint, spontaneous abortion and obstetrical outcomes [9,10,11,12,13,14]. This can be due to pooling different types of EDS in the analysis and getting the information through a self-declaring questionnaire. The last largest study published was based on an email questionnaire with no clinical validation [14]

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