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Orphanet J Rare Dis. 2016 Sep 13;11(1):124. doi: 10.1186/s13023-016-0511-2.

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

Author information

1
Unité de Gynécologie-Endocrinienne, APHP, Hôpitaux universitaires Paris Centre, Université Paris Descartes, Port Royal, 123 Bd de l'Hôpital, Paris, 75014, France.
2
Service de médecine physique et réadaptation, APHP, Hôpitaux Universitaires Paris Centre, Université Paris-Est Créteil, Hôtel Dieu, Place Jean XXIII, Paris, 75004, France.
3
Unité de Gynécologie-Endocrinienne, APHP, Hôpitaux universitaires Paris Centre, Université Paris Descartes, Port Royal, 123 Bd de l'Hôpital, Paris, 75014, France. anne.gompel@aphp.fr.

Abstract

BACKGROUND:

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. 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.

RESULTS:

We 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.

CONCLUSIONS:

Increased 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.

KEYWORDS:

Bleeding disorders; Ehlers-Danlos syndrome; Endometriosis; Hypermobility type; Premature delivery; Recurrent abortion

PMID:
27619482
PMCID:
PMC5020453
DOI:
10.1186/s13023-016-0511-2
[Indexed for MEDLINE]
Free PMC Article

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