Ehlers-Danlos syndromes (EDS) are a collection of inheritable connective tissue disorders characterised by joint hypermobility, skin stretchiness, and fragile tissue. Any of the 13 Ehlers-Danlos subtypes can result in prolapsed pelvic organs, since the connective tissue that holds us in and up can be stretchy or loose. This allows what should stay up to fall down.
There are many manifestations of Ehlers-Danlos syndromes, but here we just focus on how Ehlers-Danlos symptoms can affect gynaecological structures.
There are a high number of gynaecological complaints amongst women with Ehlers-Danlos syndromes.
The most common gynaecological complaints include:
- Heavy periods (menorrhagia)
- Period pain (dysmenorrhoea)
- Painful sex/penetration (dyspareunia)
- High rates of miscarriage and recurrent miscarriages
- More severe symptoms during puberty, premenstrually, postpartum, and while on the oral contraceptive pill
There is no observed increase in endometriosis, babies delivered by C-section or premature births.
The role of connective tissue
Connective tissue provides strength and elasticity, containing tough protein fibres (collagen, elastin) that can stretch, but have limits. Once stretched, the fibres then return to their normal position. This allows flexibility in our body for movement.
In Ehlers-Danlos syndromes, the connective tissue fibres are faulty, and do not perform as required. This means things can literally come loose in surprising places at awkward times. A body part coming loose causes damage to the connective tissue by pulling it far out of its normal scope. It could be a muscle, tendon, blood vessel, organ, gums, ligament, skin or even the eyes.
The connection of EDS with vulvodynia and vulval pain
People with EDS may have frequent pain throughout the body, with the vulva being no exception. Vulvodynia may be present as a symptom of EDS.
Risks to a foetus and mother in miscarriage, stillbirths
Tissue is very fragile in EDS, in multiple organs, including the uterus and cervix. Pregnancy with a woman with EDS has risks for both mother and baby, with more frequent complications. These complications can come at a great emotional cost, particularly in undiagnosed EDS.
- Membranes may rupture prematurely, resulting in a premature birth and rapid labour and delivery (less than four hours)
- Breech position occurs more frequently if the baby is affected by EDS, resulting in dislocations of hips/shoulders in the baby at birth
- Perineal tearing and episiotomy cuts can present healing and scarring problems, and result later in easier prolapse of the uterus or bladder
- Hemorrhage, uterine or artery rupture may occur more frequently due to blood vessel fragility
- Joint pain and laxity may increase across a pregnancy to to increased production of relaxin (which is normal but may be more pronounced in EDS)
- It is not known whether caesarean deliveries are safer than vaginal births and the resultant tears
- Genetic counselling should be obtained prior to conception if possible
- 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. Hugon-Rodin J1, Lebègue G1, Becourt S1, Hamonet C2, Gompel A3.
- Chopra, P., Tinkle, B., Hamonet, C., Brock, I., Gompel, A., Bulbena, A., & Francomano, C. (2017). Pain management in the Ehlers–Danlos syndromes. American Journal of Medical Genetics, Part C: Seminars in Medical Genetics, 175(1), 212-219. DOI: 10.1002/ajmg.c.31554