Adolescent girls with Ehlers-Danlos syndrome (EDS) often experience menstrual symptoms, but few are referred to gynecologic care, a small study suggests.
Researchers emphasized that teenagers with EDS should receive early gynecologic counseling and treatment, since these problems can severely impact their well-being.
These data also highlighted that most girls with EDS try two or more hormonal treatments to control their periods, and that long-acting reversible contraception (LARC), including hormonal intrauterine devices (IUDs), is a safe and effective option for them.
The study, “Gynecologic Management Of Pediatric And Adolescent Patients With Ehlers-Danlos Syndrome,” was published in the Journal of Pediatric and Adolescent Gynecology.
EDS is a group of rare inherited conditions that affect the connective tissue — which provides structure and support to the skin, joints, blood vessels, and various organs, including the reproductive organs.
Adult women with EDS frequently experience gynecological complications, with 72%–93% of them complaining of menstrual cramps, and 33%–76% of them reporting heavy menstrual bleeding.
These women also have a higher risk of pregnancy-associated complications, including miscarriage, ectopic pregnancies (pregnancy outside the uterus), and premature delivery.
Since hormonal treatments — particularly those containing estrogen — can negatively affect some EDS-associated comorbidities, managing gynecologic symptoms in this patient population can be a challenging process.
While several studies have reported the frequency of gynecologic symptoms in adult women with EDS and their management, no study to date has investigated these symptoms in adolescents with the disease.
Researchers in the U.S. evaluated the frequency and management of gynecologic complains in girls with EDS (under 21 years), followed at a single children’s hospital between July 1, 2007, and July 31, 2017.
Since these complaints are infrequently documented by other specialties, the team analyzed only data from girls seen by a pediatric and adolescent gynecologist.
Out of 156 girls with EDS followed during these 10 years, only 26 (16.7%) were seen by a gynecologic specialist, and were included in the analysis.
The girls’ mean age was 14.5, and 24 of them (92.3%) complained about menstrual symptoms. At least half reported menstrual cramps (57.7%) and heavy menstrual bleeding (50%). Irregular menstrual cycles were also reported by 10 girls (38.5%), and seven (26.9%) sought contraception.
Researchers noted that while girls with EDS showed similar rates of heavy menstrual bleeding to those of adult women with the disease, there was a lower, but considerable, frequency of menstrual cramps in this younger population.
These findings highlighted that “few adolescents with EDS are referred to PAG [pediatric and adolescent gynecologic providers] despite the prevalence of gynecologic complaints and potential for obstetric and gynecologic complications in this population,” the researchers wrote.
Researchers then divided the girls with menstrual complaints — 23 with hypermobile EDS and one with vascular EDS — into two groups according to the number of medications tried to control their periods. Nine were able to control their periods with only one medication (group A), while 15 girls needed to try two or more medications to achieve the same goal (group B).
Nearly half of the girls (44%) in group A controlled their periods with progestin-only pills, which was the most commonly used medication. In group B, most girls (73%) tried depot medroxyprogesterone acetate (DMPA) injections (given every 12 weeks), but hormonal IUD was the most popular final choice — used by four girls (27%).
While there were no bleeding complications associated with the insertion of LARC, including IUDs — which is a frequent concern in these patients — only one girl had vascular EDS, and this may not reflect what happens in all types of EDS.
“These [gynecologic] problems can severely impact adolescent wellbeing, so these patients should receive anticipatory counseling and early treatment of their symptoms,” the researchers wrote, adding that comorbidities in these patients “should be considered in selecting hormonal treatment.”
Due to pregnancy-associated complications in EDS patients, “contraceptive counseling and use of LARC in sexually active adolescents is recommended for patients not trying to conceive,” they added.
Future, larger studies including girls with all types of EDS are needed to confirm these findings.
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