Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers–Danlos syndrome hypermobility type) in 82 Italian patients

Abstract

Joint hypermobility syndrome (JHS) emerges as likely the most common clinical form of Ehlers-Danlos syndrome. Given the striking predominance of affected women, practitioners often face gynecologic and obstetric issues. However, their decisions are still based on personal experience rather than literature due to the lack of a consistent body of evidence. We collected a set of gynecologic and obstetric features in 82 post-puberal women with JHS attending two Italian centers. Common gynecologic findings were dysmenorrhea (82.9%), meno/metrorrhagias (53.7%), irregular menses (46.3%), and dispareunia/vulvodinia (31.7%). Forty women were nulliparous and 42 had one or more pregnancy for a total of 93 diagnosed conceptions. Of them, 16.1% were spontaneous abortions, 6.5% voluntary interruptions, 10.7% preterm deliveries, and 66.7% deliveries at term. Overall outcome of proceeding pregnancies was good with no stillbirth and fetal/neonatal hypoxic/ischemic event. Non-operative vaginal delivery was registered in 72.2%, forceps/vacuum use in 5.5% and cesarean in 22.3%. Local/total anesthesia was successfully performed in 17 pregnancies without any problem. Major post-partum complications included abnormal scar formation after cesarean or episiotomy (46.1%), hemorrhage (19.4%), pelvic prolapses (15.3%), deep venous thrombosis (4.2%), and coccyx dislocation (1.4%). Prolapses were the most clinically relevant complication and associated with episiotomy. Gathered data were discussed for practically oriented considerations.

Gynecologic and obstetric impact of the ehlers-danlos syndrome : clues from scrutinizing dermal ultrastructural alterations

“…Hence, CT disorders such as EDS possibly alter the fetal membranes inducing PPROM and miscarriages. This hypothesis is supported by the increased incidence of preterm delivery and late abortions in EDS patients [17,19,26,27]. This incidence is higher when the fetus is affected [27].…”

“…Irregular menses, metrorrhagias, severe dysmenorrhea, dyspareunia, vulvodynia and vaginal dryness are frequent in EDS women [12,[17][18][19]. Chronic pains represent the most common neurologic complaints [20].…”

“…Severe back and pelvic pains, and bleeding are the most frequent complains in EDS [17,19,27]. Major post-partum complications are seen in EDS vascular, kyphoscoliotic types, such as intestinal and vascular ruptures [17,27,[29][30][31].…”

“…Outcome of pregnancy is often unaffected in EDS hypermobile type [17,27,32]. Nevertheless, some complications are reported including abnormal fetal presentations, increased rate of suture dehiscence and delayed wound healing, uterus atonia, hemorrhage, pelvic prolapse, deep venous thrombosis and coccyx dislocation [17,19,27,32,33]. Pelvic prolapse may occur in nullipara [34].…”

Síndrome De Hipermobilidade Articular Nos Pacientes Com Disfunções Do Diafragma Da Pelve: Revisão De Literatura

“…Os critérios menores serão suficientes quando existir um familiar de primeiro grau que sofra claramente da síndro-me (7) . A pontuação pelo escore de Beighton é feita por meio da avaliação de nove pontos específicos, atribuindo um ponto a qualquer um dos seguintes: (1) oposição passiva do polegar ao aspecto flexor do antebraço (um ponto para cada lado); (2) dorsiflexão passiva do dedo mínimo, além de 90º (um ponto para cada lado); (3) hiperextensão do cotovelo além de 10º (um ponto para cada lado); (4) hiperextensão do joelho além de 10º (um ponto para cada lado); (5) flexão anterior do tronco com os joelho estendidos e as palmas descansando no chão (um ponto) (8) . De acordo com essa escala, os indivíduos com uma pontuação > 4 são considerados portadores de HA (9) .…”