title: 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.
authors: Division of Medical Genetics, Department of Molecular Medicine, Sapienza University, San Camillo-Forlanini Hospital, Rome, Italy. mcastori@scamilloforlanini.rm.it
authors: Marco
authors: M
authors: Castori
authors: Silvia
authors: S
authors: Morlino
authors: Chiara
authors: C
authors: Dordoni
authors: Claudia
authors: C
authors: Celletti
authors: Filippo
authors: F
authors: Camerota
authors: Marco
authors: M
authors: Ritelli
authors: Aldo
authors: A
authors: Morrone
authors: Marina
authors: M
authors: Venturini
authors: Paola
authors: P
authors: Grammatico
authors: Marina
authors: M
authors: Colombi
mesh_terms: D004535:Ehlers-Danlos Syndrome / Q000150:complications / Q000503:physiopathology*; D005260:Female; D005836:Genitalia, Female / Q000503:physiopathology*; D006801:Humans; D007558:Italy; D011247:Pregnancy; D011248:Pregnancy Complications / Q000503:physiopathology*