Spontaneous uterine rupture revealing vascular Ehlers-Danlos syndrome: An uncommon case report

Spontaneous Rupture of Unscarred Uterus in a Term Primagravida with Lethal Skeletal Dysplasia Fetus (Thanatophoric dysplasia). A Case Report and Review of the Literature

“… 23 , 24 Uterine rupture was also linked to uterine adenomyosis, 25 dilatation and curettage, 26 , 27 and maternal connective tissue disease, specifically Ehlers-Danlos syndrome. 28 Labor induction with misoprostol 11 , 29 and labor augmentation with oxytocin 30 were linked to uterine rupture in unscarred women. Uterine perforation during operative hysteroscopy has been associated with the possibility of uterine rupture during subsequent pregnancies.…”

Uterine rupture in women with unscarred uteri: Manifestations of underlying disorders?

“…A typical example would be Ehlers-Danlos syndrome (EDS). Slaoui et al 2 reported a preterm unscarred uterine rupture and extreme fragility of the muscular-cutaneous tissues was noted during surgery. Subsequently, a family history revealed that the woman had lost her mother and sister at 39 and 35 years, respectively, the latter because of abdominal aortic rupture.…”

“…As mentioned, the International Network of Obstetrics Survey Systems study included one women with osteogenesis imperfecta type 1. 2 In response to their question, the diagnosis in this woman was known before the pregnancy; that is, before rupture occurred. She was allowed to give birth vaginally and clinical conditions of this birth indicate that caregivers were probably not aware of the associated potential risk of uterine rupture.…”

Author reply

“…3 Literature review by Slaoui et al demonstrated a small number of cases of uterine ruptures in women with EDS, besides other severe complications, including vascular ruptures, haemorrhages, perineal and cervical ruptures and preterm birth. 4 Further, pregnancy might cause a widening of the aortic arc as an early sign of aortic dissection among these women. 5 Prof. Dr Matsubara suggested further follow up of the women in our study who had an unscarred uterine rupture with no obvious aetiology and suggested screening them for underlying conditions.…”