Myotonic dystrophy is a rare autosomal dominant degenerative neuromuscular and neuroendocrine disease which can also affect the uterine muscles. In pregnancy, issues can arise with abnormalities in all three stages of labour. We report a unique case in which a woman with classic myotonic dystrophy suffered an occult uterine rupture following post-term induction of labour and an apparently uncomplicated forceps delivery. She was diagnosed with fulminating Clostridium perfringens infection two days postpartum and required an emergency hysterectomy. We postulate that abnormal myotonic uterine contractions in labour caused focal uterine ischemic necrosis. Subsequent infection with the commensal organism Clostridium perfringens resulted in uterine infection and fulminating retroperitoneal sepsis through the disruption of the uterine wall. Despite the atypical presentation of uterine rupture and sepsis, timely recognition, judicious use of intravenous antibiotics, multidisciplinary involvement, early imaging, and undelayed surgery averted maternal mortality.

Authors: Daisy Wildash, Jackie Hawley, Dushyant Maharaj, Fali Langdana

Key Words: Myotonic dystrophy, uterine rupture, Clostridium perfringens infection, postpartum sepsis