Peripartum bilateral uterine rupture in a patient with mixed connective tissue disease with favorable outcome for the severely asphyctic newborn after hypothermia

Abstract

Information about fetal and maternal outcome in pregnant women with mixed connective tissue disease (MCTD) is rare and in part contradictory. The purpose of this study is to review published literature about MCTD in pregnancy in the context of a rare case of peripartum bilateral uterine rupture in a patient with MCTD with favorable outcome for the severely asphyctic newborn after hypothermia. The study included a selective literature review based on a PubMed search using the search terms MCTD, Sharp syndrome, uterine rupture and hypothermia, and a detailed report of our case with regard to the MCTD of the patient. Rupture to the backside of the uterus during delivery, independent of prior cesarean section, was unpredictable and its cause remains unclear. The clinical outcome of the newborn was surprisingly favorable and there were no signs of neurodevelopmental sequelae in spite of the fact that the newborn was asphyctic and had a large excess of acids in the umbilical cord blood gas analysis. The favorable outcome is due to treatment with whole body hypothermia. Any type of prior surgery of the uterus puts the patient at risk during delivery. MCTD might be a risk factor during birth. These patients should be followed closely during pregnancy and should deliver at a center, which provides all options for immediate surgical and neonatological intervention.