Abstract
INTRODUCTION: Neuromuscular dystrophies (NMD) are a group of rare genetic diseases characterized by weakness and muscular wasting. Data on NMD in pregnancy is limited. Our aim is to determine the prevalence of muscular dystrophies at delivery, and related maternal and newborn outcomes. METHODS: Using the United States Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP-NIS) from 1999 to 2013, we conducted a population-based retrospective cohort study to compare women with and without any form of NMD at delivery. We used multivariate logistic regression analyses to estimate the associated risks of maternal and neonatal outcomes. RESULTS: Of the 12,592,178 births in our cohort, 914 deliveries were to women carrying a NMD. Over the 15-year period, there was an increased prevalence rate from 3 to 10 cases per 100 000 deliveries per year (p < 0.001). Births to women with NMD were more likely to be premature (OR 1.60, 95% CI 1.26-2.02), have intrauterine growth restriction (OR 2.08,95% CI 1.50-2.90) and congenital malformation (OR 4.93, 95% CI 3.30-7.38). Compared with controls, women with muscular dystrophies were more likely to deliver by cesarean section (OR 1.87,95% CI 1.62-2.17), have forceps assisted deliveries (OR 1.76, 95% CI 1.01-3.04), were at higher risk of major morbidities including cardiac dysrhythmia (OR 4.97,95% CI 3.02-8.20) and increased requirements for blood transfusion (OR 2.44,95% CI 1.56-3.83). CONCLUSION: Pregnancies in NMD patients are associated with an increased risk of maternal and fetal morbidities and, as such, they should be cared for in a tertiary care center.