Neurofibromatosis type 1 and pregnancy complications: a population-based study

Abstract

Objective: The objective of the study was to determine whether vascular and other complications are more common in pregnant women with neurofibromatosis type 1 (NF1).

Study design: We performed a population-based retrospective cohort study using the US Nationwide Inpatient Sample, 1988-2009, defining a cohort of pregnancy-related hospitalizations with an associated diagnosis of NF1 and comparing it with the control group not associated with NF1. Multivariable logistic regression was used to adjust for suspected confounders.

Results: Among 19 million pregnancy-related admissions between 1988 and 2009, we identified 1553 associated with NF1 (prevalence 0.008%). A diagnosis of NF1 in delivering mothers was associated with gestational hypertension (adjusted odds ratio [AOR], 1.6, 95% confidence interval [CI], 1.2-2.0), preeclampsia (AOR, 2.8, 95% CI, 2.3-3.4), intrauterine growth restriction (AOR, 4.6, 95% CI, 3.7-5.6), cerebrovascular disease (OR, 8.1, 95% CI, 2.6-25.4), preterm labor (AOR, 1.6, 95% CI, 1.4-1.9), and cesarean delivery (AOR, 2.0, 95% CI, 1.8-2.3). Women with NF1 were not significantly more likely to have deep venous thrombosis/pulmonary embolism, acute cardiac events, or stillbirth or to die during their hospitalizations compared with the general obstetric population.

Conclusion: NF1 was associated with increased maternal morbidity in pregnancy (including hypertensive and cerebrovascular complications) but not increased maternal mortality. Obstetricians should be aware of the potential for increased antenatal and peripartum complications among women with NF1.

Keywords: cesarean section; epidemiology; intrauterine growth restriction; neurofibromatosis; preeclampsia.