The clinical expression and impact of multiple endocrine neoplasia 1 during pregnancy

Abstract

Context Multiple endocrine neoplasia type 1 (MEN 1) is characterised by multi‐system neoplasia including primary hyperparathyroidism and pituitary adenoma. Despite the adolescent onset of endocrinopathies, information regarding the impact of maternal MEN 1 on pregnancy is limited to case reports. Objective To explore pregnancy outcomes in MEN 1 positive women. Methods Retrospective case series of maternofoetal outcomes MEN 1 positive mothers managed at the Royal Hobart Hospital between 1967‐2018. Data were retrieved from medical records and Australian averages calculated based on the Australian Institute of Health and Welfare data. Results Twenty‐six women with MEN 1 were identified accounting for 96 pregnancies and 76 live born infants. Hyperparathyroidism was evident in 16 pregnancies. A significant increase in serum calcium in the second trimester (β=0.14, pp=0.001), hypertensive disorders (25.9% vs 7.6, p=0.018), have shorter gestations (38.1 vs 38.7 weeks, p=0.015) and have low birthweight infants (30.1% vs 6.5%, p=0.001). However, emergency caesarean deliveries (63.2% vs 52.3%) and miscarriage rate (20.8% vs 20%) were not significantly different. Conclusion Maternal MEN 1 is associated with an increased risk of gestational diabetes, hypertensive disorders and low neonatal birthweight, but not with an increased miscarriage rate. Whilst hypercalcaemia worsens during the second trimester, most pregnancies progressed without overt complications or requirement for intervention.