Multiple Endocrine Neoplasia Type 1 (MEN 1) is a rare inherited disorder caused by mutations in the tumour suppressor gene MEN 1. It is characterised by a predisposition towards the development of parathyroid, anterior pituitary and entero-pancreatic tumours. Clinically, MEN 1 is defined following development of two out of these three tumours. In patients with a known first degree relative with MEN1, the development of a single MEN associated tumour also constitutes a diagnosis, due to the autosomal dominant pattern of inheritance.
There have been no published cases of the management of MEN 1 in pregnancy. We report the first case of a 31-year-old primigravida with a confirmed diagnosis of MEN 1 prior to conception. Due to the rare nature of MEN 1 there are no guidelines on how these women should be managed. The main issues were to assess and manage potential complications, such as hypercalcaemia, diabetes mellitus, the symptoms from a pituitary tumour as well the issues around a gastrinoma and monitor fetal well being. A caesarean section was performed at 35 weeks gestation for a growth restricted fetus with raised umbilical artery dopplers. The neonate was treated with intravenous calcium secondary to hypocalcaemia. The patient and neonate recovered well.
We have demonstrated successful management of a woman with MEN1 who completed her pregnancy with few complications and a healthy neonate. It is vital for such women to be managed in the context of a multidisciplinary team setting to optimise maternal and fetal outcomes.
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