Introduction

Paragangliomas are rare neuroendocrine tumours affecting 1-8 per 1 million people and less than 7 in 100000 pregnancies. Untreated paragangliomas in pregnancy are associated with a foetal and maternal mortality of approximately 58%.

The following case highlights the importance of tailored pharmacological management in a pregnant patient with a paraganglioma prior to caesarean section.

Clinical Features

A 15 year old pregnant woman at 26 weeks gestation presented with hypertension, headache, and diaphoresis. Hypertension persisted despite treatment with first- and second-line anti-hypertensives. Pre-eclampsia screening was unremarkable; however, further biochemical analyses and scans were consistent with a rare retroperitoneal paraganglioma diagnosis. Her medical and obstetric history includes asthma, high body mass index (33kg/m²), vitamin D deficiency (25nmol/L) and insulin-dependent gestational diabetes mellitus.

Case Progress

The patient was commenced on an increasing regimen of anti-hypertensives, including phenoxybenzamine, for pre-operative management in order to minimise the risk of a hypertensive crisis during her planned caesarean section.

Drug-disease interactions were evaluated and a list of medicines that could precipitate hypertensive crisis was compiled to informatively direct and optimise her management plan. Pregnancy safety data on medicines were reviewed and discussed in a multidisciplinary setting, and the patient was counselled accordingly. She underwent a caesarean section using combined spinal epidural and post-operative fentanyl, which was considered first-line given her diagnosis.

Outcomes

The patient successfully delivered a baby girl at 31 weeks gestation weighing 1900 grams and both were transferred to their respective intensive care units. The patient’s blood pressure was adequately managed with phenoxybenzamine post-delivery. She was counselled on phenoxybenzamine safety in breastfeeding and baby was monitored for bradycardia and hypotension. She remained on anti-hypertensives and underwent tumour resection six weeks post-partum.

Conclusion

Close monitoring is required for pregnant patients with paraganglioma and tailored pharmacological management is integral to optimise maternal and foetal outcome.