Introduction: Hypertensive disorders in pregnancy are a major risk factor of maternal and fetal morbidity and mortality. Most cases of hypertension in pregnancy are due to essential hypertension. Secondary causes, such as endocrine and renal disorders, are rare with prevalence of only 0.24% (1). However, they carry greater risk of adverse outcomes. While exceedingly rare, our postpartum patient was found to have not only one but two endocrine disorders that could be responsible for her diagnosis of chronic hypertension and superimposed pre-eclampsia with severe features.
Case Study: 37-year-old G2P1102 patient now 4 months postpartum with medical history of chronic hypertension, hypokalemia, and pre-eclampsia with severe features, presents to the ED with complaints of headache, generalized weakness, and severe-range blood pressures. Patient was admitted for symptomatic hypertension and hypokalemia. Patient was also found to have metabolic alkalosis and elevated aldosterone to renin ratio. She responded well to spironolactone. Care team suspected primary hyperaldosteronism. However, CT adrenal and MRI abdomen reported left adrenal and retroperitoneal masses that were compatible with pheochromocytomas. Random catecholamine and normetanephrine measurements were negative, but a 24-hour normetanephrine was elevated. Upon discharge, patient was evaluated by outpatient urology who believed the adrenal mass is a pheochromocytoma while the retroperitoneal mass is an active aldosteronoma. Patient underwent adrenalectomy and retroperitoneal mass excision. Surgical pathology returning with the adrenal mass was an aldosteronoma while the extra-adrenal mass was a pheochromocytoma.
Discussion: This was a challenging diagnostic case because there were many factors that could complicate the picture of her hypertension: her history of chronic hypertension, her pre-eclampsia with severe features intrapartum, and the stress of being pregnant and then being a new mom. It is unclear whether the patient's pheochromocytoma and aldosteronoma were always present as the cause of her chronic hypertension or if they developed during her pregnancy. During the workup, there was inconsistency between clinical and imaging findings. Patient’s triad of resistant hypertension, hypokalemia, and metabolic alkalosis was most consistent with primary hyperaldosteronism. Elevated aldosterone to renin ratio and the patient’s response to spironolactone further solidified the suspected diagnosis. However, both CT and MRI findings reported pheochromocytoma. Based on literature, about 80% of adrenal incidentalomas are nonfunctional adenomas, 9% cause subclinical Cushing's syndrome, 4% are pheochromocytomas, and only 1% are aldosteronomas (2). Our patient has both active lesions which is extremely rare. Our case elicits the question should we consider ruling out pheochromocytomas and hyperaldosteronism in pregnant patients with resistant hypertension.
