Aim
To report a case of hypertensive crisis in early pregnancy due to phaeochromocytoma and highlight the multidisciplinary management approach and outcomes.
Methods
A 38-year-old woman at 11 weeks' gestation presented with severe hypertension and gestational diabetes. Diagnostic workup revealed markedly increased plasma metanephrines, hypercortisolism with hypokalemic alkalosis, and a 46 x 44 x 45 mm left adrenal mass. Initial treatment in the high dependency unit (HDU) included labetalol, amiloride, and a GTN patch. Due to resistant hypertension, she was transferred to the intensive care unit (ICU) where intravenous hydralazine and insulin therapy were administered. Preoperative management with phenoxybenzamine controlled blood pressure. A laparoscopic left adrenalectomy was performed at 15 weeks' gestation.
Results
Histopathology confirmed a phaeochromocytoma with low PASS and moderate GAPP scores but without significant ACTH staining. Postoperative care included IV hydocortisone, transitioning to cortisone acetate. Blood pressure and glucose levels normalized, and hypokalemia resolved. The patient weaned off glucocorticoids by 29 weeks' gestation and delivered a healthy 3.3 kg baby at 39.4 weeks via vacuum delivery. Follow-up showed normal plasma metanephrines and full recovery of the HPA axis.
Conclusion
This case demonstrates effective management of a hypertensive crisis due to phaeochromocytoma during pregnancy through a multidisciplinary approach. Early surgical intervention, performed before 24 weeks' gestation, was crucial for optimizing maternal and fetal outcomes. Despite minimal ACTH staining, clinically the patient’s ACTH and cortisol were undetectable post-operatively, suggesting a possible rare co-secreting ACTH tumor, though phaeochromocytoma crisis remains the likely diagnosis. Genetic screening is recommended due to the familial nature of one-third of phaeochromocytoma cases.
Take-home Messages