Intrapartum adrenalectomy for pheochromocytoma presenting in pregnancy: A case report

Abstract

Pheochromocytoma is one of the rare causes of hypertension in pregnancy, occurring in one in every 50,000 pregnancies. The aim of this case report is to describe an atypical presentation of pheochromocytoma at 35 weeks gestation, where it presented with right flank pain and seizures, its preoperative medical optimization, and intrapartum concurrent surgical management. It also discusses various clinical presentations of pheochromocytoma in pregnancy, appropriate laboratory and radiological investigations, and different modalities of treatment. It is important to consider the possibility of the diagnosis of pheochromocytoma when considering the different causes of high blood pressure in a term pregnancy. Prompt antenatal diagnosis and timely management are critical to reducing maternal and perinatal morbidity and mortality rates.

The Silent Killer Seeks Young Blood: A Case Report on the Perioperative Anesthetic Management of a Paraganglionoma in Pregnancy

“…A systematic review by Bancos et al [ 4 ] concluded that severe maternal complications and maternal death occurred in 7% of pregnancies with untreated PPGL. The main causes of maternal mortality include dysrhythmias, cerebrovascular incidents, and pulmonary edema [ 5 ]. The fetal mortality rate has been estimated at 26% in undiagnosed PPGL and 11% in diagnosed cases [ 4 ].…”

“…Recognizing PPGL in pregnancy is challenged by the low incidence of classic symptoms, such as paroxysmal headaches, diaphoresis, palpitations, and tachycardia [ 1 , 5 ]. Symptoms may appear for the first time during pregnancy or worsen during pregnancy, because of increased vascularity of the tumor and mechanical factors, such as pressure from the enlarging uterus, stimulating catecholamine secretion [ 6 ].…”

“…Hypertension during pregnancy is often attributed to gestational hypertension or pre-eclampsia, making the diagnosis of a PPGL more difficult [ 7 ]. In up to 50% of cases, the diagnosis of a PPGL is only made during the postpartum period [ 5 ]. When hypertension, often referred to as the "silent killer," is diagnosed in pregnancy before 20 weeks gestation, PPGL should be actively excluded as part of the workup for secondary causes of hypertension [ 1 ].…”

“…Ultrasound can sometimes be of clinical utility in finding suspected PGLs, but MRI and CT are typically needed for exact delineation prior to surgical management [ 11 ]. MRI provides superior imaging and is preferable to ultrasonography as a modality to localize the tumor in pregnancy while avoiding radiation exposure to the fetus [ 5 , 6 ]. Ultrasonography has a reported sensitivity of 89-97% for the detection of adrenal masses, while MRI additionally allows for the identification of extra-adrenal tumors [ 6 ].…”

“…When hypertension, often referred to as the "silent killer," is diagnosed in pregnancy before 20 weeks gestation, PPGL should be actively excluded as part of the workup for secondary causes of hypertension [ 1 ]. The classically described symptoms of paroxysmal headaches, diaphoresis, palpitations, and tachycardia are less common in pregnancy, challenging the consideration for PPGL [ 1 , 5 ]. Early diagnosis, localization of the tumor, and initiation of treatment are central to constructing the multidisciplinary perioperative management plan to ensure good maternal and fetal outcomes.…”