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Management of the Parturient with Pheochromocytoma and Paraganglioma: a review of 21 patients
Abstract Number: T110-512
Abstract Type: Original Research
Introduction: Management of parturients with pheochromocytoma or metabolically active paraganglioma presents a challenge to the anesthesiologist. Presentation during pregnancy is rare and diagnosis can be difficult as it can mimic other causes of hypertension in pregnancy. However, antenatal diagnosis is crucial for improved maternal and fetal outcomes as undiagnosed pheochromocytoma has high mortality for both mother and fetus.1 Surgical management is dependent on gestational age at time of diagnosis, tumor location, and characteristics.2 While most patients will have pheochromocytoma resection concomitant with delivery or post-partum, patients who fail medical management may require resection during pregnancy.3
Methods: Patients with pheochromocytoma or paraganglioma in pregnancy were identified from our medical records database. Patient demographics, timing of diagnosis, treatment during pregnancy, anesthetic management during labor, postpartum recovery location, and maternal and fetal outcomes were collected.
Results: Twenty-one distinct patients who had 40 pregnancies were included. Six patients were diagnosed during pregnancy, 7 were diagnosed postpartum, and 8 were diagnosed prior to pregnancy. Of the 8 diagnosed prior to pregnancy, 3 had pre pregnancy attempts at resection. Of the patients diagnosed prior to pregnancy or antepartum, 1 had resection of the tumor during pregnancy, 1 had resection at the time of delivery, and 13 had resection postpartum. One patient diagnosed pre pregnancy had an emergent cesarean delivery (CD) for refractory hypertension. One woman had a previable delivery for severe hypertension uncontrolled with medication. Of the patients diagnosed in the postpartum period, all 7 had symptoms during pregnancy and 3 were diagnosed with preeclampsia with severe features, representing potential premature diagnostic closure. Two of the patients diagnosed in the postpartum period had emergent CD for refractory severe hypertension. Postpartum, 8 women were observed in the intensive care unit (ICU) and 1 developed cardiogenic shock. Two women have since died from their disease.
Discussion: Management of pheochromocytoma and paraganglioma in pregnancy and peri-delivery requires a patient-specific plan designed by a multi-disciplinary team, involving obstetric anesthesiology, maternal fetal medicine, general surgery, and endocrinology. Patients often present on vasoactive medications, may need invasive monitoring, and are at high risk for morbidity and mortality. Tumor location and timing of diagnosis is key to determining risk of anesthetic complication at time of delivery. Late diagnosis and resulting delay in appropriate treatment resulted in worse outcomes, such as emergency delivery, ICU admission, and, in 1 case, cardiogenic shock.
1.Harrington JL. World J Surg (1999) 23:182–186
2.Biggar MA. Br J Surg. (2012) 100:182-190
3.Donatini G. Surg Endosc (2018) 32:3890-3900