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Management of a parturient with known MEN type 2A and recurrence of Pheochromocytoma during pregnancy
Abstract Number: F-60
Abstract Type: Case Report/Case Series
MEN 2A in pregnancy is rare with limited case reports in literature. There are no consensus guidelines regarding anesthetic technique for managing parturient with untreated Pheochromocytoma. We report a case of successful management of a pregnant patient undergoing Caesarean section with known MEN 2A with recurrence of Pheochromocytoma.
30 y/o G2P1001 white female 38 weeks of gestation with known history of MEN type 2A and recurrence of Pheochromocytoma presents for anesthetic consultation regarding her delivery. She was diagnosed with MEN type 2A seventeen years ago when she presented with medullary thyroid cancer and consequently had a total thyroidectomy at age fourteen and later developed a right Pheochromocytoma and had a right adrenalectomy at age twenty one. During the first trimester of this pregnancy, surveillance testing of urine metanephrines and catecholamines were noted to be elevated. MRI showed a well circumscribed right adrenal mass confirming a recurrent pheochromocytoma. She was prescribed alpha blockade with phenoxybenzamine but was non compliant with treatment plan and follow up with her endocrinologist. In spite of her non-compliance, she surprisingly remained normotensive during her pregnancy with no episodic hypertensive events. Upon presentation to outside hospital at 38 weeks gestational age, she was transferred to our tertiary referral hospital for optimal management. A multidisciplinary team approach was initiated that included MFM obstetrician , endocrinologist, endocrine surgeon, and obstetric anesthesiologist. A consensus decision was made to proceed with elective Caesarean section with minimal uterine manipulation during delivery to avoid surge of catecholamines with subsequent resection of Pheochromocytoma at 6-8 weeks postpartum. Neuraxial anesthesia with combined spinal epidural technique was chosen with arterial line for close hemodynamic monitoring along with use of transthoracic echo to guide fluid management. During the surgery, the patient had remained hemodynamically stable without need for major pharmacological intervention and a healthy baby was delivered. She had an uneventful postoperative course and was discharged home five days after the procedure with instruction and follow up care planned with endocrine surgery and endocrinology in anticipation for adrenalectomy at 8 weeks postpartum.
Untreated Pheochromocytoma during pregnancy can potentially be disastrous with catastrophic events to both mother and fetus leading to significant morbidity and mortality. A multidisciplinary approach considering all variables and treatments is a key to a successful outcome for these rare cases.