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A Parturient with a Possible Pheochromocytoma, Von Hippel-Lindau Disease and Preeclampsia
Abstract Number: T 55
Abstract Type: Case Report/Case Series
Intro: Pheochromocytoma (PHEO) in pregnancy is rare and hard to differentiate from chronic hypertension (HTN) or preeclampsia (PEC) (1). PHEOs can be associated with several genetic syndromes. Von Hippel-Lindau (vHL) disease is an autosomal dominant syndrome that causes neuroendocrine tumors and CNS hemangioblastomas (2).
Case: A 37-year-old, morbidly obese (BMI 42) G2P1 woman at 33 weeks gestation presented with a possible PHEO. Family history (FH) was a brother and two great uncles who died of intracranial aneurysms. Early in pregnancy, she was diagnosed with HTN, and treated with labetalol. At 31 weeks, her blood pressure increased, PEC was ruled out, and she had slight elevations in urinary norepinephrine and metanephrine. At 32 5/7 weeks, she was admitted to an outside hospital with worsening HTN. Due to the concern for PHEO she was started on phenoxybenzamine and transferred to our institution. Her paroxysmal HTN required labetelol, hydralazine, nifedipine and a nicardipine infusion. An arterial line was placed, the phenoxybenzamine continued, and propranolol added two days later. She soon developed proteinuria, was diagnosed with severe PEC, and magnesium was initiated.
Abdominal sonogram did not reveal an adrenal tumor but could not exclude an extraadrenal PHEO. Due to her FH, vHL syndrome was suspected. Due to severe PEC, the plan was for CS after only one week of alpha blockade, but at 33 6/7 weeks, her LFTs rose, and the patient underwent emergency CS after MRI confirmed the absence of lumbar spinal tumors. A 7Fr peripheral IV catheter was placed, and a CSE with a spinal dose of bupivacaine 12mg, fentanyl 15mcg, and morphine 0.2mg was performed. Intraoperative hypotension was treated with phenylephrine and vasopressin. There were no hypertensive episodes intraoperatively, and a live 1995g infant with Apgars 7, 8 was delivered. The epidural catheter was not used. She was discharged home on phenoxybenzamine and propranolol, and we are awaiting further workup for PHEO.
Discussion: A multidisciplinary team consisting of MFM, endocrinology, cardiology and obstetric anesthesiology was invaluable for this patient. Establishing the diagnosis of PHEO in the setting of pregnancy and PEC is challenging. While we wanted to postpone delivery until sympathetic blockade was adequate (classically 2 weeks), worsening PEC necessitated an earlier delivery. The risks of performing neuraxial analgesia on a patient with possible CNS hemangioblastomas include rupture of the hemangioblastoma, spinal canal bleeding and cerebral herniation resulting from dural puncture if intracranial pressure (ICP) is elevated. However, the risks of general anesthesia with PHEO include large swings in blood pressure and ICP resulting in cerebral hemorrhage (2). MRI confirmed the absence of CNS pathology in our patient, and CSE was performed when signs of end organ damage from PEC were present.
1.Biggar MA. Br J Surg 2013
2.McCarthy T. Int J Ob Anesth 2010