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Brain Tumor, Pheochromocytoma, and Cesarean Section: A Case Report of a Parturient with von Hippel-Lindau Disease
Abstract Number: FC10-113
Abstract Type: Case Report Case Series
A 28 year-old G3P0020 female at 32w3d was urgently scheduled for cesarean section in the setting of a large cerebellopontine brain mass and symptomatic pheochromocytoma. She had been diagnosed recently with VHL at another institution after presenting with vomiting and imbalance. Work-up revealed multiple tumors including a 4.2cm right cerebellopontine angle mass with mass effect on adjacent brain stem with hydrocephalus, thoracic hemangioblastomas, and a left adrenal pheochromocytoma. Given her pheochromocytoma and preeclampsia with severe features, magnesium and alpha blockade with prazosin were continued. Due to concern for increased intracranial pressure (ICP), recommendations were against neuraxial anesthesia and pushing with vaginal delivery by a multidisciplinary team. General anesthesia was induced using a modified rapid-sequence induction with fentanyl, lidocaine, propofol, and rocuronium. Sevoflurane was used for maintenance and transitioned to propofol and remifentanil infusions after delivery. EtCO2 was controlled at 30mmHg and blood pressure maintained with a phenylephrine infusion. With fascial dissection, the patient became acutely hypertensive requiring a bolus of nitroprusside. Fetal delivery was uneventful and adequate uterine tone was achieved by oxytocin infusion and intramuscular carboprost. The patient was extubated and transferred to the ICU in stable condition, and had an uncomplicated postpartum hospitalization. Approximately one month after discharge, she underwent an urgent embolization and cerebellopontine mass excision.
Von Hippel-Lindau disease (VHL) is a rare autosomal dominant genetic disorder associated with the development of malignant and benign tumors in the central nervous symptom and viscera (1). There have been successful resections of symptomatic hemangioblastomas and pheochromocytomas during pregnancy, and even combined with cesarean section (2,3). To avoid the potential for hypertensive crisis and eclampsia associated with labor and pheochromocytoma, most patients undergo cesarean section. There are reports of successful neuraxial anesthesia with VHL: risks include possible rupture of hemangioblastomas or cerebellar herniation with dural puncture in the presence of increased ICP (4). Imaging of the brain and spine along with neurosurgical consultation should be arranged before placement. With general anesthesia, hyperventilation can decrease ICP and total intravenous anesthesia has more advantageous effects on cerebral circulation, ICP, and uterine tone over volatiles. These patients should undergo delivery at tertiary care centers as the key to this patient’s outcome was the coordination of a skilled multidisciplinary team.
1. Adekola et al. Obstet Gynecol Surv. 2013;68(9):655-662.
2. Boker et al. Can J Anaesth. 2001;48(4):387-390.
3. Joffe et al. Can J Anaesth. 1993;40(9):870-874.
4. Plon et al. Clinical features, diagnosis, and management of von Hippel-Lindau disease. UpToDate. 2017.