Brain Tumor, Pheochromocytoma, and Cesarean Section: A Case Report of a Parturient with von Hippel-Lindau Disease
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, including pheochromocytoma1. Clinical manifestations develop on average in one’s mid-20s, coinciding with common childbearing years in women. The anesthetic management of the VHL parturient presents particular challenges due to possible raised intracranial pressure (ICP), spinal tumors, and hypertensive crises.
To date, there is limited literature on the diagnosis and prognosis of symptomatic VHL during pregnancy. We present a unique case of a young woman first diagnosed with VHL during her pregnancy who proceeded to undergo cesarean section in the setting of multiple classic VHL tumors, including a large cerebellopontine brain mass and pheochromocytoma.
A 28 year-old gravida 3 para 0 female at 12 weeks 3 days gestational age initially presented to an outside hospital with fever, vomiting, and gait imbalance. The work-up revealed an adrenal pheochromocytoma along with tumors in the brain, pancreas, liver, spine, and kidneys. She was diagnosed with VHL by tumor burden and positive VHL genetics panel. Alpha blockade with prazosin was initiated for pheochromocytoma management and she was initially doing well. While traveling at 32 weeks gestation, she presented to another hospital with vomiting, severe hypertension, and elevated liver enzymes, and she was transferred to our institution for tertiary care.
During the admission workup, a pan-MRI confirmed her prior known tumors, including a 4.2cm cerebellopontine angle mass, multiple thoracic hemangioblastomas, and an adrenal nodule consistent with pheochromocytoma (Figure 1). She was continued on prazosin for blood pressure control and began magnesium for a diagnosis of preeclampsia. She was evaluated by neurosurgery, urology, surgical oncology, endocrine surgery, endocrinology, and ophthalmology. Although the MRI brain showed significant mass effect on adjacent brain stem and cerebellum with developing supratentorial hydrocephalus, neurosurgery did not want to surgically intervene immediately as she was neurologically intact. They did advise against neuraxial anesthesia and pushing with vaginal delivery due to concern for further increase in ICP. Given her overall worsening clinical status, the decision was made for cesarean section at 32 weeks 3 days gestation under general anesthesia.
On physical examination, the patient had a reassuring airway with a Mallampati II, adequate neck extension, and no significant cardiopulmonary compromise. The patient was brought to the operating room, confirmed to be appropriately NPO, and given sodium citrate. Intravenous access was obtained and a pre-induction arterial line was placed. After a modified rapid-sequence induction with fentanyl, lidocaine, propofol, and rocuronium, the patient was easily intubated with cricoid pressure, indirect laryngoscopy, and a standard 7.0 cuffed endotracheal tube. General anesthesia was maintained with sevoflurane until delivery, and transitioned to propofol and remifentanil infusions for the remainder of the procedure. Magnesium was continued throughout the procedure and per neurosurgery recommendations, a goal of ETCO2 of 30mmHg was maintained. A phenylephrine infusion was used to maintain blood pressure while under general anesthesia; however with fascial dissection immediately prior to uterine incision, the patient became acutely hypertensive requiring a bolus of nitroprusside. Fetal delivery was uneventful and adequate uterine tone was achieved with 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 presented to an outside hospital with seizures, altered mental status, and hydrocephalus, requiring an urgent embolization and craniotomy for cerebellopontine mass excision. The patient and infant are currently doing well at home with multidisciplinary outpatient follow-up for multiple VHL tumors.
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 viscera1.Most cases are diangosed by a germline mutation, and the evidence of accelerated tumor growth during pregnancy is controversial. While overall fetal and maternal outcomes in VHL-related pregnancies are favorable, careful surveillance during pregnancy is recommended to assess for hemiangioblastoma growth in the central nervous system and development of pheochromocytoma that may affect delivery and anesthetic planning. If symptomatic, there have been successful resections of hemangioblastomas and pheochromocytomas during pregnancy, even combined with cesarean section2,3. If pheochromocytoma is detected during pregnancy, most patients undergo cesarean section in order to avoid the potential for hypertensive crisis associated with labor and pheochromocytoma.
There are no guidelines in the literature for providing obstetrical anesthesia in VHL patients. Since VHL is associated with multiple tumors, patient-specific risk factors and tumors are used to guide management. While 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 ICP4. Imaging of the brain and spine is recommended when an intracranial or neuraxial mass is known, or increased ICP is suspected. With general anesthesia, hyperventilation can decrease ICP and allow for combined procedures; however careful planning to avoid hemodynamic changes, ICP increases, and minimize the risk of difficult airway and aspiration is needed. 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.