Von Hippel-Lindau Syndrome (VHLS) is a rare autosomal dominant disease with incomplete penetrance and variable expression. The characteristic lesion is the capillary hemangioblastoma of the retina (60%-70% of patients) or central nervous system (CNS) (30%-70% of patients). The majority of the CNS lesions are located in the cerebellum. Renal and pancreatic cysts, hypernephroma, erythrocytosis, and pheochromocytoma (often bilateral) are also associated with the disease . This case report describes the anesthetic management of a patient admitted for excision of cerebellar mass who was incidentally found to have VHLS with bilateral pheochromocytoma.
A 22-yr-old man weighing 32 kg presented with a 5-mo history of headache and vomiting, along with blurring of vision and unstable gait for 1 mo. Headache was paroxysmal and not associated with sweating. Physical examination revealed a heart rate of 80 bpm, arterial blood pressure of 180/130 mm Hg, gross papilledema, and bilateral cerebellar signs. Routine laboratory examination revealed a hemoglobin value of 19.3 gm/dL and a hematocrit value of 60%. Chest radiograph was normal. Electrocardiogram showed normal sinus rhythm with left ventricular hypertrophy. Two-dimensional echocardiogram was normal. Computerized tomography of the brain revealed a large cystic lesion with enhancing mural nodule in the right cerebellum and two pinhead-sized nodular lesions each in either cerebellar hemispheres, which were suspected to be hemangioblastomas. There was also moderate ventriculomegaly.
Further investigations were performed to determine the cause of hypertension. An ultrasound scan of the abdomen revealed bilateral adrenal masses confirmed by computerized tomography. Twenty-four-hour urinary vanilylamandelic acid was 20 mg (normal < 8 mg). Clinical diagnosis of VHLS was made. Phenoxybenzamine 10 mg twice daily and nifedipine 10 mg twice daily were started. A right frontal reservoir was placed on the fifth day of admission for repeated aspirations of cerebrospinal fluid to treat increased intracranial tension. Despite these conservative measures for 36 h, the patient's condition did not improve, and emergency occipital craniectomy was planned. The preoperative hematocrit value was 53%, and arterial blood pressure was 160/120 mm Hg, whereas the heart rate was 58 bpm. Anesthesia was induced with meperidine 30 mg, thiopental 4 mg/kg, and lidocaine 80 mg. Vecuronium 6 mg was administered to facilitate endotracheal intubation. Four minutes before endotracheal intubation, nitrous oxide 50% and isoflurane 0.5% in 50% oxygen were administered via a face mask. Anesthesia was maintained with nitrous oxide 66%, oxygen 33%, and 0.25%-0.5% isoflurane. An epidural catheter was placed at the L3-4 interspace and advanced 5 cm cephalad. A mixture of 10 mL of 2% lidocaine and 10 mL of 0.5% bupivacaine was administered through the catheter. Fifteen minutes later, the arterial blood pressure was 140/90 mm Hg. The patient was placed in the prone position, avoiding any compression over the abdomen. Intraoperatively, usual monitoring was used, including central venous pressure via a catheter passed through an antecubital vein. The patient was hyperventilated to maintain end-tidal CO2 at 25-30 mm Hg. The surgery lasted for 6 h. An encapsulated mass in the right cerebellum was totally excised. The estimated blood loss was 600 mL. One unit (300 mL) of whole blood and 3 L of crystalloid were administered. The patient's postoperative course was uneventful.
Two weeks later, Roizen's  criteria for optimal preoperative preparation for pheochromocytoma surgery were satisfied. These criteria are: no in-hospital blood pressure reading higher than 165/90 mm Hg should be evident before surgery; orthostatic hypotension should be present, but blood pressure on standing should not be lower than 80/45 mm Hg; electrocardiogram should be free of ST-T changes; no more than one premature ventricular contraction should be present every 5 min. The patient underwent bilateral adrenalectomy under combined epidural and balanced general anesthesia (oxygen, nitrous oxide, and isoflurane) with monitoring as indicated in the first surgery. Postoperatively, the patient had persistent hypokalemia for 3 days, which was corrected by IV potassium replacement.
At the time of discharge from the hospital, the patient was normotensive and was receiving steroid replacement therapy. Histopathological examination of surgical specimens revealed cerebellar hemangioblastoma and well circumscribed pheochromocytomas of the adrenals.
A MEDLINE literature search did not reveal the anesthetic management of a patient with VHLS presenting with a scenario as reported in this case. Tempelhoff and Modica  described anesthetic management of a patient with multiple posterior fossa tumors and an active pheochromocytoma. After adequate preoperative preparation with phenoxybenzamine followed by the addition of oral propranolol, the patient was scheduled for excision of cerebellar hemangioblastomas. After the induction of anesthesia, the patient developed cardiac arrest after an attempt at pulmonary artery catheterization. After successful cardiopulmonary resuscitation, surgery was postponed and performed on the following day in prone position under balanced general anesthesia consisting of nitrous oxide 70%, oxygen 30%, isoflurane 0.25%, 1 g of thiopental total, sufentanil, and vecuronium. Intraoperative control of blood pressure required the use of phentolamine and labetalol infusions. Two weeks later, the patient underwent pheochromocytoma surgery uneventfully . Two cases describing the anesthetic management of VHLS patients undergoing cesarean section have been reported in the literature. Matthews and Halshaw  described a patient who previously underwent an operation for cerebellar hemangioblastoma and presented for cesarean section. Lumbar epidural blockade was used. Joffe et al.  described a pregnant patient who was prepared with phenoxybenzamine and propranolol since 27th week to control hypertension secondary to pheochromocytoma. General anesthesia was administered for elective cesarean section combined with pheochromocytoma surgery. After delivery of the baby by cesarean section, pheochromocytoma surgery was performed. Hypertension during surgery was controlled using sodium nitroprusside, labetalol, and esmolol.
This case, therefore, presents a unique anesthetic challenge. The essential dilemma was whether to perform intracranial surgery or pheochromocytoma surgery separately or simultaneously. If the intracranial surgery was performed first, exaggerated hypertensive responses may have caused excessive bleeding during or after surgery from the tumor bed. If adrenalectomy was performed first, the vasodilators (sodium nitroprusside, phentolamine) used to control hypertensive responses may have further increased intracranial tension pressure. Even the use of inhaled drugs such as enflurane or isoflurane can increase intracranial pressure by cerebral vasodilatory action. Because the goals of intracranial surgery (i.e., control increased intracranial pressure) and pheochromocytoma (i.e., control raised blood pressure) are different, the option of performing both surgeries at the same time does not seem to be a reasonable choice. Even if both procedures are performed simultaneously, the problem of which one to perform first arises because surgical positions are different (prone, sitting, or lateral for cerebellar surgery and supine for pheochromocytoma surgery).
Intracranial surgery after stabilization with phenoxybenzamine and nifedipine followed by pheochromocytoma surgery was planned for another time. Because the patient deteriorated neurologically, emergency excision of cerebellar mass was contemplated. The choice of position during surgery caused yet another problem. Hemodynamic responses caused by the sitting position may not be tolerated by the patient, and there is the potential for abdominal compression in the prone position, leading to excessive endocrine discharges from the adrenal glands. The prone position was preferred in this case, and we paid particular attention to avoid any undue compression over the abdomen. The rationale of epidural blockade for neurosurgery was to avoid IV vasodilators and to avoid the use of higher concentration of isoflurane, which may have further increased the preexisting increased intracranial tension.
1. Rengachary SS, Blount JP. Hemangioblastomas. In: Wilkins RH, Rengachary SS, eds. Neurosurgery. New York: McGraw Hill, 1996:1205-19.
2. Roizen MF. Anesthetic implications of concurrent diseases. In: Miller RD, ed. Anesthesia. New York: Churchill Livingstone, 1994:903-1014.
3. Tempelhoff R, Modica PA. Anesthetic management of a patient with multiple posterior fossa tumors and an active pheochromocytoma: case report and review of the literature. Anesth Rev 1988;25:13-20.
4. Matthews AJ, Halshaw J. Epidural anaesthesia in von Hippel-Lindau disease: management of childbirth and anaesthesia for caesarean section. Anaesthesia 1986;41:853-5.
5. Joffe D, Robbins R, Benjamin A. Caesarean section and phaechromocytoma resection in a patient with von Hippel Lindau disease. Can J Anaesth 1993;40:870-4.