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From the Department of Anesthesia, University of Manitoba, Winnipeg, Manitoba, Canada.
Address Correspondence to: Dr. Bill Y. Ong, Department of Anesthesia, University of Manitoba, LB 315 – 60 Pearl Street, Winnipeg, Manitoba, R3E 1X2, Canada. Phone: 204-787-3440; Fax: 204-787-4291; Email: ong{at}cc.umanitoba.ca
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Clinical features: A 30-yr-old primigravida with VHLD at 35 weeks gestation was seen at the obstetric anesthesia clinic because she wished an epidural analgesia during labour. She had a history of headaches and dizziness. Further investigations showed an enlarged cerebellar hemangioblastoma with significant local mass effects. A combined Cesarean section delivery and posterior fossa craniotomy was performed at 37 weeks gestation. A general anesthetic with fentanyl, rocuronium, nitrous oxide, oxygen and isoflurane was given for Cesarean section delivery. After delivery, isoflurane was reduced and propofol infusion at 4–8 mg•kg–1•hr–1 was initiated. The patient had an uneventful operative course and recovery.
Conclusions: Patients with VHLD may have worsening of preexisting lesions or develop other lesions during pregnancy. Some asymptomatic lesions can increase the risk for anesthesia complications. These patients need comprehensive assessment before administration of anesthesia.
MANY patients with von Hippel-Lindau disease (VHLD) have hemangioblastomas of the cerebellum and spinal cord. While many patients have completed pregnancies without any complication, some develop new symptoms or have exacerbations of preexisting lesions during pregnancy.1 A 30-yr-old primigravida with VHLD at 35 weeks gestation was found to have an enlarged cerebellar hemangioblastoma with significant local mass effects. There were evidence of uncal and trans-tentorial herniation as well as displacement of the brain stem. Due to the life threatening neurological condition, a craniotomy was deemed necessary at 37 weeks gestation. After a full discussion by the perinatal and surgical teams and with the consent of the patient, a Cesarean section delivery and a craniotomy were performed. We report the assessment and anesthetic management of this patient with VHLD and an intracranial mass lesion for Cesarean section delivery and posterior fossa craniotomy.
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). The largest, about 2 cm in diameter, was in the inferior aspect of the left cerebellar hemisphere and had mixed cystic and solid composition. There was no significant mass effect at that time.
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At 36 weeks gestation, a repeat MRI scan of the brain and the spine showed multiple hemangioblastomas. The cystic component of the left cerebellar hemisphere lesion had increased in size to 5.5 cm in diameter (Figure.2). There was a significant local mass effect resulting in left-sided tonsillar herniation (Figure 3), mild upward trans-tentorial herniation of the superior cerebellar vermis, and displacement of the brain stem towards the right and anteriorly. The patient's clinical symptoms and radiographic findings indicated that the neurologic condition might be life threatening and urgent neurosurgical intervention was needed. The perinatal and neurosurgical teams discussed the options with the patient. A combined Cesarean section delivery and posterior fossa craniotomy at 37 weeks gestation was agreed on.
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A male baby with Apgar Scores of 5, 7, 9 at one, five, ten minutes was delivered three minutes after induction of anesthesia. The baby required one dose of im naloxone (0.04 mg) for weak respiratory efforts two minutes after delivery.
After delivery of the infant, the patient received ampicillin 2 gm, midazolam 2 mg, and sufentanil 15 µg iv. An iv infusion of oxytocin 40 units•L–1 was initiated at a rate of 100 ml•hr–1.
After closure of the uterus and abdomen, the patient was positioned in a three-quarter prone (park bench) position. Mannitol 40 gm and dexamethasone 10 mg were administered. Isoflurane was reduced to 0.4% (ET) and a propofol infusion was initiated at a rate of 4–8 mg•kg–1•hr–1, to minimize the risk of postpartum hemorrhage. Initially, the brain was tense and the neurosurgical dissection was difficult because of the three previous craniotomies. The largest cyst in the left cerebellar hemisphere was drained and resected. The estimated blood loss for the two procedures was 1200 ml, of which 400 ml were lost during the craniotomy. At the end of surgery, the patient was turned to the supine position. Neuromuscular block was reversed with neostigmine 3 mg, and glycopyrolate 0.6 mg. After transfer to the postanesthesia care unit, the propofol infusion was stopped and the patient received fentanyl 50 µg, as well as lidocaine 80 mg. Within ten minutes, the patient was awake and the endotracheal tube was removed without any coughing. The postoperative course was uneventful.
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The retinal and central nervous system (CNS) hemangioblastomas are vascular units without endothelial tight junctions, so they leak plasma and blood. In 80% of the cerebellar lesions, the tumours are nodules in the wall of cysts. Some CNS lesions progress rapidly over a period of 2–4 yr, others progress slowly over many years, and some even become static.3 The retinal tumours are associated with retinal exudate.4 Even with early and aggressive treatment, as in our case, the retinal lesions can lead to glaucoma and blindness.
Most of the patients with VHLD have no problems during pregnancy but some may have worsening of existing lesions or develop new lesions. Grimbert et al.,5 surveyed 30 women with VHLD, who completed 56 pregnancies. While most of the pregnancies had a favourable outcome, three of 56 pregnancies had complications. One patient developed intra-cranial hypertension at 32 weeks gestation from her cerebellar hemangioblastoma. The second patient developed eclampsia associated with pheochromocytoma at 32 weeks gestation. The third patient developed acute abdominal pain at 34 weeks gestation related to pancreatic cystadenoma. Joffe et al.1 reported a 35-yr-old parturient with VHLD. She was diagnosed with pheochromocytoma at 22 weeks gestation, and required treatment with phenoxybenzamine and propranolol during her pregnancy. Ogasawara et al.3 described a 23-yr-old patient who presented at 35 weeks of gestation with paraplegia secondary to acute intramedulary hemorrhage from a spinal hemangioma at the thoracic (T) 4–5 level. The patient had Cesarean section delivery under epidural anesthesia despite the presence of an asymptomatic lesion at T7–8 level. Further prospective studies are needed to determine how often complications develop during pregnancy in patients with VHLD.
Case presentation
The enlargement of the cerebellar lesion in our patient was an unexpected development. The extensive bilateral retinal lesions made it difficult to assess the presence of papilledema. The diagnosis was made with high index of suspicion and MRI.
Despite the patient's initial wish for a vaginal delivery, the medical staff were concerned about the stress of labour, fluctuations in blood pressure and further increases in intracranial pressure. With the symptoms of intracranial mass effect at 36 weeks, neurosurgical intervention was needed. One option was to perform the craniotomy, monitor the fetus in the perioperative period and deal with labour and/or complication if that occurred. We were concerned about the possible adverse effects of a prolonged anesthetic and surgery, as well as hemodynamic and metabolic changes on the fetus. If labour started during the craniotomy or within a short time after the craniotomy, the mother might suffer more complications. After full discussion by the perinatal and neurosurgical teams, and with the patient's consent, a combined Cesarean section delivery and posterior fossa craniotomy were performed.
Anesthetic management
The usual rapid sequence induction for general anesthesia in pregnant patient may not be tolerated in patients with raised intracranial pressure.6 We used a non-depolarizing agent to achieve muscular relaxation in two to three minutes, while ventilating the lungs during that time. This allowed an appropriate depth of anesthesia with isoflurane, and maintained low arterial carbon dioxide level. The patient's favourable airway, absence of symptoms of gastro esophageal reflux, preoperative administration of ranitidine, non-particulate antacid and metoclopramide, and application of cricoid pressure decreased the risk of pulmonary aspiration. Bédard et al.7 reported on anesthesia for Cesarean section for a parturient with an acoustic neuroma compressing the pons, midbrain and fourth ventricle. The authors applied cricoid pressure as consciousness was lost and ventilated the patient's lungs with positive pressure by mask before tracheal intubation. There is little evidence in the literature to indicate whether any approach is preferable.
In summary, patients with VHLD may have worsening of preexisting lesions or develop other lesions during pregnancy. Some asymptomatic lesions can increase the risk of anesthesia complications. These patients need a comprehensive assessment before the administration of anesthesia.
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Accepted for publication December 18, 2000.
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2
Wang A, Sinatra RS. Epidural anesthesia for cesarean section in a patient with von Hippel-Lindau disease and multiple sclerosis. Anesth Analg 1999; 88: 1083–4.
3 Ogasawara KK, Ogasawara EM, Hirata G. Pregnancy complicated by Von Hippel-Lindau disease. Obstet Gynecol 1995; 85(part 2): 829–31.[Abstract]
4 Wing GL, Weiter JJ, Kelly PJ, Albert DM, Gonder JR. von Hippel-Lindau disease. Angiomatosis of the retina and central nervous system. Ophthalmology 1981; 88: 1311–4.[Medline]
5 Grimbert P, Chauveau D, Richard S, Rémy SRP, Grünfeld JP. Pregnancy in von Hippel-Lindau disease. Am J Obstet Gynecol 1999; 180: 110–1.[Medline]
6 Walls RM. Rapid-sequence intubation in head trauma. Ann Emerg Med 1993; 22:1008–13.[Medline]
7
Bédard JM, Richardson MG, Wissler RN. General anesthesia with remifentanil for Cesarean section in a parturient with an acoustic neuroma. Can J Anesth 1999; 46: 576–80.
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  L. P. Wang and M. J. Paech Neuroanesthesia for the Pregnant Woman Anesth. Analg., July 1, 2008; 107(1): 193 - 200. [Abstract] [Full Text] [PDF]
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