Recommendations regarding the use of spinal and epidural anaesthesia for patients with von Hippel-Lindau disease are unclear [1,2,3] and even contradictory [4,5] as a result of the possible presence of vascular malformations (haemangioblastomas) in the spinal cord, retinas and other parts of the central nervous system. We report a patient with von Hippel-Lindau disease (cerebellar cystic haemangioblastoma and retinal haemangioblastoma) in whom Caesarean section was successfully managed with epidural anaesthesia
A 23-year-old female patient, gravida 1, parturition 0 (first pregnancy), presented for Caesarean section at 38 weeks' gestation. For 4 years she had been under the care of an ophthalmologist because of blindness in the right eye due to detachment of her retina as a result of a haemangioblastoma in that eye. Magnetic resonance imaging showed haemangioblastomas in both retinas and cerebellum; a renal cyst was also revealed. We decided to perform epidural anaesthesia with sedation to permit Caesarean section. Informed consent was obtained from the patient and we explained that a regional block might cause disruption of her haemangioblastomas.
The patient's vital signs were normal and no impairment of the cardiovascular or respiratory systems was detected; airway examination was normal. The patient was blind in her right eye with a detached retina. There was a large cystic (5 × 5 cm) haemangioblastoma in the cerebellum (Figures 1 and 2). Radiography showed there were no lesions in the spinal cord and there was no evidence of tumour blocking the flow of cerebrospinal fluid as cerebral fluid flow dynamics were within normal limits.
Preoperative blood pressure was 120/80 mmHg, heart rate 75 beats min−1. Electrocardiography (ECG) showed normal sinus rhythm; haemoglobin concentration was 90 g dL−1, haematocrit 34%, glucose 4.3 mmol dL−1 and plasma electrolyte and protein concentrations, liver enzymes and blood urea were within normal limits.
Midazolam 1 mg intravenously (i.v.) was given before insertion of an epidural catheter. The patient received oxygen by facemask, and monitoring consisted of pulse oximetry, non-invasive arterial pressure and electrocardiogram. Hartmann's solution 500 mL was given to preload the circulation. A 20-gauge catheter was inserted for a distance of 3 cm into the epidural space via a 17-gauge Tuohy needle placed at the L3-4 interspace. A test dose of 3 mL lidocaine 2% with epinephrine 1:200 000 was given through the catheter; incremental doses of lidocaine were given to produce anaesthesia to the T5 level. Caesarean section was then performed and a healthy infant delivered with Apgar scores of 8 and 9 at 1 and 5 min, respectively. Both mother and baby had an uncomplicated postoperative course and were discharged home. Two months' later the patient underwent neurosurgery to resect the lesion in the brain.
Von Hippel-Lindau disease is a rare autosomal dominant disease with incomplete penetrance and variable expression. The characteristic lesion is capillary haemangioblastomas in the retinas (60–70% of patients) or in other parts of the central nervous system (30–50% of patients) . It is also associated with renal cell carcinoma, pancreatic cyst and tumours, and phaeochromocytoma [7,8]. The von Hippel-Lindau disease gene seems to be a tumour suppressive gene with loss of function leading to unchecked cell growth and tumourigenesis . The majority of the lesions are located in the cerebellum and occur in about 60% , and in the brain stem in 14–50%, of patients with von Hippel-Lindau disease. Accessible lesions are removed surgically [9,10]. There is a 3–10% recurrence rate after an apparent complete excision. Computerized axial tomography scanning, magnetic resonance imaging and angiography are the imaging techniques of choice, although lesions may be missed [10–12]. Renal and pancreatic cysts, hypernephroma, erythrocytosis, and phaeochromocytoma (often bilateral) are also associated with the disease . Complications include blindness, progressive neurological impairment and death. The prevalence of this disease is estimated to be 1:35 000 to 1:40 000 .
We were particularly concerned with the possibility of intracerebellar lesions in this woman and the risk of a cerebrovascular accident induced by a rise in arterial pressure. No specific anaesthetic agents are contraindicated in von Hippel-Lindau disease, and so anaesthetic management should be tailored according to any relevant findings (such as phaeochromocytoma or intracranial lesions) . We used epinephrine as a component of the epidural test dose in this normotensive patient because in our and others' experience any inadvertent intravascular injection of epinephrine 15 µg is likely to cause an increase in mean arterial pressure of only about 15–20 mmHg, and the drug mainly affects the heart rate [13–15]. The consequences of systemic lidocaine injection would have more detrimental effects both for the baby and the mother.
There are few reported cases of the anaesthetic management of patients with von Hippel-Lindau disease. Matthews and his colleagues described the successful use of epidural anaesthesia for elective Caesarean section in a patient who had a previous resection of cerebellar haemangioblastoma . Ercan and his colleagues described the complications encountered during resection of cerebellar haemangioblastoma in a patient who also had an active phaeochromocytoma . Wang and his colleagues  and Ogasawara and his colleagues  described the successful use of epidural anaesthesia for Caesarean section in patients with von Hippel-Lindau disease. Kuhnigk and his colleagues also reported concomitant surgery for a haemangioblastoma and Caesarean section in a patient with von Hippel-Lindau disease .
We believe that the anaesthetic technique for this condition should be chosen on an individual basis. In our pregnant patient with vascular malformations of the cerebellum and retinas, the epidural technique avoided the need for general anaesthesia and the pressor effects of laryngoscopy.
In conclusion, epidural anaesthesia was successfully performed in a patient with von Hippel-Lindau disease and cerebellar cystic haemangioblastoma without neurological sequelae. Epidural anaesthesia need not be excluded in patients with von Hippel-Lindau disease based solely on this diagnosis: the choice of anaesthesia technique should be made after careful evaluation of the extent of the patient's disease, including a review of radiological studies of the central nervous system. Consideration needs to be given to the nature of surgical procedure, the circumstances surrounding the surgery and to the patient's wishes.
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