Secondary Logo

Journal Logo

Clinical letter

Epidural anaesthesia for Caesarean section in a patient with von Hippel-Lindau disease

Demiraran, Y.1; Özgön, M.2; Utku, T.1; Bozkurt, P.1

Author Information
European Journal of Anaesthesiology: May 2001 - Volume 18 - Issue 5 - p 330-332

Abstract

Introduction

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

Presentation

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.

Figure 1.
Figure 1.:
Sagittal magnetic resonance image, cystic cerebellar haemangioma.
Figure 2.
Figure 2.:
Horizontal magnetic resonance image, cystic cerebellar haemangioma.

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.

Discussion

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) [6]. 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 [8]. The majority of the lesions are located in the cerebellum and occur in about 60% [9], 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 [6]. Complications include blindness, progressive neurological impairment and death. The prevalence of this disease is estimated to be 1:35 000 to 1:40 000 [8].

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) [6]. 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 [4]. Ercan and his colleagues described the complications encountered during resection of cerebellar haemangioblastoma in a patient who also had an active phaeochromocytoma [16]. Wang and his colleagues [17] and Ogasawara and his colleagues [18] 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 [19].

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.

References

1 Bader AM. Neurologic and neuromuscular disease. In: Chestnut DH, ed. Obstetric Anaesthesia. Principles and Practice. St. Louis: Mosby, 1994: 920–941.
2 Santo AC, Petrovsky BM, Kaplan GP. Neurologic and muscular disease. In: Datta S, ed. Anaesthetic and Obstetric Management of High-Risk Pregnancy. St. Louis: Mosby, 1991: 135–168.
3 Grance C. Miscellaneous conditions. In: Gambling DR, Douglas MJ, eds. Obstetric Anaesthesia and Uncommon Disorders. Philadelphia: Saunders, 1998: 431–432.
4 Matthews AJ, Halshaw J. Epidural anaesthesia in von Hippel-Lindau disease: management of childbirth and anaesthesia for caesarean section. Anaesthesia 1986; 42: 853–855.
5 Joffe D, Robbins R, Benjamin A. Caesarean section and phaeochromocytoma resection in a patient with von Hippel-Lindau disease. Can J Anaesth 1993; 40: 870–874.
6 Martz DG, Shreibman DL, Matjasko MJ. Neurological diseases. In: Katz J, Benumof JL, Kadis LB, eds. Anesthesia and Uncommon Diseases, 3rd edn. Philadelphia, USA: Saunders 1996.
7 Decker HJH, Weidt EJ, Brieger J. The von Hippel-Lindau tumor suppressor gene: a rare and intriguing disease opening new insight into basic mechanisms of carcinogenesis. Cancer Genet Cytogenet 1997; 93: 74–83.
8 Choyke PLK, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau disease: genetic, clinical, and imaging features. Radiology 1995; 194: 629–642.
9 Nibbelink DW, Peters BH, McCormick WF. On the association of phaeochromocytoma and cerebellar hemangioblastoma. Neurology 1969; 19: 455–459.
10 Rawe SE, Van Gilder JC, Rothman SLG. Radiographic diagnostic evaluation and surgical treatment of multiple cerebellar, brain stem and spinal cord hemangioblastomas. Surg Neurol 1978; 9: 337–341.
11 Hes FJ, Feldberg MA. Von Hippel -Lindau disease. Strategies in early detection (renal, adrenal, pancreatic masses). Eur Radiol 1999; 9: 598–610.
12 Miyazaki T, Yamashita Y, Yoshimatsu S, Tsuchigame T, Takahashi M. Renal cell carcinomas in von Hippel Lindau disease; tumor detection and management. Comput Med Imaging Graph 2000; 24: 105–113.
13 Guinard JP, Mulroy MF, Carpenter RL, Knopes KD. Test doses: optimal epinephrine content with and without acute beta adrenergic blockade. Anesthesiology 1990; 73: 386–392.
14 Schoenwald PK, Whalley DG, Schluchter MD. The hemodynamic responses to an intravenous test dose in vascular surgery patients. Anesth Analg 1995; 80: 864–868.
15 Liu SS, Carpenter R. Hemodynamic responses to intravascular injection of epinephrine containing epidural test doses in adults during general anesthesia. Anesthesiology 1996; 84: 81–87.
16 Ercan M, Kahraman S, Basgul E, Aypar U. Anaesthetic management of a patient with von Hippel-Lindau disease: a combination of bilateral phaeochromocytoma and spinal cord haemangioblastoma. Eur J Anaesthesiol 1996; 13: 81–83.
17 Ogasawara KK, Ogasawara EM, Hirata G. Pregnancy complicated by von Hippel-Lindau disease. Obstet Gynecol 1995; 85: 829–831.
18 Wang A, Sinatra RS. Epidural anaesthesia for caesarean section in a patient with von Hippel-Lindau disease and multiple sclerosis. Anesth Analg 1999; 88: 1083–1084.
19 Kuhnigk H, Danhauser-Leistner I. Caesarean section with subsequent craniotomy in the area of the posterior cranial fossa. Anaesthesiol Intensivmed Notfallmed Schmersther 1994; 29: 184–187.
Keywords:

NERVOUS SYSTEM DISEASES; NEUROCUTANEOUS SYNDROMES; von Hippel-Lindau disease; NEOPLASMS; VASCULAR TISSUE; haemangioma; HAEMANGIOMA; CAPILLARY; haemangioblastoma; anaesthesia; epidural anaesthesia; cerebellar haemangioblastoma.

© 2001 European Academy of Anaesthesiology