Epidural anesthesia for cesarean section in a patient with von Hippel-Lindau disease and multiple sclerosis.

Abstract

Recommendations regarding the use of spinal and epidural anesthesia for patients with von Hippel-Lindau (VHL) disease are unclear [1-3] and even contradictory [4,5] due to the possible presence of vascular malformations (hemangioblastomas) in the spinal cord. We report a case of a patient with VHL disease and coexisting multiple sclerosis whose cesarean section was successfully managed with epidural anesthesia. Case Report A 45-yr-old patient, gravida 2, para 1, presented for repeat cesarean section at 38 wk gestation. She had a history of VHL disease that was manifested by hemangioblastomas in her retinae and cerebellum, pheochromocytomas, and pancreatic carcinoma. Twenty years previously, pheochromocytomas had been discovered when she developed a hypertensive crisis during her first pregnancy, requiring delivery of her child by cesarean section under general anesthesia. She subsequently underwent bilateral adrenalectomies and has been receiving chronic steroid replacement therapy. Her pancreatic carcinoma was excised via a pylorus-sparing Whipple procedure 2 yr earlier, which resulted in severe gastroesophageal reflux. The patient's multiple sclerosis symptoms had been mild and intermittent, consisting mainly of heat-induced fatigue and lower extremity weakness. Her medications included prednisone 7.5 mg and fludrocortisone acetate 0.05 mg/d. The patient's vital signs were within normal limits, as were her cardiovascular, respiratory, and airway examinations. A recent total spine magnetic resonance image revealed gadolinium-enhanced lesions in the dorsal spinal cord at T8-9 and at L2 that were "suggestive of very small hemangioblastomas" (Figure 1). There was no cord compression, and the vertebral bodies and intervertebral spaces appeared normal.Figure 1: Sagittal magnetic resonance image of the patient's lumbar spine. Spinal hemangiomas appear as white opacities between the arrows at T8 and L2. The site of epidural needle insertion is delineated by the asterisk at L3-4.To avoid the stress of labor and to minimize the risk of disrupting central nervous system (CNS) hemangioblastomas during contractions, delivery by cesarean section was planned. The patient expressed a very strong desire to be awake for the delivery. Radiologic consultations suggested that the risk of lacerating a spinal cord lesion during neuraxial anesthesia was minimal because these hemangioblastomas were small and well documented as being distant from the site of needle insertion. Despite a clear understanding that regional anesthesia might cause disruption of an hemangioblastoma, which might result in paralysis, the patient wished to proceed with regional anesthesia and gave informed consent. Hydrocortisone (100 mg IV) was administered preoperatively. After IV hydration, a 20-gauge catheter was inserted 3 cm into the epidural space via a 17-gauge Tuohy-Weiss needle placed at the L3-4 interspace. After a 3-mL test dose of 2% lidocaine with epinephrine 1:200,000 was given via the catheter, incremental injections of alkalinized 2% lidocaine with epinephrine were administered to achieve a T4 level. A cesarean section was then performed yielding a healthy infant with Apgar scores of 9 and 9 at 1 and 5 min, respectively. Surgery and recovery were uneventful. The patient was ambulating 8 h later, and her neurologic examination remained normal. She denied having any neurologic sequelae 2 mo after her surgery. Discussion VHL disease is an autosomal dominant disorder with variable penetrance characterized by hemangioblastomas of the retina, cerebellum, and spinal cord. It is also associated with renal cell carcinomas, pancreatic cysts and tumors, and pheochromocytomas [6,7]. The VHL gene seems to be a tumor-suppressive gene with "loss of function leading to unchecked cell growth and tumorigenesis" [7]. Complications include blindness, progressive neurological impairment, and death. The prevalence of this disease is estimated as 1:35,000 to 1:40,000 [7]. The main anesthetic concerns for a patient with VHL disease involve complications arising from pheochromocytomas and CNS hemangioblastomas [8]. Pheochromocytomas are present in approximately 7%-19% of patients diagnosed with VHL disease [6], and perioperative mortality can be 25%-50% if they remain undiscovered until the time of surgery [9]. Because CNS hemangioblastomas may be present, using neuraxial anesthesia in patients with VHL disease is controversial. The first report of a patient with VHL disease receiving an epidural anesthetic appeared in 1986 [4]. The authors stated that an epidural needle and catheter were unlikely to disrupt intramedullary lesions in the posterior columns of the spinal cord [4]. However, the authors of a subsequent article asserted that both spinal and epidural techniques are contraindicated in all patients with VHL disease because of the inherent risk of rupturing a hemangioblastoma [5]. We believe that the choice of anesthetic technique must be evaluated on an individual basis. In our pregnant patient with vascular malformations of the spinal cord and active gastroesophageal reflux, the epidural technique avoided the need for general anesthesia and the risks of laryngoscopy. Although there is a chance of injuring a spinal cord hemangioma by perforating the dura with an epidural needle or by passing a catheter intrathecally into a lesion, this probability was low based on the radiographic documentation of their size and locations. Spinal anesthesia was not chosen because we believed that a needle directed intrathecally has a greater potential of contacting a spinal cord lesion than a needle or catheter intended for the epidural space. In conclusion, epidural anesthesia was successfully performed in a patient with VHL disease and multiple sclerosis without neurologic sequelae. Epidural anesthesia should not be ruled out in patients with VHL disease based solely on this diagnosis. Rather, the choice of anesthesia technique should be determined after careful evaluation of the extent of the patient's disease, including a review of radiologic studies of the CNS. Consideration should also be given to the nature of the surgical procedure, to the circumstances surrounding the surgery, and to the patient's desires.