Anesthetic Management of Parturient with Tuberous Sclerosis
Abstract Number: 252
Abstract Type: Case Report/Case Series
INTRODUCTION:Tuberous sclerosis(TS)is an autosomal dominant disease described as a triad of seizures,mental retardation,and central facial skin eruption.The disease process involves a constellation of benign hamartomatous proliferative lesions and malformations occurring in virtually every organ of the body. We are aware of only a few reported cases of tuberous sclerosis during pregnancy, and none mentioned specific anesthetic management.This report describes the anesthetic management of a pregnant patient affected by TS.
CASE REPORT:A 26-year-old, 62 in, 86 kg, G1P0, at 40 5/7 weeks gestation presented to the labor and delivery suites in spontaneous active labor and with cervical dilation of 4cm. The diagnosis was made at the age of 6 months following episodes of seizures,and the typical facial skin lesions. She has residual but stable-appearing intracranial tubers on MRI of the brain.She also has bilateral renal angiomyolipomas with no impairment of renal function. On exam, she was mildly retarded with cutaneous lesions of TS on her face. Vital signs were stable. There was no focal neurologic deficit. Laboratory data including EKG were all within normal limits. Patient requested labor epidural analgesia. We placed two large-bore(16g)IV lines, and we made sure to have cross matched blood available. Placement of labor epidural at L3-L4 interspace was uneventful. Effective analgesia(sensory blocked at T10 level)was established using 0.2% Ropivacaine 8mL and Fentanyl 100mcg. The epidural block was maintained with a continuous infusion of 0.11% Bupivacaine with Fentanyl 2mcg/mL at a rate of 10mL per hour. Over the course of the next 8 hours, the epidural was bolused three times with 10 cc of 0.2% Ropivacaine and Fentanyl 100mcg for breakthrough pain. After the last bolus, she did not get satisfactory relief despite a sensory block of at least T10 level. At this time, the obstetric team confirmed a failed trial of labor and a decision was made to proceed to cesarean section. Because of uncertainty of the quality of analgesia from her epidural, we decided to perform a general anesthetic for the cesarean section. Propofol and Succinylcholine were used for rapid sequence induction and anesthesia was maintained with Desflurane.She delivered a healthy newborn. She was discharged home on postoperative day three.
DISCUSSION:Patients with TS have a wide expression in the severity of the disease that may present a challenge to the anesthesiologist. As shown in this report, our patient exhibited the classic triad of seizures, mental retardation, and facial skin lesions but she did not have any focal neurologic deficit, hydrocephalus, renal dysfunction, dysrhythmias, or pulmonary dysfunction which is of importance to any anesthetic management. This patient, however, had renal angiomylipomas that can spontaneously rupture and hence we made sure to have appropriate bore IV in place and blood readily available.
REFERENCES:Lee J, et al.Br J Anaesth 1994