Anestesia em Paciente com Doença de Steinert. Relato de Caso* Anesthesia in a Patient with Steinert Disease. Case Report

SUMMARY Araujo FS, Bessa Jr RC, Castro CHV, Cruvinel MGC, Santos D —Anesthesia in a Patient with Steinert Disease. Case Report. BACKGROUND AND OBJECTIVES: Steinert disease is the mostcommon muscular dystrophy of the adult. Due to its multisystemcharacteristic, the perioperative management of these patients is achallenge to the anesthesiologist. The aim of this report was topresent a case of hemorrhoidectomy in a patient with musculardystrophy and to discuss the several anesthetic implications involved. CASE REPORT: A man patient, 58 years old, with Steinert disease,who underwent hemorrhoidectomy. Subaracnoid block with hyper-baric bupivacaine (saddle block with puncture at L 3 -L 4 with 0.5%bupivacaine [5 mg]) associated with sedation with propofol (1 µg.mL -1 target using a target-controlled infusion pump). Dypirone (1.5 g)and local infiltration with 0.5% ropivacaine (150 mg) were used forthe postoperative analgesia. Intraoperatively, the patient developedmyotonic crisis (10 minutes after being placed on the litothomyposition) that was controlled by sedation (the target concentrationwas increased to 1.5 µg.mL