Anesthetic management of a patient with Bardet-Biedl syndrome and dilated cardiomyopathy.

Abstract

Bardet-Biedl syndrome is a rare autosomal recessive disease characterized by renal abnormalities, obesity, dysmorphic extremities, retinal dystrophy, and hypogenitalism, as well as cardiac abnormalities, diabetes mellitus, hypertension and mental retardation. Renal failure is the leading cause of death and survival is substantially reduced. We describe the anesthetic management of a patient with Bardet-Biedl syndrome, dilated cardiomyopathy and fractured right femur and tibia requiring open reduction and internal fixation. A combined spinal-epidural (CSE) block was performed; 7.5 mg of bupivacaine and 20 microg of fentanyl were administered into the subarachnoid space. Postoperative analgesia was obtained with an epidural infusion mixture of bupivacaine (0.125%) and fentanyl (1 microg/mL). Hemodynamic status was monitored by direct measurement of intra-arterial blood pressure and central venous pressure. The perioperative course was uneventful.