Home > Journals > Minerva Anestesiologica > Past Issues > Minerva Anestesiologica 2007 March;73(3) > Minerva Anestesiologica 2007 March;73(3):191-4

CURRENT ISSUE
 

JOURNAL TOOLS

eTOC
To subscribe
Submit an article
Recommend to your librarian
 

ARTICLE TOOLS

Reprints
Permissions

 

CASE REPORTS   Freefree

Minerva Anestesiologica 2007 March;73(3):191-4

Copyright © 2007 EDIZIONI MINERVA MEDICA

language: English

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

Mahajan R., Kumar Batra Y., Kumar S., Kumar Grover V.

Post Graduate Institute of Medical Education and Research (Pgimer), Chandigarh, India


PDF


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 µg of fentanyl were administered into the subarachnoid space. Postoperative analgesia was obtained with an epidural infusion mixture of bupivacaine (0.125%) and fentanyl (1 µg/mL). Hemodynamic status was monitored by direct measurement of intra-arterial blood pressure and central venous pressure. The perioperative course was uneventful.

top of page