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A Journal on Anesthesiology, Resuscitation, Analgesia and Intensive Care

Official Journal of the Italian Society of Anesthesiology, Analgesia, Resuscitation and Intensive Care
Indexed/Abstracted in: Current Contents/Clinical Medicine, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 2,036

Frequency: Monthly

ISSN 0375-9393

Online ISSN 1827-1596


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


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

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.

language: English


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