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VOLUME 5 , ISSUE 4 ( October-December, 2019 ) > List of Articles

CASE REPORT

Cochlear Implantation Surgery in Jervell and Lange-Nielsen Syndrome Patients: Our Experience

Rajashree D Godbole, Joyshankar Jana, Prithviraj Gauri, Amol B Mhetre

Keywords : Cardiac arrhythmias, Jervell and Lange-Nielsen syndrome, Prolonged QT interval, Torsades de pointes

Citation Information : Godbole RD, Jana J, Gauri P, Mhetre AB. Cochlear Implantation Surgery in Jervell and Lange-Nielsen Syndrome Patients: Our Experience. J Med Sci 2019; 5 (4):96-98.

DOI: 10.5005/jp-journals-10045-00133

License: CC BY-NC 4.0

Published Online: 03-10-2020

Copyright Statement:  Copyright © 2019; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Jervell and Lange-Nielsen syndrome represents a rare autosomal recessive cause of congenital deafness. The affected patients have prolongation of QT interval on electrocardiogram. These patients may present with cardiac arrhythmias, syncopal episodes, and predisposition to sudden death. These patients are predisposed to polymorphic ventricular tachycardia–torsades de pointes leading to syncope and sudden death. Surgical stress in the perioperative period, anxiety, loud sounds, and fear may trigger fatal arrhythmias. We are reporting anesthetic management of two pediatric patients posted for cochlear implantation surgery.


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