Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report

Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. In addition, patients mig...

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Main Authors: Ali S Faris, Qutaiba A Tawfic, Leo Jeyaraj
Format: Article
Language:English
Published: Korean Society of Anesthesiologists 2014-10-01
Series:Korean Journal of Anesthesiology
Subjects:
Online Access:http://ekja.org/upload/pdf/kjae-67-283.pdf
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author Ali S Faris
Qutaiba A Tawfic
Leo Jeyaraj
author_facet Ali S Faris
Qutaiba A Tawfic
Leo Jeyaraj
author_sort Ali S Faris
collection DOAJ
description Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. In addition, patients might have cerebellar ataxia, a high content of protein in the cerebrospinal fluid, proximal myopathy, multiple endocrinopathies, and renal tubular acidosis. We herein report the successful obstetric analgesic and anesthetic management of a 28-year-old parturient patient with KSS who required labor analgesia and proceeded to deliver by cesarean section. We extrapolate that regional analgesia/anesthesia might be beneficial for reducing the metabolic demands associated with the stress and pain of labor in patients with KSS. Efficient postoperative analgesia should be provided to decrease oxygen requirements.
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spelling doaj.art-0bff36b0894d44f78b7051edf5f0031f2022-12-21T18:18:59ZengKorean Society of AnesthesiologistsKorean Journal of Anesthesiology2005-64192005-75632014-10-0167428328610.4097/kjae.2014.67.4.2837934Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case reportAli S Faris0Qutaiba A Tawfic1Leo Jeyaraj2Department of Anesthesiology, The Ottawa Hospital and University of Ottawa, Ottawa, Canada.Department of Anesthesiology, The Ottawa Hospital and University of Ottawa, Ottawa, Canada.Department of Anesthesiology, The Ottawa Hospital and University of Ottawa, Ottawa, Canada.Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. In addition, patients might have cerebellar ataxia, a high content of protein in the cerebrospinal fluid, proximal myopathy, multiple endocrinopathies, and renal tubular acidosis. We herein report the successful obstetric analgesic and anesthetic management of a 28-year-old parturient patient with KSS who required labor analgesia and proceeded to deliver by cesarean section. We extrapolate that regional analgesia/anesthesia might be beneficial for reducing the metabolic demands associated with the stress and pain of labor in patients with KSS. Efficient postoperative analgesia should be provided to decrease oxygen requirements.http://ekja.org/upload/pdf/kjae-67-283.pdfkearns-sayre syndromemitochondrial myopathiesobstetric anesthesia
spellingShingle Ali S Faris
Qutaiba A Tawfic
Leo Jeyaraj
Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
Korean Journal of Anesthesiology
kearns-sayre syndrome
mitochondrial myopathies
obstetric anesthesia
title Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_full Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_fullStr Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_full_unstemmed Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_short Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_sort obstetric anesthesia considerations in kearns sayre syndrome a case report
topic kearns-sayre syndrome
mitochondrial myopathies
obstetric anesthesia
url http://ekja.org/upload/pdf/kjae-67-283.pdf
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AT qutaibaatawfic obstetricanesthesiaconsiderationsinkearnssayresyndromeacasereport
AT leojeyaraj obstetricanesthesiaconsiderationsinkearnssayresyndromeacasereport