Korean J Anesthesiol.  2014 Oct;67(4):283-286. 10.4097/kjae.2014.67.4.283.

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

• Faris AS
• Tawfic QA
• Jeyaraj L

• KMID: 1937690
• DOI: http://doi.org/10.4097/kjae.2014.67.4.283

Abstract

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.

Keyword

Kearns-Sayre syndrome; Mitochondrial Myopathies; Obstetric anesthesia

MeSH Terms

Acidosis, Renal Tubular
Adult
Analgesia
Anesthesia, Obstetrical*
Cerebellar Ataxia
Cerebrospinal Fluid
Cesarean Section
Female
Humans
Kearns-Sayre Syndrome*
Mitochondrial Myopathies
Muscular Diseases
Ophthalmoplegia
Oxygen
Pregnancy
Retinitis Pigmentosa
Oxygen