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.
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

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.
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