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

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.

Obstetric involvement in mitochondrial disorders: A review

“…In a 26-year-old primigravida with KSS the baby had to be delivered by Cesarean section in gestational week 36 because of failure of labor progression. [22]…”

Anesthetic management of a parturient with Kearns–Sayre syndrome, dual-chamber and VVI implantable defibrillator pacemaker/defibrillator, and preeclampsia for cesarean delivery: A case report and review of the literature

“…Literature shows that both general and regional anesthesia have been used successfully in patients with KSS [ Table 1 ]. [ 8 9 10 11 12 13 ] It is advisable to maintain adequate oxygen balance, stable cardiovascular function, and good gas exchange. [ 15 ] In addition, normothermia and normoglycemia should be maintained during the anesthetic management of patients with mitochondrial myopathies, to avoid metabolic stress-induced increased demand of energy production by the diseased mitochondria.…”

“…Special anesthetic problems in patients with mitochondrial myopathies such as myocardial conduction disturbances, postoperative muscle hypotonia, and possible increased susceptibility to malignant hyperthermia can be avoided using regional blocks, when applicable. [ 8 10 13 14 ] Neuraxial analgesia has the potential to reduce the perioperative oxygen consumption during labor by 25%, particularly in parturients with KSS. [ 8 ]…”

“…[ 8 10 13 14 ] Neuraxial analgesia has the potential to reduce the perioperative oxygen consumption during labor by 25%, particularly in parturients with KSS. [ 8 ]…”

“…Few published reports have addressed the perioperative anesthetic management and potential complications. [ 8 9 10 11 12 13 14 ] We review these and make recommendations for the anesthetic managements of this rare clinical syndrome.…”