Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
نویسندگان
چکیده
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.
منابع مشابه
Hypoparathyroidism as the First Mani-Festation of Kearns-Sayre Syndrome: A Case Report
Kearns-Sayre syndrome is a mitochondrial myopathy, which was first described by Tomas Kearn in 1958. Diagnostic symptoms of this condition include retinitis pigmentosa, chronic progressive external ophthalmoplegia, and one or more of the following factors: cardiac conduction system diseases, cerebellar ataxia, and cerebrospinal fluid (CSF) with protein content above 100 mg/dL. The nature of thi...
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Introduction Herein, we describe the interesting case of a 13-year-old female with Kearns-Sayre syndrome (KSS) who underwent a successful T3 ~ L3 posterior spinal instrumentation and fusion (PSIF). KSS is a rare mitochondrial myopathy with only 226 cases reported in published literature as of 1992. It is the result of deletions in mitochondrial DNA and manifests as a triad of: 1) chronic progre...
متن کاملKearns-Sayre syndrome--a case report.
A case of Kearns - Sayre Syndrome characterized by a triad of external ophthalmoplegia, retinal dystrophy and cardiomyopathy is discussed. Ocular examination and cardiologic screening of family members is requested.
متن کاملHypoparathyroidism as the First Manifestation of Kearns-Sayre Syndrome: A Case Report
OBJECTIVE Kearns-Sayre syndrome is a mitochondrial myopathy, which was first described by Tomas Kearn in 1958. Diagnostic symptoms include retinitis pigmentosa, chronic and progressive external ophthalmoplegia plus one or more of following factors: heart conduction system disorders, cerebellar ataxia, or cerebrospinal fluid (CSF) protein content above 100 mg/dL. The nature of this uncommon dise...
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