Abstract
BackgroundSialidosis is an autosomal recessive glycoprotein storage disorder, caused by neuraminidase deficiency which leads to abnormal intracellular accumulation and urinary excretion of sialylated oligosaccharides, resulting in various morphological and functional disorders. Only a few reports have described the anesthetic managements of patients with sialidosis.Case presentationA 49-year-old woman with type 1 sialidosis suffered from all limb contractures, an ocular cherry-red spot, and myoclonic seizures of the limbs. She had been cognitively normal. She was separately scheduled for mastectomy under total intravenous anesthesia and total hysterectomy under combined general and epidural anesthesia uneventfully.ConclusionsOur patient with type 1 sialidosis received both general and epidural anesthesia uneventfully. Anesthesiologists should carefully assess patients with sialidosis and give careful consideration to individually tailored anesthetic managements.
Highlights
Sialidosis is an autosomal recessive glycoprotein storage disorder, caused by neuraminidase deficiency which leads to abnormal intracellular accumulation and urinary excretion of sialylated oligosaccharides, resulting in various morphological and functional disorders
Sialidosis is an autosomal recessive glycoprotein storage disorder, caused by neuraminidase deficiency which leads to material rich in sialic acid to accumulate in various tissues and organs, leading to morphological and functional disorders
Sialidosis is an autosomal recessive glycoprotein storage disorder caused by neuraminidase deficiency resulting from a mutation in the neuraminidase gene, located on the 6p21.3 imprinted region [4]
Summary
We uneventfully provided both general and epidural anesthesia for a patient with type 1 sialidosis.
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