Anesthetic management for mastectomy and total hysterectomy in a 49-year-old woman with type 1 sialidosis: a case report

JA Clinical Reports

Masumi Itagaki¹  Ryoji Iida¹  Nami Sugaya¹  Takeshi Maeda¹  Tomonori Furuya²  Takahiro Suzuki³ 

[1] Department of Anesthesiology, Nihon University Hospital, 1-6 Kanda, Surugadai, Chiyoda-ku, 101-8309, Tokyo, Japan;Department of Anesthesiology, Nihon University Hospital, 1-6 Kanda, Surugadai, Chiyoda-ku, 101-8309, Tokyo, Japan;Department of Anesthesiology, Nihon University School of Medicine, 30-1 Oyaguchi, Kamimachi, Itabashi-ku, 173-8610, Tokyo, Japan;Department of Anesthesiology, Nihon University School of Medicine, 30-1 Oyaguchi, Kamimachi, Itabashi-ku, 173-8610, Tokyo, Japan;

关键词: Sialidosis;    Lysosomal storage disease;    Total intravenous anesthesia;    Epidural anesthesia;   

DOI  :  10.1186/s40981-021-00425-z

来源: Springer

【 摘 要 】

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.

【 授权许可】

CC BY