Anesthetic Management of a Four-Year-Old Child Affected by Marinesco-Sjogren Syndrome Scheduled for Cataract Surgery: A Case Report

Abstract

Marinesco-Sjogren syndrome (MSS) is a rare autosomal recessive disorder with different clinical signs and symptoms. Neurological dysfunction, especially in the cerebellum, ataxia, dysarthria, muscle atrophy, progressive hypotonia, skeletal deformity with scoliosis, mental retardation, and failure to thrive are common. Congenital cataracts and strabismus may need to be corrected via surgery. This disorder requires unique anesthetic consideration for myopathy, malignant hyperthermia, and respiratory depression. The neurodevelopmental delay also makes anesthetic management challenging in these patients. Muscle weakness and poor respiratory reserve in these patients may be associated with adverse effects if muscle relaxants are used during anesthesia. Case reports of anesthetic management of this syndrome are scarce. We report a child with MSS scheduled for lens removal surgery and anesthetic management.