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

Dr Azar Ejmalian; Elham Memary; Nader Nassiri

doi:10.5812/jcma-151427

Journal of Cellular & Molecular Anesthesia

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https://doi.org/10.22037/jcma.v7i4.38067

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

Author(s):

Dr Azar Ejmalian^1,*,

Elham Memary²,

Nader Nassiri³

1Department of Anesthesiology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran

2Anesthesiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

3Ophthalmology Research Center, Labbafi Nejad Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Journal of Cellular & Molecular Anesthesia:Vol. 8, issue 1; e151427

Published online:Jun 05, 2022

Article type:Case Report

How to Cite:Dr Azar EjmalianElham MemaryNader NassiriAnesthetic Management of a Four-Year-Old Child Affected by Marinesco-Sjogren Syndrome Scheduled for Cataract Surgery: A Case Report.J Cell Mol Anesth.8(1):e151427.https://doi.org/10.22037/jcma.v7i4.38067.

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.

Keywords

Cataract

Cerebellar ataxia

Spinocerebellar Degenerations

Muscular Diseases

References

1.
References are in the PDF file of the article.

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Author(s):

Dr Azar Ejmalian:[PubMed][Scholar]
Elham Memary:[PubMed][Scholar]
Nader Nassiri:[PubMed][Scholar]