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

Image Credit:J Cell Mol Anesth

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

Authors

Dr Azar Ejmalian1,*, 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
*Corresponding Author: Department of Anesthesiology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran Email: azar.ejmalian@gmail.com

Journal of Cellular & Molecular Anesthesia:Vol. 8, issue 1; e151427
Published online:Jun 05, 2022
Article type:Case Report
How to Cite:Ejmalian DA, Memary E, Nassiri N. Anesthetic Management of a Four-Year-Old Child Affected by Marinesco-Sjogren Syndrome Scheduled for Cataract Surgery: A Case Report. J Cell Mol Anesth. 2023;8(1):e151427. doi: 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.

References

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

Copyright

© 2023, Author(s). This open-access article is available under the Creative Commons Attribution 4.0 (CC BY 4.0) International License (https://creativecommons.org/licenses/by/4.0/), which allows for unrestricted use, distribution, and reproduction in any medium, provided that the original work is properly cited.

Similar Articles

16
Mar
2023
General Anesthesia in Schwartz-Jampel Syndrome: A Case Report

General Anesthesia in Schwartz-Jampel Syndrome: A Case Report

Pooya Derakhshan,
Azadeh Habibi,
Saied Amniati

Derakhshan P, Habibi A, Amniati S. General Anesthesia in Schwartz-Jampel Syndrome: A Case Report. Anesth Pain Med. 2023;13(3):e129305. doi: https://doi.org/10.5812/aapm-129305

13
May
2023
Sjogren Larsson Syndrome in Twins: Case Report

Sjogren Larsson Syndrome in Twins: Case Report

Gabriela Roncada Haddad,
Mariana Righetto de Re Lai,
Mariana Lopes Amaral Barbosa,
Andre Luis Moura Balbino

Haddad GR, de Re Lai MR, Barbosa MLA, Balbino ALM. Sjogren Larsson Syndrome in Twins: Case Report. J Skin Stem Cell. 2023;10(2):e135792. doi: https://doi.org/10.5812/jssc-135792

22
Oct
2022
Anesthetic Management of a 9-Years-Old Child Affected by Al-Raqad Syndrome Scheduled for Cataract Surgery: A Case Report

Anesthetic Management of a 9-Years-Old Child Affected by Al-Raqad Syndrome Scheduled for Cataract Surgery: A Case Report

Azar Ejmalian,
Nader Nassiri,
Shahram Sayadi,
Dariush Abtahi,
Elham Memary

Ejmalian A, Nassiri N, Sayadi S, Abtahi D, Memary E. Anesthetic Management of a 9-Years-Old Child Affected by Al-Raqad Syndrome Scheduled for Cataract Surgery: A Case Report. J Cell Mol Anesth. 2023;8(2):e149687. doi: https://doi.org/10.22037/jcma.v8i2.39304

18
Apr
2015
Primary Sjogrens Syndrome in a Child

Primary Sjogrens Syndrome in a Child

K. Jagadish Kumar,
Anush S. Kudakesseril,
CS Sheeladevi,
HV Sowmya

Jagadish Kumar K, Kudakesseril AS, Sheeladevi C, Sowmya H. Primary Sjogrens Syndrome in a Child. Inn J Pediatr. 2015;25(2):e254. doi: https://doi.org/10.5812/ijp.254

28
Jun
2021
Normo-Calcemic Tetany in a Patient with Hypokalemic Periodic Paralysis of Sjogren’s Syndrome: Neuromuscular Excitability Secondary to the ‘Forgotten Cation’

Normo-Calcemic Tetany in a Patient with Hypokalemic Periodic Paralysis of Sjogren’s Syndrome: Neuromuscular Excitability Secondary to the ‘Forgotten Cation’

Urmila Anandh,
Pradeep Mishra,
Hilal Malla

Anandh U, Mishra P, Malla H. Normo-Calcemic Tetany in a Patient with Hypokalemic Periodic Paralysis of Sjogren’s Syndrome: Neuromuscular Excitability Secondary to the ‘Forgotten Cation’. Arch Neurosci. 2021;8(2):e105542. doi: https://doi.org/10.5812/ans.105542

More by these authors

Dr Azar EjmalianPubMedScholar
Elham MemaryPubMedScholar
Nader NassiriPubMedScholar
Share
Cited by
Metrics
Indexed in