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

authors:

avatar Azar Ejmalian ORCID 1 , * , avatar Nader Nassiri 2 , avatar Shahram Sayadi ORCID , avatar Dariush Abtahi , avatar Elham Memary ORCID 3

Department of Anesthesiology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
Ophthalmology Research Center, Labbafi Nejad Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Assistant Professor:Anesthesiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

how to cite: 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. https://doi.org/10.22037/jcma.v8i2.39304.

Abstract

Al-Raqad syndrome (ARS) is a novel and extremely rare autosomal recessive disorder. This syndrome affects many organs, mainly the central nervous and musculoskeletal systems. Al-Raqad syndrome?s manifestations include neurodevelopmental delays and a characteristic phenotype including craniofacial anomalies. This disorder, which is the result of a mutation in the Decapping Enzyme, Scavenger (DCPS) gene, was first described in 2015. We present a 9-year-old child affected with this syndrome, who suffers from severe neurodevelopmental delays, scheduled for cataract surgery. The anesthesia management focused on neurodevelopmental defects and craniofacial and musculoskeletal abnormalities. The reports of this syndrome are scarce, and to our knowledge, the present case is the first report of anesthesia management of this syndrome.