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Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI

PURPOSE: Although clinical ophthalmologic findings have been reported, no study documented magnetic resonance imaging (MRI) findings in mucopolysaccharidosis (MPS) type VI. The aim of this study was to determine the ophthalmologic imaging findings of MPS type VI in the pediatric age group retrospect...

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Autores principales: Yavuz, Ozlem Ozkale, Ayaz, Ercan, Yıldız, Yılmaz, Karaosmanoglu, Ayca Akgoz, Bulut, Elif, Sivri, H. Serap Kalkanoglu, Oguz, Kader K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Turkish Society of Radiology 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9682595/
https://www.ncbi.nlm.nih.gov/pubmed/36218154
http://dx.doi.org/10.5152/dir.2022.21372
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author Yavuz, Ozlem Ozkale
Ayaz, Ercan
Yıldız, Yılmaz
Karaosmanoglu, Ayca Akgoz
Bulut, Elif
Sivri, H. Serap Kalkanoglu
Oguz, Kader K.
author_facet Yavuz, Ozlem Ozkale
Ayaz, Ercan
Yıldız, Yılmaz
Karaosmanoglu, Ayca Akgoz
Bulut, Elif
Sivri, H. Serap Kalkanoglu
Oguz, Kader K.
author_sort Yavuz, Ozlem Ozkale
collection PubMed
description PURPOSE: Although clinical ophthalmologic findings have been reported, no study documented magnetic resonance imaging (MRI) findings in mucopolysaccharidosis (MPS) type VI. The aim of this study was to determine the ophthalmologic imaging findings of MPS type VI in the pediatric age group retrospectively. METHODS: Brain MRIs of 10 patients with MPS type VI and 49 healthy children were evaluated independently by two pediatric radiologists for the following characteristics: globe volume, ocular wall thickness, and optic nerve sheath diameter for each orbit. The means of the measurement of each group were compared by using an independent t-test. Agreement and bias between reviewers were assessed by intra-class correlation coefficients (ICC). RESULTS: A total of 59 children [32 girls (54.23%), 27 boys (45.77%); age range, 4-16 years; mean age, 10.37 ± 3.73 years] were included in the study. Statistical analysis revealed smaller eyeballs and thicker ocular walls of patients with MPS type VI (P < .001 and P < .001, respectively). However, there was no statistically significant difference in terms of optic nerve sheath diameter between the two groups (P = .648). CONCLUSION: Patients with MPS type VI displayed reduced globe volumes and increased ocular wall thicknesses compared to the healthy children. Therefore, we recommend that ophthalmologic imaging findings might prove to be an auxiliary tool in the diagnosis of MPS patients.
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spelling pubmed-96825952022-12-02 Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI Yavuz, Ozlem Ozkale Ayaz, Ercan Yıldız, Yılmaz Karaosmanoglu, Ayca Akgoz Bulut, Elif Sivri, H. Serap Kalkanoglu Oguz, Kader K. Diagn Interv Radiol Original Article PURPOSE: Although clinical ophthalmologic findings have been reported, no study documented magnetic resonance imaging (MRI) findings in mucopolysaccharidosis (MPS) type VI. The aim of this study was to determine the ophthalmologic imaging findings of MPS type VI in the pediatric age group retrospectively. METHODS: Brain MRIs of 10 patients with MPS type VI and 49 healthy children were evaluated independently by two pediatric radiologists for the following characteristics: globe volume, ocular wall thickness, and optic nerve sheath diameter for each orbit. The means of the measurement of each group were compared by using an independent t-test. Agreement and bias between reviewers were assessed by intra-class correlation coefficients (ICC). RESULTS: A total of 59 children [32 girls (54.23%), 27 boys (45.77%); age range, 4-16 years; mean age, 10.37 ± 3.73 years] were included in the study. Statistical analysis revealed smaller eyeballs and thicker ocular walls of patients with MPS type VI (P < .001 and P < .001, respectively). However, there was no statistically significant difference in terms of optic nerve sheath diameter between the two groups (P = .648). CONCLUSION: Patients with MPS type VI displayed reduced globe volumes and increased ocular wall thicknesses compared to the healthy children. Therefore, we recommend that ophthalmologic imaging findings might prove to be an auxiliary tool in the diagnosis of MPS patients. Turkish Society of Radiology 2022-09-01 /pmc/articles/PMC9682595/ /pubmed/36218154 http://dx.doi.org/10.5152/dir.2022.21372 Text en © Copyright 2022 authors https://creativecommons.org/licenses/by-nc/4.0/ Content of this journal is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. (https://creativecommons.org/licenses/by-nc/4.0/)
spellingShingle Original Article
Yavuz, Ozlem Ozkale
Ayaz, Ercan
Yıldız, Yılmaz
Karaosmanoglu, Ayca Akgoz
Bulut, Elif
Sivri, H. Serap Kalkanoglu
Oguz, Kader K.
Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI
title Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI
title_full Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI
title_fullStr Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI
title_full_unstemmed Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI
title_short Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI
title_sort increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type vi
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9682595/
https://www.ncbi.nlm.nih.gov/pubmed/36218154
http://dx.doi.org/10.5152/dir.2022.21372
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