Diagnostic and Interventional Radiology
Pediatric Radiology - Original Article

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

1.

Department of Radiology, Ankara Bilkent City Hospital, Ankara, Turkey

2.

Department of Radiology, Diyarbakır Children Hospital, Diyarbakır, Turkey

3.

Division of Metabolism and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey

4.

Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey

Diagn Interv Radiol 2022; 28: 516-521
DOI: 10.5152/dir.2022.21372
Read: 435 Downloads: 111 Published: 18 November 2021

PURPOSE
lthough 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.

You may cite this article as: Yavuz OO, Ayaz E, Yıldız Y, et  al. Increased ocular wall thickness and decreased globe volume in children with mucopolysaccharidosis type VI. Diagn Interv Radiol. 2022;28(5):516-521.

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