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In Vivo Confocal Microscopy and Anterior Segment Optical Coherence Tomography Findings in Two Cases with Mucopolysaccharidoses

ABSTRACT: The mucopolysaccharidoses are a group of disorders caused by inherited defects in lysosomal enzymes resulting in widespread intracellular and extracellular accumulation of glycosaminoglycans. Due to the mucopolysaccharidoses subtype, glycosaminoglycans can be deposited in many organs and tissues including cornea. In this report, we presented in vivo confocal microscopy and anterior segment optical coherence tomography findings in a 39-year old man with Scheie syndrome and a 41-year old woman with Morquio syndrome (Heidelberg Retina Tomograph 3 Rostock module, Germany) and reviewed the literature. On in vivo confocal microscopy, there were multiple small and larger hyperreflective deposits in the epithelium, Bowman layer and anterior stroma and abnormally shaped, elongated keratocytes with hyporeflective round structures, which might be vacuoles in the anterior-mid stroma. In anterior segment optical coherence tomography images, accumulation of glycosaminoglycans deposits lead to an increased hypereflective appearance throughout the thickened cornea.

SUBMITTER: Karakucuk Y

PROVIDER: S-EPMC7338749 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature

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<i>In Vivo</i> Confocal Microscopy and Anterior Segment Optical Coherence Tomography Findings in Two Cases with Mucopolysaccharidoses

Karaküçük Yalçın Y Bozkurt Banu B Şahin Merve M Okudan Süleyman S

Turkish journal of ophthalmology 20200601 3

The mucopolysaccharidoses are a group of disorders caused by inherited defects in lysosomal enzymes resulting in widespread intracellular and extracellular accumulation of glycosaminoglycans. Due to the mucopolysaccharidoses subtype, glycosaminoglycans can be deposited in many organs and tissues including cornea. In this report, we presented <i>in vivo</i> confocal microscopy and anterior segment optical coherence tomography findings in a 39-year old man with Scheie syndrome and a 41-year old wo ...[more]

PMID: 32631021

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Project description:Fuchs uveitis (FU) is a chronic and often unilateral ocular inflammation and characteristic iris atrophic changes, other than heterochromia, are common in FU and are key to the correct diagnosis in many cases. With the advent of anterior segment optical coherence tomography (AS-OCT), some investigators attempted to quantitatively study these atrophic changes; mostly by introducing various methods to measure iris thickness in AS-OCT images. We aimed to present an automated method in an observational case series to measure the smoothness index (SI) of the iris surface in AS-OCT images. The ratio of the length of the straight line connecting the most peripheral and central points of the anterior iris border (in nasal and temporal sides) to the actual length of this border on AS-OCT images, was defined as SI. In a uveitis referral center, twenty-two eyes of 11 patients with unilateral Fuchs uveitis (FU) (7 female) and 22 eyes of 11 healthy control subjects underwent AS-OCT imaging. Image J and a newly developed MATLAB algorithm were used for manual and automated SI measurements, respectively. Agreement between manual and automated measurements was evaluated with Bland-Altman analysis and interclass correlation coefficient. The inter-eye difference of SI was compared between the FU group and the control group. Automated mean overall SI was 0.868 ± 0.037 and 0.840 ± 0.039 in FU and healthy fellow eyes, respectively (estimated mean difference = - 0.028, 95% CI [- 0.038, - 0.018], p < 0.001). Bland- Altman plots showed good agreement between two methods in both healthy and FU eyes. The interclass correlation coefficient between the manual and automated measurements in the FU and healthy fellow eyes was 0.958 and 0.964, respectively. The inter-eye difference of overall SI was 0.029 ± 0.015 and 0.012 ± 0.008 in FU group and control group, respectively (p = 0.01). We concluded that the automated algorithm can rapidly and conveniently measure SI with results comparable to the manual method.

Project description:PurposeTo measure by ultralong scan depth optical coherence tomography (UL-OCT) dimensional changes in the anterior segment of human eyes during accommodation.DesignEvaluation of diagnostic test or technology.ParticipantsForty-one right eyes of healthy subjects with a mean age of 34 years (range, 22-41 years) and a mean refraction of -2.5 ± 2.6 diopters were imaged in 2 repeated measurements at minimal and maximal accommodations.MethodsA specially adapted and designed UL-OCT instrument was used to image from the front surface of the cornea to the back surface of the crystalline lens. Custom software corrected the optical distortion of the images and yielded the biometric measurements. The coefficient of repeatability and the intraclass correlation coefficient were calculated to evaluate the repeatability and reliability.Main outcome measuresAnterior segment parameters and associated repeatability and reliability upon accommodation. The dimensional results included central corneal thickness (CCT), anterior chamber depth and width (ACD, ACW), pupil diameter (PD), lens thickness (LT), anterior segment length (ASL = ACD + LT), lens central position (LCP = ACD + 1/2LT), and horizontal radii of the lens anterior and posterior surface curvatures (LAC, LPC).ResultsRepeated measurements of each variable within each accommodative state did not differ significantly (P>0.05). The coefficients of repeatability (CORs) and intraclass correlation coefficients for CCT, ACW, ACD, LT, LCP, and ASL were excellent (1.2%- 3.59% and 0.998-0.877, respectively). They were higher for PD (18.90%-21.63% and 0.880-0.874, respectively) and moderate for LAC and LPC (34.86%-42.72% and 0.669-0.251, respectively) in the 2 accommodative states. Compared with minimal accommodation, PD, ACD, LAC, LPC, and LCP decreased and LT and ASL increased significantly at maximal accommodation (P<0.05), whereas CCT and ACW did not change (P>0.05).ConclusionsThe UL-OCT measured changes in anterior segment dimensions during accommodation with good repeatability and reliability. During accommodation, the back surface of the lens became steeper as the lens moved forward.Financial disclosure(s)The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Dataset Information

In Vivo Confocal Microscopy and Anterior Segment Optical Coherence Tomography Findings in Two Cases with Mucopolysaccharidoses

Publications

<i>In Vivo</i> Confocal Microscopy and Anterior Segment Optical Coherence Tomography Findings in Two Cases with Mucopolysaccharidoses

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