In vivo confocal microscopy of the cornea in Morquio syndrome

Stewart, Stephen; McGhee, Charles N J; Patel, Dipika V.

doi:10.1038/eye.2012.155

Cited by 10 publications

(8 citation statements)

References 5 publications

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“…Stewart et al 10 reported laser confocal findings of a 7-year-old boy with MPS-IVA who had very mild diffuse, fine granular corneal stromal appearance and good visual acuity. IVCM demonstrated a diffuse hyperreflectivity immediately posterior to the Bowman’s layer and in the anterior and middle stroma.…”

Section: Discussionmentioning

confidence: 99%

“…In the literature, there are only a few studies about the use of IVCM and anterior segment optical coherence tomography (AS-OCT) in MPSs. 7 , 8 , 9 , 10 , 11 , 12 Herein, we report the clinical, AS-OCT, and laser IVCM findings of 2 patients with MPS-IS and MPS-IVA. Our aim was to compare our findings with previous studies in the literature, determine whether these imaging devices can show the typical histopathological features of MPS, and demonstrate the differences between MPS-IS and MPS-4A.…”

Section: Introductionmentioning

confidence: 99%

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

Karaküçük¹,

Bozkurt²,

Şahin³

et al. 2020

tjo

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Karaküçük¹,

Bozkurt²,

Şahin³

et al. 2020

tjo

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“…On confocal microscopy, the stroma contained poorly differentiated cells and empty keratocyte vacuoles (Patel et al 2005). Stewart et al (2012) compared the image of a 7-year-old cornea with type IV MPS to a normal cornea. Stewart et al (2012) compared the image of a 7-year-old cornea with type IV MPS to a normal cornea.…”

Section: Confocal Microscopy and Its Role In Mpsmentioning

confidence: 99%

“…Another report by Mocan et al (2007) showed two siblings with type VI that had similar vacuoles in the posterior stroma despite a well-maintained endothelium. Stewart et al (2012) compared the image of a 7-year-old cornea with type IV MPS to a normal cornea. As well as abnormal stromal morphology throughout the cornea, the anterior stroma directly behind Bowman's layer was found to have lost a majority of its transparency due to an irregular arrangement of stromal cells that were hyper reflective with vacuolated keratocyte nuclei.…”

Section: Confocal Microscopy and Its Role In Mpsmentioning

confidence: 99%

Use of new imaging in detecting and monitoring ocular manifestations of the mucopolysaccharidoses

Javed

Aslam

Ashworth

2016

Acta Ophthalmologica

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The aim of this review is to summate the eye involvement in patients with mucopolysaccharidoses (MPS) using current ocular imaging techniques, their advantages and disadvantages and how they may aid diagnosis, management and monitoring. We critically reviewed the current literature surrounding MPS and recent imaging technology as well as histology. Primary searches of PubMed and Web of Science were performed. We reviewed all papers on the topic published and summarized the findings of each medical device as well as the advantages and disadvantages of using these for the MPS patient. We discussed the potential of each of these devices to monitor potential ocular pathology in the MPS cohorts in the order of MPS subtype. We reviewed imaging techniques involving use of the Iris Camera, Pentacam, Optical Coherence Tomography (OCT) as well as ultrasound and Heidelberg OCT. The need for reliable objective quantification of eye findings in MPS has led to utilization of new imaging technologies described here, and future use will enhance our understanding of the unique eye features in MPS. In particular, we note that the Pentacam and iris camera are able to provide objective measurements of corneal haze and monitor ocular response to treatment.

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“…In vivo confocal microscopy of corneas affected by MPS has revealed round, vacuolated keratocyte nuclei, predominantly in the posterior stroma (Fig. c, 7b) . Diffuse irregular hyper‐reflectivity may be observed in the anterior stroma, immediately posterior to Bowman's layer (Fig.…”

Section: Carbohydratementioning

confidence: 99%