Management of Corneal Clouding in Patients with Mucopolysaccharidosis

Mucopolysaccharidoses (MPS) are a rare group of lysosomal storage disorders characterized by the accumulation of incompletely degraded glycosaminoglycans (GAGs) in multiple organ systems including the eye. Visual loss occurs in MPS predominantly due to corneal clouding and retinopathy, but the sclera, trabecular meshwork and optic nerve may all be affected. Despite the success of therapies such as enzyme replacement therapy (ERT) and hematopoietic stem-cell transplantation (HSCT) in improving many of the systemic manifestations of MPS, their effect on corneal clouding is minimal. The only current definitive treatment for corneal clouding is corneal transplantation, usually in the form of a penetrating keratoplasty or a deep anterior lamellar keratoplasty. This article aims to provide an overview of corneal clouding, its current clinical and surgical management, and significant research progress

Clinical Utility of Artificial Intelligence Algorithms to Enhance Wide-Field Optical Coherence Tomography Angiography Images

The aim of this paper is to investigate the clinical utility of the application of deep learning denoise algorithms on standard wide-field Optical Coherence Tomography Angiography (OCT-A) images. This was a retrospective case-series assessing forty-nine 10 × 10 mm OCT-A1 macula scans of 49 consecutive patients attending a medical retina clinic over a 6-month period. Thirty-seven patients had pathology; 13 had none. Retinal vascular layers were categorised into superficial or deep capillary plexus. For each category, the retinal experts compared the original standard image with the same image that had intelligent denoise applied. When analysing the Superficial Capillary Plexus (SCP), the denoised image was selected as “best for clinical assessment” in 98% of comparisons. No difference was established in the remaining 2%. On evaluating the Deep Capillary Plexus (DCP), the denoised image was preferred in 35% of comparisons. No difference was found in 65%. There was no evidence of new artefactual features nor loss of anatomical detail in denoised compared to the standard images. The wide-field denoise feature of the Canon Xephilio OCT-A1 produced scans that were clinically preferable over their original OCT-A images, especially for SCP assessment, without evidence for causing a new artefactual error