Involvement of the Anterior Segment of the Eye in Patients with Mucopolysaccharidoses: A Review of Reported Cases and Updates on the Latest Diagnostic Instrumentation.

Mucopolysaccharidoses (MPS) are a heterogeneous group of rare inherited disorders, characterized by the lack or malfunction of lysosomal enzymes necessary for glycosaminoglycan (GAGs) catabolism, and their subsequent accumulation in many tissues and organs throughout the body. An overview of the current knowledge of corneal and anterior segment manifestations in patients with MPS was provided and clinical guidelines for their diagnosis and management were furnished. The anterior segment of the eye is usually involved in every subtype of MPS, with major complications including varying degrees of corneal opacification and raised intraocular pressure (IOP) with development of glaucoma. Their recognition and management can be very useful in the diagnosis of MPS. Novel techniques are available to objectively measure the grade and extent of corneal clouding and give information about the anatomy of the anterior chamber and the structures of the angle beyond the clouded cornea. It is advisable to take advantage of this new instrumentation in order to obtain thorough information on the ocular involvement and its related anterior chamber complications for a better management of patients with MPS, both in terms of visual prognosis and therapeutic outcome.

Cytomegalovirus anterior uveitis: long-term follow-up of immunocompetent patients.

BACKGROUND: We aimed to report on the clinical findings and long-term prognosis of patients with cytomegalovirus (CMV) anterior uveitis. METHODS: This was a retrospective observational study on 15 immunocompetent patients with CMV anterior uveitis and a follow-up longer than 24 months (mean: 62.1 ± 28.5 months). RESULTS: Uveitis was unilateral and hypertensive in all cases, with acute relapsing having the characteristics of Posner-Schlossman syndrome in nine (60 %) and chronic in nine patients (40 %), three of whom were clinically classified as Fuchs' heterocromic iridocyclitis (20 %). All patients received topical antiviral and corticosteroid therapy, with six patients also receiving systemic therapy with valganciclovir or acyclovir. The mean number of uveitis relapses significantly decreased, before and after anti-CMV therapy, from 0.23 ± 0.17 to 0.03 ± 0.03 (p < 0.001), without significant differences among patients treated with topical therapy alone or combined topical and systemic therapy. Cataracts developed in nine out of 13 patients (69.2 %). A chronic raise in intraocular pressure (IOP) was found in 13 patients (86.6 %), with nine requiring surgery (60 %). At the end of the follow-up, all patients had a quiescent uveitis, with ten of them requiring topical low dose steroid therapy (66.6 %) and combined with systemic acyclovir in four cases. Eight patients (53.3 %) were on antiglaucomatous therapy. The last mean IOP value was 14.9 ± 3.6 mmHg (range 8-21 mmHg), and visual acuity was 0.89 ± 0.21. CONCLUSIONS: CMV-associated anterior uveitis has a fairly good long-term visual prognosis. Antiviral therapy can reduce the frequency of relapses, but cataracts and a chronic raise in IOP are frequent complications often requiring a surgical approach.