No abstract

Sturge–Weber syndrome has been included in the group of phakomatoses that is characterized by hamartomas involving the brain, skin, and eyes. The characteristic facial port-wine stain, involving the first branch of the trigeminal nerve and the embryonic vasculature distribution in this area, leads to several ocular complications of the anterior segment and can involve the eyelids and conjunctiva. The posterior segment of the eyes is also affected with diffuse choroidal hemangiomas. However, the most frequent ocular comorbidity is glaucoma with a prevalence rate ranging from 30%–70%. Glaucoma is related to anterior chamber malformations, high episcleral venous pressure (EVP), and changes in ocular hemodynamics. Glaucoma can be diagnosed at birth, but the disease can also develop during childhood and in adults. The management of glaucoma in Sturge–Weber syndrome patients is particularly challenging because of early onset, frequently associated severe visual field impairment at the time of diagnosis, and unresponsiveness to standard treatment. Several surgical approaches have been proposed, but long-term prognosis for both intraocular pressure control and visual function remains unsatisfactory in these patients. Choroidal hemangiomas may also lead to visual impairment thorough exudative retinal detachment and macular edema. Treatment of exudative hemangioma complications is aimed at destructing the tumor or decreasing tumor leakage.

A retrospective study on 1,417 uveitic patients referred to the Ocular Immunovirology Service of the University of Rome ‘La Sapienza’ was carried out to determine the occurrence of the various forms of uveitis. To detect also the possible changing patterns of uveitis during the last 25 years the obtained data were compared with those reported in two previous studies performed by the same author, at the same institution in 1978 and in 1985. Anterior uveitis was the most frequent anatomical type of intraocular inflammation (49.12%); intermediate uveitis was diagnosed in 12.42% of all the cases and showed an increased incidence over the years (p < 0.0001). Posterior and diffuse uveitis accounted for 22.1 and 16.37% of the cases. An improvement in diagnostic definition has been confirmed by the significant decrease in the percentage of ‘idiopathic uveitis’ from 56.8 to 38.1% (p < 0.0001). Associated infectious conditions were detected in 17.43% of the cases. Toxoplasma gondii was the most common etiologic agent of uveitis (6.63%). An associated systemic disease was diagnosed in 15.03% of uveitic patients, and 6% of them were affected with BehÇet’s disease. Specific ocular diseases and clinical entities increased from 7.8 and 14.68% to 29.42% in the last 25 years (p < 0.0001), the most frequent being pars planitis (11.99%) and Fuchs’ heterochromic iridocyclitis (8.32%).

In this article, we report a literature-based metanalysis we have conducted to outline the clinical features of the HLA-B27 Acute Anterior Uveitis (AAU). The examined material was based on observational studies in which participants were affected by Acute Anterior Uveitis and divided into HLA B27+ and HLA B27-. We performed a search on articles with the words "HLA B27 uveitis" dated before May 2014. Among these, 29 articles were selected for a second review. After a further evaluation, 22 articles were analyzed. The clinical characteristics studied in the metanalysis were: (1) systemic disease; (2) sex distribution; (3) laterality; (4) visual acuity; (5) hypopion; (6) anterior chamber's fibrin; (7) elevated intraocular pressure (IOP) during inflammation; (8) glaucoma; (9) posterior synechiae; (10) cataract; (11) cystoid macular edema; (12) papillitis. We have calculated a relative risk (RR) for each outcome measured. The results obtained remark some of the peculiar features linked to the HLA B27 Acute Anterior Uveitis, such as strong association with ankylosing spondylitis (RR = 6.80) and systemic diseases (RR = 9.9), male prevalence (RR = 1.2), unilateral (RR = 1.1) or alternating bilateral (RR = 2.2) involvement, hypopion (RR = 5.5), fibrinous reaction and even papillitis (R = 7.7). Simultaneous bilateral (RR = 0.3) AAU is more frequent in HLA-B27 negative form. We report higher risk of elevated IOP and glaucoma (RR = 0.6) in B27- Acute Anterior Uveitis. No significant difference between HLA B 27 positive and negative AAU was observed according to final visual acuity and complications such as posterior synechiae, cataract, and maculare edema. We trust that this will inform on the clinical evaluation and therapeutic decision in addressing a still ill-defined ophthalmologic condition.