Childhood pars planitis; clinical features and outcomes

To evaluate the demographic and clinical features of childhood pars planitis, and to determine the therapeutic and visual outcomes of the disease. Medical records of pediatric patients [less than 16 years of age at diagnosis] with pars planitis and at least 6 months of follow-up who were referred to Labbafinejad Medical Center, Tehran, Iran over a 22 year period were reviewed. Overall, 117 eyes of 61 patients including 51 [83.6%] male subjects were included. Mean age at the time of diagnosis was 7.8 +/- 3.2 [range, 3-16] years. Mean best corrected visual acuity [BCVA] was 0.88 +/- 0.76 logMAR at presentation which improved to 0.39 +/- 0.51 logMAR at final visit [P<0.001]. Endotheliitis was present in 23 [19.6%] eyes and was significantly more prevalent in subjects younger than 9 years [P=0.025]. Cataract formation [41.9%] and cystoid macular edema [19.7%] were the most prevalent complications. Univariate regression analysis showed that better baseline visual acuity [OR=0.38, 95%CI 0.21-0.70, P=0.002], age older than 5 years at disease onset [OR=0.36, 95%CI 0.14-0.9, P=0.029], absence of endotheliitis [OR=0.39, 95%CI 0.15-0.99, P-0.047] and female gender [OR=3.77, 95%CI 1.03-13.93, P=0.046] were significantly associated with final BCVA of 20/40 or better. Childhood pars planitis was much more common among male subjects. Endotheliitis may be a sign of inflammation spillover and is more prevalent in younger patients. Visual prognosis is favorable in most patients with appropriate treatment

Idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody

To report a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis [IRVAN] syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody [P-ANCA]. A 51-year-old man presented with loss of vision in his right eye since many years ago and blurred vision in his left eye over the past year. Ophthalmologic examination revealed optic atrophy and old vascular sheathing in the right eye and blurred disc margin, macular exudation, flame shaped hemorrhages, retinal vascular sheathing and multiple aneurysms at arterial bifurcation sites in the left eye, findings compatible with IRVAN syndrome. On systemic workup, the only notable finding was P-ANCA positivity. IRVAN syndrome may be a retinal component of P-ANCA associated vasculitis