Secondary glaucoma in CAPN5-associated neovascular inflammatory vitreoretinopathy.

OBJECTIVE: The objective of this study was to review the treatment outcomes of patients with secondary glaucoma in cases of autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV), a hereditary autoimmune uveitis due to mutations in CAPN5. PATIENTS AND METHODS: A retrospective, observational case series was assembled from ADNIV patients with secondary glaucoma. The main outcome measures were intraocular pressure (IOP), visual acuity, use of antiglaucoma medications, ocular surgeries, and adverse outcomes. Perimetry and optic disk optical coherence tomography (OCT) were also analyzed. RESULTS: Nine eyes of five ADNIV patients with secondary glaucoma were reviewed. Each received a fluocinolone acetonide (FA) implant for the management of posterior uveitis. Following implantation, no eyes developed neovascular glaucoma. Five eyes (in patients 1, 2, and 5) required Ahmed glaucoma valve surgery for the management of steroid-responsive glaucoma. Patient 2 also developed angle closure with iris bombe and underwent laser peripheral iridotomy. Patient 4 had both hypotony and elevated IOP that required periodic antiglaucoma medication in the FA-implanted eye. Patient 3 did not develop steroid-response glaucoma in either eye. Optic disk examinations were obscured by fibrosis and better assessed with OCT. CONCLUSION: ADNIV patients show combined mechanism secondary glaucoma best assessed by OCT of the optic disk. The FA implants have reduced uveitic and neovascular glaucoma. Nevertheless, IOP management remains complex due to steroid-response glaucoma, angle closure glaucoma, and hypotony.

Surgical management of fibrotic encapsulation of the fluocinolone acetonide implant in CAPN5-associated proliferative vitreoretinopathy.

OBJECTIVE: To review fibrosis of fluocinolone acetonide (FA) implants in subjects with CAPN5 autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV). METHODS: A retrospective case series was assembled from ADNIV patients in which there was fibrotic encapsulation of a fluocinolone acetonide implant. CAPN5 genotypes and surgical repair techniques were reviewed. RESULTS: Two eyes of two ADNIV patients developed a fibrotic capsule over the fluocinolone acetonide implant. Both patients had Stage IV disease. Patient A had a c.731T > C mutation in the CAPN5 gene and patient B had a c.728G > T mutation. The fibrotic membrane was surgically excised and the implant function was restored. CONCLUSION: The exuberant fibrotic response in later stages of ADNIV may be resistant to local immunosuppression with steroids. Surgical excision of fibrotic membranes over FA implants can reestablish local steroid delivery in cases of severe proliferative vitreoretinopathy.

Retinal vessel width measurement at branchings using an improved electric field theory-based graph approach.

The retinal vessel width relationship at vessel branch points in fundus images is an important biomarker of retinal and systemic disease. We propose a fully automatic method to measure the vessel widths at branch points in fundus images. The method is a graph-based method, in which a graph construction method based on electric field theory is applied which specifically deals with complex branching patterns. The vessel centerline image is used as the initial segmentation of the graph. Branching points are detected on the vessel centerline image using a set of detection kernels. Crossing points are distinguished from branch points and excluded. The electric field based graph method is applied to construct the graph. This method is inspired by the non-intersecting force lines in an electric field. At last, the method is further improved to give a consistent vessel width measurement for the whole vessel tree. The algorithm was validated on 100 artery branchings and 100 vein branchings selected from 50 fundus images by comparing with vessel width measurements from two human experts.

Inhibition of neovascularization but not fibrosis with the fluocinolone acetonide implant in autosomal dominant neovascular inflammatory vitreoretinopathy.

OBJECTIVE To review the effect of the fluocinolone acetonide implant in subjects with autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV), an inherited autoimmune uveitis. METHODS A retrospective case series was assembled from patients with ADNIV who received fluocinolone acetonide implants. Visual acuity and features of ADNIV, including inflammatory cells, neovascularization, fibrosis, and cystoid macular edema, were reviewed. RESULTS Nine eyes of 5 related patients with ADNIV with uncontrolled inflammation were reviewed. Follow-up ranged from 21.7 to 56.7 months. Visual acuity at implantation ranged from 20/40 to hand motion. Preoperatively, 8 eyes had vitreous cells (a ninth had diffuse vitreous hemorrhage). Eight eyes had cystoid macular edema, 7 had an epiretinal membrane, and 3 had retinal neovascularization. Following implantation, vitreous cells resolved in all eyes and neovascularization regressed or failed to develop. Central macular thickness improved in 4 eyes. During the postoperative course, however, visual acuity continued to deteriorate, with visual acuity at the most recent examination ranging from 20/60 to no light perception. There was also progressive intraocular fibrosis and phthisis in 1 case. Four eyes underwent cataract surgery. Six of the 7 eyes without previous glaucoma surgery had elevated intraocular pressure at some point, and 3 of these required glaucoma surgery. CONCLUSIONS The fluocinolone acetonide implant may inhibit specific features of ADNIV such as inflammatory cells and neovascularization but does not stabilize long-term vision, retinal thickening, or fibrosis. All eyes in this series required cataract extraction, and more than half required surgical intervention for glaucoma. Further studies may identify additional therapies and any benefit of earlier implantation.

Effect of the oxygen saturation target on clinical characteristics of early- versus late-onset retinopathy of prematurity.

PURPOSE: To investigate the relationship between postmenstrual age of onset of retinopathy of prematurity (ROP) and the need for treatment, while examining the effects of two different neonatal oxygen saturation protocols on this relationship. METHODS: A retrospective chart review was conducted for eligible inborn infants born before and after the institution of a new oxygen protocol adjusting target oxygen saturation from 90%-99% to 85%-93%. Early versus late-onset ROP was defined as first presence of any stage disease on examination at <36 versus ≥ 36 weeks' postmenstrual age, respectively. RESULTS: The median birth weight/postmentrual age of infants was 840 g per 26.1 weeks (early-onset ROP) versus 952.5 g per 28 weeks (late-onset ROP; P < 0.01 vs P < 0.01). ROP developed in 119 of 369 (32.2%) of high oxygen target infants, and 100 of 373 (26.8%) infants in the low-target group (P = 0.11). Cumulatively, 35 of 144 (24.3%) of early-onset and 8 of 69 (11.6%) of late-onset patients required treatment (P = 0.03). Maximal severity of disease after treatment, including retinal detachment frequency, was similar in early- and late-onset patients, independent of the oxygen protocol (P = 1.00). CONCLUSIONS: The clinical behavior of type 1 ROP is similar in early-and late-onset disease, regardless of oxygen saturation targets. Type 1 ROP disease occurred in 11.6% of patients with late-onset ROP.

A twisted mess.

A 12-year-old girl presented with painless decreased vision in the left eye. Dilated fundus examination was consistent with Wyburn-Mason syndrome, and subsequent neuroimaging demonstrated a retro-orbital arteriovenous malformation. The etiology of the vision loss is discussed, as well as management options of Wyburn-Mason syndrome.

Certolizumab pegol therapy for rheumatoid arthritis-associated scleritis.

PURPOSE: To report the successful treatment of rheumatoid arthritis (RA)-associated scleritis with the tumor necrosis factor alpha antagonist certolizumab pegol. METHODS: Retrospective case report of therapeutic interventions for a 47-year-old woman with scleritis and RA. After not tolerating treatment with other tumor necrosis factor alpha inhibitors and rituximab, or after that treatment failed, certolizumab pegol was administered in an attempt to achieve better control of her disease process. RESULTS: Six months after beginning the new therapy, it was well-tolerated and the patient's RA and scleritis were quiescent. CONCLUSIONS: Certolizumab pegol may successfully treat RA-associated scleritis, even in a patient who has not tolerated therapy with other tumor necrosis factor alpha antagonists or in whom that treatment has failed.

Effect of decreasing target oxygen saturation on retinopathy of prematurity.

INTRODUCTION: The authors of previous studies suggest that the oxygenation status of premature infants contributes to the development of retinopathy of prematurity (ROP). In this study we compared the incidence and severity of ROP before and after institution of a new neonatal intensive care unit oxygen protocol. METHODS: A retrospective chart review was performed of all eligible inborn patients screened for ROP during the 2 years immediately before (Group 1) to and the 2 years after (Group 2) the initiation of a new oxygen protocol. In the new protocol, target oxygen saturation was adjusted from 90%-99% to 85%-93%. Treatment criteria adhered to Early Treatment for Retinopathy of Prematurity guidelines for the study's duration. RESULTS: There were 387 infants in Group 1 and 386 infants in Group 2 (descriptive statistics adjusted for correlation due to multiple births). Mean birth weights (BWs) and gestational ages were 1,194 g and 29.2 weeks (ranges, 525-2,085 g; 23 2/7-39 6/7 weeks) for Group 1 and 1,139 g and 28.9 weeks (ranges, 520-2,500 g; 22 6/7-35 3/7 weeks) for Group 2 (p = 0.02/0.10). ROP developed in 32.7% of infants in Group 1 and 27.8% in Group 2 (p = 0.17). The incidence of ROP requiring treatment was 19.9% in Group 1 and 20.5% in Group 2 (p = 0.91). Subanalysis of infants with BW ≤ 1,000g (Group 1, n = 119; Group 2, n = 141) revealed ROP incidence of 75.1% versus 57.1%, respectively (p < 0.01); treatable disease occurred in 37.5% and 21.9% of affected infants (p = 0.19). CONCLUSIONS: Lowering target oxygen saturation for inborn premature infants was associated with decreased incidence of ROP only in infants with BW ≤ 1,000 g. Severity of disease, including need for treatment, was similar in both groups.