Laser photocoagulation at birth prevents blindness in Norrie's disease diagnosed using amniocentesis.

OBJECTIVE: To report the first case of prophylactic laser treatment to prevent blindness in a patient who was diagnosed with Norrie's disease by genetic testing with amniocentesis. DESIGN: Case report. PARTICIPANTS: A 2-year-old white boy with Norrie's disease. METHODS: A 37-week gestational age male with a family history of Norrie's disease was born via Cesarean section after the mother had undergone prenatal amniocentesis fetal-genetic testing at 23 weeks of gestation. A C520T (nonsense) mutation was found in the Norrie's disease gene. After examination under anesthesia confirmed the diagnosis on the first day of life, laser photocoagulation was applied to the avascular retina bilaterally. The patient was followed closely by ophthalmology, pediatrics, and occupational therapy departments. MAIN OUTCOME MEASURES: Functional outcome, as documented by Teller visual acuity and formal occupational therapy testing, and anatomic outcome, as documented by Retcam photography and fluorescein angiography. RESULTS: Complete regression of extraretinal fibrovascular proliferation was observed 1 month after laser treatment. No retinal detachment had occurred to date at 24 months. Teller visual acuity at 23 months of life was 20/100 in both eyes. The patient's vision and developmental milestones were age appropriate. CONCLUSIONS: Pre-term genetic diagnosis with immediate laser treatment after birth may preserve vision in individuals affected with Norrie's disease.

CONCURRENT SCLERAL BUCKLE AND INTRAVITREAL BEVACIZUMAB FOR ADVANCED RETINOPATHY OF PREMATURITY-RELATED RETINAL DETACHMENT.

BACKGROUND: The preoperative use of intravitreal bevacizumab in Stage 4 or 5 retinopathy of prematurity (ROP) can reduce vascular endothelial growth factor load and bleeding risk; however, it can induce traction and exacerbate a tractional retinal detachment. Concurrent placement of a scleral buckle may reduce these risks and obviate future vitrectomy. METHODS: A retrospective analysis of eyes treated for Stage 4 and 5 ROP with concurrent intravitreal bevacizumab and scleral buckle was performed. Retinal reattachment and need for vitrectomy were studied. RESULTS: Thirteen eyes from 10 infants were treated with concurrent intravitreal bevacizumab and scleral buckle. Nine eyes were Stage 4a, 2 were Stage 4b, and 2 were Stage 5. All eyes with Stage 4 ROP achieved macular attachment at last follow-up. All the eyes with 4a ROP achieved retinal reattachment: 4 (44.4%) with buckle alone and 5 (55.6%) with subsequent vitrectomy. All 4 eyes with Stage 4b or 5 ROP required vitrectomy. CONCLUSION: Concurrent intravitreal bevacizumab and scleral buckle may serve as a bridge to vitrectomy and, in some instances, may obviate the need for subsequent intervention.

Hypoxic-ischemic injury induces macrophage inflammatory protein-1alpha expression in immature rat brain.

BACKGROUND AND PURPOSE: Macrophage inflammatory protein (MIP)-1alpha is a well-characterized monocyte chemoattractant; its role in regulating monocyte and microglial recruitment and activation in the injured neonatal brain is unknown. We evaluated the impact of acute hypoxic-ischemic (HI) brain injury on the expression of MIP-1alpha in neonatal rat brain. METHODS: To elicit forebrain ischemic injury, 7-day-old (P7) rats underwent right carotid ligation, followed by 2.5 hours of 8% oxygen exposure. We used an enzyme-linked immunosorbent assay and immunohistochemistry to detect MIP-1alpha; double-labeling immunofluorescence assays were analyzed with confocal microscopy to identify cellular sources of MIP-1alpha. Immunocytochemistry assays were also used to detect 2 MIP-1alpha receptors, CCR1 and CCR5. RESULTS: We found marked increases in tissue concentrations of MIP-1alpha in the HI cerebral hemisphere, peaking from 8 to 72 hours after lesioning. Immunocytochemistry assays revealed that MIP-1alpha was constitutively expressed in physiologically activated microglia; from 8 to 120 hours after lesioning, MIP-1alpha immunoreactive monocytes and microglia accumulated in the lesion territory. In immunoreactive cells, MIP-1alpha was diffusely distributed throughout the cytoplasm at early post-HI time intervals; by 72 hours, MIP-1alpha immunoreactivity was typically concentrated adjacent to the nucleus, a pattern indicative of active MIP-1alpha production. In P7 to P12 brain, many cells expressed MIP-1alpha receptors; both CCR1 and CCR5 immunoreactivity were localized to endothelium and ependyma; CCR1-immunoreactive astrocytes and neurons and CCR5-immunoreactive microglia were also identified. CONCLUSIONS: These data implicate MIP-1alpha as a mediator of the complex and sustained inflammatory response initiated by perinatal HI braininjury.

An occult case of giant cell arteritis presenting with combined anterior ischemic optic neuropathy and cilioretinal artery occlusion.

A 61-year-old female presented with a moderate decrease in vision in the left eye. The patient denied any other ocular or systemic symptoms related to giant cell arteritis. Visual acuity was 20/50 in the left eye with a 2+ relative afferent pupillary defect and markedly abnormal color vision. Dilated fundus examination and flourescein angiography revealed optic disc edema as well as a cilioretinal artery occlusion. Erythrocyte sedimentation rate was only slightly elevated. Subsequent biopsy of the superficial temporal artery confirmed the diagnosis of giant cell arteritis. Cilioretinal arteries are anatomical variants derived from the short posterior ciliary arteries. Arteritic anterior ischemic optic neuropathy typically results from thrombotic occlusion of the short posterior ciliary arteries. Consequently, arteritic occlusion of the short posterior ciliary arteries can result in concomitant occlusion of the cilioretinal artery. This case highlights the situation where clinical symptoms were not suspicious for giant cell arteritis but the presence of an anterior ischemic optic neuropathy and a cilioretinal artery occlusion was virtually pathognomonic for giant cell arteritis.

Reactivation of retinopathy of prematurity after bevacizumab injection.

OBJECTIVE: To report late reactivation and progression of retinopathy of prematurity (ROP) after intravitreal bevacizumab monotherapy. METHODS: Retrospective review of 9 patients (17 eyes) with recurrence of ROP after initial treatment with intravitreal bevacizumab monotherapy. Data collected included (1) location and stage of ROP activity, (2) number and timing of treatments, and (3) structural outcomes. RESULTS: Mean age at treatment-requiring recurrence was 49.3 weeks (SD, 9.1 weeks; minimum, 37 weeks; maximum, 69 weeks) postmenstrual age (PMA). The mean time between initial treatment and treatment-requiring recurrence was 14.4 weeks, with a minimum of 4 and maximum of 35 weeks. Fives eyes progressed to retinal detachment (4 eyes stage 5, 1 eye stage 4a). Age at retinal detachment ranged from 49 to 69 weeks PMA with a median of 55 weeks PMA and mean of 58.4 weeks PMA. No eye that received laser treatment for recurrence progressed to retinal detachment. CONCLUSIONS: Although intravitreal bevacizumab treatment is effective in inducing regression of ROP, the effect may be transient. Recurrence can occur later in the course than with conventional laser therapy. Late retinal detachment can occur despite early regression. Longterm favorable structural outcome may require extended observation and retreatment. Laser may be a useful treatment for recurrences.

Intravitreal bevacizumab for neovascular glaucoma.

PURPOSE: To report 6-month and 1 year outcomes of eyes treated for neovascular glaucoma (NVG) with intravitreal bevacizumab injection and panretinal laser (PRP) compared to those receiving PRP alone. DESIGN: retrospective, consecutive case series. METHODS: Charts of patients with NVG from retinal ischemia and at least 6 months of follow-up were reviewed. Patients were treated with one injection of 1.25 mg intravitreal bevacizumab followed by PRP or with PRP alone. The primary outcome was the long-term angle anatomy. Secondary measures included intraocular pressure (IOP), visual acuity, patient compliance, and control of systemic diseases. RESULTS: Fourteen eyes of 12 patients treated with bevacizumab and PRP and 15 eyes of 11 patients treated with PRP alone were included in the study. Mean sectors of open angle at baseline was 1.31 in the bevacizumab group and 1.47 in the retinal ablation group (P = 0.73). Mean sectors of open angle was 2.14 and 1.18 in the bevacizumab and retinal ablation groups, respectively (P < 0.05) at 6-month follow-up, and 2.27 and 1.18, respectively (P < 0.05) at 1-year follow-up. Mean baseline IOP was 32.3 mmHg (+/-14.8) in the bevacizumab group and 31.8 mmHg (+/-13) in the PRP group (P = 0.75). At 6-month follow-up, the mean IOP was 18.28 mmHg (+/-10) in the bevacizumab group and 23.33 mmHg (+/-14.6) in the PRP group (P = 0.05), and 19.12 mmHg (+/-6.8) and 26.2 mmHg (+/-18) (P = 0.1), respectively at 1-year follow-up. Nineteen patients were judged to be noncompliant, 10 had uncontrolled diabetes and 7 had uncontrolled hypertension. CONCLUSIONS: This study documents better long-term preservation of open angle and IOP control in eyes receiving bevacizumab along with PRP. We stress that NVG is still associated with poor visual acuity outcomes.

Extended-wear silicone hydrogel soft contact lenses in the management of moderate to severe dry eye signs and symptoms secondary to graft-versus-host disease.

PURPOSE: To determine the safety and efficacy of the Focus NIGHT & DAY (CIBA Vision, Duluth, GA) silicone hydrogel contact lens in the management of refractory, moderate to severe dry eye signs and symptoms secondary to graft-versus-host disease (GVHD). METHODS: Seven patients with GVHD and moderate to severe dry eye disease as determined by the Ocular Surface Disease Index (OSDI) questionnaire were fitted with a near plano Focus NIGHT & DAY soft contact lens (SCL) used on a 7-night continuous-wear basis. Visual acuity, objective measures of dry eye disease (i.e., Schirmer I, tear breakup time, and corneal fluorescein staining), and OSDI scores were compared before SCL wear and after 1 week and 1 month of SCL wear. RESULTS: There was significant improvement in subjective assessment of dry eye symptoms (initial vs. 1-month OSDI score, 76.8 +/- 13.6 vs. 31.2 +/- 17.8, P<0.0005, paired t test). In addition, patients had significant improvement in best-corrected visual acuity after 1 month of SCL wear (initial vs. 1-month logMAR visual acuity for the right eye, 0.23 +/- 0.050 vs. 0.04 +/- 0.027, P<0.007; initial vs. 1-month logMAR visual acuity for the left eye, 0.22 +/- 0.049 vs. 0.04 +/- 0.020, P<0.007, analysis of variance, Dunnett post hoc). There were no significant changes in results of Schirmer I testing, corneal fluorescein staining, or tear breakup time. No adverse events or complications of SCL wear were observed. CONCLUSIONS: The Focus NIGHT & DAY contact lens can improve subjective dry eye symptoms and visual acuity in patients with refractory dry eye disease secondary to GVHD.