Reversible Cerebral Vasoconstriction Syndrome Presenting With Visual Field Defects.

A 45-year-old woman with a history of depression and anxiety, treated with selective serotonin reuptake inhibitors (SSRIs), experienced acute, recurrent, and severe bifrontal headaches. Over time, she developed a left homonymous hemianopia and mental status changes. MRA revealed segmental vasoconstriction of cerebral arteries in multiple vascular distributions. She was treated with a calcium-channel blocker and magnesium, and there was resolution of her symptoms and cerebrovascular changes. Her clinical course and neuroimaging findings were consistent with reversible cerebral vasoconstriction syndrome. Although rare, this disorder frequently manifests with visual complaints. Although the prognosis is generally favorable, patients with this syndrome require prompt diagnosis and care directed to avoid complications including stroke, seizure, and subarachnoid hemorrhage.

Ocular and orbital pain for the headache specialist.

Ocular pain is most commonly associated with redness and inflammation; however, eye pain can also occur in the absence of grossly visible pathology. Pain in the quiet eye can be the first sign of a number of threatening conditions. Many of these conditions such as intermittent angle closure glaucoma, carotid artery dissection, idiopathic intracranial hypertension, and giant cell arteritis can lead to permanent vision loss or blindness. In this review, ocular history and examination techniques are summarized. The article also reviews pertinent ocular, orbital, referred, and other causes of eye pain in the quiet eye. The neurologist and headache specialist should recognize when consultation with an ophthalmologist is necessary.

Traumatic Retinal Detachment in Patients with Self-Injurious Behavior: An International Multicenter Study.

PURPOSE: To describe the clinical characteristics, surgical outcomes, and management recommendations in patients with traumatic rhegmatogenous retinal detachment (RRD) resulting from self-injurious behavior (SIB). DESIGN: International, multicenter, retrospective, interventional case series. PARTICIPANTS: Patients with SIB from 23 centers with RRD in at least 1 eye. METHODS: Clinical histories, preoperative assessment, surgical details, postoperative management, behavioral intervention, and follow-up examination findings were reviewed. MAIN OUTCOME MEASURES: The rate of single-surgery anatomic success (SSAS) was the primary outcome. Other outcomes included new RRD in formerly attached eyes, final retinal reattachment, and final visual acuity. RESULTS: One hundred seven eyes with RRDs were included from 78 patients. Fifty-four percent of patients had bilateral RRD or phthisis bulbi in the fellow eye at final follow-up. The most common systemic diagnoses were autism spectrum disorder (35.9%) and trisomy 21 (21.8%) and the most common behavior was face hitting (74.4%). The average follow-up time was 3.3 ± 2.8 years, and surgical outcomes for operable eyes were restricted to patients with at least 3 months of follow-up (81 eyes). Primary initial surgeries were vitrectomy alone (33.3%), primary scleral buckle (SB; 26.9%), and vitrectomy with SB (39.7%), and 5 prophylactic SBs were placed. Twenty-three eyes (21.5%) with RRDs were inoperable. The SSAS was 23.1% without tamponade (37.2% if including silicone oil), and final reattachment was attained in 80% (36.3% without silicone oil tamponade). Funnel-configured RRD (P = 0.006) and the presence of grade C proliferative vitreoretinopathy (P = 0.002) correlated with re-detachment. The use of an SB predicted the final attachment rate during the initial surgery (P = 0.005) or at any surgery (P = 0.008. These associations held if restricting to 64 patients with ≥12 months followup. Anatomic reattachment correlated with better visual acuity (P < 0.001). CONCLUSIONS: RRD resulting from SIB poses therapeutic challenges because of limited patient cooperation, bilateral involvement, chronicity, and ongoing trauma in vulnerable and neglected patients. The surgical success rates were some of the lowest in the modern retinal detachment literature. The use of an SB may result in better outcomes, and visual function can be restored in some patients.

Frequency of Rhegmatogenous Retinal Detachment after Intravitreal Therapy in Neovascular Age-Related Macular Degeneration.

PURPOSE: To identify characteristics of neovascular age-related macular degeneration (nAMD) patients undergoing pars plana vitrectomy (PPV) after rhegmatogenous retinal detachment (RRD), including changes to injection intervals. DESIGN: Single-center retrospective, consecutive review. PARTICIPANTS: All patients with RRD receiving anti-vascular endothelial growth factor treatment for nAMD from January 1, 2014, through October 30, 2018. METHODS: Billing codes were used to identify RRD that occurred within 90 days of a previous intravitreal injection for nAMD. MAIN OUTCOME MEASURES: Outcome measures included the quadrant of the retinal break(s), visual acuity at the time of RRD and final follow-up, and postoperative injection frequency. RESULTS: An exact total of 203 000 intravitreal injections for nAMD were administered. Seventeen eyes from 17 patients demonstrated RRDs, giving a rate of 1 RRD per 11 941 intravitreal injections (0.0084%) within 90 days of intravitreal injection. Patients received a mean of 27.56 injections in the superotemporal quadrant before RRD. Of known retinal breaks, the superotemporal quadrant was involved most frequently (10 of 16 eyes [62.5%]). Six patients (35.3%) required a second surgery. Of patients requiring postoperative injections, the average interval increased from 7.18 weeks to 9.17 weeks after surgery. Eleven of 17 patients (64.7%) either increased their injection intervals or required no further injections, 3 maintained similar intervals, and 3 decreased intervals. The average number of injections in the 6 months before RRD (n = 84) and the 6 months after the first injection after PPV (n = 47) was 4.94 ± 1.89 and 2.76 ± 2.44, respectively (P = 0.009). CONCLUSIONS: Based on these results, the 90-day rate of RRD in nAMD patients receiving intravitreal injections is low. Rhegmatogenous retinal detachments in these patients may be more difficult to repair. Although many physicians worry about injection frequency in vitrectomized eyes because of a presumed increased medication clearance, this study found that most patients received fewer injections after surgery.

Reconsidering the Postoperative Day 0 Visit for Retina Surgery.

BACKGROUND AND OBJECTIVE: This study compares outcomes of patients who were examined on postoperative (PO) day 0 and PO day 1 following retina surgery. PATIENTS AND METHODS: A nonrandomized, comparative, retrospective series of one vitreoretinal surgeon was conducted on 166 patients with a PO day 0 visit (approximately 5 hours following surgery) and 428 patients with a PO day 1 visit. RESULTS: Among patients examined at PO day 0, 4.6% had hypotony (intraocular pressure [IOP] ≤ 5 mm Hg) and 1.8% needed intervention for elevated IOP (IOP ≥ 30 mm Hg) compared with 1.6% and 4.0% of patients, respectively, examined on PO day 1. CONCLUSION: Following retina surgery, there is a similar percentage of patients with abnormal IOP when patients are examined on PO day 0 when compared with PO day 1. However, the rate of hypotony was statistically higher on PO day 0, and there was a trend toward more elevated IOP on PO day 1. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e52-e56.].

Multi-modal imaging and anatomic classification of the white dot syndromes.

The white dot syndromes (WDS) are a diverse group of posterior uveitidies that share similar clinical findings but are unique from one another. Multimodal imaging has allowed us to better understand the morphology, the activity and age of lesions, and whether there is CNV associated with these different ocular pathologies. The "white dot syndromes" and their uveitic masqueraders can now be anatomically categorized based on lesion localization. The categories include local uveitic syndromes with choroidal pathology, systemic uveitic syndromes with choroidal pathology, and multifocal choroiditis with outer retinal/choriocapillaris pathology with uveitis and without uveitis. Neoplastic and infectious etiologies are also discussed given their ability to masquerade as WDS.

Hermansky-Pudlak syndrome (HPS5) in a nonagenarian.

Hermansky-Pudlak syndrome (HPS) is an autosomal-recessive disorder clinically characterized by oculocutaneous albinism, bleeding diatheses, and lysosomal accumulation of ceroid lipofuscin, which in some cases may cause granulomatous colitis and pulmonary fibrosis. Any of these complications could result in a shortened life span for patients with HPS. We report a 92-year-old man with HPS 5 who, to our knowledge, is the oldest patient with HPS documented in the literature. This report highlights the importance of typing HPS to counsel patients regarding disease prognosis.