Progression of retinitis pigmentosa on multimodal imaging: The PREP-1 study.

IMPORTANCE: It is important to establish reliable outcome measures to detect progression in retinitis pigmentosa (RP). BACKGROUND: To evaluate progression of RP using multimodal imaging, including spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF) and microperimetry (MP). DESIGN: Retrospective longitudinal study at a tertiary teaching hospital. PARTICIPANTS: 205 eyes of 106 patients with RP with 1 to 5 y of follow-up. METHODS: Demographics and visual acuity (VA) were recorded, and each modality was graded at baseline and every annual follow-up. SD-OCT was graded for the width of ellipsoid zone (EZ), FAF was graded for the diameter and area of the hyperautofluorescent ring (if present), and MP was graded for mean, central and paracentral sensitivity. Spearman's correlation was used to measure correlations at baseline. Mixed effects models were used to estimate the annual change of each parameter, adjusted for disease duration. MAIN OUTCOME MEASURES: Rate of progression. RESULTS: The median VA at baseline was 75 letters and was positively correlated with mean and central sensitivity (r: 0.372 and 0.394; P = 0.01 for both). All parameters (except paracentral sensitivity) were strongly correlated with each other (r: 0.673-0.991; P < 0.001 for all). The annual rates of change for each parameter were as follows: VA, -2.3 letters (P < 0.001); EZ, -151 µm (P < 0.001); ring diameter, -132 µm (P < 0.001); ring area, -0.4 mm2 (P < 0.001); mean sensitivity, -0.3 dB (P < 0.001); central sensitivity, -0.7 dB (P < 0.001); paracentral sensitivity, -0.4 dB (P < 0.001). CONCLUSIONS AND RELEVANCE: Structural and functional measures are well correlated in RP and can reliably measure disease progression within the course of a year.

Epidemiology of Primary Ophthalmic Inpatient Admissions in the United States.

PURPOSE: To determine the national estimates, demographics, and costs of inpatient eye care in the United States (US). DESIGN: Retrospective cross-sectional study. METHODS: National Inpatient Sample (NIS), a representative sample of all US community hospitals, was used to analyze inpatient admissions with a primary ophthalmic diagnosis from 2001 to 2014. National estimates of the most prevalent diagnoses were determined, and descriptive statistics were calculated for demographics and costs. RESULTS: From 2001 to 2014, there were an estimated 671 324 inpatient admissions (male patients, 51.6%; mean [standard deviation] age, 44.5 [27.3] years) in the US owing to an ophthalmic disorder-an annual rate of 16 per 100 000 population. The Mid-Atlantic region had the highest rate. Most admissions were owing to nontraumatic disorders (75.3%), classified as emergencies (41.8%), and covered by public insurance (48.9%). The median length of stay was 2 days and mortality was 0.2%. The total inflation-adjusted cost over the 14-year period was $5.9 billion. The most prevalent diagnosis was orbital cellulitis (14.5%), followed by orbital floor fracture (9.6%) and eyelid abscess (6.0%). Most diagnoses were infectious (28.0%) and the majority were attributed to external disease (24.3%). A total of 31.1% of all patients had an ophthalmic procedure, pars plana vitrectomy (4.8%) being the most common one. CONCLUSION: There were around 48 000 ophthalmic inpatient admissions in the US costing $421 million every year. Orbital pathology, namely infection and trauma, was the leading cause of admissions. Implementing interventions to decrease the incidence of these conditions may significantly reduce the burden of inpatient ophthalmic care.

Progression of Retinitis Pigmentosa as Measured on Microperimetry: The PREP-1 Study.

PURPOSE: To evaluate yearly progression of retinitis pigmentosa (RP) using microperimetry (MP) performed on Nidek MP1 (NAVIS Software v1.7; Nidek Technologies, Padova, Italy). DESIGN: Retrospective longitudinal study. PARTICIPANTS: RP patients with consecutive MP tests (using the same test settings). METHODS: Data were collected as part of the Photoreceptor Cell Death in Retinitis Pigmentosa Retrospective (PREP-1) study. Visual acuity, fixation stability, mean sensitivity, and regional sensitivity were assessed at baseline and at yearly follow-up appointments. Regional sensitivity was calculated based on 2 methods. Method 1 involved topographical division into central macula (CM) and paracentral macula (PM). Method 2 involved functional division into the edge of scotoma (ES) and the seeing retina (SR). Linear mixed-effects models were used to assess the annual rate of change for each parameter, adjusted for disease duration. MAIN OUTCOME MEASURES: Annual rate of change of visual acuity, fixation stability, and retinal sensitivities (mean sensitivity and regional sensitivities using methods 1 and 2). RESULTS: In total, 75 eyes of 39 patients (median age, 56 y; males, 57%) with a follow-up period ranging from 1 to 4 years were reviewed. Visual acuity at baseline was positively correlated with all retinal sensitivity parameters, most strongly with CM sensitivity (r = 0.545, P < 0.001). There was no change in visual acuity (P = 0.075) or fixation stability (P = 0.371) per year. All retinal sensitivity parameters had a significant decline per year (P < 0.001), with a decline of 0.4 decibel (dB) for mean sensitivity, 0.6 dB for CM, 0.3 dB for PM, 1.3 dB for ES, and 1.1 dB for SR. Method 2 identified the greatest number of cases, with a significant decline in regional sensitivity. CONCLUSION: MP can detect significant changes in regional sensitivity over a 1-year period in patients with RP, even as visual acuity and fixation remain stable. An individualized approach to analyzing retinal sensitivity derived from MP may offer a useful outcome measure for future clinical trials.