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Purpose: To measure the accuracy of image-based retinopathy of prematurity (ROP) diagnosis by pediatric ophthalmology fellows., Methods: This was a comparative case series of expert versus nonexpert clinicians in image-based ROP diagnosis. An atlas of 804 retinal images was captured from 248 eyes of 67 premature infants with a wide-angle camera (RetCam-II, Clarity Medical Systems, Pleasanton, CA). Images were uploaded to a study website from which an expert pediatric retinal specialist and five pediatric ophthalmology fellows independently provided a diagnosis (no ROP, mild ROP, type 2 ROP, or treatment-requiring ROP) for each eye. Two different retinal specialists experienced in ROP examination served as additional controls. Primary outcome measures were sensitivity and specificity of image-based ROP diagnosis by fellows compared to a reference standard of image-based interpretation by the expert pediatric retinal specialist. Secondary outcome measure was intraphysician reliability., Results: For detection of mild or worse ROP, the mean (range) sensitivity among the five fellows was 0.850 (0.670-0.962) and specificity was 0.919 (0.832-0.964). For detection of type 2 or worse ROP by fellows, mean (range) sensitivity was 0.527 (0.356-0.709) and specificity was 0.938 (0.777-1.000). For detection of treatment-requiring ROP, mean (range) sensitivity was 0.515 (0.267-0.765) and specificity was 0.949 (0.805-1.00)., Conclusions: Pediatric ophthalmology fellows in this study demonstrated high diagnostic specificity in image-based ROP diagnosis; however, sensitivity was lower, particularly for clinically significant disease., (Copyright © 2011. Published by Mosby, Inc.)

Purpose: To examine the relationship between rate of vascular change and plus disease diagnosis., Design: Retrospective observational case-control study., Methods: Wide-angle images were taken bilaterally from 37 infants at 31 to 33 weeks and 35 to 37 weeks postmenstrual age (PMA). The semi-automated Retinal Image multiScale Analysis system was used to measure parameters for all arteries and veins: integrated curvature, diameter, and tortuosity index. A reference standard diagnosis (plus vs not plus) was defined for each eye by consensus of 5 experts at 35 to 37 weeks PMA. Weekly rate of change in parameters was compared in eyes with plus vs not plus disease. Receiver operating characteristic area under the curve (AUC) was calculated for plus disease detection based on 1) weekly rates of parameter change between 31 to 33 weeks and 35 to 37 weeks PMA and 2) parameter values at 35 to 37 weeks only., Results: Weekly rates of change in all venous parameters were significantly different in eyes with plus vs not plus disease, particularly for tortuosity index (P < .0004) and diameter (P = .018). Using weekly rate of change, AUC for plus disease detection was highest for venous tortuosity index (0.819) and venous diameter (0.712). Using the 35 to 37-week PMA image only, AUC was highest for venous integrated curvature (0.952) and diameter (0.789)., Conclusion: Rate of change in venous, but not arterial, parameters is correlated with plus disease development in this data set. This did not appear to contribute information beyond analysis of an image at 35 to 37 weeks PMA only., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to report a case of acute retinal necrosis after combined cataract surgery and intracameral triamcinolone acetonide injection in a healthy elderly patient., Methods: Testing used was a clinical examination including fundus photographs, fluo-rescein angiography, and serologic testing., Results: A 75-year-old healthy white woman undergoing cataract extraction received an injection of intracameral triamcinolone acetonide as a substitute for postoperative topical steroids. Two weeks later, the patient developed acute retinal necrosis, which responded well to systemic antiviral therapy., Conclusion: Acute retinal necrosis is a rare, but potentially devastating, complication that may be associated with intraocular triamcinolone acetonide injection.

Purpose: The purpose of this study was to measure the accuracy of retinopathy of prematurity (ROP) diagnosis by retinal fellows., Methods: An atlas of 804 retinal images was captured from 248 eyes of 67 premature infants with a wide-angle camera (RetCam-II, Clarity Medical Systems, Pleasanton, CA). Images were uploaded to a study Web site, from which an expert pediatric retinal specialist and 7 retinal fellows independently provided a diagnosis (no ROP, mild ROP, type 2 ROP, or treatment-requiring ROP) for each eye. The sensitivity and specificity of each retinal fellow were calculated and subsequently compared with a reference standard of diagnosis by an expert pediatric retinal specialist., Results: For detection of type 2 or worse ROP by fellows, mean (range) sensitivity was 0.751 (0.512-0.953), and specificity was 0.841 (0.707-0.976). For detection of treatment-requiring ROP, mean (range) sensitivity was 0.914 (0.667-1.000), and specificity was 0.871 (0.678-0.987)., Conclusion: In general, fellows showed high accuracy for detecting ROP. However, 3 of 7 fellows achieved <80% sensitivity for diagnosis of type 2 or worse ROP, and 2 of 7 achieved <90% sensitivity for diagnosis of treatment-requiring ROP. This could lead to undermanagement and undertreatment of clinically significant disease and raises potential concerns about the quality of ROP screening examinations performed by less-experienced examiners.

Background/aims: To assess accuracy of telemedical retinopathy of prematurity (ROP) diagnosis by trained non-expert graders compared with expert graders., Methods: Eye examinations (n=248) from 67 consecutive infants were captured using wide-angle retinal photography (RetCam-II, Clarity Medical Systems, Pleasanton, California, USA). Non-expert graders attended two 1-h training sessions on image-based ROP diagnosis. Using a web-based telemedicine system, 14 non-expert and three expert graders provided a diagnosis for each eye: no ROP, mild ROP, type 2 pre-threshold ROP or treatment-requiring ROP. All diagnoses were compared with a reference standard of dilated indirect ophthalmoscopy by an experienced paediatric ophthalmologist., Results: For detection of type 2 or worse ROP, the mean (range) sensitivities and specificities were 0.95 (0.94-0.97) and 0.93 (0.91-0.96) for experts, 0.87 (0.71-0.97) and 0.73 (0.39-0.95) for resident non-experts, and 0.73 (0.41-0.88) and 0.91 (0.84-0.96) for student non-experts, respectively. For detection of treatment-requiring ROP, the mean (range) sensitivities and specificities were 1.00 (1.00-1.00) and 0.93 (0.88-0.96) for experts, 0.88 (0.50-1.00) and 0.84 (0.71-0.98) for resident non-experts, and 0.82 (0.42-1.00) and 0.92 (0.83-0.97) for student non-experts, respectively., Conclusions: Mean sensitivity and specificity of trained non-experts were lower than that of experts, although several non-experts had high accuracy. Development of methods for training non-expert graders may help support telemedical ROP evaluation.

Purpose: To compare performance of single-image vs multiple-image telemedicine examinations for retinopathy of prematurity (ROP) diagnosis., Design: Prospective comparative study., Methods: A total of 248 eyes from 67 consecutive infants underwent wide-angle retinal imaging by a trained neonatal nurse at 31 to 33 weeks and/or 35 to 37 weeks postmenstrual age (PMA) at a single academic institution. Data were uploaded to a web-based telemedicine system and interpreted by three masked retinal specialists. Diagnoses were provided based on single images, and subsequently on multiple images, from both eyes of each infant. Findings were compared to a reference standard of indirect ophthalmoscopy by a pediatric ophthalmologist. Primary outcome measures were recommended follow-up interval, presence of plus disease, presence of type-2 or worse ROP, and presence of visible peripheral ROP., Results: Among the three graders, mean sensitivity/specificity for detection of infants requiring follow-up in less than one week were 0.85/0.93 by single-image examination and 0.91/0.88 by multiple-image examination at 35 to 37 weeks PMA. Mean sensitivity/specificity for detection of infants with type-2 or worse ROP were 0.82/0.95 by single-image examination and 1.00/0.91 by multiple-image examination at 35 to 37 weeks PMA. Mean sensitivity/specificity for detection of plus disease were 1.00/0.86 by single-image examination and 1.00/0.87 by multiple-image examination at 35 to 37 weeks PMA. There were no statistically-significant intragrader differences between accuracy of single-image and multiple-image telemedicine examinations for detection of plus disease., Conclusions: Single-image and multiple-image telemedicine examinations perform comparably for determination of recommended follow-up interval and detection of plus disease. This may have implications for development of screening protocols, particularly in areas with limited access to ophthalmic care.

Objective: To prospectively measure accuracy, reliability, and image quality of telemedical retinopathy of prematurity (ROP) diagnosis., Methods: Two-hundred forty-eight eyes from 67 consecutive infants underwent wide-angle retinal imaging by a trained neonatal nurse at 31 to 33 weeks' and/or 35 to 37 weeks' postmenstrual age (PMA) using a standard protocol. Data were uploaded to a Web-based telemedicine system and interpreted by 3 expert retinal specialist graders who provided a diagnosis (no ROP, mild ROP, type 2 prethreshold ROP, treatment-requiring ROP) and an evaluation of image quality for each eye. Findings were compared with a reference standard of indirect ophthalmoscopy by an experienced pediatric ophthalmologist., Results: At 35 to 37 weeks' PMA, sensitivity and specificity for diagnosis of mild or worse ROP were 0.908 and 1.000 for grader A, 0.971 and 1.000 for grader B, and 0.908 and 0.977 for grader C. Sensitivity and specificity for diagnosis of type 2 prethreshold or worse ROP were 1.000 and 0.943 for grader A, 1.000 and 0.930 for grader B, and 1.000 and 0.851 for grader C. At 35 to 37 weeks' PMA, weighted kappa for intergrader reliability was 0.791 to 0.889, and kappa for intragrader reliability for detection of type 2 prethreshold or worse ROP was 0.769 to 1.000. Image technical quality was rated as "adequate" or "possibly adequate" for diagnosis in 93.3% to 100% of eyes., Conclusion: A telemedicine system using nurse-captured retinal images has the potential to improve existing shortcomings of ROP management, particularly at later PMAs.

Purpose: It is unclear whether protein C deficiency is associated with retinal venous occlusive disease., Design: We performed a cross-sectional study., Methods: Members of a protein C-deficient family, either deficient or nondeficient, with a history of nonocular venous thrombosis were included. All participants completed questionnaires regarding their medical and ophthalmic histories. Each subject underwent dilated direct ophthalmoscopic and binocular indirect ophthalmoscopic examinations by a single vitreoretinal specialist., Results: None of the 18 family members with a known history of nonocular thrombosis-12 with and 6 without protein C deficiency- manifested stigmas of recent or chronic retinal vascular occlusive disease., Conclusions: This study showed no evidence of involvement of the retinal vascular bed in a family with an increased risk of nonocular venous thrombosis attributable to the deficiency of protein C.

Background/aim: A lack of data exists concerning the development of late postoperative, non-proliferative vitreoretinopathy (PVR), rhegmatogenous retinal detachments (RRDs) after successful posterior segment intraocular foreign body (PSIOFB) removal. The authors present a series of PSIOFB cases over several years with posterior hyaloid separation resulting in RRD in two patients, 4 and 8 months after initial injury and vitrectomy. This report aims to increase awareness concerning the possibility of late RRDs complicating PSIOFB injuries and to emphasise careful long term observation., Methods: Medical records of consecutive cases referred for presumed PSIOFB injury during a 4 year period were retrospectively reviewed. All eyes referred for presumed PSIOFB injuries were included., Results: 11 patients were included in the series. Two patients had eyes so badly injured by large PSIOFBs that primary globe closure was followed within days by enucleation. Nine patients underwent pars plana vitrectomy for PSIOFB removal. Two patients experienced late RRDs that were managed with excellent long term visual outcomes., Conclusions: Late RRD may occur following successful removal of PSIOFBs, even several months after initial management. These RRDs may be successfully managed with a variety of methods, depending upon the extent and location of the detachment and causative break as well as surgeon comfort and preference.

Aim: A cross sectional (prevalence) study was performed to assess the usefulness and sensitivity of commonly employed criteria to identify infants for routine ophthalmoscopic screening for retinopathy of prematurity (ROP)., Methods: At a tertiary care centre between 1 January 1992 and 30 June 1998, experienced vitreoretinal specialists screened 438 premature infants for ROP. Retinal maturity and the presence of ROP were determined by indirect ophthalmoscopic examinations., Results: Of the eligible infants surviving 28 days, 276 (91.7%) of 301 infants with birth weights

Purpose: To describe a new method for salvaging externally exposed silicone scleral exoplants (buckles) to avoid removal and the consequent risk of retinal redetachment., Design: A retrospective, noncomparative, interventional case series., Participants: Four patients with exposed, clinically uninfected scleral buckles after initial surgery for the treatment of rhegmatogenous retinal detachment., Intervention: Processed human donor pericardium patch grafts (Tutoplast; Innovative Ophthalmic Products, INC:, Costa Mesa, CA) were used to cover exposed areas of scleral buckles in concert with conjunctivoplasty., Main Outcome Measures: Scleral buckle preservation was the goal of this new treatment strategy., Results: In four eyes treated with pericardial patch grafts to cover segments of exposed scleral buckles, three (75%) were managed successfully with one surgery. One eye (25%) had this treatment method fail and required scleral buckle removal., Conclusions: Processed human donor pericardium patch grafting is one useful way to avoid removing exposed scleral buckles, and consequently, sparing patients the risk of recurrent retinal detachment.

Purpose: To determine the in vitro permeability of the sclera to high molecular weight compounds and the relationship between scleral permeability and molecular size., Methods: Fresh rabbit sclera was mounted in a two-chamber diffusion apparatus, and its permeability to sodium fluorescein, fluorescein isothiocyanate (FITC)-conjugated bovine serum albumin, FITC-IgG, and FITC dextrans ranging in molecular weight from 4 to 150 kDa was determined by fluorescence spectrophotometry. Electron microscopy was used to assess the impact of the experimental design on scleral ultrastructural integrity. The effect of the diffusion apparatus on scleral hydration was examined. Rabbit scleral permeability was compared with previously reported data for human and bovine sclera., Results: Scleral permeability decreased with increasing molecular weight and molecular radius, consistent with previous human and bovine data. Molecular radius was a better predictor of scleral permeability than molecular weight. The sclera was more permeable to globular proteins than to linear dextrans of similar molecular weight. The experimental apparatus did not alter scleral ultrastructure. Permeability of rabbit sclera was similar to human sclera but greater than bovine sclera., Conclusions: Large molecules, such as IgG, diffuse across sclera in a manner consistent with porous diffusion through a fiber matrix. Transscleral delivery of immunoglobulins and other large compounds to the choroid and retina may be feasible.

Purpose: This study aimed to present two patients with delayed-onset, endogenous fungal keratitis after treatment for fungal endophthalmitis after cataract surgery., Methods: The authors retrospectively reviewed the clinical course of two patients in whom deep stromal fungal keratitis developed 2 weeks and 3 months, respectively, after apparent successful aggressive therapy for fungal endophthalmitis. Before the onset of the keratitis, both patients underwent vitrectomies with intraocular injection of 7.5 to 10.0 mcg amphotericin B, as well as treatment with systemic fluconazole and topical antifungal therapy. In case 1, a pre-existing prosthetic intraocular lens and lens capsular bag were removed at the time of vitrectomy, whereas in case 2, the intraocular lens was left in place and a posterior capsulectomy was performed., Results: The keratitis worsened in both patients, despite intensive systemic and topical antifungal therapy. Both patients underwent therapeutic penetrating keratoplasties. In case 1, this resulted in successful resolution of the infection and no recurrences 3 months after the transplant. The corneal transplant was complicated by an expulsive choroidal hemorrhage in the other patient. Fusarium solani was cultured from the initial vitrectomy specimen in patient 1, and although it was not cultured from the keratitis, septate hyphal elements were present on histopathologic examination. The causative organism in case 2 was Acremonium kiliense, which was cultured from both the original vitrectomy specimen and the deep corneal stromal infiltrate., Conclusions: Fungal organisms may not be eradicated completely from eyes with endophthalmitis despite aggressive therapy and apparent initial successful treatment. These patients need to be monitored for prolonged periods, and treatment should be reinitiated at the earliest sign of recrudescence of infection.

Purpose: To demonstrate ophthalmic findings of bacterial endocarditis in a patient with a cardiac structural anomaly and to illustrate the potential usefulness of transesophageal echocardiography in the examination of such patients., Methods: We examined a patient with bacteremia who had white-centered retinal hemorrhages. Complete clinical, laboratory, and echocardiographic evaluations were performed., Results: Streptococcal bacteremia was proven and, by using transesophageal echocardiography, a ventricular false chorda was demonstrated., Conclusions: Patients with white-centered retinal hemorrhages should undergo thorough systemic examinations. In this unusual case of such hemorrhages caused by bacteremia in a patient with a ventricular false chorda, modern, high-resolution transesophageal echocardiography played an important role in confirming the diagnosis.

Purpose: Four patients presented after cataract surgery with delayed-onset endophthalmitis caused by Acremonium kiliense with in vitro sensitivity to amphotericin B. In all patients, ocular infection was recalcitrant to single-dose intravitreous amphotericin B injection. The authors reviewed the management of endophthalmitis caused by A. kiliense and presented treatment recommendations., Methods: The authors retrospectively evaluated a cluster of four patients with delayed-onset postoperative endophthalmitis after phacoemulsification with posterior chamber intraocular lens implantation. All patients underwent vitreous sampling, intravitreous injection of amphotericin B, and systemic administration of fluconazole. Pars plana vitrectomy was performed in all patients for management of either primary (1 eye) or persistent infection (3 eyes). Two patients with persistent infection also underwent surgical explanation of their posterior chamber intraocular lens., Results: Worsening infection developed in three of three eyes that underwent vitreous aspiration with intravitreous injection 5 micrograms amphotericin B. These patients subsequently responded to vitrectomy followed by additional intravitreous amphotericin B injection. One eye underwent primary vitrectomy and intravitreous injection of 7.5 micrograms amphotericin B. Although treatment of the initial infection was successful, fungal keratitis developed in this patient 3 months after presentation. Visual outcome was variable, ranging from visual acuity of 20/25 to no light perception with follow-up of 2 years. Epidemiologic investigation suggested a common environmental source for the A. kiliense organisms., Conclusions: Single-dose administration of intravitreous amphotericin B was inadequate treatment for fungal endophthalmitis caused by A. kiliense. Vitrectomy with repeated intravitreous administration of amphotericin B may be necessary to eradicate intraocular function caused by this organism.

Battered/shaken baby syndrome is a clinical and pathologic diagnosis based on clinical examination, central nervous system dysfunction, and intracranial, optic nerve sheath, and retinal hemorrhages in infants under the age of three years. This report describes a case in which the battered/shaken baby syndrome was suspected because of an unusual parental reaction to an acute, mortal illness in their seven-week-old baby, as well as an acute intracranial hemorrhage coupled with the discovery of optic nerve sheath hemorrhages at necropsy. Thorough microscopic study, however, uncovered an unusual subarachnoid vascular malformation, the rupture of which led to the death of the infant. This case underscores the importance of complete postmortem examinations in cases of suspected child abuse, and teaches caution in jumping to hasty conclusions.

A 15-year-old girl presented with a left lower eyelid tumor consistent with a nonresolving chalazion. The specimen was biopsied. Histopathologic examination revealed eosinophilic granuloma, an entity that rarely presents on the eyelid. The histopathologic features of eosinophilic granuloma--a member of the histiocytosis X group of diseases--are reviewed. Our patient responded well to incisional biopsy and has required no additional treatment.