Incidence and visual outcomes of acute endophthalmitis post intravitreal injection of anti-vascular endothelial growth factors in a single referral center.

BACKGROUND: To describe the incidence and factors predicting visual outcome in patients with infectious endophthalmitis following intravitreal anti-VEGF injection. METHODS: Retrospective, single-site, cohort study. Patients with acute endophthalmitis within 6 weeks of intravitreal anti-VEGF injection who were referred to our practice after inciting injection or were injected by us between January 2010 and July 2017 were included. All patients received intravitreal antibiotics with either vitreous/anterior chamber tap (TAP) or pars plana vitrectomy. Visual outcomes pre/post treatment, baseline variables (age, gender, ocular disease) and cultures results were studied. RESULTS: Seventy eyes of 69 patients were included. Presenting VA was the strongest factor associated with final visual outcome after adjusting for other variables including culture status and baseline VA (p = .0002). Cultures were positive in 62.8% of eyes and were associated with worse visual outcome (p = .0087). Growth of Streptococcus or microorganisms other than coagulase negative Staphylococci (CNS) was also associated with worse prognosis, regardless of baseline and presenting VA (p = .0002). The crude incidence of post-injection endophthalmitis was 0.028% in our practice (40 eyes in 143,628 injections) during the study time. No significant difference was found between pre-filled bevacizumab versus ranibizumab or aflibercept drawn from a vial. CONCLUSIONS: In a large, single center, retrospective study, the incidence of acute endophthalmitis post anti-VEGF injection was relatively low. Worse visual acuity at presentation of endophthalmitis and growth of Streptococcus or organisms other than CNS were associated with the worst visual outcomes.

Delayed-Onset Propionibacterium acnes Endophthalmitis in the Absence of Capsular Complex Following Scleral-Fixated Intraocular Lens: A Case Report.

Purpose: This work reports a case of delayed-onset Propionibacterium acnes endophthalmitis in a patient with scleral-fixated intraocular lens (IOL), successfully treated with intravitreal antibiotics and steroids. Methods: Patient underwent complete ophthalmic examinations over 2 years. Vitreous cultures, optical coherence tomography, anterior segment and fundus photographs, and fluorescein angiogram were performed. Results: A 78-year-old man with dislocated IOL underwent IOL removal and scleral-fixated AO60. He did well for 2 months but returned 9 months later with vision loss. Examination revealed low-grade inflammation and multiple IOL opacities. Vitreous culture grew P acnes. He was treated with intravitreal clindamycin followed by dexamethasone for macular edema without IOL explantation. A year after treatment no inflammation or macular edema was noted. Conclusion: To our knowledge, this is the first case of P acnes endophthalmitis following scleral sutured IOL. Treatment with intravitreal injections alone, without IOL explantation, was effective, possibly because of the absence of capsular complex.

The Expanded Clinical Spectrum of Coxsackie Retinopathy.

The authors present two cases of coxsackie retinopathy that presented with the classic findings of acute outer retinal disruption that evolved to an outer retinal scar. In these two cases, the classic lesions displayed an extramacular distribution. The authors provide detailed multimodal imaging in both cases highlighting the nature of the lesions. It is important to consider coxsackie virus infection in the differential diagnosis of extramacular lesions with outer retinal disruption. The term "unilateral acute idiopathic maculopathy" should be replaced with "coxsackie retinopathy" to better align with the expanded clinical spectrum and the known etiology of this disorder. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:529-532.].

A proposal of an algorithm for the diagnosis and treatment of recurrence or treatment failure of retinopathy of prematurity after anti-VEGF therapy based on a large case series.

PURPOSE: To provide a diagnostic algorithm of recurrence and treatment failure after intravitreal bevacizumab (IVB) injection for retinopathy of prematurity type 1 (ROP1) and the stepwise therapeutic approach for both conditions. METHODS: Retrospective chart review of all patients diagnosed with ROP1 initially treated with IVB in 6 tertiary referral centers of Toluca and Mexico City from 2005 to 2017. Treatment failure was defined as persistence or progression of neovascularization, elevation of the ridge, worsening of plus disease, or retinal crunch within the first week after treatment. Recurrence was defined as the new appearance of plus disease, an elevated ridge, or pathological new vessels after an initial regression of ROP following treatment. Therapy was observation, switch of anti-VEGF agent, retinal photocoagulation, vitrectomy, or a combination of two or more, depending on the severity of findings. RESULTS: A total of 672 patients who received intravitreal bevacizumab injection for ROP1 treatment were included. Of these, 2.5% (17 patients) failed to treatment, 6.8% (46 patients) developed a recurrence for ROP, and 5.5% (37 patients) carried a misdiagnosis of recurrence and were diagnosed with other than ROP1 after examination. Based on the severity of findings, patients with recurrence or treatment failure were further treated by observation, repeat anti-VEGF intravitreal injection (bevacizumab or other), laser photocoagulation, vitrectomy, or a combination of these. Based on the treatment results, a therapeutic algorithm was created. CONCLUSIONS: Intravitreal injection of anti-VEGFs for the treatment of ROP warrants close follow-up as some of these patients may have treatment failure or recurrence of the disease. It is crucial to differentiate between them to avoid a misdiagnosis and offer the correct treatment. We propose a novel algorithm for the follow-up and treatment approach of ROP1 following initial treatment with IVB. This algorithm offers a summary of our recommendations based on a large case series of ROP1 patients. It is meant to grow and expand as more clinical evidence becomes available.

Comparison of training effect on tremor using 2 training modules.

PURPOSE: To compare training effect of 2 training models-a surgical simulator anti-tremor module and a paper version-on tremor and time-to-task completion. SETTING: Ophthalmology Department, Veterans Affairs Boston Healthcare System, Jamaica Plain, Massachusetts, USA. DESIGN: Prospective crossover study. METHODS: Trainees completed simulator and paper training modules (baseline test, 3 training sessions, posttraining test, and final test) with their dominant and nondominant hands. The change in tremor, number of paper errors, and time-to-task completion in dominant and nondominant hands were compared. The 2 training modules were compared using nonparametric tests. RESULTS: The study comprised 19 trainees. There was a moderate correlation between average tremor values (simulator, 3-dimensional module) and paper errors (paper, 2-dimensional module) (Spearman ⍴ = 0.35, P < .0001). Practice on the simulator or paper modules did not reduce tremor significantly from baseline to final tasks for both hands combined (P = .12, simulator; P = .2, paper). Practice on the training modules improved time-to-task completion in the simulator module and paper module (both P < .0001). The improvement in time from baseline to final tasks was greater in the nondominant hands in the simulator module (improvement 64.5% over baseline time) than in the paper module (53.6% over baseline time). CONCLUSION: Practice might not reduce tremor but improved the outcome measure of time, and results suggest that trainees can learn to compensate for tremor in both hands, which is important in bimanual microsurgery.

Outcomes after cataract surgery in eyes with pseudoexfoliation: Results from the Veterans Affairs Ophthalmic Surgery Outcomes Data Project.

OBJECTIVE: To compare clinical outcomes of cataract surgery in eyes with and without pseudoexfoliation (PXF). DESIGN: Retrospective deidentified data analysis. PARTICIPANTS: A total of 123 PXF and 4776 non-PXF eyes of patients who underwent cataract surgery. METHODS: We compared data on visual acuity, Visual Function Questionnaire (VFQ)-based quality of life, and complications in PXF and non-PXF eyes from the Veterans Affairs (VA) Ophthalmic Surgery Outcomes Data Project across 5 VA medical centres. RESULTS: Pupillary expansion devices were used in 31 (25.2%) PXF cases and 398 (8.4%) non-PXF cases (p < 0.0001). Capsular tension rings were used in 6 (4.9%) PXF cases and 55 (1.2%) non-PXF cases (p < 0.004). The following complications occurred more frequently in PXF cases: zonular dehiscence without vitrectomy (4 [3.3%] PXF cases vs 40 [0.8%] non-PXF cases p = 0.02), persistent inflammation (28 [24.1%] vs 668 [14.5%]; p = 0.007), and persistent intraocular pressure elevation (5 [4.3%] vs 68 [1.5%]; p = 0.03). Best corrected visual acuity (BCVA) improved in both groups after 1 month, but 87 (83.7%) PXF cases achieved postoperative BCVA better than or equal to 20/40 compared to 3991 (93.8%) non-PXF cases (p = 0.0003). There was no significant difference in the postoperative composite VFQ scores between PXF (82.1 ± 16.9) and non-PXF cases (84.2 ± 16.8, p = 0.09). CONCLUSIONS: Several complications occurred more frequently in the PXF group compared to the non-PXF group, and fewer PXF cases achieved BCVA better than or equal to 20/40. Despite this, both groups experienced similar improvement in vision-related quality of life after cataract surgery.

CONSTRUCT AND FACE VALIDITY OF THE EYESI INDIRECT OPHTHALMOSCOPE SIMULATOR.

PURPOSE: To evaluate construct and face validity of the Eyesi Binocular Indirect Ophthalmoscope Simulator. METHODS: The performance of 25 medical students (Group A) was compared with that of 17 ophthalmology and optometry trainees (Group B) on the Eyesi Binocular Indirect Ophthalmoscope Simulator. During the course of a single session, each participant viewed an orientation module followed by an instruction session and a demonstration case, and performed 6 cases of progressively increasing difficulty (4 levels) and a 10-question face validity questionnaire. Outcomes included total score, total examination time, percent retina examined, and duration of eye exposure to light. RESULTS: Group B achieved significantly better total scores than Group A on all difficulty levels (P = 0.02, P = 0.001, P = 0.001, and P = 0.0001, for Levels 1-4, respectively) and had a significantly faster mean duration of examination (8 minutes 58 seconds vs. 5 minutes 21 seconds, P < 0.0001). Medical students reported higher scores in the face validity questionnaire for the simulator experience being helpful at orienting them to true indirect ophthalmology, and that further training on the simulator would improve their skills in the clinic (P = 0.03 for all). CONCLUSION: The Eyesi Binocular Indirect Ophthalmoscope Simulator has significant construct and face validity and shows promise for medical education.

Management of Corneal Bee Sting Injuries.

PURPOSE: To review the management of keratitis after corneal bee stings and to report a case of deep stromal corneal infiltrate secondary to a retained bee stinger managed conservatively in a patient who presented three days after unsanitary manipulation of the stinger apparatus. METHODS: Case report and review of literature. RESULTS: A 57-year-old male beekeeper was evaluated for pain, blurry vision, and photosensitivity after a corneal bee sting. Of note, the venom sac had been removed with dirty tweezers three days prior to his visit. On exam, a focal infiltrate with diffuse edema was seen surrounding a retained bee stinger in the peripheral cornea. Trace cells in the anterior chamber were also noted. Based on a high suspicion for infectious keratitis, a conservative treatment strategy was elected. Administration of broad-spectrum topical antibiotics with concomitant abstention of corticosteroids led to rapid resolution of the symptoms. Over 16 months of follow-up, the stinger has remained in situ without migration and the patient has maintained 20/20 visual acuity without complications. There is debate on the preferred method for the management of corneal injury secondary to bee stings, especially when it is associated with a retained stinger. We herein present our findings in our appraisal of reported cases. CONCLUSION: In the aftermath of an ocular bee sting, close surveillance for inflammation and infection is essential. Individual manifestations of these injuries vary in timing, type, and severity; therefore, the accessibility of the stinger and the evolving clinical picture should guide therapeutic decisions.

American Society of Anesthesiologists classification in cataract surgery: Results from the Ophthalmic Surgery Outcomes Data Project.

PURPOSE: To explore the association of American Society of Anesthesiologists (ASA) classification with cataract surgery outcomes. SETTING: Five Veterans Affairs Medical Centers, United States. DESIGN: Retrospective observational cohort study. METHODS: The study analyzed the outcomes of cataract surgery cases. Corrected distance visual acuity (CDVA), unanticipated events, and vision-related quality of life (VRQL) were assessed using the National Eye Institute Visual Function Questionnaire (NEI-VFQ), comparing ASA classes I through IV. For some analyses, ASA classes I and II were designated as Group A and ASA classes III and IV were designated Group B. RESULTS: Of the 4923 cases, 875 (17.8%) were in Group A, 4032 (81.9%) were in Group B, and 16 (0.3%) had missing data. The mean CDVA and mean composite NEI-VFQ score improved after cataract surgery in both groups (P < .0001); however, Group A had a better mean postoperative CDVA and postoperative VFQ composite scores than Group B (P < .0001, both outcomes). A higher ASA class was associated with an increased risk for 2 unanticipated events; that is, clinically significant macular edema (CSME) (Group A: 4 [0.47%] versus Group B: 50 [1.28%]; adjusted odds ratio [OR], 3.02; 95% confidence interval [CI], 1.02-13.05; P = 0.04) and readmission to the hospital within 30 days (2 [0.23%] versus 56 [1.41%]; OR, 8.26; 95% CI, 1.71-148.62; P = .004) CONCLUSIONS: Among United States veterans, the ASA classification could be an important predictor of VRQL and visual outcomes. In this cohort, it was associated with an increased risk for 2 serious unanticipated events-CSME and readmission to the hospital-both costly, unwanted outcomes. FINANCIAL DISCLOSURE: Dr. Vollman is a consultant to Forsight Vision5. None of the authors has a financial or proprietary interest in any material or method mentioned.

Longitudinal trends in use of targeted therapies for treatment of malignant neoplasms of the eye: a population-based study in Taiwan.

OBJECTIVES: This study examined the recent trend in use and costs of antineoplastic agents for treatment of eye malignancies in Taiwan from 2009 to 2012. We also forecasted use and costs of targeted therapies up to and including year 2016 based on the current patterns. DESIGN: Retrospective observational study focusing on the usage of targeted therapies for treatment of eye malignancy. SETTING: The monthly claims data for eye malignancy-related antineoplastic agents were retrieved from Taiwan's National Health Insurance Research Database (2009-2012). MAIN OUTCOME MEASURES: We calculated the number of prescriptions and costs for each class of medications, and analysed their time trends. In addition, using a time series design with ARIMA models, we estimated the market share by prescription volume and the proportion of costs for targeted therapies for year 2016. RESULTS: The market share by prescription volume of targeted therapies grew from 1.56% in 2009 to 9.98% in 2012 among all antineoplastic agents, and the proportion of costs for targeted therapies rose from 15.12% in 2009 to 58.88% in 2012. Especially, the proportion of costs for protein kinase inhibitors grew from 25.62% to 45.28% among all antineoplastic agents between 2010 and 2012. The market share by prescription volume and the proportion of costs for targeted therapies for treatment of eye malignancies were predicted to reach 27.33% and 91.39% by the fourth quarter in 2016, respectively. CONCLUSIONS: This is the first study that examined and forecasted use and costs of targeted therapies for treatment of eye malignancies in Taiwan. Our findings indicate that, compared with other classes of drugs, targeted therapies are having a more and more relevant share among all treatment strategies for eye malignancies in Taiwan, and due to their high costs they are likely to cause great economic burden.

Outcomes of cataract surgery with residents as primary surgeons in the Veterans Affairs Healthcare System.

PURPOSE: To explore visual outcomes, functional visual improvement, and events in resident-operated cataract surgery cases. SETTING: Veterans Affairs Ophthalmic Surgery Outcomes Database Project across 5 Veterans Affairs Medical Centers. DESIGN: Retrospective data analysis of deidentified data. METHODS: Cataract surgery cases with residents as primary surgeons were analyzed for logMAR corrected distance visual acuity (CDVA) and vision-related quality of life (VRQL) measured by the modified National Eye Institute Vision Function Questionnaire and 30 intraoperative and postoperative events. In some analyses, cases without events (Group A) were compared with cases with events (Group B). RESULTS: The study included 4221 cataract surgery cases. Preoperative to postoperative CDVA improved significantly in both groups (P < .0001), although the level of improvement was less in Group B (P = .03). A CDVA of 20/40 or better was achieved in 96.64% in Group A and 88.25% in Group B (P < .0001); however, Group B had a higher prevalence of preoperative ocular comorbidities (P < .0001). Cases with 1 or more events were associated with a higher likelihood of a postoperative CDVA worse than 20/40 (odds ratio, 3.82; 95% confidence interval, 2.92-5.05; P < .0001) than those who did not experience an event. Both groups had a significant increase in VRQL from preoperative levels (both P < .0001); however, the level of preoperative to postoperative VRQL improvement was significantly less in Group B (P < .0001). CONCLUSION: Resident-operated cases with and without events had an overall significant improvement in visual acuity and visual function compared with preoperatively, although this improvement was less marked in those that had an event. FINANCIAL DISCLOSURE: None of the authors has a financial or proprietary interest in any material or method mentioned.

Ophthalmic Surgical Simulation in Training Dexterity in Dominant and Nondominant Hands: Results From a Pilot Study.

PURPOSE: To determine whether a structured training program using the validated EYESI surgical simulator improves dexterity in nondominant (ND) hands. SETTING: Academic tertiary referral center. DESIGN: Nonrandomized, prospective study. METHODS: Subjects who chose to participate and provided informed consent completed a structured simulation training program, which included a baseline test, 3 sessions of repeated tasks, and a final test on capsulorhexis in dominant (D) and ND hands. Participants completed demographic and satisfaction questionnaires. Performances at each session were recorded. We compared overall scores at baseline and at the end of the study, and analyzed trends over time. Statistical analysis was performed using JMP by SAS. RESULTS: Overall, 14 subjects completed the training program. In all, 3 (21.4%) were attending physicians and 11 (78.6%) were trainees. There was a significant improvement in the average overall scores (baseline vs. final) in both the D hand (33.4 vs. 46.5; p < 0.05) and the ND hand (28.9 vs. 47.7; p < 0.001). The structured training program demonstrated significantly faster performance times in both hands at the end of the study (D p< 0.001, ND p < 0.02). However, the learning curve was significantly steeper in the ND hand (p < 0.01). Participants agreed that simulation training improved the ND hand dexterity. CONCLUSIONS: We found a significantly greater trend for improvement in the ND compared with the D hand. These results suggest that an elaborate, structured curriculum targeting teaching dexterity results in better simulated performance.

Cataract Surgery Outcomes in Glaucomatous Eyes: Results From the Veterans Affairs Ophthalmic Surgery Outcomes Data Project.

PURPOSE: To compare visual acuity outcomes, vision-related quality of life, and complications related to cataract surgery in eyes with and without glaucoma. DESIGN: Retrospective cohort study. METHODS: Cataract surgery outcomes in cases with and without glaucoma from the Veterans Affairs Ophthalmic Surgical Outcomes Data Project were compared. RESULTS: We identified 608 glaucoma cases and 4306 controls undergoing planned cataract surgery alone. After adjusting for age, pseudoexfoliation, small pupil, prior ocular surgery, and anterior chamber depth, we found that glaucoma cases were more likely to have posterior capsular tear with vitrectomy (odds ratio [OR] 1.8, P = .03) and sulcus intraocular lens placement (OR 1.65, P = .03) during cataract surgery. Glaucoma cases were more likely to have postoperative inflammation (OR 1.73, P < .0001), prolonged elevated intraocular pressure (OR 2.96, P = .0003), and additional surgery within 30 days (OR 1.92, P = .03). Mean best-corrected visual acuity (BCVA) and Visual Function Questionnaire (VFQ) scores significantly improved after cataract surgery in both groups (P < .0001), but there were larger improvements in BCVA (P = .01) and VFQ composite scores (P < .0001) in the nonglaucoma vs the glaucoma group. A total of 3621 nonglaucoma cases (94.1%) had postoperative BCVA 20/40 or better, compared to 466 glaucoma cases (89.6%) (P = .0003). CONCLUSIONS: Eyes with glaucoma are at increased risk for complications and have more modest visual outcomes after cataract surgery compared to eyes without glaucoma. Despite this, glaucoma patients still experience significant improvement in vision-related outcomes after cataract extraction. Further study is needed to explore potential factors that influence cataract surgery outcomes in glaucomatous eyes.

Acute pancreatitis in intraductal papillary mucinous neoplasms: A common predictor of malignant intestinal subtype.

BACKGROUND: Acute pancreatitis (AP) is a common presenting symptom of intraductal papillary mucinous neoplasms (IPMN). Our objective was to evaluate the clinical and pathologic features of IPMN that present with AP. We hypothesized that the intestinal epithelial subtype, which produces a highly viscous mucin containing MUC2 glycoprotein, is associated with the development of AP. METHODS: We performed a case-control study, evaluating 325 patients with IPMN resected at our institution. Clinicopathologic features were assessed for patients with and without a history of AP and compared between the 2 groups. RESULTS: A history of AP was found in 69 patients (21%). Of those, 33 (48%) experienced a single episode, and some patients had as many as 10 distinct attacks. Three patients presented with necrotizing pancreatitis requiring operative debridement. After resection, recurrent AP occurred in only 14%. A history of AP was associated with younger age (61 vs 70, P < .001), main duct involvement (odds ratio [OR] 1.73, 95% confidence interval [95% CI] 1.00-3.01; P = .049), intestinal subtype (OR 4.84, 95% CI 2.71-8.67; P < .001), and high-grade dysplasia (OR 1.82, 95% CI 1.07-3.11; P = .028). On multivariate analysis, AP was an independent predictor of intestinal subtype (OR 4.69, 95% CI 2.48-8.84, P < .001), malignancy (OR 1.97, 95% CI 1.07-3.63, P = .029), and main duct involvement (OR 1.87, 95% CI 1.02-3.43, P = .044). CONCLUSION: AP is a frequent presenting symptom of IPMN. These patients are younger and have greater odds of harboring malignant intestinal-type IPMN involving the main pancreatic duct.

Management of mydriasis and pain in cataract and intraocular lens surgery: review of current medications and future directions.

The maintenance of mydriasis and the control of postoperative pain and inflammation are critical to the safety and success of cataract and intraocular lens replacement surgery. Appropriate mydriasis is usually achieved by topical and/or intracameral administration of anticholinergic agents, sympathomimetic agents, or both, with the most commonly used being cyclopentolate, tropicamide, and phenylephrine. Ocular inflammation is common after cataract surgery. Topical steroids and nonsteroidal anti-inflammatory drugs are widely used because they have been proved effective to control postsurgical inflammation and decrease pain. Topical nonsteroidal anti-inflammatory drugs have also been shown to help maintain dilation. However, use of multiple preoperative drops for pupil dilation, inflammation, and pain control have been shown to be time consuming, resulting in delays to the operating room, and they cause dissatisfaction among perioperative personnel; their use can also be associated with systemic side effects. Therefore, ophthalmologists have been in search of new options to streamline this process. This article will review the current medications commonly used for intraoperative mydriasis, as well as pain and inflammation control. In addition, a new combination of ketorolac, an anti-inflammatory agent, and phenylephrine, a mydriatic agent has recently been designed to maintain intraoperative mydriasis and to reduce postoperative pain and irritation from intraocular lens replacement surgery. Two Phase III clinical trials evaluating this combination have demonstrated statistically significant differences when compared to placebo in maintaining intraoperative mydriasis (P<0.00001) and in reducing pain in the early postoperative period (P=0.0002). This medication may be of benefit for use in cataract and lens replacement surgery in the near future.

Intraoperative floppy iris and prevalence of intraoperative complications: results from ophthalmic surgery outcomes database.

PURPOSE: To estimate the prevalence of untoward events during cataract surgery with the use of pupillary expansion devices and intraoperative floppy iris (IFIS). DESIGN: Retrospective analysis of 4923 cataract surgery cases from the Veterans Affairs Ophthalmic Surgical Outcomes Data Project. METHODS: Outcomes from 5 Veterans Affairs medical centers were analyzed, including use of alpha-blockers (both selective and nonselective), IFIS, intraoperative iris trauma, intraoperative iris prolapse, posterior capsular tear, anterior capsule tear, intraoperative vitreous prolapse, and use of pupillary expansion devices. P values were calculated using the χ(2) test. RESULTS: A total of 1254 patients (25.5%) took alpha-blockers preoperatively (selective, 587; nonselective, 627; both, 40). Of these 1254 patients, 428 patients (34.1%) had documented IFIS. However, 75.2% of patients with IFIS (428/569) had taken alpha-blockers preoperatively (P < .00001). A total of 430 patients (8.7%) had a pupillary expansion device used during their cataract surgery, of which 186 patients (43.4%) had IFIS (P < .0001). Eighty-six patients with IFIS had at least 1 intraoperative complication and 39 patients with IFIS had more than 1 intraoperative complication (P < .001). CONCLUSIONS: The use of either selective or nonselective alpha-antagonists preoperatively demonstrated a significant risk of IFIS. Nonselective alpha-antagonists caused IFIS at a higher prevalence than previously reported. This study did demonstrate statistically significant increased odds of surgical complications in patients with IFIS vs those without IFIS in all groups (those taking selective and nonselective alpha-antagonists and also those not taking medications).

Scleral necrosis in a patient with congenital erythropoietic porphyria.

PURPOSE: To report on a patient with congenital erythropoietic porphyria who presented with scleral necrosis. METHOD: Case report. RESULTS: A 34-year-old man with a long history of congenital erythropoietic porphyria was referred to us for evaluation of necrotizing scleritis of the right eye. The patient presented with a 3-month duration of eye pain and redness, which initially responded to oral and topical corticosteroids. However, upon corticosteroid taper, the symptoms quickly recurred. The patient was initially tried on oral azathioprine therapy, which failed to induce resolution of the symptoms. Full serological investigation did not suggest that the necrotic process was secondary to an immunologically driven process, and we proceeded with scleral biopsy and patch graft. The biopsy disclosed atrophic changes of the sclera with calcium-like plaques, without evidence of inflammation. Aggressive strategy of sun avoidance was implemented and immunosuppressive therapy discontinued. One year after the surgery, the patient remains asymptomatic without recurrence of ocular disease. CONCLUSIONS: This case highlights the usefulness of tissue biopsy in porphyria when one is uncertain as to whether a necrotizing process is driven by inflammation or phototoxic damage. When the underlying mechanism is clearly identified, the appropriate therapy can then be instituted to prevent further damage.