Simple hamartoma of the retinal pigment epithelium with macular edema.

PURPOSE: Presumed congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare intraocular finding that is described as a focal, nodular, jet black lesion. These lesions frequently occur at or near the macula and have no known association with changes in the surrounding neurosensory retina, retinal pigment epithelium, or choroid, nor have they been related with exudation or hemorrhage. Until now, there have been no cases reported of CSHRPE with associated vascular activity. CASE REPORT: A 14-year-old Hispanic adolescent girl with a presumed CSHRPE presented with adjacent macular edema. The patient was treated with an off-label intravitreal bevacizumab injection to decrease the edema. Two months later, the patient presented with improved visual acuity and decreased macular edema. CONCLUSIONS: This is the first documented case of macular edema secondary to vascular activity being associated with these rare lesions. When CSHRPE is found to have secondary vascular activity and associated macula edema, treatment of intravitreal bevacizumab may lead to improved visual and anatomical outcomes.

Endophthalmitis caused by Achromobacter xylosoxidans after cataract surgery.

PURPOSE: To report Achromobacter xylosoxidans as a cause of both acute-onset and delayed-onset postoperative endophthalmitis after cataract surgery. METHODS: A noncomparative consecutive case series of patients with culture-proven A. xylosoxidans endophthalmitis between 1970 and 2012. Cataract surgery and intraocular lens placement were performed in all patients before endophthalmitis. Positive cultures were obtained from the vitreous, capsular bag, and/or the removed intraocular lens. RESULTS: The clinical diagnosis was confirmed in four patients with positive cultures. Two patients with endophthalmitis had a preliminary culture report of Pseudomonas species. In addition to receiving intravitreal antibiotics, all patients underwent capsulectomy and intraocular lens removal at the time of pars plana vitrectomy. Visual acuity at last follow-up was 20/40 or better in 2 (50%) of the 4 patients, but the remaining 2 patients were 20/200 or worse. CONCLUSION: A. xylosoxidans may be a cause of acute, recurrent, and delayed-onset postoperative endophthalmitis after cataract surgery. Complete capsulectomy and intraocular lens removal can be considered in recurrent and recalcitrant patients.

Tapioca melanoma of the iris without iris heterochromia.

PURPOSE: A case of a teenage girl with tapioca melanoma of the iris is presented. This case is unusual, as the patient did not have heterochromia and did not present with elevated intraocular pressure. CASE REPORT: A 14-year-old female patient presented with an amelanotic, multinodular, multifocal lesion of the right iris. Pathology confirmed a diagnosis of tapioca melanoma using immunohistologic staining. The patient underwent enucleation of her right eye and has been free of metastatic disease 3 years later. CONCLUSIONS: Tapioca melanoma of the iris must be included among the other differential diagnoses when examining patients with amelanotic iris lesions, even when iris heterochromia is not clearly evident.

New Approaches to Overcoming Antimicrobial Resistance in Endophthalmitis.

Endophthalmitis is a rare but vision-threatening infection characterized by marked inflammation of intraocular fluids and tissues, uncommonly seen following surgery and intravitreal injection. Antimicrobials are used worldwide in the prophylaxis and treatment of bacterial and fungal infections of the eye and are standard treatment in the preoperative and postoperative care of surgical patients. However, antimicrobials are reported to be overprescribed in many parts of the world, which contributes to antimicrobial resistance (AMR). AMR complicates the prophylaxis and treatment of endophthalmitis. This article examines the prevalence and mechanisms of AMR in ocular microorganisms, emphasizing the importance of understanding AMR patterns for tailored treatments. It also explores prophylaxis and management strategies for endophthalmitis, with a discussion on the use of intracameral antibiotic administration. The use of prophylactic intracameral antibiotics during cataract surgery is common in many parts of the world but is still controversial in some locations, especially in the US. Finally, it highlights the role of stewardship in ophthalmology and its benefits in the treatment of endophthalmitis.

Retinoblastoma.

PURPOSE OF REVIEW: Retinoblastoma is the most common malignant intraocular tumor of childhood. Treatment and diagnostic modalities associated to this condition are changing rapidly as our understanding of this condition crystallizes. The purpose of this review is to provide an update of the current understanding of retinoblastoma. RECENT FINDINGS: Knowledge on tumorigenesis and genomic expression has expanded tremendously with the development of a mouse model for retinoblastoma. Tumor hypoxia has been identified as a significant step in the tumor progression and a novel target for future treatments. Current globe-sparing therapies, including periocular carboplatin, selective ophthalmic artery chemoreduction, intravitreal melphalan, and focal consolidation are being used and investigated actively. Diagnosis and the management of retinoblastoma is also undergoing major advances including wide-field photography, autofluorescence, and high-resolution optical coherence tomography. SUMMARY: Progressive advances in the understanding of retinoblastoma pathogenesis continue to lead treatment strategies. Improvements in the diagnosis and management of retinoblastoma are improving morbidity and mortality associated to this condition in the developed nations. However, it is of outmost importance to flatten the international boundaries to offer prompt care to retinoblastoma children in underdeveloped communities.

Bilateral vitreomacular traction syndrome associated with topical pilocarpine 1% ophthalmic solution.

To describe a case of transient bilateral vitreomacular traction syndrome associated with topical 1% pilocarpine ophthalmic solution in both eyes as a treatment for advanced glaucoma. Observations: Spectral-domain OCT demonstrated bilateral vitreomacular traction syndrome after initiation of topical 1% pilocarpine solution in both eyes for advanced glaucoma. Follow-up imaging revealed the resolution of vitreomacular traction after cessation of the drug without a complete posterior vitreous detachment. Conclusions and Importance: In the advent of new pilocarpine formulations, this case raises the concern of vitreomacular traction syndrome as a serious potential sequela of long-term topical pilocarpine use.

Adjuvant Intravitreal Bevacizumab for Retinal Neovascularization in Eales' Disease Associated With Latent Mycobacterium Tuberculosis.

We report a case of bilateral Eales' disease managed with intravitreal bevacizumab. A 32-year-old woman with a history of bacillus Calmette-Guerin vaccine, administered when she was 10 years old, presented with a five-day history of a scotoma in the temporal field of her right eye. A dilated fundus exam and fluorescein angiography showed bilateral retinal peripheral capillary non-perfusion, retinal neovascularization in the right eye, and deep intraretinal hemorrhages in the left eye. Her laboratory workup resulted in a positive QuantiFERON-TB Gold test (Cellestis Ltd, Carnegie, Victoria, Australia). Chest computed tomography showed a calcified granuloma in her right lung. Angiographic-guided pan-retinal photocoagulation was performed, and intravitreal injections of bevacizumab (1.25 mg/0.05 mL) were administered in both eyes over the course of three months. The intraretinal hemorrhages resolved after three months of therapy. Three months following treatment, the patient showed normal fundus findings without any evidence of recurrence and a visual acuity of 20/20 in both eyes. Intravitreal bevacizumab in combination with angiography-guided pan-retinal photocoagulation may be efficacious in select patients with Eales' disease.

Update on current pharmacologic therapies for diabetic retinopathy.

INTRODUCTION: Diabetic retinopathy is a major cause of visual loss worldwide. The most important clinical findings include diabetic macular edema (DME) and proliferative diabetic retinopathy (PDR). AREAS COVERED: PubMed was used for our literature review. Articles from 1995 to 2023 were included. Pharmacologic treatment of diabetic retinopathy generally involves the use of intravitreal anti-vascular endothelial growth factor (VEGF) therapy for DME and PDR. Corticosteroids remain important second-line therapies for patients with DME. Most emerging therapies focus on newly identified inflammatory mediators and biochemical signaling pathways involved in disease pathogenesis. EXPERT OPINION: Emerging anti-VEGF modalities, integrin antagonists, and anti-inflammatory agents have the potential to improve outcomes with reduced treatment burdens.

Clinical Outcomes of Combined Phacoemulsification With Intraocular Lens Placement and Microincision Vitrectomy in Adult Vitreoretinal Disease.

Purpose: To evaluate the safety and clinical outcomes of combined phacoemulsification with intraocular lens (IOL) placement and microincision vitrectomy surgery (MIVS) in adult patients with concomitant cataract and vitreoretinal disease. Methods: A consecutive series of patients with comorbid vitreoretinal disease and cataract who had combined phacoemulsification with IOL placement and MIVS was retrospectively analyzed. The main outcome measures were visual acuity (VA) and intraoperative and postoperative complications. Results: The analysis comprised 648 eyes of 611 patients. The median follow-up was 26.9 months (range, 12-60 months). The most common vitreoretinal pathology was intraocular tumor (53%). The best-corrected Snellen VA improved from 20/192 at baseline to 20/46 at the 12-month follow-up. The most frequent intraoperative complication was capsule tear (3.9%). The most common postoperative adverse events after 3 months of follow-up (mean, 24 months) were vitreous hemorrhage (3.2%) and retinal detachment (1.8%). No patient developed endophthalmitis. Conclusions: Combined phacoemulsification with IOL placement and MIVS is a safe, effective technique to manage a broad range of vitreoretinal diseases in patients with significant cataract.

Visual loss associated with influenza A: a case report and review of literature.

A 24-year-old female presenting with influenza A pneumonia and simultaneous visual loss was hospitalized. A complete ophthalmological examination was performed three weeks later. Best corrected visual acuity was 20/70 (right eye) and 20/30 (left eye). A dilated fundus exam revealed bilateral vitreous cells and marked bilateral optic nerve swelling with associated peripapillary hemorrhages. A submacular hemorrhage was seen in the right eye. Spinal tap opening pressure was 490 mmHg, with normal cerebrospinal fluid cell counts. Eight months after the initial clinical presentation, the patient was asymptomatic, with normal posterior poles and a best corrected visual acuity of 20/30 in the right eye and 20/25 in the left eye. Although papilledema can produce peripheral retinal hemorrhages secondary to extensive retinal venous congestion, the presence of bilateral vitritis and elevated influenza serum titers suggested that the patient might be suffering from influenza retinopathy. Vitreous polymerase chain reaction could potentially aid in the diagnosis of influenza retinopathy.

Intravitreal bevacizumab for peripapillary subretinal neovascular membrane associated to papilledema: a case report.

The purpose of this report is to document the use of bevacizumab as treatment of peripapillary subretinal neovascular membrane associated to idiopathic intracranial hypertension. This case reports a 31-year-old obese female with chronic papilledema due to idiopathic intracranial hypertension that developed an acute submacular hemorrhage due to a choroidal neovascular membrane in her left eye. Two separate intravitreal injections of bevacizumab (1.25 mg/0.5 ml) were administered with six weeks apart. Fourteen weeks after the initial injection her visual acuity improved to 20/40 in her left eye with associated angiographic resolution of the hemorrhage. Bevacizumab appears to be an effective option for patients who have subretinal neovascular membranes due to papilledema. Although choroidal neovascularization may have different pathogenic mechanisms, our case report demonstrates encouraging results without adverse ocular or systemic side effects.

Clinical spectrum of blunted foveal contour.

Foveal hypoplasia is the absence of a foveal depression and the presence of the ganglion cell layer in the foveola. A spectrum of clinical characteristics, including normal or variably decreased visual acuity, has been described in patients with blunted foveal contours. Multiple systemic and ophthalmologic conditions including albinism, aniridia, nanophthalmos, prematurity, and fovea plana have been associated with this anomaly. This article illustrates select clinical conditions characterized by a blunted foveal contour. Given the heterogeneity of findings, a thorough medical history and detailed physical and ocular examinations are usually sufficient for the clinician to make the correct diagnosis.

Optical Coherence Tomography Angiography Findings in Sclerochoroidal Calcification.

Optical coherence tomography angiography (OCT-A) is a modern non invasive imaging technique that may aid in the evaluation of retinal diseases through the assessment of chorioretinal vasculature. The literature regarding OCT-A in sclerochoroidal calcification is scarce. We present the case of a 57-year-old female who was referred to the clinic due to an atypical choroidal lesion in the right eye. OCT-A showed an apparent decrease in the vascular flow of the choroidal/choriocapillaris layers and an apparent increase in the vascular flow of the deep retinal layers. A mass effect may be seen in OCT-A en face imaging that may create artifactual vascular flow pattern. This case reports the first OCT-A findings of sclerochoroidal calcification.

Atypical Event Profiling of Intra-Arterial Chemotherapy.

Purpose: Three cases of atypical events following intra-arterial chemotherapy for the treatment of retinoblastoma are presented. Methods: Case report. Results: One patient had acute orbital swelling with proptosis, another with extravasation of the chemotherapeutic agent, and the final with total ipsilateral hearing loss. Conclusions: These cases underscore the importance of maintaining close follow-up when using intra-arterial chemotherapy for treatment of retinoblastoma.

Multimodal treatment of Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome.

PURPOSE: To report a Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome. OBSERVATIONS: A 64 year-old woman with prior diagnosis of retinal dystrophy presented with decreased vision in the right eye (OD). Ophthalmologic examination was remarkable for bilateral arteriolar attenuation, mid-peripheral bony-spicules, and waxy disc pallor. Coats-like exudative vitreoretinopathy and cystoid macular edema were present OD. Genetic testing showed a homozygous pathogenic mutation in gene NR2E3, variant c.932G>A (p.Arg311Gln), consistent with Goldmann-Favre syndrome. Targeted laser ablation and combination intravitreal therapy were effective in decreasing macular edema. CONCLUSIONS AND IMPORTANCE: A Coats-like exudative vitreoretinopathy may occur in the setting of Goldmann-Favre syndrome. Targeted laser ablation in combination with intravitreal therapy can be efficacious in select patients.

Acute macular neuroretinopathy in dengue virus serotype 1.

PURPOSE: To report a case of acute macular neuroretinopathy (AMN) associated with dengue virus serotype 1 infection. OBSERVATION: An 18-year-old Puerto Rican female was evaluated due to painless paracentral scotomas in each eye that developed after being hospitalized for dengue fever a week before. Clinical examination and multimodal imaging revealed bilateral hypopigmented macular lesions, hyperreflectivity at the outer nuclear and photoreceptor layer, and reduced flow signal in the deep capillary plexus. Additionally, hypoautofluorescent parafoveal lesions were found in the left eye. AMN was diagnosed. Two-month follow-up after the initial evaluation showed resolution of symptoms but persistence of some findings on optical coherence tomography. CONCLUSIONS AND IMPORTANCE: Patients with dengue virus serotype 1 may develop paracentral scotomas with classic AMN findings and obtain complete symptomatic recovery without treatment.

Risk Factors Leading to Enucleation or Evisceration in Infectious Endophthalmitis.

Endophthalmitis treatment consists of intravitreal antibiotics injections and, in selected circumstances, pars plana vitrectomy. However, severe or refractory cases may require an enucleation or evisceration (ENEV). Our study seeks to identify risk factors leading to enucleation or evisceration in patients with infectious endophthalmitis. A retrospective chart review of subjects with a clinical diagnosis of infectious endophthalmitis was undertaken. The affected eyes were stratified into groups: those that underwent ENEV and those in which the eyeball was preserved (EP). The groups were compared using statistical analyses. In total, 69 eyes diagnosed with infectious endophthalmitis were included in the study. There was a higher frequency of exogenous infectious endophthalmitis in the ENEV group versus the EP group. Postsurgical infectious endophthalmitis was lower in the ENEV than in the EP group. A visual acuity of no light perception was more common in the ENEV compared to the EP group. Panophthalmitis was more frequent in the ENEV versus the EP group. Our findings suggest that eyes with endophthalmitis presenting with a visual acuity of no light perception, panophthalmitis, or exogenous etiology have a higher risk of requiring ENEV. In addition, eyes with a postsurgical etiology may be at a lower risk of requiring ENEV.

Characteristics Upon Presentation of Patients with Type 2 Macular Telangiectasia in Puerto Rico.

OBJECTIVE: To describe the systemic disease associations and clinical features upon initial presentation of a cohort of patients with type 2 macular telangiectasia who live in Puerto Rico. METHODS: A retrospective review of patients with macular telangiectasia was performed in 4 private retina practices in Puerto Rico. The demographic and clinical characteristics were recorded. RESULTS: Twenty-one patients who were diagnosed with macular telangiectasia were included in the analysis. The median age of presentation was 62 years; 86% were female, and all patients were Hispanics. The median visual acuity at presentation was 20/50. A prior medical diagnosis of type II diabetes mellitus was found in 15 (71.4%) patients, essential hypertension in 12 (57.1%), and dyslipidemia in 9 (42.9%). All patients had bilateral disease. The most common ocular findings were the presence of right-angle vessels in 32 (76.2%) eyes and angiographic hyperfluorescence temporal to the fovea, found in 22 (52.4%) of the affected eyes. One eye had evidence of choroidal neovascularization. CONCLUSION: Our cohort showed a higher prevalence of type 2 diabetes in patients with type 2 macular telangiectasia than in other cohorts. It also supports the findings of other studies showing that macular telangiectasia patients are more likely to have type 2 diabetes and hyperlipidemia. However, the increased prevalence of diabetes and hyperlipidemia may be due to selection bias, and further studies are needed to assess the significance of these findings.

Vitreous cultures and antibiotic analysis in Puerto Rican endophthalmitis patients.

OBJECTIVE: To analyze positive vitreous cultures and their respective antibiotic sensitivities in patients with endophthalmitis in Puerto Rico. METHODS: We conducted a retrospective cohort study of vitreous cultures from all of the patients with a clinical diagnosis of endophthalmitis at the Puerto Rico Medical Service Administration Center in San Juan, Puerto Rico, from August 2009 to July 2010. Positive isolates were selected for analysis. A retrospective chart review was performed to establish the mechanism involved in the development of endophthalmitis. RESULTS: Forty-three patients underwent vitreous cultures for a diagnosis of endophthalmitis, of which 16 patients had positive cultures. Seventy-eight percent of the isolates were bacterial and 22% fungal. Staphylococcus genus was identified in 38% of patients. All of the Staphylococcus epidermidis and Streptococcus pneumonia isolates were resistant to oxacillin; 66% of the Staphylococcus aureus isolates were also resistant to oxacillin. All of the Gram-positive isolates in our study were sensitive to vancomycin. All of the gram-negative isolates were sensitive to ceftazidime. Twenty-nine percent of the post-traumatic endophthalmitis cases were fungal in origin. CONCLUSION: The majority of endophthalmitis cases in our study were bacterial in origin, and the Staphylococcus genus was the most common type of organism identified. In our cohort, post-traumatic endophthalmitis was the most common mechanism leading to infection. Vancomycin in combination with ceftazidime appears to be adequate for the empiric treatment of all cases of bacterial endophthalmitis in our population. Anti-fungal agents should be considered as adjuvant empiric treatment in patients with post-traumatic endophthalmitis.