Diagnostic implications of Gardner Syndrome, case report of a familial adenomatous polyposis (FAP) variant, for eye care professionals.

INTRODUCTION: Gardner Syndrome (GS) is a variant of Familial Adenomatous Polyposis (FAP). FAP is characterized by several precancerous adenomatous intestinal polyps while GS has additional distinct extraintestinal features such as congenital hypertrophy of retinal epithelium (CHRPE), which we describe here. PRESENTATION OF CASE: 42-year-old male with GS presenting with flashes and floaters observed to have CHRPE-like lesions characteristic of GS. DISCUSSION: Subtle CHRPE findings differentiate pathological, described in the present case, from non-pathological etiologies and may guide further management. CONCLUSION: Here we present the signs and symptoms that raise suspicion for GS associated with CHRPE and how to approach management late in the disease presentation.

Relationship Between Macular Thickness and Visual Acuity in the Treatment of Diabetic Macular Edema With Anti-VEGF Therapy: Systematic Review.

Purpose: To examine the relationship between central macular thickness (CMT) measured by optical coherence tomography (OCT) and visual acuity (VA) in patients with center-involving diabetic macular edema (DME) receiving antivascular endothelial growth factor (anti-VEGF) treatment. Methods: Peer-reviewed articles from 2016 to 2020 reporting intravitreal injections of bevacizumab, ranibizumab, or aflibercept that provided data on pretreatment (baseline) and final retinal thickness (CMT) and visual acuity (VA) were identified. The relationship between relative changes was assessed via a linear random-effects regression model controlling for treatment group. Results: No significant association between the logarithm of the minimum angle of resolution (logMAR) VA and CMT was found in 41 eligible studies evaluating 2667 eyes. The observed effect estimate was a 0.12 increase (95% CI, -0.124 to 2.47) in logMAR VA per 100 µm reduction in CMT after treatment change. There were no significant differences in logMAR VA between the anti-VEGF treatment groups. Conclusions: There was no statistically significant relationship between the change in logMAR VA and change in CMT as well as no significant effect of the type of anti-VEGF treatment on the change in logMAR VA. Although OCT analysis, including measurements of CMT, will continue to be an integral part of the management of DME, further exploration is needed on additional anatomic factors that might contribute to visual outcomes.

Idiopathic retinal arterial occlusive vasculitis in the setting of multiple arterial occlusions.

PURPOSE: We present a patient with vaso-occlusive retinal vasculitis to summarize this uncommon entity and review the clinical features and management challenges applicable to patients with retinal vasculitis. OBSERVATIONS: A 76-year-old male presented with sudden-onset severe central vision loss. On examination, vitreous hemorrhage, neovascularization of the optic nerve, peripheral segmental periphlebitis, vessel sclerosis, vascular sheathing, and retinal hemorrhages were observed, and a diagnosis of active vaso-occlusive retinal vasculitis was made. The patient then underwent a complete infectious, inflammatory, and neoplastic workup which returned negative. The patient was treated with locally with a sub-Tenon's injection of 40 mg triamcinolone on presentation and later with oral prednisone. At three-month follow-up, vision improved to 20/300 with regressing neovascularization and clearing of vitreous hemorrhage in the right eye (OD). CONCLUSIONS: Considering novel associations of occlusive retinal vasculitis, it is important to recognize that idiopathic occlusive retinal vasculitis, although uncommon, can occur and represents a prototypical disease form. It is imperative that these patients have a complete infectious, inflammatory, and neoplastic workup owing to the possible overlap of masquerade clinical signs and symptoms.

Epiretinal membrane and cystoid macular edema as predictive factors of recurrent proliferative vitreoretinopathy.

PURPOSE: Proliferative vitreoretinopathy (PVR) is the most common cause of recurrent retinal detachment (RD). We sought to determine the predictive factors of recurrent PVR formation and the need for additional vitreoretinal surgical intervention after uncomplicated primary RD repair. METHODS: This is a retrospective single-center case-control study of consecutive patients with PVR formation after uncomplicated RD repair. Logistic regression was used to assess factors associated with recurrent PVR formation. RESULTS: Thirty-seven eyes (37 patients) who had recurrent RD secondary to PVR formation were included. Among those, 27 eyes needed one additional surgery, whereas the remainder 10 eyes required two or more additional surgeries. In the univariate analysis, patients who had cystoid macular edema (CME) after the second surgery were 8.33 times (crude odds ratio [COR], 95% confidence interval [CI]: 1.23-56.67, p=0.0302) more likely to have recurrent PVR formation compared to those who did not have CME after the second surgery. Similarly, those who had epiretinal membrane (ERM) after the second surgery were 8.00 times (COR, 95% CI: 1.43-44.92, p=0.0182) more likely to have recurrent PVR formation compared to those who did not have ERM after the second surgery. In the multivariate analysis, patients who had ERM after the second surgery were 8.20 times (adjusted odds ratio [AOR], 95% CI: 1.08-62.40, p=0.0422) more likely to develop recurrent PVR compared to those who did not have ERM after the second surgery, when adjusted for age, sex, and CME after the second surgery. CONCLUSION: ERM and CME are potential predictive factors for recurrent PVR formation after uncomplicated primary RD repair. Early recognition and treatment of ERM and CME may be critical to prevent subsequent PVR formation and improve visual outcomes.

Comparison of microbiology and visual outcomes of patients undergoing small-gauge and 20-gauge vitrectomy for endophthalmitis.

BACKGROUND: The role of pars plana vitrectomy (PPV) for endophthalmitis has evolved over recent decades but the literature is lacking on comparisons between small-gauge and 20-gauge vitrectomy. OBJECTIVE: To evaluate evolving etiological and microbiological trends in patients undergoing vitrectomy for endophthalmitis and to compare culture-positive rates and visual outcomes between small-gauge (23- and 25-gauge) and 20-gauge instrumentation during vitrectomy for endophthalmitis. METHODS: Ten-year retrospective comparative case series and prospective laboratory in vitro testing. Tertiary care academic referral center. Patients who underwent PPV for endophthalmitis between 2003 and 2013. Vitreous biopsies were obtained in all cases. The effect of vitrectomy gauge (20-, 23-, and 25-gauge) and vitreous cutting rate (1,500 and 5,000 cuts per minute) on the viability of bacterial culture was evaluated in an in vitro prospective laboratory investigation. MAIN OUTCOME MEASURES: Comparison of etiology, microbiology culture-positive rates, and visual outcomes between small-gauge and 20-gauge instrumentation in patients undergoing PPV for infectious endophthalmitis. RESULTS: A total of 61 cases of vitrectomy for endophthalmitis were identified over a 10-year period; of these, 34 were treated with small-gauge (23- and 25-gauge) vitrectomy and 27 were treated with 20-gauge vitrectomy. In the small-gauge group, 12 cases (35.3%) yielded culture-positive results versus 20 cases (74.1%) with culture positivity in the 20-gauge cohort (P=0.002). The most common cause of endophthalmitis was cataract surgery and the most frequently identified organism was coagulase-negative Staphylococci in both groups. There was no significant difference in mean postoperative visual acuities between groups (P=0.33). Etiological trends indicate an increase in endophthalmitis due to intravitreal injection in the small-gauge group (n=9) compared to the 20-gauge group (n=3) (P=0.001). In vitro laboratory testing revealed no significant difference in rates of culture growth for different vitrectomy gauge sizes or vitreous cutting speeds. CONCLUSION AND RELEVANCE: Small-gauge vitrectomy for endophthalmitis yields final visual outcomes comparable to 20-gauge instrumentation. A significant difference in culture-positive rates was observed between small-gauge and 20-gauge instrumentation for vitrectomy in endophthalmitis; however, laboratory testing indicates this is not related to either vitreous gauge size or cutter speed. Intravitreal injections are emerging as a common etiology of vitrectomy for endophthalmitis.

Nosocomial keratitis caused by methicillin-resistant Staphylococcus aureus: case report and preventative measures.

A 47-year-old African-American woman was admitted to the intensive care unit of our community hospital for respiratory failure secondary to severe decompensated heart failure, requiring intubation. In the ensuing days, she developed a methicillin-resistant Staphylococcus aureus (MRSA) infection of the cornea, despite no growth of MRSA in multiple blood, sputum, and urine cultures. This unexpected corneal infection complicated her hospital stay, and increased morbidity and disease-related cost. Risk factors, warning signs, and preventative measures for MRSA keratitis secondary to lagophthalmos (inability to completely close one's eyelids) are outlined in this case report. Implementing simple precautions such as taping eyelids shut or using artificial lubrication may reduce patient morbidity and disease-related costs. These recommendations are directed to non-ophthalmic clinicians who provide care to patients in settings where MRSA colonization is widespread.

Oral mineralocorticoid antagonists for recalcitrant central serous chorioretinopathy.

PURPOSE: To evaluate the effect and tolerance of oral mineralocorticoid antagonists, eplerenone and/or spironolactone, in recalcitrant central serous chorioretinopathy. METHODS: Retrospective consecutive observational case series. Primary outcome measures included central macular thickness (CMT, µm), macular volume (MV, mm(3)), Snellen visual acuity, and prior treatment failures. Secondary outcomes included duration of treatment, treatment dosage, and systemic side effects. RESULTS: A total of 120 patients with central serous chorioretinopathy were reviewed, of which 29 patients were treated with one or more mineralocorticoid antagonists. The average age of patients was 58.4 years. Sixteen patients (69.6%) were recalcitrant to other interventions prior to treatment with oral mineralocorticoid antagonists, with an average washout period of 15.3 months. The average duration of mineralocorticoid antagonist treatment was 3.9±2.3 months. Twelve patients (52.2%) showed decreased CMT and MV, six patients (26.1%) had increase in both, and five patients (21.7%) had negligible changes. The mean decrease in CMT of all patients was 42.4 µm (range, -136 to 255 µm): 100.7 µm among treatment-naïve patients, and 16.9 µm among recalcitrant patients. The mean decrease in MV of all patients was 0.20 mm(3) (range, -2.33 to 2.90 mm(3)): 0.6 mm(3) among treatment-naïve patients, and 0.0 mm(3) among recalcitrant patients. Median visual acuity at the start of therapy was 20/30 (range, 20/20-20/250), and at final follow-up it was 20/40 (range, 20/20-20/125). Nine patients (39.1%) experienced systemic side effects, of which three patients (13.0%) were unable to continue therapy. CONCLUSION: Mineralocorticoid antagonist treatment had a positive treatment effect in half of our patients. The decrease in CMT and MV was much less in the recalcitrant group compared to the treatment-naïve group. An improvement in vision was seen only in the treatment-naïve group. Systemic side effects, even at low doses, may limit its usage in some patients.

Long-term outcomes in patients undergoing vitrectomy for retinal detachment due to viral retinitis.

PURPOSE: To determine the outcomes in patients with rhegmatogenous retinal detachment (RRD) secondary to viral retinitis. PATIENTS AND METHODS: This was a retrospective, consecutive, noncomparative, interventional case series of 12 eyes in ten patients with RRD secondary to viral retinitis. Results of vitreous or aqueous biopsy, effect of antiviral therapeutics, time to retinal detachment, course of visual acuity, and anatomic and surgical outcomes were investigated. RESULTS: There were 1,259 cases of RRD during the study period, with 12 cases of RRD secondary to viral retinitis (prevalence of 0.95%). Follow-up was available for a mean period of 4.4 years. Varicella zoster virus was detected in six eyes, herpes simplex virus in two eyes, and cytomegalovirus in two eyes. Eight patients were treated with oral valacyclovir and two patients with intravenous acyclovir. Lack of optic nerve involvement correlated with improved final visual acuity of 20/100 or greater. Pars plana vitrectomy (n=12), silicone-oil tamponade (n=11), and scleral buckling (n=10) provided successful anatomic retinal reattachment in all cases, with no recurrent retinal detachment and no cases of hypotony during the follow-up period. CONCLUSION: Varicella zoster virus was the most frequent cause of viral retinitis, and lack of optic nerve involvement was predictive of a favorable visual acuity prognosis. Vitrectomy with silicone-oil tamponade and scleral buckle placement provided stable anatomical outcomes.

Scleral buckle hemorrhagic cyst masquerading as an orbital tumor.

We present a case of a 41-year-old man who was referred for evaluation of a choroidal tumor with a remote history of scleral buckle placement for traumatic retinal detachment. Ocular imaging, echography, and magnetic resonance imaging could not rule out a neoplastic process so the patient was taken for surgical exploration where a hemorrhagic cyst was discovered. This is the first case in the literature of a silicone scleral buckle-associated hemorrhagic cyst presenting as orbital mass.