Early Detection of Optic Nerve Changes on Optical Coherence Tomography Using Deep Learning for Risk-Stratification of Papilledema and Glaucoma.

BACKGROUND: The use of artificial intelligence is becoming more prevalence in medicine with numerous successful examples in ophthalmology. However, much of the work has been focused on replicating the works of ophthalmologists. Given the analytical potentials of artificial intelligence, it is plausible that artificial intelligence can detect microfeatures not readily distinguished by humans. In this study, we tested the potential for artificial intelligence to detect early optic coherence tomography changes to predict progression toward papilledema or glaucoma when no significant changes are detected on optical coherence tomography by clinicians. METHODS: Prediagnostic optical coherence tomography of patients who developed papilledema (n = 93, eyes = 166) and glaucoma (n = 187, eyes = 327) were collected. Given discrepancy in average cup-to-disc ratios of the experimental groups, control groups for papilledema (n = 254, eyes = 379) and glaucoma (n = 441, eyes = 739) are matched by cup-to-disc ratio. Publicly available Visual Geometry Group-19 model is retrained using each experimental group and its respective control group to predict progression to papilledema or glaucoma. Images used for training include retinal nerve fiber layer thickness map, extracted vertical tomogram, ganglion cell thickness map, and ILM-RPE thickness map. RESULTS: Trained model was able to predict progression to papilledema with a precision of 0.714 and a recall of 0.769 when trained with retinal nerve fiber layer thickness map, but not other image types. However, trained model was able to predict progression to glaucoma with a precision of 0.682 and recall of 0.857 when trained with extracted vertical tomogram, but not other image types. Area under precision-recall curve of 0.826 and 0.785 were achieved for papilledema and glaucoma models, respectively. CONCLUSIONS: Computational and analytical power of computers have become an invaluable part of our lives and research endeavors. Our proof-of-concept study showed that artificial intelligence (AI) algorithms have the potential to detect early changes on optical coherence tomography for prediction of progression that is not readily observed by clinicians. Further research may help establish possible AI models that can assist with early diagnosis or risk stratification in ophthalmology.

Orbital Apex Syndrome Secondary to Invasive Aspergillus Infection: A Case Series and Literature Review.

BACKGROUND: Invasive fungal sinusitis carries high morbidity and mortality and often poses a diagnostic challenge. Orbital apex syndrome (OAS) is not an uncommon presentation in the setting of invasive fungal sinusitis. Delays in diagnosis and appropriate treatment can result in permanent visual dysfunction and, potentially, death. We present 2 cases of OAS secondary to invasive sinus aspergillosis, detailing the diagnostic process, treatment, and outcome for both patients. Subsequently, we present a review of the literature and combined analysis of our 2 patients plus 71 cases from previously published reports. METHODS: Literature review was performed to identify demographic, diagnostic, clinical, and treatment data of patients with OAS caused by Aspergillus species. RESULTS: The review resulted in 52 included articles with 71 patients, plus our 2 reported patients, leading to a total of 73 subjects included in the analysis. The average age of patients at presentation was 59.9 years. A combination of visual disturbance and pain (headache and/or periocular pain) was the most common presentation reported (46 cases; 63%). Diabetes mellitus was reported in 15 cases (21%), with more than half specifically noted to have poorly controlled diabetes. After diabetes, the second most common cause of immunocompromise was chronic steroid use (n = 13; 18%). Empiric antifungal treatment was started in 10 patients (14%), while 25 patients (34%) were first treated with systemic steroids due to a concern for an inflammatory etiology. Time to diagnosis from initial presentation was on average 7.4 weeks (range of 0.3-40 weeks). Approximately 78% of the cases (57 of 73) had biopsies with histology that confirmed Aspergillus fungal morphology, and 30/73 (41%) had diagnostic fungal cultures. The majority of the cases received monotherapy with intravenous (IV) amphotericin B (36 patients; 49%) and IV voriconazole (19 patients; 26%), with a combination of the 2 or more antifungal agents being used in 11 patients (15%). Forty patients (55%) showed signs of clinical improvement with treatment, while 33 (45%) patients did not experience any improvement or continued to deteriorate, and 23 (32%) died in the course of their reported follow-up. CONCLUSIONS: The present cases illustrate well the challenge in the diagnosis and treatment of OAS due to invasive sinus aspergillosis. Our review and analysis of 73 cases support the notion that a high index of suspicion leading to early biopsy with histology and fungal culture is paramount for diagnosis. Early empiric antifungal treatment and debridement can potentially reduce morbidity and mortality.

The Use of Systemic Steroids in the Treatment of Herpes Zoster Ophthalmicus-Related Ophthalmoplegia: Case Report and Case Meta-analysis.

PURPOSE: The role of systemic steroids in the treatment of ophthalmoplegia in the setting of herpes zoster ophthalmicus (HZO) is controversial. We conducted a case report-based meta-analysis to investigate the role of systemic steroid in the recovery of efferent dysfunctions in HZO. DESIGN: Case-report based meta-analysis. METHOD: We report a case of herpes zoster ophthalmicus-related ophthalmoplegia (HZORO) in which systemic steroid led to complete resolution of external ophthalmoplegia. We further identified subjects from published cases of HZO-related ophthalmoplegia by searching PubMed and Google Scholar, which elicited 42 articles (49 cases) after excluding those younger than 18 years or with incomplete follow-up data. With the present case, a total of 50 cases are included in the analysis. Main outcome measure is the recovery of efferent dysfunction at the last known follow-up, coded as 1 for complete recovery or 0 for noncomplete recovery. We performed multivariable linear regression and Cox proportional hazards analysis to determine the contribution of steroid duration on the status of complete recovery. RESULTS: Multivariable linear regression showed significant association between duration of steroid treatment and status of complete recovery (P < .001). Cox proportional hazards analysis showed a hazard ratio of 1.1 (P = .04), indicating that longer treatment duration increased chance of complete recovery. Age, gender, and initial steroid dose did not contribute significantly to recovery status. CONCLUSION: Our meta-analysis suggests that extended steroid taper may aid the recovery of ophthalmoplegia in the setting of HZO and should be investigated further in the future.

Superior oblique myokymia, a review.

PURPOSE OR REVIEW: Superior oblique myokymia is a rare, monocular condition that typically presents in healthy adults. In this article, we review the proposed pathophysiology, describe the typical clinical presentation, and discuss the recommended work up and various treatment options. RECENT FINDINGS: Treatment for this condition ranges from observation and reassurance to various topical and systemic medications. Both strabismus surgery and neurosurgery are reserved for patients who fail medical therapy or are intolerant to medication secondary to side effects. SUMMARY: Due to its episodic nature, SOM is often under-diagnosed. We highlight key features in a patient's clinical history and the examination findings that suggest and support the diagnosis, with review of available treatment options.

Pathologic Study of Supernumerary Orbital Band in Type I Duane Syndrome.

BACKGROUND/AIMS: Accessory orbital bands are relatively rare and very few reports detail histopathology. Cases in the literature describe the composition of the bands as muscular and/or fibrous. The composition of the supernumerary band lying deep in the medial rectus muscle in a patient with type I Duane syndrome was investigated. METHODS: Histochemical stains were used in conjunction with polarized light for differentiating compressed collagen from muscle. Immunohistochemistry was used for verification of the presence of muscle. RESULTS: Compressed collagen appeared red using Masson trichrome staining. Collagen was positively identified by illumination with polarized light on several stains including the underutilized Sirius red dye. CONCLUSIONS: The findings of dense collagen fibers in the fibrotic band with focal striated muscle correlated with the restrictive strabismus. In concert with other cases in the literature, it is proposed that the fibrous bands are generally associated with restrictive strabismus. Bands that are muscular may or may not be associated with strabismus. Special techniques are needed to positively identify compressed collagen.

Ultrasound biomicroscopy measurement of Schlemm's canal in pediatric patients with and without glaucoma.

PURPOSE: To compare the diameter of Schlemm's canal in children with and without congenital glaucoma as measured in vivo by means of ultrasound biomicroscopy. METHODS: In this prospective single-center study of pediatric subjects (<18 years of age) the diameter of Schlemm's canal in nonglaucomatous and glaucomatous eyes was compared. An 80 MHz iUltrasound probe (iScience Interventional Inc, Menlo Park, CA) placed near the limbus was used to identify and measure the canal's diameter with special attention to the anterior segment anatomy (especially in subjects with congenital glaucoma). RESULTS: A total of 20 subjects were included; mean age of subjects without glaucoma was 6.6 ± 6.65 years; of those with glaucoma, 9.4 ± 11.80 months. The mean canal diameter in nonglaucomatous eyes was 142 ± 33.2 µm (range, 90-196 µm); in glaucomatous eyes, 64.9 ± 10.90 µm (P = 0.007). Schlemm's canal could not be identified in 50% of patients with congenital glaucoma. There was a trend toward smaller canal diameter in subjects with no glaucoma <2 years old. Mean canal diameter in nonglaucomatous eyes was 103 ± 8 µm (range, 90-115 µm) in subjects <2 years of age and 161 ± 20 µm (range, 110-196 µm) in subjects >2 years of age (P = 0.0012). CONCLUSIONS: In our pediatric study cohort the diameter of Schlemm's canal varied by age and presence of glaucoma.

Bilateral Congenital Posterior Capsular Defects and Ectopic Cataracts.

Preexisting posterior capsular defects are rare and can be associated with infantile cataracts. The authors review possible etiologies of this condition and emphasize the importance of cautious preoperative planning and surgery.

EN FACE OPTICAL COHERENCE TOMOGRAPHY OF MACULAR MICROCYSTS DUE TO OPTIC NEUROPATHY FROM NEUROMYELITIS OPTICA.

PURPOSE: To describe the multimodal imaging, including en face optical coherence tomography (OCT) and OCT angiography, findings of a case of macular microcysts associated with neuromyelitis optica. METHODS: Findings on clinical examination, color fundus photography, fluorescein angiography, fundus autofluorescence, visual fields, and OCT including en face OCT and OCT angiography are presented. RESULTS: A 12-year-old African American boy presented with bilateral optic atrophy from neuromyelitis optica. Clinical examination was notable for bilateral optic nerve head pallor. Visual fields of both eyes showed generalized depression. Fluorescein angiography and fundus autofluorescence were unremarkable. Spectral domain OCT B-scan images showed characteristic paracentral, hyporeflective, microcystic lesions in the inner nuclear layer of both eyes, and en face OCT images demonstrated a corresponding pattern of large paracentral cysts radiating peripherally into smaller diffuse cysts. Optical coherence tomographic angiography of the superficial and deep retinal capillary plexuses was unremarkable. CONCLUSION: Macular microcysts have been associated with various forms of optic atrophy, including neuromyelitis optica. Spectral domain and en face OCT imaging of the microcysts demonstrated a very characteristic pattern. Normal fluorescein and OCT angiography suggest that nonvascular causes, such as Müller cell degeneration, might contribute to the etiologic mechanism.

Binocular inhibition in strabismic patients is associated with diminished quality of life.

PURPOSE: To characterize the relationship between binocular summation (BiS) and binocular inhibition (BI) on the quality of life of adults and children with strabismus. METHODS: Strabismus patients at a single center from 2010 to 2012 were prospectively enrolled. A BiS score was measured using ETDRS and Sloan low-contrast visual acuity (LCA) protocols at 2.5% and 1.25% contrast. Patients were categorized as having BiS (binocular better than better-eye visual acuity by ≥5 letters), BI (binocular worse than better-eye visual acuity by ≥5 letters), or otherwise indeterminate visual acuity (a difference between binocular visual acuity and monocular visual acuity of the better eye of <5 letters). Quality of life was evaluated by the National Eye Institute Visual Functioning Questionnaire 25 (VFQ-25), 20-item Adult Strabismus Questionnaire (AS-20), and the Amblyopia and Strabismus Questionnaire. RESULTS: A total of 108 patients were included. There was no significant BiS or BI for high-contrast ETDRS or 2.5% LCA tests; however, a mean BiS score of -2.14 ± 7.0 letters for 1.25% LCA demonstrated significant binocular inhibition (P = 0.004) for this contrast level. The mean composite VFQ-25 score was significantly lower in subjects with BI on ETDRS (80 ± 19 vs 57 ± 7 for subjects with BiS and BI, resp. [P = 0.03]), 2.5% LCA (81 ± 14 vs 66 ± 16 for subjects with BiS and BI, resp. [P = 0.01]), and 1.25% LCA tests (91 ± 9 vs 72 ± 14 for subjects with BiS and BI, resp. [P = 0.005]). After accounting for potential covariates, significant association persisted for BI, demonstrated by 1.25% LCA (P = 0.01). With BI demonstrable at 2.5%, AS-20 scores were also significantly lower (P = 0.04). CONCLUSIONS: Strabismic patients with BI had significantly lower quality-of -life scores than those who did not, even after accounting for potential covariates and the absence of diplopia.

Surgical management for postkeratoplasty glaucoma: a meta-analysis.

PURPOSE: To perform a meta-analysis on postkeratoplasty glaucoma and directly compare the affect of trabeculectomy, cyclophotocoagulation (CPC), and glaucoma drainage device (GDD) on intraocular pressure (IOP) control and corneal graft survival. METHODS: We searched Medline (Ovid, PubMed), EMBASE, and The Cochrane Library databases for clinical articles that maintained our inclusion criteria. The primary outcome measures were IOP control and overall corneal graft survival. The secondary outcomes were failure rate of the primary glaucoma procedure and change in visual acuity. We used 1-way analysis of variance weighed by the number of participants in each study to compare the outcomes. RESULTS: Information from a total of 266 eyes was collected from 13 articles. Trabeculectomy reduced IOP by 13.6 mm Hg compared with 20.4 mm Hg with CPC and 20.2 mm Hg with GDD (P<0.001). The failure rate of glaucoma surgery was highest after trabeculectomy 37% (95% CI, 31.4%-41.9%) compared with 20.7% (95% CI, 17.6%-23.7%) after CPC and 16% (95% CI, 13.8%-18.9%) after GDD and this was statistically significant (P<0.001). GDD was associated with higher rate of corneal graft failure (35%) than either CPC or trabeculectomy (21% and 24%, P=0.001 and P<0.001, respectively). The percentage of patients who experienced worsening of vision was the highest after CPC (26%) and the least with GDD (20%). CONCLUSIONS: Our meta-analysis shows that in patients with postkeratoplasty glaucoma, GDD is associated with greater IOP control, the lowest glaucoma surgery failure rate, and less vision loss compared with other forms of glaucoma surgery. However, GDD surgery is also associated with higher rate of graft failure.

Combined surgical and sclerotherapy for an extensive venous-lymphatic orbital anomaly.

Combined venous-lymphatic anomalies (lymphangiomas) of the orbit are nonhereditary, congenital lesions. We report the case of a 6-year-old boy with an extensive right orbital venous-lymphatic anomaly and severe facial deformity who developed 3 intraconal hemorrhages over 3 months. Although the visual acuity was recovered in the first two surgical interventions, the last episode of bleeding resulted in permanent visual acuity loss. At that stage, adjunctive therapy with n-butyl-cyanoacrylate allowed for greater surgical excision. No further episodes of recurrent hemorrhage occurred and the exophthalmos was corrected, thereby improving his overall cosmesis.