Faculty Bibliography

PURPOSE/OBJECTIVE:To describe a rare case of silicone oil emulsification which occurred only three months postoperatively. We discuss the implications for postoperative counseling. METHODS:Retrospective chart review of a single patient. RESULTS:39-year-old female who presented with a right eye macula-on retinal detachment which was repaired with scleral buckle, vitrectomy, and silicone oil tamponade. Her course was complicated by extensive silicone oil emulsification within 3 months postoperatively, most likely due to shear forces induced by her daily CrossFit exercise regimen. CONCLUSION/CONCLUSIONS:Typical postoperative precautions after a retinal detachment repair include no heavy lifting or strenuous activity for one week. These restrictions may need to be more stringent and long-term for patients with silicone oil to prevent early emulsification.

BACKGROUND/OBJECTIVE/OBJECTIVE:To identify geographic and socioeconomic variables associated with residential proximity to Phase 3 ophthalmology clinical trial sites. METHODS:The geographic location of clinical trial sites for Phase 3 clinical trials in ophthalmology was identified using ClinicalTrials.gov. Driving time from each United States (US) census tract centroid to nearest clinical trial site was calculated using real traffic patterns. Travel data were crosslinked to census-tract level public datasets from United States Census Bureau American Community Survey (ACS). Cross-sectional multivariable regression was used to identify associations between census-tract sociodemographic factors and driving time (>60 min) from each census tract centroid to the nearest clinical trial site. RESULTS:There were 2330 unique clinical trial sites and 71,897 census tracts. Shortest median time was to retina sites [33.7 min (18.7, 70.1 min)]. Longest median time was to neuro-ophthalmology sites [119.8 min (48.7, 240.4 min)]. Driving >60 min was associated with rural tracts [adjusted odds ratio (aOR) 7.60; 95% CI (5.66-10.20), p < 0.0001]; Midwest [aOR 1.84(1.15-2.96), p = 0.01], South [aOR 2.57 (1.38-4.79), p < 0.01], and West [aOR 2.52 (1.52-4.17), p < 0.001] v. Northeast; and tracts with higher visual impairment [aOR 1.07 (1.03-1.10), p < 0.001)]; higher poverty levels [4th v.1st Quartile of population below poverty, aOR 2.26 (1.72-2.98), p < 0.0001]; and lower education levels [high school v. Bachelor's degree or higher aOR 1.02 (1.00-1.03), p = 0.0072]. CONCLUSIONS:There are significant geographic and socioeconomic disparities in access to ophthalmology clinical trial sites for rural, non-Northeastern, poorer, and lower education level census tracts, and for census tracts with higher levels of self-reported visual impairment.

A 23-year-old man presented with new onset horizontal diplopia 5 months after a left orbital floor fracture. Examination revealed bilateral abduction deficits and disc swelling. Urgent MRI and MRI showed no significant abnormalities in the CNS. Lumbar puncture revealed a minimally elevated opening pressure and significant leukocytosis. Additional CSF testing revealed probable Lyme meningitis. The patient responded to a course of oral doxycycline, with rapid resolution of his diplopia, abduction deficits, and disc edema.

BACKGROUND:This study aimed to identify best practices for treating 13q deletion syndrome (13q-) patients with retinoblastoma in the era of super-selective ophthalmic artery chemosurgery (OAC) and intravitreal injection therapy (IVIT). METHODS:Retrospective study of 21 eyes from 14 patients with retinoblastoma and 13q- who were treated at Memorial Sloan Kettering Cancer Center (MSKCC) between May 2006 and May 2020, with a mean follow up of 3.7 years. Ocular survival, patient survival, and treatment toxicities were assessed. RESULTS:Nine of the 12 eyes that underwent OAC/IVIT at MSKCC have been progression free for at least 1 year since their last treatments. Fifteen out of 26 OAC cycles resulted in grade 3-4 hematologic toxicity. There was one death from sepsis in the setting of intravenous chemotherapy (IVC) for metastatic disease that occurred after OAC/IVIT therapy. The 2-year Kaplan-Meier ocular survival estimate for the whole cohort was 75% and for the eyes that received OAC or IVIT at MSKCC 83%. For OAC hematologic toxicities, one platelet transfusion and two filgrastim doses were administered, and one patient was hospitalized for neutropenic fevers. CONCLUSIONS:The majority of 13q- eyes treated with OAC/IVIT-based regimens can be cured, and there were no deaths related to complications from OAC or IVIT. 13q- Patients did have increased risk of systemic treatment complications, even from super-selective chemotherapies. Despite these toxicities, only one patient developed febrile neutropenia, one patient required a blood product transfusion, and two patients received filgrastim for both OAC and IVC complications. PRÉCIS: Children with 13q deletion syndrome with retinoblastoma managed with intra-arterial and intravitreal chemotherapy have excellent patient and ocular survival with acceptable toxicity.

Purpose : Predicting progression of primary open angle glaucoma (POAG) continues to be a challenge. Recent studies have shown that macular parameters may predict glaucoma progression and disease onset equally as well or even better than the traditionally used optic nerve head (ONH) retinal nerve fiber layer (RNFL) thickness. We performed a retrospective longitudinal study to identify structural parameters which best predicted visual field (VF) loss. Methods : Subjects with POAG with at least 5 qualified Cirrus OCT (Zeiss, Dublin, CA) macular and optic nerve head (ONH) scans and 5 qualified 24-2 Humphrey VF tests (Zeiss) were enrolled to this study. All parameters from the OCT's report of the macula and ONH scans were used in the analysis. We identified subjects who were OCT ONH progressors or VF progressors using both event and trend based definitions of progression based on the Guided Progression Analyses. Students t-test was used to assess differences in baseline characteristics between groups, and mixed-effects model was used in longitudinal analysis to compare the rate of parameter change between groups. Results : 263 eyes (180 subjects) with a mean follow-up time of 2.4+/-1.8 years were included in the study. Twenty-three eyes were identified as ONH progressors, 25 eyes were identified as VF progressors, and of these, 6 eyes were both VF and ONH progressors. At baseline, only macular average RNFL and macular inferior RNFL were significantly thinner in ONH progressors compared to non-progressors. Between the VF progressors and non-progressor groups, all OCT parameters were significantly thinner at baseline in the progressors except for four focal macular measurements and disc area. Mixed-effects modeling showed that both focal macular and ONH parameters thinned at a significantly greater rate in VF progressors compared to non-progressors (Table 1). Conclusions : Focal macular retinal segmentations and focal ONH parameters thin significantly more per year in VF progressors, and VF progressors tend to have thinner baseline structural parameters

GABAergic interneurons have many important functions in cortical circuitry, a reflection of their cell diversity. The developmental origins of this diversity are poorly understood. Here, we identify rostral-caudal regionality in Wnt exposure within the interneuron progenitor zone delineating the specification of the two main interneuron subclasses. Caudally situated medial ganglionic eminence (MGE) progenitors receive high levels of Wnt signaling and give rise to somatostatin (SST)-expressing cortical interneurons. By contrast, parvalbumin (PV)-expressing basket cells originate mostly from the rostral MGE, where Wnt signaling is attenuated. Interestingly, rather than canonical signaling through β-catenin, signaling via the non-canonical Wnt receptor Ryk regulates interneuron cell-fate specification in vivo and in vitro. Indeed, gain of function of Ryk intracellular domain signaling regulates SST and PV fate in a dose-dependent manner, suggesting that Ryk signaling acts in a graded fashion. These data reveal an important role for non-canonical Wnt-Ryk signaling in establishing the correct ratios of cortical interneuron subtypes.

OBJECTIVE:The Mobile Universal Lexicon Evaluation System (MULES) is a test of rapid picture naming that is under investigation for concussion. MULES captures an extensive visual network, including pathways for eye movements, color perception, memory and object recognition. The purpose of this study was to introduce the MULES to visual assessment of patients with MS, and to examine associations with other tests of afferent and efferent visual function. METHODS:We administered the MULES in addition to binocular measures of low-contrast letter acuity (LCLA), high-contrast visual acuity (VA) and the King-Devick (K-D) test of rapid number naming in an MS cohort and in a group of disease-free controls. RESULTS:Among 24 patients with MS (median age 36 years, range 20-72, 64% female) and 22 disease-free controls (median age 34 years, range 19-59, 57% female), MULES test times were greater (worse) among the patients (60.0 vs. 40.0 s). Accounting for age, MS vs. control status was a predictor of MULES test times (P = .01, logistic regression). Faster testing times were noted among patients with MS who had greater (better) performance on binocular LCLA at 2.5% contrast (P < .001, linear regression, accounting for age), binocular high-contrast VA (P < .001), and K-D testing (P < .001). Both groups demonstrated approximately 10-s improvements in MULES test times between trials 1 and 2 (P < .0001, paired t-tests). CONCLUSION/CONCLUSIONS:The MULES test, a complex task of rapid picture naming involves an extensive visual network that captures eye movements, color perception and the characterization of objects. Color recognition, a key component of this novel assessment, is early in object processing and requires area V4 and the inferior temporal projections. MULES scores reflect performance of LCLA, a widely-used measure of visual function in MS clinical trials. These results provide evidence that the MULES test can add efficient visual screening to the assessment of patients with MS.

PURPOSE/OBJECTIVE:To report a case of bilateral toxic corneal and conjunctival epitheliopathy secondary to administration of filter paper impregnated with lysergic acid diethylamide (LSD) in the inferior conjunctival fornices. METHODS:This is a single case report of an 18-year-old man who presented to the emergency department with acute, bilateral eye pain and redness of 24 hours. The patient admitted to placing folded strips of blotting paper impregnated with LSD into the inferior fornices of his eyes the previous night. RESULTS:The patient was found to have localized bilateral corneal and conjunctival abrasions with underlying subconjunctival hemorrhage. Conjunctival abrasion was "kissing," involving the bulbar and palpebral conjunctiva, corresponding to the presumed location of the filter paper. There was no corneal stromal opacification. He was lost to follow up within 1 week of initial presentation but stated that his symptoms improved. CONCLUSIONS:To the best of our knowledge, this is the first reported case of bilateral hemorrhagic conjunctival abrasion and corneal abrasion secondary to LSD. "Kissing" conjunctival lesions, which have been previously reported with heroin use, should raise suspicion for drug abuse.