Publications

2021

Purpose: Localization of the lacrimal sac is a critical step during endoscopic dacryocystorhinostomy (endo-DCR). A "light pipe" can be used to transilluminate the lacrimal sac endonasally. We hypothesized that this may misguide the surgeon learning endo-DCR to create an osteotomy mostly posterior to the maxillary line if only the bone overlying the transillumination was to be removed, as the thinner lacrimal bone will transmit light more readily than the thicker maxillary bone of the frontal process of the maxilla that forms the anterior lacrimal sac fossa.Methods: The charts of 32 patients with primary acquired nasolacrimal duct obstruction in whom a lighted system was used during endo-DCR at Massachusetts Eye and Ear from April 2015 through October 2016 were reviewed. Patients with prior history of lacrimal surgery or trauma directly to the lacrimal sac fossa were excluded. Location of the maximal point of transillumination in relation to the maxillary line was observed and noted intraoperatively.Results: Of a total of 39 endo-DCR surgeries performed, the intraoperative transillumination point was entirely posterior to the maxillary line in 32 instances (82%).Conclusions: Use of an endocanalicular light pipe preferentially illuminates posterior to the maxillary line endonasally. The anterior lacrimal sac fossa (maxillary line and anterior as visualized endonasally) is rarely transilluminated, likely due to thicker bone in that region. Surgeons learning how to perform endo-DCR using a light pipe should be aware of this phenomenon.
Purpose: To report two cases of thyroid eye disease (TED) associated compressive optic neuropathy (CON) that resolved after treatment with teprotumumab. Observation: Two patients presented with active TED resulting in mild CON with the typical corresponding visual field (VF) defects. Both patients were initiated on intravenous (IV) corticosteroid therapy but despite treatment had persistent VF defects. Both patients were then treated with teprotumumab and demonstrated marked clinical improvement and complete resolution of TED-CON VF defects early in their infusion course. Conclusions and importance: These cases suggest that teprotumumab can be a rapid and effective treatment for TED-CON, and raises the question of whether it may be superior to IV corticosteroid therapy.
Sangermano R, Deitch I, Peter V, Ba-Abbad R, Place E, Zampaglione E, Wagner N, Fulton A, Coutinho-Santos L, Rosin B, Dunet V, AlTalbishi A, Banin E, Sousa AB, Neves M, Larson A, Quinodoz M, Michaelides M, Ben-Yosef T, Pierce E, Rivolta C, Webster A, Arno G, Sharon D, Huckfeldt R, Bujakowska K. Broadening INPP5E phenotypic spectrum: detection of rare variants in syndromic and non-syndromic IRD. NPJ Genom Med. 2021;6(1):53.
Pathogenic variants in INPP5E cause Joubert syndrome (JBTS), a ciliopathy with retinal involvement. However, despite sporadic cases in large cohort sequencing studies, a clear association with non-syndromic inherited retinal degenerations (IRDs) has not been made. We validate this association by reporting 16 non-syndromic IRD patients from ten families with bi-allelic mutations in INPP5E. Additional two patients showed early onset IRD with limited JBTS features. Detailed phenotypic description for all probands is presented. We report 14 rare INPP5E variants, 12 of which have not been reported in previous studies. We present tertiary protein modeling and analyze all INPP5E variants for deleteriousness and phenotypic correlation. We observe that the combined impact of INPP5E variants in JBTS and non-syndromic IRD patients does not reveal a clear genotype-phenotype correlation, suggesting the involvement of genetic modifiers. Our study cements the wide phenotypic spectrum of INPP5E disease, adding proof that sequence defects in this gene can lead to early-onset non-syndromic IRD.
Reversal of optic nerve head (ONH) cupping has been considered an important clinical observation that signals surgical success and control of intraocular pressure (IOP) in childhood glaucoma. Many theories based on elasticity of pediatric eyes have been proposed, including anterior movement of the elastic lamina cribrosa or shrinkage of the scleral canal. The relationship between these factors and axonal loss is unclear when reversal of cupping has been observed. Retinal nerve fiber layer (RNFL) optical coherence tomography (OCT) can help to clarify this. We present a case series of 4 pediatric patients with secondary glaucoma that demonstrated ONH cupping reversal with pre- and postoperative clinical images and RNFL OCT.
Chen T, Jin L, Zhu W, Wang C, Zhang G, Wang X, Wang J, Yang K, Cochrane G, Lamoureux EL, Friedman D, Gilbert S, Lansingh V, Resnikoff S, Zhao J, Xiao B, He M, Congdon N. Knowledge, attitudes and eye health-seeking behaviours in a population-based sample of people with diabetes in rural China. Br J Ophthalmol. 2021;105(6):806–811.
AIMS: To assess knowledge of diabetes and acceptance of eye care among people with diabetes in rural China, to improve service uptake. METHODS: Population-based study of people in Guangdong, China, with glycosylated haemoglobin A1c≥6.5% and/or known history of diabetes. Between August and November 2014, participants answered a questionnaire (based on Delphi process/previous focus groups) on medical history, demographic characteristics, self-rated health and vision, knowledge about diabetes and diabetic retinopathy, quality of local healthcare, barriers to treatment, likely acceptance of eye exams and treatment, and interventions rated most likely to improve service uptake. Presenting visual acuity was assessed, fundus photography performed and images graded by trained graders. Potential predictors of accepting care were evaluated and confounders adjusted for using logistic regression. RESULTS: A total of 562 people (9.6% (256/5825), mean age 66.2±9.84 years, 207 (36.8%) men) had diabetes, 118 (22.3%) previously diagnosed. 'Very likely' or 'likely' acceptance of laser treatment (140/530=26.4%) was lower than for eye exams (317/530=59.8%, p<0.001). Predictors of accepting both exams and laser included younger age (p<.001) and prior awareness of diabetes diagnosis (p=0.004 and p=0.035, respectively). The leading barrier to receiving diabetes treatment was unawareness of diagnosis (409/454, 97.2%), while interventions rated most likely to improve acceptance of eye exams included reimbursement of travel costs (387/562, 73.0%), video or other health education (359/562, 67.7%) and phone call reminders (346/562, 65.3%). CONCLUSIONS: Improving diagnosis of diabetes, along with incentives, education and communication strategies, is most likely to enhance poor acceptance of diabetic eye care in this setting.
Than T, Morettin C, Harthan J, Hartwick A, Huecker J, Johnson S, Migneco M, Shorter E, Whiteside M, Margolis M, Olson C, Alferez C, Zyl T, Rodic-Polic B, Storch G, Gordon M. Efficacy of a Single Administration of 5% Povidone-Iodine in the Treatment of Adenoviral Conjunctivitis. Am J Ophthalmol. 2021;
PURPOSE: To evaluate the safety and efficacy of a single, in-office administration of 5% povidone-iodine (PVP-I) compared to artificial tears (AT) for adenoviral conjunctivitis (Ad-Cs). DESIGN: Double-masked pilot randomized trial METHODS: Patients presenting with presumed adenoviral conjunctivitis were screened at 9 U.S. clinics. INCLUSION CRITERIA: ≥ 18 years of age, symptoms ≤ 4 days and a positive AdenoPlus® test. EXCLUSION CRITERIA: thyroid disease, iodine allergy, recent ocular surgery, and ocular findings inconsistent with early-stage Ad-Cs. Randomization was to a single administration of 5% PVP-I or AT in one eye and examinations on days 1-2, 4, 7, 14 and 21 with conjunctival swabs taken each visit for quantitative polymerase chain reaction. Primary outcome was percent reduction from peak viral load. Secondary outcomes were improvement in clinical signs and symptoms. RESULTS: Of 56 patients randomized, 28 had detectable viral titers at baseline. Day 4 post-treatment, viral titers in the 5% PVP-I and AT groups were 2.5% ± 2.7% and 14.4% ± 10.5% of peak respectively (p=0.020). Severity of participant-reported tearing, lid swelling and redness as well as clinician-graded mucoid discharge, bulbar redness and bulbar edema were lower in the 5% PVP-I group than AT group on Day 4 (p< 0.05). After Day 4, viral titers, severity of signs and symptoms decreased markedly in both groups and no differences between groups were detected. CONCLUSIONS: Pilot data suggest a single, in-office administration of 5% PVP-I could reduce viral load and hasten improvement of clinical signs and symptoms in patients with Ad-Cs.