Jack J. Tian

Clues from Crouzon: Insights into the potential role of growth factors in the pathogenesis of myelinated retinal nerve fibers

Purpose We present a case of bilateral extensive peripapillary myelinated retinal nerve fibers (MRNF) in an individual with Crouzon syndrome, an inherited form of craniosynostosis caused by overactivation of fibroblast growth factor receptor 2. As a secondary aim, we examine the utility of optical coherence tomography (OCT) angiography for visualization of peripapillary vasculature obscured by myelination on other imaging modalities. Methods A 24-year-old woman with Crouzon syndrome was evaluated for suspected optic neuritis in the right eye. Results Funduscopic examination and photography revealed the incidental finding of bilateral extensive peripapillary MRNF. OCT angiography provided excellent visualization of peripapillary retinal vessels, which were partially obscured by myelination on other imaging modalities. Conclusions This association of Crouzon syndrome with bilateral peripapillary MRNF may lend insight into the developmental control of optic nerve myelination, the pathogenesis of MRNF, and the potential role of growth factors in these processes. Further, OCT angiography allowed for excellent blood vessel visualization in this case of MRNF.

Comparison of 2 pupil expansion devices for small-pupil cataract surgery

UNLABELLED We compared the degree of postoperative iris distortion with the diamond-shaped Malyugin ring and the circular Visitec I-Ring iris dilators using postoperative iris photographs in a patient with bilateral small nondilating pupils. Routine phacoemulsification was performed in the right eye with the assistance of a Malyugin ring for iris dilation. One month later, the same procedure was performed in the left eye using the circular ring for iris dilation. Anterior chamber slitlamp photographs were taken of both eyes. The amount of distortion in the postoperative pupils was calculated using the ratio of the postoperative pupil area to the area of the circle of best fit. The circular ring, constructed from a softer material, resulted in 11% distortion and the Malyugin ring, 49%, suggesting that the circular ring caused less trauma to the iris. FINANCIAL DISCLOSURE None of the authors has a financial or proprietary interest in any material or method mentioned.

High Rate of Early Posterior Capsule Opacification following Femtosecond Laser-Assisted Cataract Surgery

Purpose: To compare the rates of rapid posterior capsule opacification (PCO) formation in the first 3 months following femtosecond laser-assisted cataract surgery (FLACS) to manual anterior capsulorhexis. Methods: Retrospective review of 29 cases of FLACS, comparing the rates of PCO in the first 3 months following surgery to 50 consecutive cases of manual anterior capsulorhexis. Results: Seven of the 29 FLACS cases developed PCO requiring capsulotomy at 3 months, while none of the control cases required a capsulotomy over the same time period (p < 0.05). Conclusion: There is an increased incidence of early-onset PCO following the use of femtosecond laser in cataract surgery that is otherwise unfounded in manual capsulorhexis. This suggests that the use of a femtosecond laser could increase the risk of this novel postoperative complication.

Correcting Finger Counting to Snellen Acuity

ABSTRACT In this paper, the authors describe an online tool with which to convert and thus quantify count finger measurements of visual acuity into Snellen equivalents. It is hoped that this tool allows for the re-interpretation of retrospectively collected data that provide visual acuity in terms of qualitative count finger measurements.

Fractional photothermolysis treatment of digital cutis laxa reverses hand disability

In this case study, we present a safe and novel treatment for a patient with soft tissue hand disability caused by severe and chronic lupus and cutis laxa (CL). This patient was a woman in her 50s with a 20‐year history of systemic lupus erythematous (SLE) and multiple sclerosis who developed hand disability because of the drastic loss of firmness in her soft tissue, extending from the dermis down to the ligaments. The likely cause was CL with SLE synovitis, exacerbated by corticosteroid tapering. Fractional photothermolysis (FP) LASER (Fraxel DUAL 1550/1927; Solta Medical) therapy profoundly alleviated her joint locking in addition to improving the firmness of the overlying skin to reverse her hand disability. This case illustrates a novel approach to CL hand treatment and the profound impact the treatment had on the patients disabled hand. FP therapy is quick and safe, and its medical application to skin and joints should be further explored.

Validity of Forced Eyelid Closure Test: A Novel Clinical Screening Test for Ocular Myasthenia Gravis

Background: Forced eyelid closure test (FECT) is a clinical screening test developed from the original Cogan lid twitch (CLT) sign to assist in the diagnosis of ocular myasthenia gravis (OMG), We evaluated the sensitivity and specificity of FECT compared with CLT and benchmarked to standard diagnostic tests. Methods: This study was a retrospective chart review of 48 patients using electronic medical records of those that presented with ptosis and/or diplopia at Doheny Eye Institute, University of California, Los Angeles between February 2015 and April 2016. Patients without FECT testing were excluded. FECT and CLT results, and final diagnosis were recorded. To perform FECT, the patient was asked to squeeze his or her eyelids shut for 5–10 seconds then open quickly and fixate in primary position. The excessive upward overshoot of eyelids movement indicated a positive FECT. The test was performed by a neuro-ophthalmologist before establishing the diagnosis. Patients who had equivocal test results and/or inconclusive final diagnosis were excluded. Results: Of the 48 patients studied, 18 patients (37.5%) had positive FECT; 15 of whom had a final diagnosis of OMG (83.3%). Of the 30 patients with negative FECT, 1 had OMG (3.3%). Of the 48 patients, 35 patients also had a documented CLT result (72.9%). CLT was positive in 11 of these 35 patients (31.4%), and 9 of these 11 had OMG (81.8%). Of the 24 patients with negative CLT, 2 of them had OMG (8.3%). Sensitivity and specificity of FECT were 94% and 91% (joint 95% confidence region: sensitivity × specificity = [0.70, 1] × [0.75, 1]). The relative true-positive fraction (rTPF) between FECT and CLT was 1.15; the relative false-positive fraction was 1.31. Conclusions: FECT is a simple clinical screening test with good sensitivity and specificity for OMG.

Melanoma-Associated Retinopathy 28 Years After Diagnosis

Melanoma-Associated Retinopathy 28 Years After Diagnosis To date, at least 60 case reports of melanoma-associated retinopathy (MAR) have been published, and these patients have the associated autoantibodies targeting epitopes common to melanoma and bipolar cells.1 The classic symptoms of MAR include flickering and shimmering in the peripheral vision that progresses to constriction of the visual field.1-3 In most MAR cases, the fundus photograph appears normal, and visual acuity and color vision test results are also typically normal.1,2 These findings create a diagnostic dilemma for the physician. Objective measures of bipolar cell function can be fundamental to making the diagnosis, with on-off responses during electroretinography (ERG) showing a characteristic b-wave depression.4 This result can be supported by immunohistochemical analysis, indirect immunoassay, and Western blotting for autoantibodies to bipolar cells5; however, although these laboratory tests are used in investigative studies, they have not been established as clinically useful.