Dysthyroid Orbitopathy Presenting with Gaze-Evoked Amaurosis: Case Report and Review of the Literature

Background: Gaze-evoked amaurosis (GEA) describes visual loss associated with eccentric gaze that recovers when the eye is returned to primary position. Here we describe an unusual case of bilateral GEA as the presenting feature of dysthyroid orbitopathy. This is only the third such case to be reported in the literature and the first to feature bilateral GEA in all positions of gaze without accompanying proptosis or ophthalmoplegia.

Case Presentation: A 50-year-old man who had recently commenced treatment for thyrotoxicosis presented with a 3-week history of typical GEA in both eyes in all positions of gaze. He subsequently developed a bilateral compressive optic neuropathy which was only partially responsive to high dose steroid therapy.

Conclusion: Although an uncommon presenting feature of dysthyroid orbitopathy, GEA is an ominous symptom that may precede sight-threatening optic nerve compromise. When present, early immunosuppressive and/or decompressive treatment should be considered.     

Optogenetic Inhibition of Nav1.8 Expressing Corneal Afferents Reduces Persistent Dry Eye Pain

PurposeThe aim of the present study was to investigate the contribution of Nav1.8 expressing corneal afferent neurons to the presence of ongoing pain in lacrimal gland excision (LGE)-induced dry eye.MethodsThe proton pump archaerhodopsin-3/eGFP (ArchT/eGFP) was conditionally expressed in corneal afferents using Nav1.8-cre mice. Dry eye was produced by unilateral LGE. Real time place preference was assessed using a three-chamber apparatus. A neutral, unlit center chamber was flanked by one illuminated with a control light and one illuminated with an ArchT activating light. For real-time preference, animals were placed in the neutral chamber and tracked over five 10-minute sessions, with the lights turned on during the second and fourth sessions. In other studies, movement was tracked over three 10-minute sessions (the lights turned on only during the second session), with animals tested once per day over the course of 4 days. A local anesthetic was used to examine the role of ongoing corneal afferent activity in producing place preference.ResultsThe corneal afferent nerves and trigeminal ganglion cell bodies showed a robust eGFP signal in Nav1.8-cre;ArchT/eGFP mice. After LGE, Nav1.8-cre;ArchT/eGFP mice demonstrated a preference for the ArchT activating light paired chamber. Preference was prevented with pre-application to the cornea of a local anesthetic. Nav1.8-cre;ArchT/eGFP mice with sham surgery and LGE wild-type control mice did not develop preference.ConclusionsResults indicate LGE-induced persistent, ongoing pain, driven by Nav1.8 expressing corneal afferents. Inhibition of these neurons represents a potential strategy for treating ongoing dry eye-induced pain.     

Pain sensitivity and autonomic nervous system parameters as predictors of dry eye symptoms after LASIK

PurposeDifferences in pain processing and autonomic function among patients have been implicated in the development of chronic pain after surgery. This study was designed to evaluate whether pain and autonomic metrics predict severity of chronic dry eye (DE) symptoms after LASIK, as there is increasing evidence that DE symptoms may be manifestations of persistent post-operative ocular pain.MethodsSecondary analysis of prospective randomized clinical trial. Patients were treated with either pregabalin or placebo. As no significant differences in DE symptoms were detected by treatment allocation at six months, all participants were grouped together for the present analyses. Subjects were evaluated pre-LASIK with regard to evoked pain sensitivity (utilizing quantitative sensory testing), autonomic metrics and DE and ocular pain symptoms (via validated questionnaires). Measures of DE and ocular pain were assessed post-LASIK, and the Dry Eye Questionnaire 5 (DEQ5) score 6-months after surgery was the primary outcome of interest.Results43 individuals were randomized to pregabalin (n = 21) or placebo (n = 22). 42 completed the 6-month visit. Several baseline autonomic metrics correlated with 6-month post-operative DEQ5 scores, including lower systolic (r −0.37, p = 0.02) and diastolic blood pressure (r −0.32, p = 0.04). Ocular pain at 6 months was also negatively correlated with blood pressure (r −0.31, p = 0.047). The presence of painful aftersensations was a significant predictor of chronic DE symptoms at 6 months (mean DEQ5 scores: 8.0 ± 1.9 versus 5.0 ± 5.0, p = 0.009).ConclusionsHeightened parasympathetic tone and prolonged pain sensitivity measured prior to surgery predicted greater DE symptom severity 6 months after LASIK.Trial registrationNCT02701764.     

The Use of Topical Corticosteroids for Treatment of Dry Eye Syndrome

Dry eye disease (DED) is a multifactorial disease that results in symptoms of discomfort, visual disturbance, and damage to the ocular surface. Because chronic inflammation plays an important role in DED, treatment with topical corticosteroids has been demonstrated to ameliorate the signs and symptoms of the disease. Although these agents have proven short-term efficacy, their long-term use may cause intraocular pressure elevation and cataract progression. A carefully review of the different studies shows that differences between corticosteroids may exist regarding the incidence of side effects and evidence of efficacy in DED patients.     

干眼症患者泪液中表皮生长因子的含量分析

目的验证干眼症泪液中EGF的含量及与健康人的差别。方法对干眼症患者用放射免疫法检测泪液中的EGF的含量,所得数据利用Independent Sample T Test分析,结果干眼症组泪液中EGF的含量与对照组相比都有统计学意义上的降低（P=0.012）。结论干眼症患者泪液中的EGF含量都比健康人的低,为干眼症的诊断治疗开阔了更广阔的空间。     

Ophthalmic Pathology of Nance-Horan Syndrome: Case Report and Review of the Literature

Background: Nance-Horan syndrome (NHS) is a rare X-linked disorder typified by dense congenital central cataracts, microcornea, anteverted and simplex pinnae, brachymetacarpalia, and numerous dental anomalies due in most cases to a mutation in the NHS gene. Material and Methods: We present a case of clinical manifestation and ocular pathology in a patient with NHS. This article also reviews and discusses the relevant literature. Results: Classic and novel ocular pathological findings of a young male with NHS are described, including congenital cataracts, infantile glaucoma, scleral staphyloma, and severe retinal cystoid degeneration. Conclusions: We report a new pathological finding of severe retinal cystoid degeneration in this NHS patient and confirm abnormal development of the anterior chamber angle structure. These findings, coupled with our analysis of the available NHS literature, provide new understanding of the histopathological basis of ocular abnormalities and vision loss in NHS.     

Epithelioid Angiosarcoma of the Orbit Presenting as Tolosa-Hunt Syndrome: A Clinicopathologic Case Report with Review of the Literature

The clinical diagnosis of Tolosa-Hunt syndrome was first considered in a 66-year-old man with facial pain and diplopia. A complete neuroradiologic evaluation as well as an oncologic work-up yielded normal results. Several courses of oral prednisone provided no significant benefit. Within a year the patient became clinically worse and a CT scan disclosed an abnormal area of enhancement at the left orbital apex. An orbital exploration was performed elsewhere and a histologic diagnosis of myositis was obtained. Because of further worsening the patient was re-evaluated 3 months later and a CT scan showed a mass in the left orbital apex and superior orbital fissure. A second orbital exploration was performed and a sausage-shaped mass encompassing the optic nerve was excised. By light microscopy a poorly differentiated malignant tumor was infiltrating the orbital tissues with areas of intra- and perineural invasion. The tumor cells were arranged in strands and tubules with a definite tendency to form lumens that often contained red blood cells. Electron microscopic studies disclosed features consistent with a neoplasm of endothelial cell origin displaying a polarized basal lamina and rare micropinocytotic vesicles on the luminal side. The presence of multiple, slender microvilli and sometimes tonofilaments as well as desmosomes were interpreted as epithelioid metaplasia of an angiosarcoma.     

雌激素受体基因多态性与绝经后干眼症的相关性的研究

目的:研究雌激素受体(estrogen receptor,ER)基因多态性与绝经后干眼症的相关性。方法:收集绝经后干眼症患者65例及正常对照73例血液标本,提取基因组DNA,用聚合酶链反应方法检测ER基因的XbaⅠ和PvuⅡ酶切多态性并进行统计学分析。结果:干眼症患者与正常人比较,ER基因的PvuⅡ酶切多态性存在显著性差异(P<0.05),而ER基因的XbaⅠ酶切多态性没有明显差异(P>0.05)。结论:ER基因的PvuⅡ酶切多态性与绝经后干眼症发病存在相关性,不同个体的基因差异可能影响干眼症的发生。     

A Singular Case of Congenital Self-healing Histiocytosis with Skin,Liver and Atypical Eye Involvement

Purpose: To describe a rare case of congenital self-healing Langerhans cell histiocytosis (CSHLCH) presenting with atypical eye involvement. Design: Case report. Methods: A female newborn presented with purpuric lesions over the trunk, limbs, and face. Liver ultrasonography revealed hypoechogenic lesions with blurred borders. Biomicroscopy showed right posterior synechiae with fibrinoid deposits on the lens. At 7 months she presented with right acute glaucoma. Results: Biomicroscopy showed the presence of inflammatory pseudo-membrane covering the anterior surface of the lens, iris, and iridocorneal angle. Ab externo trabeculotomy was performed; access to the anterior chamber with capsulorrhexis forceps permitted a peeling of the pseudo-membrane with normalization of the intraocular pressure. Histologic examination of the membrane revealed an inflammatory tissue with CD1a and S-100 positive histiocytic cells. Conclusions: This is the first case of CSHLCH describing acute glaucoma secondary to a pseudo-inflammatory membrane with typical histiocytic cells, occluding the iridocorneal angle.     

Case of Primary Leptomeningeal Lymphoma Presenting with Papilloedema and Characteristics of Pseudotumor Syndrome

The authors describe an immunocompetent, 50-year-old man who complained of a daily transient blurring of his vision with bilateral papilloedema. His visual acuity was 20/20 OU, and the blind spot was enlarged bilaterally. There was intracranial hypertension, but imaging for systemic and brain tumours were negative. These findings suggested a diagnosis of the pseudotumor syndrome. However, MRI showed leptomeningeal enhancement, and acetazolamide successfully resolved his visual symptoms and papilloedema. Cytology and flow cytometry of the CSF led to the final diagnosis of primary leptomeningeal lymphoma (PLML). Clinicians need to be aware that a case of PLML may be misdiagnosed as peudotumor cerebri.     

Effects of Glaucoma Medication on Dry Eye Syndrome and Quality of Life in Patients with Glaucoma

PurposeTo investigate ocular surface diseases and changes in the quality of life of patients using glaucoma medications.MethodsParticipants were divided into the normal (31 individuals, 62 eyes) and glaucoma medication (30 patients, 60 eyes) groups. Changes in tear break-up time, lipid layer thickness (LLT), corneal and conjunctival staining scores, ocular surface disease index (OSDI), and Visual Function Questionnaire 25 (VFQ-25) score were assessed for 1 year.ResultsThe change in mean LLT was lower in glaucomatous eyes than in control eyes (p = 0.019) after 1 year. The results of OSDI deteriorated (p’ = 0.008), but conjunctival staining and Schirmer test results showed improvement in glaucomatous eyes compared to those in control eyes (p’ =0.035 and 0.009, respectively). The average LLT decreased at 6 and 12 months, but there was no change at 24 months. In pairwise analysis, the decrease in LLT over the first 6 months was statistically significant (p < 0.001) and remained unchanged until 24 months. Among the VFQ items, scores for near activity and social function deteriorated over 1 year in the medication group (p’ = 0.033 and 0.015, respectively). However, there was no difference in the total VFQ score.ConclusionsSignificant reduction in LLT and deterioration of OSDI were observed in the medication group compared to the control group. However, this deterioration was observed only in the first 6 months. There was no significant difference in the VFQ total score. Nonetheless, there were significant differences in near activity and social function between the control and medication groups. Therefore, the results of this study showed that although glaucoma medication worsened eye dryness, the change was limited and did not worsen the quality of life. Glaucoma medication should be used with the consideration that they can limit near activity and social functioning.     

Proteus Syndrome: Report of a Case with Developmental Glaucoma

The purpose of this study was to report developmental glaucoma and pseudopapilledema in a patient with Proteus syndrome. We defined the presence of developmental glaucoma, right pseudopapilledema and myopia in a 4.5-year-old patient with Proteus syndrome. Marked right hemihypertrophy, lipoma, macrodactyly, and asymmetry of the limbs were observed on systemic examination. A cavernoma was also detected in magnetic resonance imaging of the brain. The patient underwent bilateral goniotomy surgery due to glaucoma. The surgical outcomes were satisfactory in both eyes. In conclusions developmental glaucoma and pseudopapilledema might be associated with Proteus syndrome.     

Bilateral Posterior Scleritis Presenting as the First Manifestation of Immunoglobulin A Nephropathy: Case Report and Review of the Literature

Purpose: We describe a case of posterior scleritis presenting as the first manifestation in a Chinese patient with immunoglobin A nephropathy (IgAN).

Methods: Retrospective review of our patient and review of the published case reports from PubMed search.

Results: A 43-year-old woman developed bilateral posterior scleritis. Microscopic hematuria and proteinuria was found by urinalysis, and a kidney biopsy revealed IgAN. The posterior scleritis was resolved with oral prednisolone. For the review, 12 cases of episcleritis (70.6%), 4 cases of scleritis (23.5%), and 1 case of posterior scleritis (5.9%) associated with IgAN were analyzed. Five patients (29.4%) had scleritis or episcleritis as the first presenting manifestation.

Conclusions: Posterior scleritis may be the first manifestation of IgAN in a patient. For any adult patient with scleral/episcleral inflammation, urinalysis should be done to rule out IgAN.     

Five-Year Retrospective Review of Cases with Benign Essential Blepharospasm and Hemifacial Spasm Presenting in a Tertiary Eye Care Center in North India

Retrospective analysis of epidemiological and clinical characteristics of patients diagnosed with benign essential blepharospasm and hemifacial spasm who reported to the oculoplasty clinic of a tertiary eye care center in north India between January 2010 and April 2015 was carried out. Dry eye, as well as all the local factors that can cause blepharospasm or hemifacial spasm, was ruled out. Systemic evaluation was done to rule out any neurological disorder. A detailed history was taken to rule out any associated psychiatric disorders as well as use of any medication which could be responsible for dystonic movements. In every patient of hemifacial spasm, magnetic resonance imaging (MRI) of the brain was done for any facial nerve compression or tumor involving posterior fossa. Botulinum type A injections were given after assessing their requirements on the basis of guidelines given by Jankovic et al.     

The Effect of Horizontal Rectus Muscle Surgery on Clinical and Eye Movement Recording Indices in Infantile Nystagmus Syndrome

Objective: To determine the relationship between horizontal rectus muscle surgery and eye movement recording in infantile nystagmus syndrome.

Methods: In this prospective, interventional, non-randomized study, patients with infantile nystagmus syndrome were assigned to one of three treatment groups. Group I with head postures of less than 20°, binocular visual acuity of less than 20/30, and heterotropia less than 30^Δ underwent recessions of all 4 horizontal rectus muscles. Group II with head postures of less than 20°, binocular visual acuity of less than 20/30, and heterotropia more than 30^Δ underwent large recessions of two horizontal rectus muscles. Group III with head postures of more than 20° underwent Kestenbaum-Anderson surgery. Baseline and follow-up evaluations included best corrected visual acuity for distance and near, cycloplegic refraction, head posture, and eye movement recordings.

Results: Fifty-eight (58)patients with a mean age of 18.7 (SD ± 9.10 years) were enrolled. Four horizontal rectus muscle recessions were performed in 29 cases, 2 rectus recession in 23, and Kestenbaum-Anderson surgery in 6 cases. Mean follow-up period after surgery was 18?±?7.4 months. Distance visual acuity improved in all three groups, but only in the 2 rectus muscle surgery group was the change significant for both monocular and binocular vision (P < 0.001). In all 3 groups speed and amplitude of nystagmus waves were changed in positive or negative direction but only in 4-recti recession decrease was statistically significant (P = 0.02 & 0.04).

Conclusion: Horizontal rectus muscle surgery in subjects with infantile nystagmus syndrome improves the visual acuity and nystagmus intensity.     

Prevalence and Risk Factors Associated with Dry Eye Syndrome among Senior High School Students in a County of Shandong Province,China

Purpose: To determine the prevalence and risk factors for dry eye syndrome (DES) among senior high school students in Shouguang, a county of Shandong Province, China.

Methods: A cross-sectional survey was conducted in July 2010, and 1902 senior high school students were selected by multi-stage sampling to answer a questionnaire assessing the prevalence and risk factors for DES. DES was defined as the presence of a previous clinical diagnosis of DES or severe symptoms (both dryness and irritation constantly or often). Risk factors associated with DES were evaluated with a χ² test and logistic regression analyses.

Results: The prevalence of DES was 23.7%. In univariate analysis, myopia (P < 0.0001), contact lens wear (P = 0.020), inadequate refractive correction (P < 0.0001), frequent self-administered topical ophthalmic medications (P < 0.0001), and poor sleep quality (P = 0.007) were significantly associated with DES. In multivariate analysis, inadequate refractive correction (P < 0.0001, odds ratio, OR 1.980; 95% confidence interval, CI 1.577–2.488), frequent self-administered topical ophthalmic medications (P < 0.0001, OR 1.838; 95% CI 1.399–2.410), and poor sleep quality (P = 0.001, OR 1.342; 95% CI 1.054–1.709) were significant risk factors for DES.

Conclusions: Senior high school students are a neglected population with a high prevalence of DES. Preventive measures directed against risks factors for DES among senior high school students may help reduce the prevalence and provide a positive impact on students’ health.     

Juvenile Xanthogranuloma Presenting with Unilateral Prominent Nodule of the Eyelid: Report of a Case and Clinicopathological Findings

Background The purpose of this study is to report and describe the clinical and histopathologic findings in a patient with juvenile xanthogranuloma presenting as a relatively large, solitary, pedunculated mass on the left upper eyelid.Case A 31-month-old Japanese girl presented with a pedunculated nodular lesion that had been developing since 4 months earlier. Otherwise, no abnormal findings were identified in either eye. In addition, no other abnormalities, including skin eruptions or nodules, were detected anywhere. The lesion was completely excised and examined histopathologically.Observations Microscopic study revealed a histiocytic proliferation accompanied by lymphocytes and occasional plasma cells and eosinophils within the dermis. A moderate number of giant cells of the foreign-body and Touton types were present. In the immunohistochemical analysis, the proliferative histiocytes and giant cells showed positive immunoreactivity for vimentin and macrophage markers and negativity for S-100 protein. The diagnosis of juvenile xanthogranuloma was made. The findings of further detailed ophthalmologic and systemic examinations and evaluations were unremarkable. Therefore, the diagnosis was made of juvenile xanthogranuloma, solitary type, occurring on the eyelid.Conclusions Although juvenile xanthogranuloma of the eyelid is uncommon and its occurrence anywhere in the eye is rare, ophthalmologists should be familiar with this entity. Juvenile xanthogranuloma should be included in the differential diagnosis of eyelid mass lesions. Jpn J Ophthalmol 2004;48:435–439 © Japanese Ophthalmological Society 2004     

The Role of Eye Movements During Reading in Patients with Age-Related Macular Degeneration (AMD)

AMD patients often have particular difficulty reading, even when the text is magnified to compensate for reduced visual acuity. This study explores whether reading performance can be explained by eye movement factors. Forty patients with advanced AMD were tested with a high-speed video eye tracker to evaluate fixation stability and saccadic eye movements. Reading speed was measured for standardized texts viewed at the critical print size. Visual acuity and contrast sensitivity were unrelated to reading speed, but fixation stability, proportion of regressive saccades and size of forward saccades were all significantly associated with reading performance, accounting for 74% of the variance. The implications of these findings for low-vision training programmes are discussed.     

噻吗心胺滴眼液治疗准分子激光手术后屈光回退

目的：观察并分析噻吗心胺滴眼液治疗准分子激光手术后屈光回退的疗效,并且初步探讨其作用机制。方法：随机对照研究。选取在乌鲁木齐市眼耳鼻喉专科医院2002 年5 月至2014 年8 月期间行准分子激光手术,术后长期随访中出现不同程度的屈光回退患者51 例（97 眼）。屈光回退度数（-0.95±0.45）D,发生回退时间（3.53±0.72）个月。将这些患者随机分为2 组：噻吗心胺组（48 眼）用0.5%噻吗心胺滴眼液规范治疗,2 次/d;人工泪液组（49 眼）给予人工泪液点眼,3 次/d。治疗时间均为1 个月。所有患者治疗前和治疗后均进行裸眼视力（UCVA）、主觉验光、非接触眼压测量。采用成组t 检验对这2 组患者的各项数据进行比较分析。结果：UCVA（LogMAR）：噻吗心胺组治疗前为0.80±0.25,治疗1 个月后为0.18±0.13,人工泪液组治疗前UCVA为0.78±0.20,治疗1 个月后为0.90±0.85,治疗后2 组差异具有统计学意义（t =6.42,P=0.005）。噻吗心胺组治疗前后眼压下降了1～4 mmHg,平均（1.41±0.39）mmHg,人工泪液组治疗前后眼压下降了-2～1 mmHg,平均（0.52±0.35）mmHg,2 组眼压下降量差异具有统计学意义（t =12.85,P=0.007）。噻吗心胺组治疗前等效球镜度（-0.95±0.42）D,治疗后（-0.13±0.20）D,人工泪液组治疗前等效球镜度（-0.93±0.43）D,治疗后（-0.90±0.42）D,治疗后2 组差异具有统计学意义（t =19.68,P=0.002）。结论：噻吗心胺滴眼液用于治疗准分子激光手术后屈光回退是安全有效的。     

Severe Intraoperative Orbital Venous Congestion during Resection of a Frontal Meningioma Presenting with Post-operative Vision Loss and Ophthalmoplegia: A Case Report

Introduction: Visual loss or papilloedema has been linked to hypocalcaemia for many years. The pathogenesis is unclear, although raised intracranial pressure has often been implicated. Case report: We report a 36-year-old gentleman with hypocalcaemia secondary to pseudohypoparathyroidism presenting as bilateral sequential (five years apart) severe visual loss with normal cerebrospinal fluid pressure. Conclusions: The most likely cause is sequential anterior ischaemic optic neuropathies (AION), perhaps precipitated by hypocalcaemia. Despite the fact that this may be a chance association, we feel it is justified for all patients with AION to undergo a metabolic screen, including serum calcium level, in order to exclude a treatable cause and prevent subsequent AION in the unaffected eye.