Comparative risk of blindness and vision-threatening ocular comorbidities in patients with Behçet's disease versus the general population.

OBJECTIVE: To compare the risk of blindness and vision-threatening ocular comorbidities in patients with Behçet's disease (BD) vs the general population. METHODS: Using 2002-2017 Korea National Health Insurance Service database, we did a population-based cohort study comparing newly diagnosed BD patients and age- and sex-matched non-BD controls at a 1:5 ratio. The primary outcome was blindness, defined as a best-corrected visual acuity of ≤20/500 in the better-seeing eye. Secondary outcomes were vision-threatening ocular comorbidities (cataract, glaucoma and retinal disorders) that require surgical interventions and incident uveitis. Cox proportional hazards models were used to estimate hazard ratios (HRs) and 95% CIs. We performed subgroup analyses by sex and BD diagnosis age. RESULTS: We included 31 228 BD patients and 156 140 controls. During a follow-up of 9.39 years, the incidence rate of blindness per 1000 person-years was 0.24 in BD and 0.02 in controls with an HR of 10.73 (95% CI 7.10, 16.22). The HR for secondary outcomes was 2.06 (95% CI 1.98, 2.15) for cataract surgery, 5.43 (4.57, 6.45) for glaucoma surgery and 2.71 (2.39, 3.07) for retinal surgery. The HR of incident uveitis was 6.19 (95% CI 5.83, 6.58). Males suffered a disproportionately higher risk of blindness than females due to greater severity rather than a lower incidence of uveitis. The risk of uveitis and blindness decreased as BD diagnosis age increased. CONCLUSIONS: In this large population-based cohort study, BD patients compared with the general population have a 10.73-fold risk of blindness in 10 years and also a substantially higher risk of diverse ocular comorbidities that pose potential threats to vision.

Prevalence of human papillomavirus in eyelid carcinoma among Koreans: a clinicopathological study.

BACKGROUND: Human papillomavirus (HPV) has been detected in eyelid sebaceous gland carcinoma (SGC) and squamous cell carcinoma (SCC), and detection rates greatly varied across studies. This study aimed to investigate the presence of HPV in eyelid SGC and SCC among Koreans and its correlation with clinicopathological characteristics. METHODS: Surgically resected eyelid samples diagnosed as SGC or SCC from January 1999 to June 2011 were identified from the pathology database of three referral centres in Korea. Clinicopathological information including origin (skin vs. tarsal conjunctiva) and treatment outcomes were retrospectively reviewed. Immunohistochemistry (IHC) for p16, HPV DNA in situ hybridisation (ISH), and polymerase chain reaction-based DNA microarray were performed in paraffin-embedded tissue sections. RESULTS: Our cohort included 34 SGC and 12 SCC cases with Asian ethnicity. HPV was detected in 4 SGC and 6 SCC by DNA microarray, while 2 SCC (16.7%) showed positivity in ISH. SCC of tarsal conjunctival origin was significantly more common in HPV-positive SCC than in HPV-negative SCC (5 of 6 vs. 0 of 6, P = 0.015, Fisher's exact test). Among samples showing positive staining in p16 IHC, HPV positivity rates were 0.0% (0/19) in SGC and 100% (3/3) in SCC. There was no significant difference in overall and local recurrence rate in eyelid SGC and SCC according to the HPV status (P > 0.99). CONCLUSIONS: HPV was found in a subset of eyelid SGC and SCC among Koreans and might be aetiologically related to SCC of tarsal conjunctival origin. Overexpression of p16 is considered to be inappropriate as an indicator of HPV infection in eyelid SGC. Further investigation is required to elucidate the transmission route and pathogenic roles of HPV.

A conjunctival-sparing surgical technique for lower eyelid cicatricial entropion repair in ocular cicatricial pemphigoid.

Purpose: To present five cases of lower eyelid cicatricial entropion secondary to ocular cicatricial pemphigoid (OCP) successfully repaired with a conjunctival-sparing surgical technique.Methods: The records of one surgeon (SKF) were reviewed to identify patients with lower eyelid cicatricial entropion secondary to OCP who underwent repair with a conjunctival-sparing technique between September 1, 2016 and October 18, 2017. The medical records were reviewed and extracted data included: age, gender, past medical history, current medical and OCP status, clinical examination, details of entropion repair surgery, and outcome.Results: Five patients (three female, two male) were included with ages ranging from 44 to 93 years. All had biopsy proven OCP, which was in remission at the time of surgery, and all were currently receiving immunomodulatory medications. All patients were symptomatic from cicatricial entropion secondary to OCP and underwent successful lower eyelid entropion repair with a conjunctival-sparing technique described herein, involving infraciliary rotation with suture fixation of the orbicularis muscle to the tarsus. Other contributing mechanisms of eyelid malposition including horizontal eyelid laxity and orbicularis oculi override were addressed simultaneously with lateral tarsal plication or orbicularis muscle debulking, resulting in 100% anatomic success and relief of symptoms with no cases of OCP reactivation, and with good durability with an average 13.9 months follow up (range 6.5-22 months).Conclusions: Successful repair of lower eyelid cicatricial entropion in immunomodulated patients with OCP can be achieved without disease reactivation using a surgical technique that spares the conjunctiva and lower eyelid retractors.

Lacrimal drainage system stenosis associated with Trastuzumab emtansine (Kadcyla®, T-DM1) administration: a case report.

BACKGROUND: Trastuzumab emtansine (Kadcyla®, T-DM1) is an antibody-drug conjugate used to treat HER2 (human epidermal growth factor receptor 2) overexpressing metastatic breast cancer. In this report, we present the first case of lacrimal drainage system stenosis identified after T-DM1 administration, and its successful treatment with a topical steroid. CASE PRESENTATION: A 36-year-old female with metastatic breast cancer was referred for excessive tearing of both eyes. She previously underwent mastectomy and was treated with multiple anti-cancer regimens. However, metastases to liver and bone were identified and T-DM1 was administered. After 2 months, epiphora developed in both eyes and the patient was referred for ophthalmologic examination. The height of the tear meniscus was increased. The fluorescein dye disappearance test (FDDT) showed a delayed clearance in both eyes. Diagnostic lacrimal probing demonstrated a lower distal canalicular stenosis in both eyes. Dacryocystography indicated multiple focal narrowing of nasolacrimal duct in the right eye and diffused narrowing of nasolacrimal duct in the left eye. Topical eyedrop of tobramycin 0.3% and dexamethasone 0.1% were prescribed four times a day. After 2 months of treatment, the patient reported relief from epiphora, and the height of tear meniscus was normalized in both eyes. CONCLUSION: T-DM1 administration in breast cancer treatment can induce lacrimal drainage system stenosis, which can be treated effectively with a topical steroid.

Effect of topical steroids on recently developed incomplete nasolacrimal duct obstruction: optical coherence tomography study.

PURPOSE: To evaluate the effect of a topical steroid instillation on recently developed incomplete nasolacrimal duct obstruction (NLDO) evaluated by using anterior segment optical coherence tomography (ASOCT). METHODS: Forty-nine eyes of 31 patients with incomplete NLDO who developed epiphora within 3 months were recruited and treated with a topical steroid for 3 months. Tear meniscus height (TMH) and area (TMA) were measured by ASOCT. The treatment was considered to be effective when there was improvement of subjective epiphora, normal tear meniscus on slit lamp examination, a fluorescein dye disappearance test with grade 0 or 1, and TMH < 250 µm on ASOCT. The treatment effectiveness and associated factors were analyzed. RESULTS: Treatment was effective in 24 eyes (49.0%) after 6 months of follow-up. TMH and TMA at 3 months were significantly decreased in the effective group on ASOCT (TMH: from 385 to 268 µm, p = 0.002, TMA: from 0.0564 to 0.0266 mm2, p = 0.001, Wilcoxon test). In the effective group, the eyes with normal tear meniscus at 1 month were significantly more frequent than those in the ineffective group (p < 0.001, Fisher's exact test). No patient in the effective group required further invasive management during 3 years of follow-up. CONCLUSIONS: Topical steroid instillation has a significant therapeutic effect on recently developed incomplete NLDO. Additional invasive procedures were avoided in half of the subjects.

Longitudinal Changes of Optic Nerve Head and Peripapillary Structure during Childhood Myopia Progression on OCT: Boramae Myopia Cohort Study Report 1.

PURPOSE: To delineate longitudinal changes in the optic nerve head (ONH) and peripapillary structure during myopia progression in childhood using spectral-domain (SD) OCT and to explore the factors associated with myopic ONH and peripapillary changes. DESIGN: Prospective cohort study. PARTICIPANTS: Twenty-three healthy children with myopia (46 eyes). METHODS: The participants underwent fundus photography, SD OCT, and axial length (AXL) measurements every 6 months for 2 years. Based on the morphologic changes of the ONH and ß-zone parapapillary atrophy (PPA), eyes were classified as group A (ONH unchanged without ß-zone PPA; 11 eyes), group B (ONH changed without ß-zone PPA at baseline; 10 eyes), group C (ONH changed with ß-zone PPA at baseline; 15 eyes), and group D (ONH unchanged with ß-zone PPA; 10 eyes). The configuration of the border tissue (BT) at the temporal margin of the ONH was assessed, and the ONH parameters, including Bruch's membrane opening distance (BMOD), border length (BL), and BT angle (BTA), were measured on horizontal SD OCT scans. MAIN OUTCOME MEASURES: Changes in ONH parameters and associated factors. RESULTS: Group B showed the greatest AXL increase per year (group B > group C > group A = group D; P < 0.001). During the follow-up periods, the BT configuration initially was changed from internally oblique to externally oblique (group B) and was stretched, resulting in optic disc ovality and γ-zone PPA development (group C). In group C, BL was increased significantly nasally and BTA was decreased significantly, whereas BMOD remained stable (P < 0.001, P < 0.001, and P = 0.100, respectively). In the multivariate analysis using the generalized linear mixed-effect model, the changes of BL and BTA were associated with axial elongation (P = 0.028 and P = 0.010, respectively). CONCLUSIONS: Development of myopic optic disc and γ-zone PPA during myopia progression was delineated using SD OCT images. During the ONH and peripapillary changes, the BL was increased nasally and the BTA was decreased, whereas the BMOD remained relatively stable. The association of axial elongation with ONH and peripapillary tissue changes may facilitate understanding of the relationship between myopia and glaucoma.

Positional Change of Optic Nerve Head Vasculature during Axial Elongation as Evidence of Lamina Cribrosa Shifting: Boramae Myopia Cohort Study Report 2.

PURPOSE: To investigate the positional change of central retinal vasculature and vascular trunk to deduce the change in the lamina cribrosa (LC) during axial elongation. DESIGN: Prospective cohort study. PARTICIPANTS: Twenty-three healthy myopic children (46 eyes). METHODS: Participants had undergone a full ophthalmologic examination and axial length measurement every 6 months for 2 years. Using spectral-domain OCT, circle scans centered around the optic disc in the glaucoma progression analysis mode, which enabled capturing of the same positions throughout the entire study period, and enhanced depth imaging of the deep optic nerve head complex were performed. Infrared imaging of the circle scans was used to measure the changes in the angles between the first and final visits. The angle between the major superior and inferior retinal arteries was measured along the circle scan twice: from the center of the circle scan and from the central retinal vascular trunk, respectively. The positional change of the retinal vascular trunk also was measured. MAIN OUTCOME MEASURES: Change in vascular angle and position of vascular trunk with axial elongation and associated factors. RESULTS: The vascular angle measured from the center of the circle scan did not change (P = 0.247), whereas the angle measured from the central retinal arterial trunk decreased with axial elongation (P < 0.001). A generalized estimating equation analysis revealed that the factors associated with angle decrease were axial elongation (P = 0.004) and vascular trunk dragging (P < 0.001). The extent of vascular trunk dragging was associated with axial elongation (P < 0.001) and increased border length with marginal significance (P = 0.053), but the extent of dragging could not be explained fully by their combination. The major directionality of dragging was mostly to the nasal side of the optic disc, with large variations among participants. CONCLUSIONS: During axial elongation, the retinal vasculature at the posterior pole was unchanged, whereas the position of the central vascular trunk was dragged nasally. Because the central retinal vascular trunk is embedded in the LC, its dragging indicates nasal shifting of the LC, which could explain the vulnerability of myopic eyes to glaucomatous optic neuropathy.

Case Series: Herpes Zoster Ophthalmicus with Acute Orbital Inflammation.

SIGNIFICANCE: Herpes zoster ophthalmicus (HZO) has variable initial manifestations, and acute orbital inflammation may be the first sign without apparent zoster rash. This case series is significant for presenting diverse clinical features and treatment options of HZO with acute orbital inflammation. PURPOSE: To report a case series of patients diagnosed as HZO with acute orbital inflammation including two cases with unique presentations. CASE REPORTS: Medical records of four patients of HZO with acute orbital inflammation were reviewed. Two men and two women with a median age of 57 years (range, 32 to 69 years) were diagnosed as having HZO with acute orbital inflammation. Initial presentations included two cases of zoster rash and two cases of orbital pain preceding vesicles. Clinical orbital findings included proptosis, ptosis, ophthalmoplegia, and decreased visual acuity. Orbital magnetic resonance image showed enlarged extraocular muscle with enhancement and optic nerve sheath enhancement in all four patients, and unilateral dacryoadenitis in one patient. All four patients were administered with systemic steroid, three patients received intravenous acyclovir, and one patient received oral acyclovir. Orbital signs improved in all patients over several months. CONCLUSIONS: Herpes zoster ophthalmicus may initially present with orbital inflammatory signs, such as acute orbital myositis, perioptic neuritis, or dacryoadenitis, without zoster rash. Physicians should be aware of acute orbital inflammation as a presenting sign of HZO.

Effects on Periocular Tissues after Proton Beam Radiation Therapy for Intraocular Tumors.

BACKGROUND: To present our experience on orbital and periorbital tissue changes after proton beam radiation therapy (PBRT) in patients with intraocular tumors, apart from treatment outcomes and disease control. METHODS: Medical records of 6 patients with intraocular tumors who had been treated with PBRT and referred to oculoplasty clinics of two medical centers (Seoul National University Hospital and Seoul Metropolitan Government-Seoul National University Boramae Medical Center) from October 2007 to September 2014 were retrospectively reviewed. The types of adverse effects associated with PBRT, their management, and progression were analyzed. In anophthalmic patients who eventually underwent enucleation after PBRT due to disease progression, orbital volume (OV) was assessed from magnetic resonance (MR) images using the Pinnacle3 program. RESULTS: Among the six patients with PBRT history, three had uveal melanoma, and three children had retinoblastoma. Two eyes were treated with PBRT only, while the other four eyes ultimately underwent enucleation. Two eyes with PBRT only suffered from radiation dermatitis and intractable epiphora due to canaliculitis or punctal obstruction. All four anophthalmic patients showed severe enophthalmic features with periorbital hollowness. OV analysis showed that the difference between both orbits was less than 0.1 cm before enucleation, but increased to more than 2 cm³ after enucleation. CONCLUSION: PBRT for intraocular tumors can induce various orbital and periorbital tissue changes. More specifically, when enucleation is performed after PBRT due to disease progression, significant enophthalmos and OV decrease can develop and can cause poor facial cosmesis as treatment sequelae.

Conjunctival granuloma with necrosis associated with exposed suture in upper double lid masquerading as ocular surface squamous neoplasia: a case report.

BACKGROUND: This study reports two cases of conjunctival granuloma with necrosis caused by an exposed suture in the upper palpebral conjunctiva masquerading as ocular surface squamous neoplasia. CASE PRESENTATION: Two patients presented with chronic conjunctival ulcerative and granulomatous lesions on the superior bulbar conjunctiva that repeatedly recurred after the mass was removed. The pathologic findings revealed the absence of malignant cells and presence of many lymphocytes, plasma cells, and histiocytes. There was no evidence of acid-fast bacilli or fungal organisms. When a past history of blepharoplasty was established, microscopic examination revealed occult exposed suture tips. After the sutures were removed, the granuloma with necrosis was resolved within a month. CONCLUSION: For all conjunctival lesions in the superior bulbar conjunctiva, a thorough examination of the ocular adnexae which includes eyelid eversion should be performed. There should be a suspicion of foreign body or exposed suture material especially when there is a non-healing ulcer.

Inflammatory pseudotumor of eyelid: a probable IgG4-related sclerosing disease clinically mimicking eyelid pilomatrixoma.

BACKGROUND: Ocular adnexal IgG4-related sclerosing disease (IgG4-SD) has been categorized as a novel disease entity. It is characterized by stromal sclerosis and an infiltration of mass-forming lymphoplasmic cells containing many IgG4-positive plasma cells. Although ocular adnexal tissue involvement has been increasingly reported, a focal nodular sub-brow mass is not typical in an IgG4-SD presentation. We report a rare case of probable ocular adnexal IgG4-SD that clinically mimicked eyelid pilomatrixoma. CASE PRESENTATION: A 42-year-old woman presented with a nodular mass in her left sub-brow area. The initial clinical impression of her lesion was eyelid pilomatrixoma. However, the final pathologic diagnosis was IgG4-SD, but extranodal marginal zone B-cell lymphoma could not be excluded. The patient underwent testing to determine tumor malignancy and systemic IgG4-SD involvement. Laboratory testing showed normal IgG and IgG4 serum levels and imaging revealed no remarkable findings. Oral prednisolone was administered and slowly tapered to manage the possible remnant lesion and to prevent disease recurrence. Two years after initiating therapy, there was no evidence of relapse. The patient is under close surveillance for signs of recurrence, systemic involvement, and potential malignant transformation. CONCLUSIONS: We found an unusual case of probable ocular adnexal IgG4-SD, which presented as a unilateral restricted mass involving the sub-brow area. Although the mass was surgically removed, systemic steroid treatment and long-term surveillance were initiated due to the possibility of recurrence, the potential association with systemic disease, and the potential development of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma.

Genome-wide DNA methylation profiles according to Chlamydophila psittaci infection and the response to doxycycline treatment in ocular adnexal lymphoma.

PURPOSE: To compare genome-wide DNA methylation profiles according to Chlamydophila psittaci (Cp) infection status and the response to doxycycline treatment in Korean patients with ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL). METHODS: Twelve ocular adnexal EMZL cases were classified into two groups (six Cp-positive cases and six Cp-negative cases). Among the 12 cases, eight were treated with doxycycline as first-line therapy, and they were divided into two groups according to their response to the treatment (four doxy-responders and four doxy-nonresponders). The differences in the DNA methylation states of 27,578 methylation sites in 14,000 genes were evaluated using Illumina bead assay technology. We also validated the top-ranking differentially methylated genes (DMGs) with bisulfite direct sequencing or pyrosequencing. RESULTS: The Infinium methylation chip assay revealed 180 DMGs in the Cp-positive group (74 hypermethylated genes and 106 hypomethylated genes) compared to the Cp-negative group. Among the 180 DMGs, DUSP22, which had two significantly hypomethylated loci, was validated, and the correlation was significant for one CpG site (Spearman coefficient=0.6478, p=0.0262). Regarding the response to doxycycline treatment, a total of 778 DMGs were revealed (389 hypermethylated genes and 336 hypomethylated genes in the doxy-responder group). In a subsequent replication study for representative hypomethylated (IRAK1) and hypermethylated (CXCL6) genes, the correlation between the bead chip analysis and pyrosequencing was significant (Spearman coefficient=0.8961 and 0.7619, respectively, p<0.05). CONCLUSIONS: Ocular adnexal EMZL showed distinct methylation patterns according to Cp infection and the response to doxycycline treatment in this genome-wide methylation study. Among the candidate genes, DUSP22 has a methylation status that was likely attributable to Cp infection. Our data also suggest that the methylation statuses of IRAK1 and CXCL6 may reflect the response to doxycycline treatment.

Expression of cell cycle regulatory proteins in eyelid sebaceous gland carcinoma: low p27 expression predicts poor prognosis.

Prognosis of eyelid sebaceous gland carcinoma is largely unpredictable and there are few practically available markers for predicting patients' prognosis. Dysregulation of cell cycle progression is strongly associated with the development of cancer and the cancer prognosis. We investigated the expression of cell cycle regulatory proteins in eyelid sebaceous gland carcinoma and estimate their value as prognostic predictors. Forty-three cases of eyelid sebaceous gland carcinoma were included in this study. Immunohistochemistry for the p53, p21, p27, cyclin E, p16, cyclin D1, and phosphorylated Rb (pRb) proteins was performed using archival paraffin blocks. Correlations between clinical features and protein expression were evaluated statistically. Nine patients showed lymph node or distant metastasis, and the remaining patients showed localized disease. High expression of p21, p27, cyclin E, and p16 was found in the majority of tumor cell nuclei, whereas these proteins were rarely expressed in the normal sebaceous glands. However, pRb was focally lost in a subset of cases. Patients showing diffuse p27 expression developed metastasis less commonly than those with negative or focal p27 expression (log-rank test, p = 0.008). Aberrant expression of cell cycle regulatory proteins was observed in eyelid sebaceous gland carcinoma, suggesting that cell cycle dysregulation is involved in the pathogenesis of this tumor. Decreased p27 expression is a predictive biomarker of an unfavorable prognosis of eyelid sebaceous gland carcinoma.

Squinting and photophobia in intermittent exotropia.

PURPOSE: To report factors associated with preoperative squinting, defined as transient eye closure in bright light, and photophobia and the factors affecting improvement of these symptoms postoperatively in intermittent exotropia. METHODS: In this retrospective study, patients (N = 99) were divided into groups according to the presence (n = 54) or absence (n = 45) of preoperative squinting and the presence (n = 64) or absence (n = 35) of photophobia. Clinical characteristics, including overaction or underaction of the oblique muscle and fundus intorsion and extorsion, were compared between the two groups. The squinting and photophobia groups were further categorized into two subgroups each according to postoperative improvement. The extended list of characteristics, including the duration from onset to surgery, postoperative angle of deviation, and fusion, was compared between the two subgroups. RESULTS: Preoperatively, 54 (54.5%) and 64 (64.6%) patients had squinting and photophobia, respectively. The coincidence of squinting and photophobia was marginally significant (p = 0.05). Postoperatively, squinting and photophobia disappeared in 64.8 and 59.4% of the patients, respectively. The photophobia group had a younger onset age of strabismus than the nonphotophobia group (39.3 vs. 56.4 months; p = 0.03). Good fusional status at the near range was more common in the nonsquinting group than in the squinting group (74.3 vs. 47.6%; p = 0.02). Superior oblique overaction was significantly more common in the squinting group than in the nonsquinting group (11.1 vs. 0%; p = 0.03). Early surgical correction and successful outcomes were associated with squinting improvement (p = 0.001 and p = 0.02, respectively). CONCLUSIONS: More than 50% of patients with intermittent exotropia had squinting or photophobia, and approximately 60% of symptomatic patients experienced improvement postoperatively. The onset of strabismus, near fusion, superior oblique overaction, and fundus intorsion were related to these symptoms. Early surgery and successful eye position realignment were beneficial for improving squinting postoperatively.

Clinicopathological features of inflammatory lesions of the lateral canthal subconjunctival area.

PURPOSE: To investigate the clinical and histopathological features of inflammatory lesions of the lateral canthal subconjunctival area. METHODS: This is a retrospective case series of 12 patients with inflammatory subconjunctival masses in the lateral canthal area. All patients included in this study were treated at Seoul National University Hospital or Seoul National University Bundang Hospital between 2006 and 2012. Clinical data were obtained from the medical records. Histopathologic findings were thoroughly reviewed. RESULTS: There was a woman predominance in the study group (10:2), and the median age at presentation was 39 years (range 33-70). Common symptoms included conjunctival injection, sticky discharge, and pain or discomfort. Histopathologically, all lesions originated from ductules of the lacrimal gland. Two cases showed cysts containing clear fluid with mild inflammation. One case showed lacrimal ductulitis without cyst formation. Nine cases showed lacrimal ductal cysts with varying periductal inflammation, and the contents were pinkish, amorphous materials in 7 cases. Embedded cilia were found in 8 cases. CONCLUSIONS: Inflammatory lesions of the lateral canthal subconjunctival area all originated from lacrimal gland ductules, showing a variable histopathologic spectrum of inflammation and cyst formation. Cilia impaction was a very frequently observed finding.

Correction of lower eyelid marginal entropion by eyelid margin splitting and anterior lamellar repositioning.

PURPOSE: There is a paucity of data in the literature on the surgical management of lower eyelid marginal entropion. In this study, the authors report outcomes of a surgical technique of eyelid margin splitting and anterior lamellar reposition in patients with lower eyelid marginal entropion. METHODS: The medical records for 30 eyelids from 22 patients with lower eyelid marginal entropion who had undergone eyelid margin splitting and anterior lamellar repositioning at Seoul National University Hospital from January 2004 to December 2012 were retrospectively reviewed. Success was defined as the lack of any lash in contact with the globe, no need for a second procedure, the complete resolution of symptoms, and acceptable cosmesis at the final follow up. RESULTS: The mean follow-up duration was 16.7 months. The split eyelid margin exhibited good wound healing in each case, but trichiasis recurred postoperatively in 3 of 30 eyelids, even though the eyelid margin was ultimately well positioned. That is, the overall success rate was 90%. Of the 3 eyelids with recurrent trichiasis, 2 required additional electrolysis to remove irritated cilia, but the other did not require to be treated. In the latter case, the trichiasis observed was fine, focal, and unaccompanied by symptoms or corneal lesions. There was no significant complication such as secondary ectropion or eyelid retraction. CONCLUSIONS: Eyelid margin splitting and anterior lamellar repositioning achieve success with a low rate of complications among patients with lower eyelid marginal entropion.

A Preliminary Evaluation of the Diagnostic Performance of a Smartphone-Based Machine Learning-Assisted System for Evaluation of Clinical Activity Score in Digital Images of Thyroid-Associated Orbitopathy.

Background: We previously developed a machine learning (ML)-assisted system for predicting the clinical activity score (CAS) in thyroid-associated orbitopathy (TAO) using digital facial images taken by a digital single-lens reflex camera in a studio setting. In this study, we aimed to apply this system to smartphones and detect active TAO (CAS ≥3) using facial images captured by smartphone cameras. We evaluated the performance of our system on various smartphone models and compared it with the performance of ophthalmologists with varying clinical experience. Methods: We applied the preexisting ML architecture to classify photos taken with smartphones (Galaxy S21 Ultra, iPhone 12 pro, iPhone 11, iPhone SE 2020, Galaxy M20, and Galaxy A21S). The performance was evaluated with smartphone-captured images from 100 patients with TAO. Three ophthalmology residents, three general ophthalmologists with <5 years of clinical experience, and three oculoplastic specialists independently interpreted the same set of images taken under a studio environment and compared their results with those generated by the smartphone-based ML-assisted system. Reference CAS was determined by a consensus of three oculoplastic specialists. Results: Active TAO (CAS ≥3) was identified in 28 patients. Smartphone model used in capturing facial images influenced active TAO detection performance (F1 score 0.59-0.72). The smartphone-based system showed 74.5% sensitivity, 84.8% specificity, and F1 score 0.70 on top three smartphones. On images from all six smartphones, average sensitivity, specificity, and F1 score were 71.4%, 81.6%, and 0.66, respectively. Ophthalmology residents' values were 69.1%, 55.1%, and 0.46. General ophthalmologists' values were 61.9%, 79.6%, and 0.55. Oculoplastic specialists' values were 73.8%, 90.7%, and 0.75. This smartphone-based ML-assisted system predicted CAS within 1 point of reference CAS in 90.7% using facial images from smartphones. Conclusions: Our smartphone-based ML-assisted system shows reasonable accuracy in detecting active TAO, comparable with oculoplastic specialists and outperforming residents and general ophthalmologists. It may enable reliable self-monitoring for disease activity, but confirmatory research is needed for clinical application.

Ophthalmic manifestations and visual outcomes of granulomatosis with polyangiitis: a retrospective multicentre study in Korea.

OBJECTIVES: To analyse the ophthalmic manifestations and treatment outcomes of Korean patients with granulomatosis with polyangiitis (GPA). METHODS: One hundred twenty patients diagnosed with GPA by rheumatologists from January 1984 to March 2019 at three referral centres were retrospectively reviewed. Patients with ophthalmic symptoms were examined by ophthalmologists and underwent orbital imaging. Ophthalmic manifestations were divided into ocular involvement and ocular adnexal involvement. Multivariable logistic regression was used to examine the factors related to ocular, ocular adnexal, and optic nerve involvement. Visual improvement was defined as a best-corrected visual acuity gain of ≥2 Snellen lines, accompanied by improvements in optic nerve function. RESULTS: Ophthalmic manifestations were observed in 50 patients (41.7%) during the median follow-up period of 6.7 years. Proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) positivity (odds ratio 3.19, 95% confidence interval 1.18-8.60) was an independent risk factor for ocular involvement, while sinonasal involvement (21.94, 2.54-189.69) and brain involvement (5.38, 1.50-19.31) were independent risk factors for ocular adnexal involvement. Antinuclear antibody (ANA) positivity was associated with optic nerve involvement (12.8, 1.80-90.5). Visual improvement occurred in 5 of 14 patients with optic nerve involvement, all of whom received intravenous (IV) immunosuppressive treatments beyond oral steroids within 2 months of visual impairment. CONCLUSIONS: Ophthalmic involvement is common in Korean GPA patients and should be considered in the presence of PR3-ANCA, sinonasal or brain involvement. Patients with positive ANA have an increased risk of optic nerve involvement, and early IV immunosuppressive treatments beyond oral steroids are necessary to improve the visual outcome.

Change of peripapillary retinal nerve fiber layer and choroidal thickness during 4-year myopic progress: Boramae Myopia Cohort Study Report 4.

AIMS: To investigate the longitudinal changes of peripapillary retinal nerve fibre layer (RNFL) and choroidal thickness during myopic axial elongation. METHODS: Peripapillary RNFL and choroidal thickness were prospectively evaluated by spectral-domain optical coherence tomography (SD-OCT) in 46 eyes of 23 myopic children over the course of 4 years. Using serial OCT images acquired based on a fixed scan circle in the glaucoma progression analysis mode, general and sectoral RNFL thicknesses were acquired at the same position and the angular location of the peak was measured. The peripapillary choroidal thickness likewise was measured at eight positions in serial OCT images. RESULTS: The mean age at the baseline was 9.6±1.7 years. The mean axial length increased from 24.80±1.28 mm to 25.64±1.35 mm. The global peripapillary RNFL thickness was 98.54±12.06 µm at baseline. The global and sectoral RNFL thicknesses did not change during the 4 years. The angular location of RNFL peaks was also stable and was located in the superotemporal (64.18±10.85°) and inferotemporal (293.98±11.62°) sectors. The global peripapillary choroidal thickness was 145.40±28.67 µm at the baseline. The global and sectoral choroidal thicknesses did not change during the 4 years. CONCLUSIONS: The peripapillary RNFL and choroidal thicknesses as well as the locations of the RNFL peaks had been preserved, during the 4-year follow-up on myopic children, when traced and measured from the same location.

Serum microRNA as a potential biomarker for the activity of thyroid eye disease.

The aim of this study is to characterize the microRNA (miRNA) expression signatures in patients with thyroid eye disease (TED) and identify miRNA biomarkers of disease activity. Total RNA was isolated from the sera of patients with TED (n = 10) and healthy controls (HCs, n = 5) using the miRNeasy Serum/Plasma Kit. The NanoString assay was used for the comprehensive analysis of 798 miRNA expression profiles. Analysis of specific miRNA signatures, mRNA target pathway analysis, and network analysis were performed. Patients with TED were divided into two groups according to disease activity: active and inactive TED groups. Differentially expressed circulating miRNAs were identified and tested using quantitative reverse transcription-polymerase chain reaction (qRT-PCR) tests in the validation cohort. Among the 798 miRNAs analyzed, 173 differentially downregulated miRNAs were identified in TED patients compared to those in the HCs. Ten circulating miRNAs were differentially expressed between the active and inactive TED groups and regarded as candidate biomarkers for TED activity (one upregulated miRNA: miR-29c-3p; nine downregulated miRNAs: miR-4286, miR-941, miR-571, miR-129-2-3p, miR-484, miR-192-5p, miR-502-3p, miR-597-5p, and miR-296-3p). In the validation cohort, miR-484 and miR-192-5p showed significantly lower expression in the active TED group than in the inactive TED group. In conclusion, the expression levels of miR-484 and miR-192-5p differed significantly between the active and inactive TED groups, suggesting that these miRNAs could serve as circulating biomarkers of TED activity, however, these findings need to be validated in further studies.