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The study aims to describe the clinical and histopathologic features of different types of dacryops and their clinical diagnostic challenges.This is a retrospective cohort study of all surgically excised cases of dacryops in 2 tertiary eye hospitals in Riyadh, Saudi Arabia.The study included 58 dacryops specimens from 55 patients with an average age of 41.2 years (range, 4-78 years). The most common location was the upper lid (60.3%), whereas the least expected location was the caruncle (6.9%). The most common site of dacryops occurrence was in the accessory lacrimal gland (55.2%), the main lacrimal gland (32.8%), and then ectopic dacryops (12%). All patients presented with lid swelling alone except for 3 patients who experienced secondary mechanical ptosis. On physical examination, conjunctival scarring existed in 4 patients (6.9%). Preoperative diagnosis of dacryops was accurate in 44.8% of the cases. Dacryops of the main lacrimal gland was accurately diagnosed clinically in all cases compared with other locations, which was statistically significant (p0.001). The causes of inaccurate clinical diagnoses were hidrocystoma (26.9%), inclusion cyst (11.5%), and dermoid cyst (7.7%), whereas the remaining cases were diagnosed as cysts without a specific subtype (53.9%). Recurrence of the lesion was observed in 2 cases (3.5%). No clinical or histopathologic factors were associated with a risk of recurrence.Dacryops can represent a diagnostic challenge to ophthalmologists. Familiarity with clinical presentations and findings is required to diagnose dacryops outside the main lacrimal gland.

Objective To correlate the histopathologic results of conjunctival specimens with clinical findings in patients with conjunctivochalasis and report the results of the paste-pinch-cut technique for management. Design Retrospective chart review. Methods SETTING: Single tertiary ophthalmological centre (Ocular Surface Diseases and Dry Eye Clinic, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Md.). Methods Twenty-five patients (32 eyes) with clinically significant conjunctivochalasis. All patients were referred for clinically significant dry eye without previous diagnosis of chalasis. Sixteen patients had an underlying inflammatory systemic condition. Intervention or Observation Procedure(s): Patients underwent surgery with paste-pinch-cut technique. Subjective dry eye symptoms and ocular surface staining scores (corneal and conjunctival staining using fluorescein and lissamine green respectively) were assessed at every visit. Main outcome measures Change in patient symptoms and ocular surface staining scores and histopathologic findings in conjunctival specimens. Results After surgery, significant improvement was achieved in dry-eye symptoms as well as both corneal and conjunctival staining scores in 29 eyes on reduced topical therapy. Only 3 eyes had persisting conjunctival lissamine staining. Light microscopic examination disclosed mild to moderate lymphoplasmocytic inflammation of the conjunctivae with areas of epithelial goblet cell loss, squamous metaplasia, stromal edema, and fibrosis. Conclusion Conjunctivochalasis appears to be associated with significant inflammation in the setting of dry eye and underlying inflammatory systemic conditions. Although topical anti-inflammatory treatment could be attempted in the initial management, surgical excision should be considered in the absence of clinical response.

Pyogenic granuloma is a common benign, vascular lesion of the skin and mucosa. Our case was a 34-year-old woman at 28 weeks of gestation. She presented with a complaint of a growing and occasionally bleeding mass on the left lower eyelid conjunctiva for approximately 3 months. On examination, a red, pedunculated fragile lesion on the medial lower eyelid conjunctiva was observed. Ophthalmologic examination revealed that visual acuity was 20/20 in both eyes. Anterior and posterior segment examination was normal bilaterally. The lesion was excised completely under local anesthesia and sent for pathological examination. Histopathological examination revealed multiple vascular structures with small-diameters in the fibrous stroma. Vessels showed strong CD31 expression in immunohistochemical staining. Kaposi sarcoma was excluded due to negative HHV-8 staining. These findings were diagnostic of pyogenic granuloma. The frequency of pyogenic granuloma increases during pregnancy and surgical excision is important in diagnosis and treatment of these patients. This is the first reported case of conjunctival pyogenic granuloma during pregnancy. RESUMO O granuloma piogênico é uma lesão vascular benígna comum da pele e da mucosa. Neste caso, uma mulher de 34 anos com 28 semanas de gestação, apresentou queixa de massa em crescimento e sangramento ocasional na conjuntiva inferior da pálpebra esquerda por cerca de 3 meses. No exame, detectou-se uma lesão frágil, pedunculada vermelha na conjuntiva na porção medial da pálpebra inferior No exame oftalmológico, a acuidade visual foi 20/20 em ambos os olhos. O exame dos segmentos anterior e posterior foi normal bilateralmente. A lesão foi excisada totalmente com anestesia local e enviada para exame patológico. No exame histopatológico, múltiplas estruturas vasculares de pequeno diâmetro foram observadas no estroma fibroso. Os vasos apresentaram forte expressão de CD31 na coloração imuno-histoquímica. O sarcoma de Kaposi foi excluído devido à negatividade da coloração do HHV-8. Esses achados foram diagnósticos para granuloma piogênico. A frequência de granuloma piogênico aumenta durante a gestação e a excisão cirúrgica é importante no diagnóstico e tratamento desses pacientes. Este é o primeiro caso relatado de granuloma piogênico conjuntival ocorrido durante a gestação.

Purpose To assess the prevalence of ophthalmic findings in patients with Darier disease, an autosomal dominant genetic skin disorder, in an effort to evaluate the need for eye examinations in the management of the disease. Design Prospective observational case series. Methods Thirty-six individuals with Darier disease were evaluated by both ocular assessment questionnaire and a comprehensive ophthalmic examination (visual acuity, refraction, external examination, and slit-lamp examination) with emphasis on the eyelids, conjunctiva, and cornea. In addition, questionnaire-based medical interview and skin examination were conducted. Results According to the medical questionnaire, 39% of patients reported eye problems, 36% dry eye, and 42% eye fatigue after prolonged reading. Ocular examination revealed Darier disease lesions on the eyelids in 55% of the patients, blepharitis in 44%, conjunctival hyperemia in 28%, and short tear film break-up time in 83%. There was no significant relationship between any of these ophthalmic findings and systemic retinoid therapy, sex, or age. Conclusions The high prevalence of blepharitis and dry eye highlights the importance of ophthalmologic evaluation of patients with Darier disease.

A previously healthy 69-year-old man presented to the emergency department with right orbital pain and blurred vision for 1 day. He denied fever or recent trauma history. On examination, he had subconjunctival haemorrhage, chemosis and proptosis (figure 1). His neurologic examination revealed right-sided ophthalmoplegia. His vital signs were stable, and the rest of the physical examination and laboratory study were unremarkable. Figure 1 Photograph of the patient showing chemosis and proptosis of his right eye. What is the most likely diagnosis for this patient? 1. Orbital cellulitis. 2. Orbital …

Purpose: In this study, we wanted to retrospectively evaluate the effect of the use of topical brimonidine on intraoperative bleeding and surgical hemostasis before strabismus surgery. Methods: Bri...

Purpose The aim of this work was to study the clinical presentation, management and outcomes of orbital and adnexal amyloidosis. Methods This retrospective analysis included all the patients diagnosed with orbital and adnexal amyloidosis between January 1990 and December 2019. Positive staining with Congo Red and apple-green birefringence on polarized light microscopy established the diagnosis. Data analyzed included demographic profile, varied presentations, management, and outcome. Results Thirty-three eyes of 26 patients were included. The male:female ratio was 1:1. The mean age of the study population was 42.6 ± 16 years. The median duration of symptoms was two years. Unilateral involvement was seen in 19 eyes (right = 11, left = 8). The most common presenting feature was acquired ptosis. Eyelid was the most commonly affected site followed by orbit and conjunctiva. Two patients had systemic involvement in the form of multiple myeloma and lymphoplasmacytic lymphoma. Complete excision was done in seven (26.9%) cases while 19 (73.1%) cases underwent debulking. Three patients underwent ptosis surgery. The median duration of follow-up was 1.5 years. Three cases had recurrence and underwent repeat surgery. Conclusion Orbit and adnexa is a rare site for amyloidosis. It is usually localized; however it can occur as a part of systemic amyloidosis. Eyelid is the most common site of involvement and patients usually present as eyelid mass or ptosis. Complete excision is difficult and most of the patients usually undergo debulking surgery. All patients should undergo screening for systemic amyloidosis.

Analysis of lacrimal glands in eyes with severe dryness following Stevens-Johnson syndrome (SJS) showed normal acinar morphology, secretory activity and myoepithelial cells; indicating that ocular dryness in SJS is not because of primary glandular destruction.

SIGNIFICANCE In this study, assessments of conjunctival redness were performed to evaluate whether patients with or without dry eye disease (DED) could be discriminated based on this measure. Our findings suggest that subjectively grading redness by quadrant, as opposed to automated en face measurements, may be more suitable for this purpose. PURPOSE This study aimed to quantify bulbar redness using the validated bulbar redness (VBR) grading scale and an automated objective method (Oculus Keratograph 5M; K5M) in participants with DED and non-DED controls. METHODS Participants with DED (Ocular Surface Disease Index score ≥20 and Oxford scale corneal staining ≥2) and controls (Ocular Surface Disease Index score ≤10 and corneal staining ≤1) attended two study visits. In part 1A of visit 1, baseline bulbar redness was graded with the VBR scale in each conjunctival quadrant of both eyes, followed by automated measurements of temporal and nasal redness with the K5M. This was immediately followed by part 1B, during which a topical vasoconstrictor was instilled into both eyes. Redness assessments were repeated 5 and 30 minutes after instillation with both instruments. Participants returned 14 days later for visit 2, where the same assessments as for visit 1A were repeated. RESULTS Seventy-four participants (50 DED and 24 controls) completed the study. There were statistically significant differences in redness between the DED and control groups when assessed with the VBR scale (14/16 comparisons; all, P < .05), whereas no significant differences in K5M-derived redness between the DED and non-DED groups were found at any location or time point. Both subjective and objective instruments detected statistically significant reductions in redness 5 and 30 minutes after instillation of the vasoconstrictor (all, P < .01). CONCLUSIONS Although both subjective and objective instruments were sensitive to detecting changes in redness induced by vasoconstriction, statistically significant differences in redness between DED and control groups were only found using the VBR scale.

Background Because atopic dermatitis does not heal completely, associated severe atopic keratoconjunctivitis (AKC) and vernal keratoconjunctivitis (VKC) often require long-term treatment. This study aims to evaluate the long-term outcomes of using 0.1% tacrolimus eye drops to treat these severe allergic conjunctival diseases. Methods Two-hundred-and-seventy eyes of 135 patients diagnosed with AKC or VKC from April 2004 to April 2014 were screened retrospectively. Patient demographics and objective signs were extracted from the electronic medical records. The severity of 10 objective signs, related to the palpebral and bulbar conjunctiva, limbus, and cornea, and intraocular pressure (IOP) were observed at baseline, at 2 weeks, 1, 2, 3, 6, and 12 months after starting treatment, and every 1 year thereafter (average use period: 8.4 ± 2.9 years). Safety was evaluated based on the incidence and severity of adverse events. Results 12 patients (AKC; 7 cases, VKC; 5 cases) who were treated with 0.1% tacrolimus eye drops were enrolled in this study. The total score of clinical signs significantly decreased after 2 weeks and remained effective thereafter. Tacrolimus eye drops elicited a statistically significant difference in the mean total clinical scores and IOP over the course of treatment (P Conclusions Topical tacrolimus may provide effective and long-term improvement in clinical signs of severe AKC and VKC cases that refractory to standard conventional treatment. Trial registration: University Hospital Medical Information Network (UMIN) 000034460.

Purpose To evaluate morphological alterations of meibomian glands (MGs) in the dry anophthalmic socket syndrome (DASS). Methods Fifteen unilateral anophthalmic patients wearing cryolite glass prosthetic eyes were enrolled. All patients with clinical blepharitis or other significant eyelid abnormalities were excluded. In vivo laser scanning confocal microscopy (LSCM) of the MGs in the lower eyelids both on the anophthalmic side and the healthy fellow eye was performed to quantify acinar unit density, acinar unit diameter, acinar unit area, meibum secretion reflectivity, the inhomogeneous appearance of the glandular interstice, and inhomogeneous appearance of the acinar walls. Results The lower eyelids of the anophthalmic sockets revealed a significant reduction of the acinar unit density (p = 0.003) as well as a significantly more inhomogeneous appearance of the periglandular interstices (p = 0.018) and the acinar unit walls (p = 0.015) than the healthy fellow eyelid. However, there were no significant differences regarding the acinar unit diameter, acinar unit area, and meibum secretion reflectivity of the MGs on the anophthalmic side compared to the healthy fellow eyelid (p ≥ 0.05, respectively). Conclusions The eyelids of anophthalmic sockets without clinical blepharitis demonstrate a reduced density of MG acinar units and a more inhomogeneous appearance of the periglandular interstices and the acinar unit walls. This can cause meibomian gland dysfunction contributing to DASS and suggests early treatment of these symptomatic patients, even in the clinical absence of any blepharitis signs.

Ocular surface diseases (OSD) can cause serious visual deterioration and discomfort. Commercial artificial tear solution containing hyaluronic acid (HA) show excellent biocompatibility and unique viscoelastic characteristics. Here, we developed a novel HA membrane (HAM) by chemical crosslinking using 1,4-butanediol diglycidyl ether for the effective treatment of OSDs. The main purpose of HAMs is to provide sustained release of HA to modulate the wound healing response in OSDs. The safety and efficacy of HAMs were investigated using primary cultured human corneal epithelial cells and various OSD rabbit models. In the dry state, the HAM is firm, transparent, and easy to manipulate. When hydrated, it swells rapidly with high water retention and over 90% transmission of visible light. Human corneal epithelial cells and rabbit eyes showed no toxic response to HAM. Addition of HAMs to the culture medium enhanced human corneal epithelial cell viability and expression of cell proliferation markers. Investigation of HAM wound healing efficacy using mechanical or chemical corneal trauma and conjunctival surgery in rabbits revealed that application of HAMs to the ocular surface enhanced healing of corneal epithelium and reduced corneal limbal vascularization, opacity and conjunctival fibrosis. The therapeutic potential of HAMs in various OSDs was successfully demonstrated.

Objectives To determine the presenting features of ocular surface disease in patients with atopic dermatitis (AD) treated with dupilumab at a tertiary, university hospital. To establish the need for treatment of dupilumab-associated ocular surface disease and report any long-term effects on the ocular surface. Methods A retrospective analysis of consecutive patients treated with dupilumab for AD between January 2017 and August 2019 was undertaken. Data were collected on demographics, incidence and type of ocular disease features, natural history and treatment. Results A total of 50% (14/28) patients developed ocular symptoms with a mean time of onset of 6.75 (±6.1) weeks from starting dupilumab. Of these, 69% (9/13) were diagnosed with conjunctivitis associated with cicatrisation in two patients and periorbital skin changes in four. Of these nine, four had prior history of atopic keratoconjunctivitis. All were treated with topical steroids; two required additional ciclosporin drops. In all, 67% (6/9) patients went on to have on-going ocular inflammation requiring maintenance drops at a mean of 16 (±6.9) months of follow-up. All patients had improvement in their AD severity; only one patient discontinued dupilumab due to ocular side effects. Conclusion The rate of dupilumab-associated ocular surface disease was 32%. Periorbital skin changes and conjunctival cicatrisation were noted in association with conjunctivitis. Ocular surface disease improved on topical steroids and ciclosporin but 67% of patients needed on-going treatment. Close liaison with an ophthalmologist should be considered in those patients who develop conjunctivitis or have a past history of severe ocular surface disease.

Purpose To report occurrence of cicatrizing conjunctivitis as an extraglandular ocular manifestation of primary Sjogren's syndrome (SS). Methods Medical charts of all patients with SS evaluated at two tertiary ophthalmological referral centers were reviewed. Patients who demonstrated clinical findings of cicatrizing conjunctivitis were included in this review. Patient and disease-related data including ocular complications, therapies and outcomes were collected. Results Eight patients with a diagnosisis of SS were noted to have cicatrizing conjunctivitis findings over a period of 11 years (between 2009 and 2020). Mean age of patients was 79. All patients had a negative immunoreactant deposition in conjunctival biopsy. Mean follow-up time was 6 years (range, 18–197 months). Three patients had progression of conjunctival scarring. Worsening of vision occurred in 4 patients due to corneal complications, including ulceration, perforation and scarring. Conclusions SS is an under-recognized etiology of severe progressive cicatrizing conjunctivitis that can lead to ocular morbidity and loss of vision without appropriate management.

Purpose: To describe the immediate consequences of SARS-CoV-2 and the COVID-19 pandemic on the ocular surface and eye-care professionals, and to discuss the need for a mandatory switch from currently performed tele-screening to true teleconsultation for remote ocular surface assessment. Main findings: Ophthalmologists have been largely impacted by the COVID-19 sanitary crisis, due to both the ocular manifestations of SARS-CoV-2 and to the high contagiousness of the virus. The proximity of ophthalmologists to their patients have pushed eye-care providers to readapt their practices and develop alternatives to face-to-face consultations. However, teleconsultation has some major limitations and drawbacks, especially for ocular surface assessment that relies on high-quality graphic data for adequate diagnosis. Tele-screening, on the other hand, emphasizes on the importance of history-taking and listening to the patient in order to adequately prioritize appointments based on the presumed degree of emergency. Conclusion: Despite all the enthusiasm, tele-screening as currently performed with the available tools is still not capable of completely replacing a standard ophthalmic examination for the assessment of ocular surface diseases. While waiting for new emerging technologies and future implementation of imaging modalities and artificial intelligence, decision making algorithms can help eye-practitioners remotely screen their patients to assess the optimal time for follow-up appointments.

PURPOSE To determine the frequency, symptoms and risk factors for adverse reactions to two-times instillation of 1% cyclopentolate in children. STUDY DESIGN Prospective, observational study. METHODS The subjects were 646 patients who underwent cycloplegic refraction with cyclopentolate (mean age; 7.0 ± 3.5 years, age range; 0-15 years). Five minutes after instillation of 0.4% oxybuprocaine hydrochloride, a 1% cyclopentolate eye drop was instilled twice, with an interval of 10 min. Fifty minutes later, two certified orthoptists evaluated adverse reactions using a questionnaire and interviewed the patients' guardians. The relationship between the adverse reaction rates and age, gender, additional instillation, complications of the central nervous system (CNS), time of day and season were analysed using binominal and polytomous logistic regression models. RESULTS The overall frequency of adverse reactions was 18.3% (118/646 patients). The main symptoms included conjunctival injection (10.5%, 68/646), drowsiness (6.8%, 44/646) and facial flush (2.2%, 14/646). The odds ratio (OR) of conjunctival injection increased with patient's age (p

Background Ocular graft-versus-host disease (GVHD) is one of the most severe complications of hematopoietic stem cell transplantation. It manifests as an impairment of the ocular surface, such as severe dry eye disease, and deteriorates the recipient's visual function and quality of life. We encountered an "overlap syndrome" of ocular GVHD, which is characterized by the presence of both acute and chronic GVHD symptoms. In this report, we present the treatment progress of the overlap syndrome in a case with ocular GVHD. Case presentation A 57-year-old man with acute myeloblastic leukemia underwent hematopoietic stem cell transplantation. Six weeks after the treatment, the recipient complained of eye pain and discharge. He was diagnosed with the overlap syndrome due to low tear volume, severe corneal epithelitis, hyperemia, and a pseudomembrane on the conjunctiva. Immune cells infiltration, fibrinoid degeneration, fibroblastic and spindle-shaped cells, and fibrosis were observed in the pathology of the pseudomembrane. The recipient was treated with topical immunosuppression and pseudomembrane removal. One week after the initial treatment, ocular GVHD improved. Twelve weeks after the treatment, the topical steroid was discontinued due to the elevation of intraocular pressure. Conclusions The assessment of conjunctival pseudomembrane in ocular GVHD is important to determine the stage of the case and to assess systemic GVHD. Furthermore, prompt removal of the pseudomembrane after diagnosis is an appropriate management to reduce the symptoms of ocular GVHD. The combination of topical steroids and immunosuppressive agents is suggested to be an effective treatment in management of overlap syndrome.

Severe conjunctival diseases can cause significant conjunctival scarring, which seriously limits eye movement and affects patients' vision. Conjunctival reconstruction remains challenging due to the lack of efficient methods for stem cells enrichment. This study indicated that p75 positive conjunctival epithelial cells (CjECs) were mainly located in the basal layer of human conjunctival epithelium and showed an immature differentiation state in vivo. The p75 strongly positive (p75++) CjECs enriched by immuno‐magnetic beads exhibited high expression of stem cell markers and low expression of differentiated keratins. During continuous cell passage cultivation, p75++ CjECs showed the strongest proliferation potential and were able to reconstruct the conjunctiva in vivo with the most complete structure and function. Exogenous addition of NGF promoted the differentiation of CjECs by increasing nuclear localization of SALL2 in p75++ CjECs while proNGF played an opposite role. Altogether, p75++ CjECs present stem cell characteristics and exhibit the strongest proliferation potential so can be used as seed cells for conjunctival reconstruction, and NGF‐p75‐SALL2 signaling pathway was involved in regulating the differentiation of CjECs., p75++ CjECs(conjunctival epithelial cells) were enriched by immuno‐magnetic beads and their stem cell properties were detected. After 10 days of culture on amniotic membrane, conjunctiva constructed by p75++/− CjECs was transplanted to a rabbit model of conjunctival defect to test the repair effect. Exogenous addition of NGF promoted the differentiation of CjECs by increasing nuclear localization of SALL2 in p75++ CjECs.

To investigate the role of miRNA in the pathogenesis underlying ocular surface complications in patients with Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) in the chronic stage. Using oligonucleotide microarrays, we performed comprehensive miRNA analysis of the conjunctival epithelium of SJS/TEN patients with severe ocular complications (SOC) in the chronic stage (n = 3). Conjunctival epithelium of patients with conjunctival chalasis (n = 3) served as the control. We confirmed the down- and up-regulation of miRNA of interest by quantitative real-time polymerase chain reaction (RT-PCR) assays using the conjunctival epithelium from 6 SJS/TEN with SOC patients and 7 controls. We focused on miRNA-455-3p, which is significantly upregulated in the conjunctival epithelium of the SJS/TEN patients, and investigated its function by inhibiting miR-455-3p in primary human conjunctival epithelial cells (PHCjEs). Comprehensive miRNA expression analysis showed that the expression of 5 kinds of miRNA was up-regulated more than fivefold, and that the expression of another 5 kinds of miRNA was down-regulated by less than one-fifth. There was a significant difference between the SJS/TEN patients and the controls [analysis of variance (ANOVA) p

Allergic conjunctival diseases (ACDs) are inflammatory diseases of the conjunctiva and cornea caused predominantly by the IgE-mediated immediate hypersensitivity response. Allergic conjunctival diseases include allergic conjunctivitis, vernal keratoconjunctivitis (VKC), atopic keratoconjunctivitis (AKC), and giant papillary conjunctivitis. In clinical practice of ACDs, an ocular allergy test using biomarker measurement is a crucial examination technique for diagnosing, evaluating severity, and determining the efficacy of medical treatment. The ocular allergy test includes the tear test for evaluating the concentration of biomarkers in tears and an ocular surface test for assessing the expression levels of messenger ribonucleic acid (mRNA) biomarkers on the ocular surface. The clinical usefulness of several biomarkers has been demonstrated in patients with ACDs; specifically, eosinophil cationic protein and eotaxin-2 as eosinophilic inflammation biomarkers; interleukin-4 and thymus and activation regulated chemokine (CCL17/TARC) as Th2 inflammation biomarkers; eotaxin, tumor necrosis factor-alpha and soluble IL-6 receptor as giant papillae biomarkers; and osteopontin and periostin as allergic inflammation and remodeling biomarkers. Furthermore, the ocular allergy test, quantitative evaluation methods using biomarkers have allowed for better understanding of the immunological and pathophysiological mechanisms of ACDs. Therefore, the search for a biomarker is important to make an ocular allergy test useful. In previous ocular allergy tests, the biomarkers for allergic inflammation in patients with chronic ACDs including VKC and AKC were substantial. However, the selection of biomarkers associated with the early phase reaction of immediate hypersensitivity and innate immunity responses needs to be addressed in future investigations.

Purpose: Ocular complications are common in the critical care setting but are frequently missed due to the focus on life-saving organ support. The SARS-CoV-2 (COVID-19) pandemic has led to a surge in critical care capacity and prone positioning practices which may increase the risk of ocular complications. This article aims to review all ocular complications associated with prone positioning, with a focus on challenges posed by COVID-19. Materials and Methods: A literature review using keywords of “intensive care”, “critical care”, “eye care”, “ocular disorders”, “ophthalmic complications,” “coronavirus”, “COVID-19,” “prone” and “proning” was performed using the electronic databases of PUBMED, EMBASE and CINAHL. Results: The effects of prone positioning on improving respiratory outcomes in critically unwell patients are well established; however, there is a lack of literature regarding the effects of prone positioning on ocular complications in the critical care setting. Sight-threatening ophthalmic disorders potentiated by proning include ocular surface disease, acute angle closure, ischemic optic neuropathy, orbital compartment syndrome and vascular occlusions. Conclusions: COVID-19 patients may be more susceptible to ocular complications with increased proning practices and increasing demand on critical care staff. This review outlines these ocular complications with a focus on preventative and treatment measures to avoid devastating visual outcomes for the patient.

To assess the reliability and validity of gray level co-occurrence matrices (GLCM) in the quantification of choriocapillaris and describe GLCM features in normal and eyes with resolved acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and serpiginous choroiditis (SC).In this, multicenter, reliability, validity and comparative study; OCTA was performed on eyes with resolved APMPPE and SC and normal individuals. CC texture classification, low flow area measurements and GLCM feature extraction were performed.A total of 13 normal, 8 APMPPE and 15 SC eyes were analyzed. All GLCM parameters demonstrated an excellent reliability. GLCM parameters were differently distributed across the three groups. Decision-tree based on the random forest predictive model provided an overall accuracy of 86% in classifying the three groups using GLCM features.These data demonstrated an excellent reliability and validity of GLCM features in quantifying the choriocapillaris in healthy and diseased eyes.

Purpose In recent decades, the medical and surgical treatment of limbal stem cell deficiency (LSCD) has evolved significantly through the incorporation of innovative pharmacological strategies, surgical techniques, bioengineering, and cell therapy. With such a wide variety of options, there is a need to establish a global consensus on the preferred approaches for the medical and surgical treatment of LSCD. Methods An international LSCD Working Group was established by the Cornea Society in 2012 and divided into subcommittees. Four face-to-face meetings, frequent email discussions, and teleconferences were conducted since then to reach agreement on a strategic plan and methods after a comprehensive literature search. A writing group drafted the current study. Results A consensus in the medical and surgical management of LSCD was reached by the Working Group. Optimization of the ocular surface by eyelid and conjunctival reconstruction, antiinflammatory therapy, dry eye and meibomian gland dysfunction treatment, minimization of ocular surface toxicity from medications, topical medications that promote epithelialization, and use of a scleral lens is considered essential before surgical treatment of LSCD. Depending on the laterality, cause, and stage of LSCD, surgical strategies including conjunctival epitheliectomy, amniotic membrane transplantation, transplantation of limbal stem cells using different techniques and sources (allogeneic vs. autologous vs. ex vivo-cultivated), transplantation of oral mucosal epithelium, and keratoprosthesis can be performed as treatment. A stepwise flowchart for use in treatment decision-making was established. Conclusions This global consensus provides an up-to-date and comprehensive framework for the management of LSCD.

The definition, classification, pathogenesis, test methods, clinical findings, criteria for diagnosis, and therapies of allergic conjunctival disease are summarized based on the Guidelines for Clinical Management of Allergic Conjunctival Disease 2019. Allergic conjunctival disease is defined as “a conjunctival inflammatory disease associated with a Type I allergy accompanied by some subjective or objective symptoms.” Allergic conjunctival disease is classified into allergic conjunctivitis, atopic keratoconjunctivitis, vernal keratoconjunctivitis, and giant papillary conjunctivitis. Representative subjective symptoms include ocular itching, hyperemia, and lacrimation, whereas objective symptoms include conjunctival hyperemia, swelling, folliculosis, and papillae. Patients with vernal keratoconjunctivitis, which is characterized by conjunctival proliferative changes called giant papilla accompanied by varying extents of corneal lesion, such as corneal erosion and shield ulcer, complain of foreign body sensation, ocular pain, and photophobia. In the diagnosis of allergic conjunctival diseases, it is required that type I allergic diathesis is present, along with subjective and objective symptoms accompanying allergic inflammation. The diagnosis is ensured by proving a type I allergic reaction in the conjunctiva. Given that the first-line drug for the treatment of allergic conjunctival disease is an antiallergic eye drop, a steroid eye drop will be selected in accordance with the severity. In the treatment of vernal keratoconjunctivitis, an immunosuppressive eye drop will be concomitantly used with the abovementioned drugs.

Crizotinib, a targeted molecular therapy drug which inhibits tyrosine kinase, is approved for treatment of non-small cell lung carcinoma which has some ocular side effects like photopsia and delayed dark adaptation.This report documents a unique case of persistent conjunctival chemosis likely due to side effects of crizotinib therapy. A 64-year-old gentleman on crizotinib for metastatic adenocarcinoma of the lung presented with conjunctival chemosis in right eye which appeared 1 month after uneventful clear corneal phacoemulsification surgery. The patient was on crizotinib 250 mg twice a day started 2 months before cataract surgery. Clinical examination revealed marked inferior bulbar conjunctival edema of the right eye. Anterior segment optical coherence tomography, slit-lamp photographs, and magnetic resonance imaging orbit and systemic investigation were done to rule out other causes of conjunctival edema. Magnetic resonance imaging shows conjunctival and preseptal edema around both eye and thinning of the optic nerve in the right eye. Anterior segment optical coherence tomography revealed elevated hyper-reflective thickened conjunctival layer with dilated empty thin wall dark spaces of varying caliber. Chemosis was persisted for the next 3 months and not responded to oral acetazolamide, topical steroid and decongestive eyedrops. After 3 months, crizotinib was discontinued by oncologist due to drug intolerance and surprisingly within 1 week, the conjunctival edema was disappeared totally. To the best of the authors' knowledge, this is the first reported case of persistent unilateral non-inflammatory conjunctival chemosis caused by crizotinib. The physicians should be vigilant about these complications.

This retrospective observational study compared long-term topographic changes after recurrent- and primary-pterygium surgery depending on pterygium size. Patients who underwent recurrent-pterygium excision between 2002–2013 and age, sex, and pterygium size-matched controls who underwent primary-pterygium surgery were included (33 eyes of 33 patients in each group). Pterygium size was graded per advancing edge position

Purpose: Rho-associated kinase-inhibitor ripasudil 0.4% eye drops are reportedly effective for the reduction of intraocular pressure (IOP) in glaucoma patients. However, the previous studies invest...

Purpose To report an unusual case of corneal whorl-like opacity patterns in a patient of primary localized conjunctival amyloidosis. Methods Case report and review of literature. Results A 35-year old man with a case of primary localized conjunctival amyloidosis presented with corneal whorl-like opacity patterns and underwent conjunctival and limbal biopsies. Histopathological evaluation showed dense deposition of amorphous, hyaline, eosinophilic material with week periodic acid-Schiff positivity and congophilia on congo red stain. There was birefringence under a polarized microscope. These findings were suggestive of amyloid deposition. tumor-associated calcium signal transducer-2 mutation was negative ruling out gelatinous drop-like corneal dystrophy. Systemic evaluation was negative for primary systemic amyloidosis. A diagnosis of primary localized conjunctival amyloidosis with corneal whorl-like opacity patterns was confirmed. Conclusions This is an unusual case report of primary localized conjunctival amyloidosis, presenting as corneal whorl-like opacity patterns. Primary localized conjunctival amyloidosis usually involves the fornix and then spreads to lids causing lid thickening, blepharoptosis, subconjunctival, or orbital mass. In our case, it probably started around limbus, involving palisades of Vogt and caused corneal whorl-like opacity patterns which were the presenting feature.

Purpose To evaluate the immediate effects on cornea and conjunctiva of a cryosurgical method based on the application of a dimethyl ether, propane and isobutene solution (DMEPI, Histofreezer® ), and of Liquid Nitrogen (LN2 ; Cry-Ac Cryogun® ). Methods A total of 52 isolated pig eyes were obtained at the slaughterhouse, preserved for less than one hour in Ringer Lactate and divided into four groups. The first group included eyes treated with two applications of DMEPI in the center of the cornea, the second group eyes treated with DMEPI on the third eyelid's conjunctiva, the third group eyes treated with LN2 on the third eyelid's conjunctiva and the fourth group included eyes treated with LN2 on the central cornea. Each cryogen application (0.8 cm area) lasted 40 seconds. Each group included one (untreated) control eye. The eyes were submitted for routine histopathological evaluation. Histological alterations were recorded and scored with a semiquantitative scoring system. Results No tissue alteration was detected on the conjunctiva, in any of the groups. Treated corneas showed mild to moderate cytoplasmic vacuolization of epithelial cells, shrinkage and hypereosinophilia of small groups of basal epithelial cells and stromal cleft formation. Conclusions Soft cryosurgery caused no severe acute histologically detectable damage to cornea and conjunctiva in isolated pig eyes, and no significant difference was observed when LN2 and DMEPI treatments were compared. Further in vivo studies should be performed in order to verify possible delayed effects and the clinical efficacy of DMEPI cryosurgery in specific corneal and/or conjunctival diseases.

Purpose: To demonstrate the reliability of conjunctival biopsy analyzed by direct immunofluorescence (DIF) and supplemented with avidin-biotin complex immunoperoxidase (ABC) in diagnosing oMMP, and...

Background A cyclodialysis cleft often leads to direct communication between the anterior chamber and the suprachoroidal space. It is a rare condition that is encountered with blunt trauma, and less commonly, after surgery. Hypotony is the major sequelae that may lead to hypotonous maculopathy, optic disc edema, corneal folds, and astigmatism. These may cumulatively lead to visual loss. We describe how endoscopy in a cyclodialysis repair allowed us to accurately locate the cleft and guided its appropriate management avoiding unnecessary cryopexy. Case presentation A 41-year-old male experienced a traumatic cyclodialysis cleft, which resulted in persistent hypotony. Pars plana vitrectomy was performed to treat vitreous hemorrhage. Scleral indentation was attempted to visualize the cyclodialysis cleft. However, the depression distorted the visualization. Intraocular endoscopy was therefore used to evaluate the cleft. Guided by this assessment, only intraocular gas tamponade was used to reposition the ciliary body. The patient’s intraocular pressure was restored to 13 mmHg 3 days after the operation, and OCT confirmed cleft closure 1 month after the operation. Conclusion Endoscopy-assisted repair of cyclodialysis is an approach that enhances visualization and can guard against common causes of persistent cleft and hypotony, as well as reveal the causes of recurrent failure. Hence, it can eliminate unnecessary cryopexy that might worsen the hypotonous state. In our case, intraocular endoscopy was effective for the evaluation of a cyclodialysis cleft and the subsequent selection of an appropriate management technique, gas tamponade, that was more conservative than other approaches initially considered.

Allergic conjunctival diseases (ACDs) are a group of ocular allergies that include allergic conjunctivitis, atopic keratoconjunctivitis, vernal keratoconjunctivitis, and giant papillary conjunctivitis. Although a large body of information exists on the pathophysiology of ACDs, this has not yet lead to the development of clear recommendations and guidelines for the diagnosis of ACDs or development of conclusive and objective diagnostic tools. Identification of objectively measurable biomarkers that represent the molecular and cellular mechanisms associated with ACDs will be an important step toward achieving these aims. This is a comprehensive review of biological markers that have the potential to become "biomarker(s)" for ACDs and aid in the classification, diagnosis, and development of new therapeutic strategies for these group of allergic conditions.

Purpose To investigate the details of the long-term progression of ocular surface cicatrization in eyes with ocular sequelae caused by Stevens-Johnson syndrome (SJS)/toxic epidermal necrosis (TEN). Methods In 105 eyes of 66 patients with SJS/TEN, the severity of ocular sequelae was retrospectively evaluated using an ocular surface grading score (OSGS) at 2 time points separated by a greater than 5-year interval. The OSGS included 7 ocular surface components (conjunctivalization, neovascularization, opacification, keratinization, symblepharon, and upper/lower conjunctival-sac shortening) evaluated into grades 0 to 3 (maximum total OSGS: 21), with the worsening of each component and total OSGS evaluated at the greater than 5-year interval. Moreover, we evaluated whether upper-tarsus and lid-margin scarring are factors that affect ocular surface cicatrization progression. Results In 35 (33.3%) of 105 eyes, the total OSGS worsened during the follow-up period. Partial conjunctivalization (score 1-2) progressed more frequently to total conjunctivalization (score 3) than to no conjunctivalization (score 0) (OR [95% CI]; 5.6 [1.6-20.3]). Partial keratinization (score 1-2) also had a high risk of progression into total keratinization (41.0 [6.3-266.5]). In all cases, keratinization progressed only in the eyes with total conjunctivalization (conjunctivalization score 3). Severity of upper-tarsus scarring or lid-margin scarring affected the worsening of the total OSGS. Conclusions In 66 patients with chronic-phase SJS/TEN, ocular surface cicatrization progressed in 33.3% of the 105 eyes during the long-term follow-up period of over 5 years. More than 50% of the partial conjunctivalization eyes progressed toward total conjunctivalization. The partial keratinization eyes had a high possibility of progressing to total keratinization; that is, the so-called "end-stage" status.

Purpose: A prospective, open-label study in 20 professional swimmers evaluated the efficacy and safety of an ophthalmic solution containing crosslinked hyaluronic acid, coenzyme Q10, and vitamin E TPGS in releasing eye irritation and restoring ocular surface damages after prolonged exposure to chlorinated water. Methods: Individually, one eye was instilled with the ophthalmic solution and the other used as a comparator. Eye drops were self-administered three times a day for 2 months. Tear film breakup time (primary endpoint), Schirmer I test, beating of eyelashes/min, tear osmolarity, corneal and conjunctival staining with fluorescein, Ocular Surface Disease Index questionnaire, subject satisfaction, visual acuity (secondary endpoints), and Efron Grading Scale were evaluated at screening/baseline (V1), week 1 (V2), week 2 (V3), week 4 (V4), and week 8 (V5). Results: After 2 months, breakup time test significantly improved in the treated eyes (+1.67 s) compared to control (−3.00 s) ( p = 0.0002). Corneal and conjunctival surfaces of treated eyes recovered significantly compared to control eyes when assessed by fluorescein staining ( p Conclusion: The adequate combination of crosslinked hyaluronic acid, coenzyme Q10, and vitamin E TPGS, contained in the ophthalmic solution VisuXL®, has been shown to protect ocular surface from potential damages originating from prolonged exposure to chlorinated water. VisuXL may represent a compelling treatment in other situations beyond dry eye syndrome.

PURPOSE: To demonstrate the marginal corneal vascular remodelling using optical coherence tomography angiography (OCTA) after pterygium surgery. METHODS: Twenty-two eyes of 19 patients (8 males, 11 females; age, 58.68 ± 0.34 years) with primary grade-T3 nasal pterygium were enroled in this study. The eyes underwent excision of the pterygium followed by a free limbal-conjunctival autograft. OCTA was performed in the nasal limbal area before surgery and at 10 days, 1 month, and 3 months after surgery. The scans were analyzed in terms of postoperative vascular remodelling of the autograft and marginal corneal vascular arcades (MCAs). RESULTS: Preoperatively, the pterygium presented as abnormal centripetal vascular growth in OCTA scans. The conjunctival vessel density in the nasal quadrant was 29.26% ± 1.00%, 15.80% ± 0.83%, 19.80% ± 0.88%, and 20.26% ± 0.89% before and 10 days, 1 month, and 3 months, respectively, after surgery (F = 1.55, P

Purpose To report our 14-year experience with orbital exenteration and assess risk factors for poor prognosis by focusing on conjunctival melanoma. Patients and method A retrospective study was conducted in our tertiary care centre (Jules Gonin Eye Hospital, Lausanne, Switzerland) between 2003 and 2017. Inclusion criteria were patients aged ≥18 years with a follow-up >12 months, without metastatic spread at the time of surgery. Data recorded were age, gender, tumour histology, surgical technique, postoperative complications, surgical margin status, local recurrence, postoperative radiation beam therapy and metastatic status. Results Twenty-five patients with a mean age of 63.2 years (38–92) were included. Conjunctival melanoma was the most frequently identified tumour (n = 14, 56%) followed by conjunctival squamous cell carcinoma (n = 4, 16%), sebaceous carcinoma (n = 3, 12%), choroidal melanoma (n = 2, 8%) and basal cell carcinoma (n = 2, 8%). Eighteen tumours (72%) originated from the conjunctival tissue. Clear surgical margins were achieved in 21 (84%) patients. Fourteen (56%) patients experienced distant metastases and died from metastatic spread after a mean follow-up of 52.3 months (6–120). The 1-, 3- and 5-year overall survival (OS) was 96%, 72% and 60%, respectively. In the univariate analysis, positive surgical margins, local recurrence and metachronous metastases were associated with a decreased OS (p = 0.002, p = 0.005 and p = 0.007, respectively). In the multivariate analysis, positive surgical margins and metachronous metastases were also associated with a decreased OS (p = 0.02 and p = 0.042, respectively). Conjunctival melanoma was not associated with a poorer prognosis (p = 0.280). Conclusion Free surgical margins are needed to increase OS. To achieve clearer surgical margins, neoadjuvant targeted therapies/immunotherapies may be considered.

PURPOSE To evaluate the clinical findings and ocular surface changes in patients with ankylosing spondylitis (AS). METHODS This prospective study involved 45 patients with AS (group 1) and 28 healthy subjects (group 2). Patients in group 1 were in the inactive period. The study subjects underwent a complete ophthalmic examination. The right eyes of the subjects in each group were tested for the study including slit-lamp examination, Schirmer I test, tear break-up time (TBUT), conjunctival impression cytology (CIC), and ocular surface disease index (OSDI). Results between the two groups were compared. RESULTS The mean Schirmer I test result was found as 12.2±8 mm in group 1 and 20.3±9.9 mm in group 2 (P

Purpose To evaluate the effectiveness of conjunctival cyst removal using a modified method of argon laser photoablation. Methods Seventeen cases of symptomatic conjunctival cysts were included. After staining the surface of a conjunctival cyst with a dark purple marker, the cyst was incised using a 26-gauge needle. Low-energy argon laser photoablation was then applied around the incision site for a mean of 100 times. Results During a mean follow-up period of 13.3 months (range 6-28 months), all conjunctival cysts were successfully corrected by applying either one or 2 laser sessions. Complete resolution occurred after a single laser session in 14 eyes (82.4%). There were 3 cases of recurrence with medium to large cysts, and repeating the same procedure produced surgical success in all of these cases; the conjunctival cyst recurred again after the second laser session in one of the 3 eyes, but this spontaneously regressed without further treatment. No postoperative complications such as conjunctival scarring or persistent ocular irritation were observed. Conclusions Our modified method of argon laser photoablation uses staining of the conjunctival cyst surface to increase the amount of thermal laser energy absorbed by the target. This novel technique is simple and effective for treating conjunctival cysts in an outpatient clinic.

Conjunctival orbital cysts are rare; they are typically either conjunctival dermoid or conjunctival epithelial cysts - congenital or acquired (inclusion). We describe the case of a 15-month-old girl presenting with strabismus and proptosis who had a retrobulbar intraconal cystic lesion displacing the optic nerve, with an adjacent middle cranial fossa anomaly. Aspiration of the orbital cyst tested positive for asialotransferrin, raising the suspicion of a direct communication with cerebrospinal fluid (CSF). Subsequent fine cut CT scanning disproved any connection with the intracranial space, and the cyst was excised complete and intact. Histopathology showed a conjunctival epithelial cyst. To our knowledge, this is the first case report in the literature of an asialotransferrin positive pediatric orbital conjunctival epithelial cyst. It is of clinical relevance as it explores the possibility of either a false positive asialotransferrin or potentially a prior developmental communication with the subarachnoid space. These two diagnostic possibilities are discussed.

Ambient air pollution is a well-recognized risk for various diseases including asthma and heart diseases. However, it remains unclear whether air pollution may also be a risk of ocular allergic diseases. Using a web-based, nation-wide, cross-sectional study design, we examined whether the level of ambient air pollution is significantly associated with the prevalence of ocular allergic diseases. A web-based questionnaire was posted to invite the participants who are members of the Japan Ophthalmologist Association and their family members. The answers from 3004 respondents were used to determine whether there were significant associations between the level of the pollutants and the prevalence of ocular allergic diseases. The study period was between March to May 2017. The data of the air pollutants during 2012 to 2016 were obtained from the National Institute for Environmental Studies. The prevalence of allergic diseases was calculated by post stratification and examined for significant associations with the level of pollutants using multiple logistic regression analyses. The prevalence of seasonal allergic conjunctivitis, perennial allergic conjunctivitis, atopic keratoconjunctivitis (AKC), and vernal keratoconjunctivitis (VKC) in Japan was 45.4%, 14.0%, 5.3%, and 1.2%, respectively. The high prevalence of the severe forms of allergic conjunctivitis, including AKC and VKC, were significantly associated with the levels of the air pollutants. The prevalence of AKC was significantly associated with the levels of NO2 with an odds ratio (OR) of 1.23 (per quintile). The prevalence of VKC was significantly associated with the levels of NOx and PM10 with ORs of 1.72 and 1.54 respectively. The significant associations between the prevalence of AKC and VKC and the levels of air pollutants indicate that clinicians need to be aware that air pollutants may pose serious risks of vision threatening severe ocular allergy.

The purpose of this case report is to describe a new surgical method for sutureless placement of the amniotic membrane with a symblepharon ring in a pediatric patient with acute toxic epidermal necrolysis (TEN). A 1-year-old girl developed severe ocular surface inflammation with large corneal and conjunctival epithelial defects secondary to TEN. She was treated by applying a large (4 cm x 4 cm) amniotic membrane graft and non-sterile symblepharon ring under sedoanalgesia at bedside in the intensive care unit. The ocular surface was completely epithelized by post-treatment week 6 in the right and week 8 in the left eye. Two years after amniotic membrane transplantation, both eyes were quiet with no symblepharon, scar formation, or limbal stem cell deficiency. Performing bilateral amniotic membrane transplantation under a symblepharon ring at bedside provided sufficient acute coverage of the ocular surface and led to excellent clinical outcomes by reducing inflammation and protecting the ocular surface.

To describe the long-term effect of a treatment protocol for ocular involvement in acute Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), including focused ocular examination and pathology-appropriate use of lubrication, topical corticosteroids, topical antibiotics, and amniotic membrane transplantation (AMT).Retrospective, comparative case series.A total of 48 patients (96 eyes) were included in this study. Nine of 48 patients (18 eyes) had acute SJS/TEN from 2000 to 2007 and did not receive protocol care (Group I). Thirty-nine of 48 patients (78 eyes) had acute SJS/TEN from 2008 to 2017 and received protocol care (Group II). The main outcome measures were best-corrected visual acuity (BCVA) at final follow-up visit and incidence of complications in the chronic phase.No eyes in Group I received AMT for SJS/TEN, compared to 87% of qualifying eyes in Group II (P.0001) There was a significant difference in the proportion of eyes with BCVA ≥20/40 at last follow-up between Group I and Group II (33% vs 92%, P .001). The proportion of eyes with vision-threatening complications in the chronic phase was significantly higher in Group I versus Group II (67% vs 17%, P = .002), with most complications occurring in the first 2 years after disease onset in both groups.A specific protocol for acute ocular care in SJS/TEN, including aggressive use of AMT, was highly successful in reducing corneal blindness and severe vision-threatening complications of the disorder.

To investigate the prevalence of meibomian gland dysfunction (MGD) in patients presenting with subjective dry eye-related symptoms at their first-time consultation in a Norwegian specialized ocular surface clinic. Additionally, to explore the accuracy of the ocular surface disease index score (OSDI) as an extensively applied tool to assess the severity of dry eye symptoms and MGD diagnosis. Patients with subjective dry eye-related complaints (n = 900) attending the clinic for the first time, from 2012 to 2016, were included in the study. At the baseline, patients completed the OSDI questionnaire. Subsequently, objective clinical tests, including fluorescein break-up time (FBUT), Schirmer-I test, ocular surface staining (OSS), and meibomian gland function assessment using gland expressibility and meibum quality were performed. The association between MGD and its severity in relation to symptom severity defined by OSDI-score was examined. MGD was found in 93.8% of the study group. MGD prevalence was not significantly different between groups based on age (p = 0.302) or sex (p = 0.079). There was a significant association between severity of MGD and dry eye-related symptoms (p = 0.014). OSS was significantly higher in patients with severe symptoms (p = 0.031). Sensitivity and specificity of positive symptom-score (OSDI ≥ 13) for disclosing MGD were 85.5% and 30.4%, respectively. MGD was highly prevalent, not associated with age and sex. OSDI ≥ 13 had high sensitivity and high positive predictive value (PPV), but low specificity and negative predictive value (NPV) for disclosing MGD. This underscores the importance of meibomian gland assessment in patients with dry eye-related symptoms.

Purpose Ocular manifestations of immunoglobulin G4 (IgG4)-related disease are common in children although remain ill-defined because of the disease's rarity. We describe a pediatric case of IgG4-related orbital disease (IgG4-ROD) who presented with persistent conjunctival infiltration before developing lacrimal gland enlargement 3 years later. Methods This was a case report. Results An 8-year-old girl developed forniceal salmon-patch-like conjunctival lesions in her left eye that were refractory to topical corticosteroids. Investigations, including an orbital MRI and 2 conjunctival biopsies, were negative for lymphoma. She was treated with topical corticosteroids and then nonsteroidal antiinflammatory drops. The lesions decreased mildly, and no new lesion emerged. After 3 years, the patient developed a ptosis, new salmon-patch conjunctival lesions, and papillae. Vision deteriorated to 20/80 because of severe punctate epithelial erosions in the left eye, and the Schirmer test was significantly reduced. A repeat MRI revealed an enlarged left lacrimal gland. A biopsy was performed and was compatible with IgG4-ROD. An elevated IgG4 level of 4.61 g/L was also found. The patient was successfully treated with oral prednisone but flared on tapering the dosage. Rituximab was therefore initiated with excellent clinical response, and prednisone was discontinued. Vision returned to 20/20 after aggressive lubrification, punctal plugs, and autologous serum eye drops. Tear function came back to normal, and local treatments were stopped. Conclusions This case describes a pediatric case of IgG4-ROD presenting initially with conjunctival follicular reaction, later developing lacrimal gland involvement. Therefore, it is important to consider IgG4-ROD in chronic atypical follicular conjunctival lesions in children, even in the absence of orbital disease.

PURPOSE To evaluate the clinical presentation, anatomical location, and histological features of congenital conjunctival cysts of the orbit. The location and the histological features of inflammation in these patients were compared with those for 293 orbital dermoid cysts. PATIENTS AND METHODS Retrospective review of the clinical details, imaging, and histopathology for patients who had excision of conjunctival cysts from their orbit between 1992 and 2020; patients with a history of trauma or surgery were omitted. RESULTS Twelve patients (7 male; 58%) with congenital conjunctival cysts were identified, the patients presenting at an average age of 16 years (median 26; range 1-61) with a symptoms for a mean duration of 20 months (median 24; range 6-36). The commonest symptoms were peribulbar lump (6/12 patients; 50%), and eyelid swelling and blepharoptosis (6/12 patients; 50%). An orbitaxl mass was palpable in 10 patients (83%), 3 patients (25%) had mild proptosis (1-3 mm), and the cysts were most commonly located superiorly (6/12 patients; 50%) or superonasally (3/12; 25%) in the anterior half of the orbit. Imaging was performed in 7 cases, this showing an intimate relation to the common sheath of the superior rectus/levator complex in 3 patients (25%) and to the trochlea in 1 (8%). All cysts were excised completely, and no patient had postoperative complications or recurrence. Chronic mild and nonspecific inflammation was evident within the cyst wall in 7 cases (54%), but-unlike 55% of the 293 dermoid cysts-none showed granuloma formation. CONCLUSION Congenital conjunctival cysts are rare and usually present with a palpable mass in the upper eyelid sulcus. A significant proportion of these cysts have an intimate relationship with the trochlea, or the superior rectus, levator palpebrae or superior oblique muscles and, to minimize the risk of postoperative diplopia or ptosis, particular care must be exercised during surgery.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse drug reactions with high mortality rates. Its sequelae, such as blindness, persist even after recovery. Patients with SJS/TEN should be accurately diagnosed and receive appropriate treatment as soon as possible. Therefore, identifying the factors for severity prediction is necessary. We aimed to clarify the clinical parameters and biological markers that can predict acute severe ocular complications (SOCs) in SJS/TEN. This retrospective cross-sectional study enrolled 47 patients with SJS/TEN who were divided into two groups according to ocular severity at acute onset: non-severe ocular complications group (n = 27) and severe ocular complications group (n = 20). Multivariate logistic regression analysis revealed that disease severity (body surface area detachment ≥ 10%) was a predictive factor for acute SOCs, and older age (≥ 60 years) was marginally significantly predictive of SOCs. Serum biomarker levels of S100A8/A9 and granulysin were marginally significant and tended to increase in the SOC group. Therefore, during the early acute stage, focusing on disease severity, patient age, and serum inflammatory biomarkers (S100A8/A9 and granulysin) might help predict SOC progression in patients with SJS/TEN who need prompt and aggressive ocular management to prevent severe ocular sequelae.

BACKGROUND/OBJECTIVES Laryngo-onycho-cutaneous syndrome (LOC) is a rare subtype of junctional epidermolysis bullosa (JEB), featuring aberrant granulation tissue formation in the skin, larynx, and eyes. So far, three mutations including the specific (founder) mutation in exon 39 of LAMA3 (c.151dup) have been identified, but sparse data exists regarding the natural history, the genotype-phenotype correlation, and its differentiation from other JEB types. METHODS We reviewed our pediatric EB database to identify English children with clinical and genetically diagnosed LOC within the last 15 years. Their demographic, clinical, and laboratory data were examined. We searched three databases for case reports of LOC between January 1986 and November 2020 and extracted clinical and molecular details. RESULTS We identified 6 LOC patients, all female (mean age 5.4 years). Periungual hypergranulation and skin fragility were the earliest presenting signs (0-3 months), followed by laryngeal stenosis, symblepharon (mean onset 10.7 and 11.8 months, respectively), and dental abnormalities. Five children developed anemia at an average of 19.2 months. We identified 22 published studies in English with 31 cases. CONCLUSIONS This study delineates the disease course of LOC and highlights the overlap with some forms of JEB. Classical signs/symptoms including anemia appear early in life. Genetic analysis revealed three new LOC-associated variants and underscores the finding that interpretation of skin immunolabeling and molecular diagnostics can be challenging. We provide recommendations on management of this complex syndrome.

This prospective study aimed at determine whether eye irrigation removes ocular foreign bodies (FBs) and whether ocular pain predicts FBs. Emergency department patients complaining of ocular FBs were enrolled. In the irrigation group (n = 52), pain was evaluated with a visual analog scale before and after irrigation, and the presence of FBs was determined under a slit-lamp. In the nonirrigation group (n = 27), the evaluations were performed upon arrival. The corneal FB retention rate was found significantly lower in the irrigation (13/52, 25%) than in the nonirrigation groups (13/27, 48%; P = 0.04). After irrigation, those without FBs had more patients experiencing pain reduction (67%) compared to those with retained FBs (46%; P = 0.14) and had a greater magnitude of change in pain score (mean ± SD, − 2.6 ± 2.7 vs. − 0.7 ± 1.4; P = 0.02). An improvement in ocular pain score ≥ 5 points after irrigation predicted the absence of FBs with a negative predictive value of 100%. Eye irrigation significantly lowered corneal FB retention; if ocular pain decreased considerably, the probability of retained FBs was low, making irrigation-associated pain score reduction a feasible diagnostic method to exclude FB retention without needing specialized ophthalmic examinations.

Cicatricial conjunctival diseases (CCDs), are a diverse group of ocular surface diseases characterized by chronic scarring of the conjunctiva. These diseases can cause significant ocular morbidity. They are life-long once acquired and can be debilitating, painful diseases leading to visual loss. A recent international consensus of ocular surface disease experts have placed emphasis on the need of validated clinical disease scoring systems for CCDs, important for the objective evaluation of disease severity, outcomes of therapies, and longitudinal monitoring of disease. This review aims to describe the various published clinical disease scoring systems available for CCDs and evaluates the benefits and limitations of each system. It can be used as a guide for clinicians managing patients with CCDs and for researchers evaluating potential therapies in clinical trials.