Late capsular blockage syndrome: Clinical and anterior segment optical coherence tomography characteristics.

BACKGROUND: Late capsular blockage syndrome (CBS) is a rare phenomenon which is found after cataract surgery. The mechanism, anterior segment optical coherence tomography (OCT) presentation, and clinical characteristics are not well studied. METHODS: We studied patients who developed late CBS in Taipei Veterans General Hospital from 2012 to 2019. Age, sex, systemic disease, ocular disease, interval between cataract surgery and CBS, axial length, type of intraocular lens implanted, grading of posterior capsular opacity, refraction, visual acuity, and anterior segment OCT findings were documented. Patients are categorized into two groups according to anterior segment OCT findings. All patients underwent Nd:YAG laser capsulotomy and were prescribed a low-dose topical steroid for 7 days. Postcapsulotomy refraction and visual acuity were recorded. All the clinical data were compared in the two groups. RESULTS: This study included 18 eyes with late CBS. Patients' median age was 80 (range, 54-92) years. The mean duration between cataract surgery and CBS diagnosis was 80.28 (range 15-136) months. According to anterior segment OCT findings, we subcategorized the patients into two groups: gravel appearance (n = 7) and milky (n = 11) appearance. After laser capsulotomy, mean visual acuity improved 0.18 ± 0.10 on the LogMAR. A significant myopic shift in refraction after laser capsulotomy was noted in the gravel appearance group compared to the milky appearance group ( p = 0.027). No patient developed complications or needed further treatment for CBS during the median follow-up of 14.5 months (range 1-84 months). CONCLUSION: High-resolution anterior segment OCT is useful for analyzing patients with late CBS. Our study implies that the two types of CBS presentation indicate different causes of late CBS formation, as well as distinct clinical presentation and postlaser capsulotomy refractive outcome. In addition, YAG laser capsulotomy is a safe late CBS treatment modality.

Management of Delayed Complications of Hydrogel Scleral Buckles.

(1) Background: hydrogel scleral buckles (HSB)-related complications can happen decades after implantation, although this material has been retrieved for a long time. Due to its fragile texture, ensuring the complete removal of this material and avoiding complications are challenging. Incomplete removal, iatrogenic complication, recurrent retinal detachment, and infection could occur. (2) Methods: chart review of patients who developed delayed HSB-related complications and received removal of HSB in Taipei Veterans General Hospital from 2004 to 2021. The presenting symptoms, prior diagnosis before referral, clinical findings, image features, surgical technique, operative findings, and outcome were analyzed. Detailed surgical procedure and tips for removal were demonstrated in the study. (3) Results: a total of eleven patients were identified. The presenting symptoms include limitations to extraocular movement (ten eyes, 90.9%), ocular redness (eight eyes, 72.7%), ocular fullness (eight eyes, 72.7%), pain (six eyes, 54.5%), and exposed ocular foreign body (five eyes, 45.5%). Of note, six patients (54.5%) have monocular glaucoma and four of them have intractable high intraocular pressure. All patients underwent surgeries to smoothly remove swollen HSB via transcutaneous or transconjunctival approach. Most symptoms improved after surgery and no cases developed surgical-related complications. (4) Conclusions: although HSB have been off the market for decades, delayed complications are still emerging. Clinicians should remain alert for potential complications for patients with prior HSB surgeries. Early diagnosis and meticulous management can help to safely remove the expanded HSB and reduce the associated complications.

Plug-related canaliculitis: a rare or underdiagnosed disease?

CLINICAL RELEVANCE: Conjunctivitis, chalazion and blepharitis are routinely managed by optometrists. However, it is especially important to consider the diagnosis of canaliculitis in patients with chronic or recurrent conditions. BACKGROUND: This study aimed to report the clinical features, radiological findings and treatment outcomes in patients with plug-related canaliculitis. METHODS: This retrospective study included patients with canaliculitis secondary to plug insertion between 2007 and 2020. All data regarding epidemiological characteristics, clinical presentation, isolated microorganisms, computed tomography imaging findings, treatment, and outcomes were analysed. RESULTS: A total of 20 plug-related canaliculitis from 19 patients (18.3%) among all 109 cases of canaliculitis were identified. All patients with plug-related canaliculitis were females with a past history of lacrimal plug insertion for dry eye (mean age: 58.2 years). Most patients were initially treated as conjunctivitis with the mean time lapse to a diagnosis of 5.2 months. The average time from plug insertion to onset of symptoms was 5.1 years. Eighteen patients underwent canaliculotomy, and one patient received lacrimal irrigation. Plugs were identified in 18 cases, with SmartPlug in 13 cases (72%), followed by EaglePlugTM (two cases), Herrick Lacrimal Plug (two cases), and migrated FCI Painless Plug (1 case). Cultures of discharge, concretions, and/or infected plugs mostly revealed Pseudomonas aeruginosa (42%). Orbital computed tomography in four cases with SmartPlug revealed central radiolucency with surrounding soft-tissue enhancement. No recurrent canaliculitis was observed throughout a mean follow-up period of 13.7 months. No patient needed re-plugging after canaliculotomy and plug removal, with only one required additional lubricants for recurrent dry eye. CONCLUSION: Plug-related canaliculitis is often underdiagnosed due to late onset and similar symptoms to common ocular diseases. Awareness of plug insertion history as well as meticulous removal of the plug, concretion and/or granulation tissue is important for early diagnosis and to ensure a good outcome.

The Clinical Feature and Treatment Outcome of Ocular Melanoma: A 34-Year Experience in a Tertiary Referral Center.

Malignant melanoma can arise from melanocytes in various structures of the eye, orbit, and ocular adnexa. We reviewed the clinical features and long-term results of all subjects with histologically proved melanoma originating from any of the ocular and periocular structures in a tertiary referral center. Overall, 88 patients including 47 men were recruited. The tumor was primarily located in the uvea, followed by the conjunctiva, orbit, eyelid, and lacrimal sac. Patients with uveal melanoma were diagnosed at a relatively younger age (47.0 years), while those with orbital and eyelid melanomas were older at presentation (79.5 years and 78.5 years, respectively). The overall local recurrence rate was 9% at a median follow-up of 41.0 months, among which orbital and eyelid melanomas recurred most commonly. The overall mortality rate was 41% in a median duration of 27.2 months (IQR, 13-58 months) from diagnosis, with the highest for lacrimal sac melanoma, followed by melanoma of the orbit, uveal, conjunctiva, and eyelid. Despite prompt local control, the risk for metastasis and mortality was high. Therefore, efficient modalities for early diagnosis and treatment of ocular melanoma are necessary.

Curcumin Suppresses TGF-ß1-Induced Myofibroblast Differentiation and Attenuates Angiogenic Activity of Orbital Fibroblasts.

Orbital fibrosis, a hallmark of tissue remodeling in Graves' ophthalmopathy (GO), is a chronic, progressive orbitopathy with few effective treatments. Orbital fibroblasts are effector cells, and transforming growth factor ß1 (TGF-ß1) acts as a critical inducer to promote myofibroblast differentiation and subsequent tissue fibrosis. Curcumin is a natural compound with anti-fibrotic activity. This study aims to investigate the effects of curcumin on TGF-ß1-induced myofibroblast differentiation and on the pro-angiogenic activities of orbital fibroblasts. Orbital fibroblasts from one healthy donor and three patients with GO were collected for primary cell culture and subjected to myofibroblast differentiation under the administration of 1 or 5 ng/mL TGF-ß1 for 24 h. The effects of curcumin on TGF-ß1-induced orbital fibroblasts were assessed by measuring the cellular viability and detecting the expression of myofibroblast differentiation markers, including connective tissue growth factor (CTGF) and α-smooth muscle actin (α-SMA). The pro-angiogenic potential of curcumin-treated orbital fibroblasts was evaluated by examining the transwell migration and tube-forming capacities of fibroblast-conditioned EA.hy926 and HMEC-1 endothelial cells. Treatment of orbital fibroblasts with curcumin inhibited the TGF-ß1 signaling pathway and attenuated the expression of CTGF and α-SMA induced by TGF-ß1. Curcumin, at the concentration of 5 µg/mL, suppressed 5 ng/mL TGF-ß1-induced pro-angiogenic activities of orbital fibroblast-conditioned EA hy926 and HMEC-1 endothelial cells. Our findings suggest that curcumin reduces the TGF-ß1-induced myofibroblast differentiation and pro-angiogenic activity in orbital fibroblasts. The results support the potential application of curcumin for the treatment of GO.

Smartphone-based diabetic macula edema screening with an offline artificial intelligence.

BACKGROUND: Diabetic macular edema (DME) is a sight-threatening condition that needs regular examinations and remedies. Optical coherence tomography (OCT) is the most common used examination to evaluate the structure and thickness of the macula, but the software in the OCT machine does not tell the clinicians whether DME exists directly. Recently, artificial intelligence (AI) is expected to aid in diagnosis generation and therapy selection. We thus develop a smartphone-based offline AI system that provides diagnostic suggestions and medical strategies through analyzing OCT images from diabetic patients at the risk of developing DME. METHODS: DME patients receiving treatments in 2017 at Taipei Veterans General Hospital were included in this study. We retrospectively collected the OCT images of these patients from January 2008 to July 2018. We established the AI model based on MobileNet architecture to classify the OCT images conditions. The confusion matrix has been applied to present the performance of the trained AI model. RESULTS: Based on the convolutional neural network with the MobileNet model, our AI system achieved a high DME diagnostic accuracy of 90.02%, which is comparable to other AI systems such as InceptionV3 and VGG16. We further developed a mobile-application based on this AI model available at https://aicl.ddns.net/DME.apk. CONCLUSION: We successful integrated an AI model into the mobile device to provide an offline method to provide the diagnosis for quickly screening the risk of developing DME. With the offline property, our model could help those nonophthalmological healthcare providers in offshore islands or underdeveloped countries.

Change of Eyelid Parameters in Patients With Marin-Amat Syndrome.

PURPOSE: Marin-Amat syndrome is an acquired facial synkinesis manifesting as involuntary eyelid closure on jaw movement. The authors investigate the clinical features, especially the quantitative changes in eyelid parameters of patients with Marin-Amat syndrome. METHODS: Patients with Marin-Amat syndrome between 2015 and 2017 in a medical center were collected. Clinical features and the change of eyelid parameters, including margin reflex distance 1 (MRD-1), margin reflex distance 2 (MRD-2), and palpebral fissure height, were evaluated. RESULTS: There were 5 men and 3 women with a mean age of 76 years. All had a history of facial palsy. The mean time to onset of Marin-Amat syndrome was 4.4 years after facial palsy. Seven patients (87.5%) developed subsequent ipsilateral facial spasm after facial palsy. Most patient complaints were ptosis (62.5%) and ptosis on eating (37.5%). The mean palpebral fissure height of involved eyes decreased from 5.88 to 2 mm on jaw opening (p = 0.011), which resulted from decrease in MRD-1 (from 2.06 to 0.06 mm, p = 0.012) and MRD-2 (from 3.81 to 1.94 mm; p = 0.012). Botulinum toxin A (Botox) injection into the periorbital orbicularis muscle in 6 patients significantly relieved the change of palpebral fissure height on jaw opening compared with that before injection (9.9% vs. 68.6 %, p = 0.027). CONCLUSIONS: Most patients with Marin-Amat syndrome present with ptosis and might be overlooked or underestimated. The reduction in palpebral fissure height in our patients with Marin-Amat syndrome was due to involuntary orbicularis oculi muscle contraction, resulting in decrease of both the MRD-1 and MRD-2 on jaw opening.

Mitochondrial transport mediates survival of retinal ganglion cells in affected LHON patients.

The mutations in the genes encoding the subunits of complex I of the mitochondrial electron transport chain are the most common cause of Leber's hereditary optic neuropathy (LHON), a maternal hereditary disease characterized by retinal ganglion cell (RGC) degeneration. The characteristics of incomplete penetrance indicate that nuclear genetic and environmental factors also determine phenotypic expression of LHON. Therefore, further understanding of the role of mutant mitochondrial nicotinamide adenine dinucleotide dehydrogenase subunit proteins and nuclear genetic factors/environmental effects in the etiology of LHON is needed. In this study, we generated human-induced pluripotent stem cells (hiPSCs) from healthy control, unaffected LHON mutation carrier, and affected LHON patient. hiPSC-derived RGCs were used to study the differences between affected and unaffected carriers of mitochondrial DNA point mutation m.11778G > A in the MT-ND4 gene. We found that both mutated cell lines were characterized by increase in reactive oxygen species production, however, only affected cell line had increased levels of apoptotic cells. We found a significant increase in retrograde mitochondria and a decrease in stationary mitochondria in the affected RGC axons. In addition, the messenger RNA and protein levels of KIF5A in the LHON-affected RGCs were significantly reduced. Antioxidant N-acetyl-L-cysteine could restore the expression of KIF5A and the normal pattern of mitochondrial movement in the affected RGCs. To conclude, we found essential differences in the mutually dependent processes of oxidative stress, mitochondrial transport and apoptosis between two LHON-specific mutation carrier RGC cell lines, asymptomatic carrier and disease-affected, and identified KIF5A as a central modulator of these differences.

Immunoglobulin G4-related ophthalmic disease.

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized inflammatory disease of unknown etiology. It characterized by distinctive histopathological appearance of dense IgG4-positive lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis in one or more organs, simultaneously or subsequently. In cases of ocular adnexal involvement, unique clinicohistopathological features were delineated by recent studies, and IgG4-related ophthalmic disease (IgG4-ROD) is generally recognized as the disease name. A significant proportion of previous labeled idiopathic orbital inflammations and Mikulicz's disease are now consistent with a diagnosis of IgG4-ROD. Increasing studies have accumulated regarding its epidemiology, diagnosis, clinical features, treatment, and the association between lymphoma. In this review, we summarize our present understanding of IgG4-ROD.

Conjunctival papilloma: Clinical features, outcome, and factors related to recurrence.

PURPOSE: The purpose of the study was to evaluate the clinical features, treatment, and outcome of patients with conjunctival papilloma. MATERIALS AND METHODS: Twenty-two patients (22 eyes) with biopsy-proven conjunctival papilloma between January 2005 and January 2015 in a tertiary medical center were retrospectively reviewed. Clinical profiles, treatment, outcome, and factors related to recurrence were evaluated. RESULTS: There were 16 males (73%) and 6 females (27%), with a mean age of 47 years. The most common location of conjunctival papilloma was the caruncle (43%), followed by palpebral conjunctiva (29%), bulbar conjunctiva (14%), and fornix (14%). Recurrence developed in five patients (22.7%). The risk of postoperative recurrence was significantly related to the presence of bulbar conjunctival papilloma with corneal involvement (P = 0.043) and surgical excision alone (P = 0.039). One case with multiple recurrences developed nonkeratinizing carcinoma. Two young females developed conjunctival papilloma even after receiving human papillomavirus vaccinations. CONCLUSION: The recurrence of conjunctival papilloma is not uncommon, especially for those patients underwent surgical excision alone. Surgical excision with adjunctive therapy and long-term follow-up is rational for the treatment of conjunctival papilloma.

Primary ductal adenocarcinoma of lacrimal gland: Two case reports and review of the literature.

A 64-year-old male presented with progressive proptosis of the left eye for 3 months. Orbital computed tomography (CT) demonstrated a 3.9 cm infiltrative mass over the superotemporal quadrant of the left orbit. Pathology of biopsy revealed a ductal adenocarcinoma of lacrimal gland with positive immunohistochemical staining for androgen receptor (AR), cytokeratin-7 (CK7), and gross cystic disease fluid protein 15 (GCDFP-15). The patient received orbital exenteration and adjuvant chemoradiotherapy. No recurrence or metastasis was noted 27 months after treatment. Another case was a 64-year-old male who came for proptosis of the right eye and diplopia for 3 weeks. Orbital CT revealed a 5 cm infiltrated right superotemporal orbital mass with destruction of the lateral and inferior orbital walls. Biopsy showed primary ductal adenocarcinoma of lacrimal gland with positive immunohistochemical staining for CK7, AR, and epidermal growth factor receptor. The patient underwent exenteration and concomitant chemoradiotherapy. However, lung and neck metastasis was noted 21 months after surgery. Collectively, 26 cases in the literature were reviewed. The mean age was 57 years old and male was prevalent (73%). Most immunohistological staining showed positive for AR (46%), CK7 (46%), Ki-67 (38%), and GCDFP-15 (35%). More than half of the patients developed metastasis and one-third of the patients died of disease. Early diagnosis, treatment, and long-term follow-up are required for this aggressive tumor.

Change of Serum IgG4 in Patients with Ocular Adnexal Marginal Zone B Cell Lymphoma Associated with IgG4-Related Ophthalmic Disease After Treatment.

PURPOSE: To investigate the change of serum IgG4 concentrations correlated with clinical evolution in patients with ocular adnexal marginal zone B cell lymphoma associated with IgG4-related ophthalmic disease (IgG4-ROD). METHODS: Three consecutive patients with histopathologically confirmed ocular adnexal marginal zone B cell lymphoma associated with IgG4-ROD were evaluated. Two patients received radiotherapy and 1 patient received steroid therapy. Treatment outcome was evaluated by clinical symptoms, radiologic examination, and change of serum IgG4 level in these patients. RESULTS: All patients had elevated serum IgG4 before treatment (462, 338, and 780 mg/dL respectively.) The 2 patients who received radiotherapy achieved complete remission and the serum IgG4 decreased to 345 and 92 mg/dL, respectively. The patient who underwent systemic steroid achieved partial remission and the serum IgG4 decrease to 161 mg/dL. CONCLUSION: Our study showed elevated serum IgG4 in all patients with ocular adnexal marginal zone B cell lymphoma associated with IgG4-ROD. In addition, the elevated serum IgG4 may decrease or keep stable after treatment, accompanied by improvement in clinical symptoms and reduction of lesions.

HMB-45 negative angiomyolipoma of the orbit: a case report and review of the literature.

BACKGROUND: Angiomyolipoma is a benign mesenchymal tumor composed of variable amounts of smooth muscle, adipose tissue and thick-walled blood vessels, and usually named PEComas (perivascular epithelioid cell tumors). PEComas share overlapping histopathological features with epithelioid cells along a perivascular distribution and characteristic immunohistochemistry with coexpression of myoid and melanocytic markers (HMB-45 /or Melan-A). We report the first case of primary orbital angiomyolipoma with negative melanocytic marker. CASE PRESENTATION: An 80-year-old Asian woman had a 2-year history of progressive swelling in the left upper eyelid. External examination revealed 3 cm of relative proptosis of the left eye and a palpable mass in the left superonasal orbit. Computed tomographic scan demonstrated a circumscribed, heterogeneous orbital mass. Excision biopsy was done and the histological finding demonstrated the orbital mass was composed of mature adipocytes, intermingled with spindle or oval-shaped cells, and accompanied by thick-walled blood vessels. Immunohistochemically, tumor cells were positive for CD34 and HHF-35, but negative for cytokeratin, HMB-45 and Melan-A. The diagnosis of angiomyolipoma was made. No recurrence was noted at 2-year follow-up. CONCLUSION: In our case, the HMB-45 negativity may be explained by the rarity of the epithelioid cells, and the HMB-45 positivity is often weaker or absent in spindle cells. Angiomyolipoma, although rare, should be added to the differential diagnosis of space-occupying orbital lesion.

Clinical features, microbiological profiles and treatment outcome of lacrimal plug-related canaliculitis compared with those of primary canaliculitis.

AIMS: To compare the clinical features and treatment outcome between lacrimal plug-related canaliculitis and primary canaliculitis. METHODS: Patients with plug-related canaliculitis and primary canaliculitis between 2007 and 2014 in a medical centre were collected. Charts were reviewed for clinical features, microbiological profiles, time lapse between plug insertion and symptom onset, type of plug and outcomes. RESULTS: Of 76 eligible cases collected, 13 were plug-related canaliculitis and 63 were primary canaliculitis. The most common presenting symptom was discharge in both groups (85% and 79%, respectively). The average time interval from plug insertion to symptoms onset was 5.5 years. Most canaliculitis developed in women, especially for plug-related canaliculitis, when compared with primary canaliculitis (100% vs 65.1%; p=0.015). The most common isolated microorganism was Pseudomonas aeruginosa in plug-related canaliculitis (46%) and Streptococcus in primary canaliculitis (28%), respectively. Isolation of Pseudomonas was significantly higher in plug-related canaliculitis than in primary canaliculitis (46% vs 12%; p=0.029). Most plug-related canaliculitis resolved after removal of plugs by canaliculotomy (12 cases, 93%). Most identified plug was SmartPlug (seven cases), followed by EaglePlug (two cases) and Herrick Lacrimal Plug (two cases). There was no recurrence in patients with plug-related canaliculitis, however, recurrence developed in seven patients (11%) with primary canaliculitis. CONCLUSIONS: In comparison with primary canaliculitis, plug-related canaliculitis appear to be more prevalent in women and show a different microbiological profile. Retrieval of infected plug by canaliculotomy and adequate antibiotics can achieve a good outcome. Long-term follow-up is required because canaliculitis may develop several years after plug insertion.

Comparison of the Clinical Characteristics and Outcome of Benign and Malignant Eyelid Tumors: An Analysis of 4521 Eyelid Tumors in a Tertiary Medical Center.

We retrospectively reviewed the clinical features and outcome of benign and malignant eyelid tumors from 1995 to 2015 in a tertiary medical center. Among 4,521 histologically confirmed eyelid tumors, 4,294 (95.0%) were benign tumors and 227 (5.0%) were malignant tumors. The mean age at diagnosis was significantly higher in patients with malignant lid tumors than those with benign lid tumors (72.5 and 55.4 years, resp., p < 0.001). The most common benign eyelid tumors were intradermal nevus (21.1%), followed by seborrheic keratosis (12.6%) and xanthelasma (11.2%). The most common malignant eyelid tumors were basal cell carcinomas (57.8%), followed by sebaceous gland carcinomas (21.1%) and squamous cell carcinomas (10.1%). There was a relative male predominance (63.4% and 49.2%, resp., p < 0.001) and higher recurrence rate (11.9% and 4.4%, resp., p < 0.001) in malignant lid tumors as compared with those of benign lid tumors. Twenty-two patients (9.7%) received orbital exenteration/enucleation. Eight patients (3.5%) with malignant lid tumors died of disease. Patients with eyelid melanoma were associated with a high mortality rate (25.0%). It is important to differentiate between benign and malignant eyelid tumors, because they may cause cosmetic disfigurement and severe morbidity, especially in those with malignant eyelid tumors.

Large gene deletion and changes in corneal endothelial cells in a family with choroideremia.

PURPOSE: We provided the first report of an association between changes in corneal endothelial cells, retina, and choriocapillaris in a choroideremia family. METHODS: Four members of an Asian choroideremia family, comprising two affected patients and two carriers, were evaluated. All participants underwent complete eye examinations, including visual acuity (VA), slit-lamp examination, ophthalmoscopy, perimetry, and electrophysiology tests. In addition, images of corneal endothelium were captured with a noncontact specular microscope. Genomic DNA amplification and whole-genome cytogenic array analysis were used to confirm the diagnosis of choroideremia and determine the molecular basis of the phenotype. RESULTS: In the affected patients, funduscopy revealed characteristic features of RPE and chorioretinal atrophy. The slit-lamp biomicroscopy disclosed unexpected pigmented punctate lesions in the corneal endothelium in one of them. Surprisingly, specular microscopy detected decreased endothelial cell density (ECD) with features of pleomorphism and polymegethism. Genomic DNA analysis revealed large deletion (~4.5 mega base pairs) of the entire CHM gene and encompassed region. In carriers, funduscopy revealed stippling pigmentary change despite normal electrophysiological results. Specular microscopy also disclosed reduced ECD with features of pleomorphism and polymegethism. CONCLUSIONS: To our knowledge, this is the first description of corneal changes in choroideremia patients. The loss of corneal ECD is conspicuous and is accompanied by pleomorphism and polymegethism in this family. The observed changes in corneal endothelium may be associated with larger encompassed regions of the CHM gene defect or dysfunction in the blood-aqueous barrier. It warrants further investigation and clarification of the pathophysiology and associations between retinal and corneal changes in choroideremia.

Ocular adnexal IgG4-related disease: clinical features, outcome, and factors associated with response to systemic steroids.

PURPOSE: The aim of this study was to investigate clinical characteristics, outcome, and factors associated with response to systemic administration of steroids in patients with ocular adnexal immunoglobulin G4 (IgG4)-related disease. METHODS: This was a retrospective evaluation of 11 patients with histopathologically verified ocular adnexal IgG4-related diseases at a medical center in Taiwan between January 2006 and December 2012. Clinical features and outcome, including serial change of serum IgG4 and clinical factors related to response to steroids systemically were evaluated. RESULTS: Seven men and four women, mean age 54.5 years and mean follow-up of 33.5 months, were evaluated. Elevated serum IgG4 levels (>135 mg/dl) were observed in ten patients (91%). Lacrimal gland involvement was noted in eight (72.7%), followed by orbit, extraocular muscles, and eyelids. Seven patients (63.6%) had bilateral ocular lesions and eight (72.7%) had extraorbital involvement. Eight of ten patients who underwent systemic steroid treatment responded well in the early phase. Recurrence developed in five patients (45%), requiring repeat steroid therapy and adjunctive treatment. Median serum levels of IgG4 reduced from 540 to 101 mg/dl in ten patients after systemic corticosteroid administration. Patients with lower serum IgG4 and IgG4:IgG ratio at diagnosis were associated with poor response (p = 0.037). CONCLUSIONS: Ocular adnexal IgG4-related disease predominantly involved the lacrimal glands bilaterally and was usually associated with high serum IgG4 levels and multiple organ involvement. Most patients responded well to steroid therapy, which was accompanied by a marked decrease in serum IgG4.

Microsporidial keratitis in patients with hot springs exposure.

This retrospective study included 10 eyes of 9 patients diagnosed with microsporidial keratitis. All of them were known to contract this disease after taking baths in hot springs. The disease was diagnosed based on detecting microsporidia in corneal scrapings using Gram stain and the modified Kinyoun's acid-fast stain. The specimens from the last six patients were subjected to PCR and then sequencing. All of them revealed that the microorganism identified has a high similarity to Vittaforma corneae. Repeated debridement of the epithelial lesions successfully eradicated the microsporidial infection in all nine patients.