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PurposeObservationsConclusionsUveal effusion syndrome (UES) is an exudative detachment of ciliary body, retina and choroid. Various underlying causes leads to UES-like drugs (topiramate), inflammation and hypotony. Wnt gene involvement has never been associated with UES. We report a case of bilateral UES being misdiagnosed as Vogt–Koyanagi–Harada syndrome (VKH), with Wnt gene dysfunction as the underlying trigger.A 32-year-old male presented with diminution of vision in his left eye. He was found to have choroidal detachment with retinal detachment in his left eye. Choroidal detachment was noted in the right eye. Various ocular imaging including fundus fluorescein angiography and ocular coherence tomography was done. He was misdiagnosed as a case of VKH syndrome, for which he was treated with systemic immunomodulatory therapy. However, a subclinical response was made to revisit the diagnosis. Ultrasound biomicroscopy showed supraciliary effusion. Systemic and genetic evaluation led to the detection of Wnt10A pathway mutation.UES is an entity of diagnostic challenge. Careful and thorough systemic evaluation is required to clinch the diagnosis. We reported the first case of bilateral UES recalcitrant to corticosteroids, with Wnt10A gene mutation. [ABSTRACT FROM AUTHOR]

Abstract is missing (Correspondence)

This report describes a unique case of systemic diffuse large B-cell lymphoma (DLBCL) with initial ocular manifestations of bilateral optic disc edema and serous retinal detachment (SRD). A 29-year-old man presented with altered color vision in the left eye, mild fever, weakness, and headache, followed by bilaterally reduced visual acuity. Anterior segment and vitreous examinations showed no inflammation with sluggish response of light reflex. His fundus examination revealed bilateral multiple SRDs and optic disc swelling with choroidal thickening. On fluorescein angiography, pinpoint hyperfluorescence, associated dye pooling, and optic disc staining with leakage were found bilaterally. Laboratory studies revealed elevated C-reactive protein and mild leukocytosis with neutrophil predominance. He was provisionally diagnosed with probable Vogt-Koyanagi-Harada syndrome and received methylprednisolone pulse therapy. Five days later, his systemic condition deteriorated following initial ocular symptom improvement. Whole-body computerized tomography revealed clustered lymphadenopathies, which were interpreted as DLBCL after lymph node biopsy. His ocular condition improved after DLBCL chemotherapy. We hope to promote early recognition with appropriate workups through this case and literature review.

Abstract Background Vogt‒Koyanagi‒Harada (VKH) disease is a multifactorial systemic autoimmune disorder against melanocytes that is characterized by panuveitis. Familial occurrence of VKH disease is rare. Here, we report two cases of a father and his son with characteristic manifestations of VKH disease. Case presentation A 53-year-old male with typical clinical symptoms of VKH disease was referred to Tangshan Eye Hospital. Examination showed the presence of ciliochoroidal effusion and exudative retinal detachment in both eyes. The patient was given intravenous methylprednisolone 120 mg for 2 days and intravenous methylprednisolone 80 mg for 1 day followed by 48 mg (1 mg/kg/day) oral methylprednisolone daily, accompanied by oral azathioprine 50 mg daily. Cycloplegic agent (0.5% tropicamide three times daily [TID]) was added. The patient was free of symptoms and recurrence within more than 1-year-follow-up period, the best corrected visual acuity (BVCA) was increased and maintained in both eyes with complete resolution of subretinal fluid. One year and nine months later, case 2 (his son) also presented with the typical clinical symptoms of VKH disease at 29 years of age. The son also recovered from VKH disease after routine and standard treatment. Conclusions To the best of our knowledge, this is the first VKH disease case report of a father-son relationship. Although genetic factors have been demonstrated to be involved in the pathogenesis of VKH disease, the different inheritance modes of VKH patients need to be further explored and studied.

Purpose: To report the outcome of surgical intervention for inflammatory, exudative retinal detachment (ERD). Methods: A retrospective analysis of eyes with ERD that underwent vitrectomy. Results: Twelve eyes (10 patients) with ERD, non-responsive to medical therapy, underwent vitrectomy. The mean age was 35.7 ± 17.7 years. Five eyes (42%) had Vogt–Koyanagi–Harada disease, three (25%) had presumed tuberculosis (TB), two (17%) pars planitis, and one (8%) had sympathetic ophthalmia. The mean time of vitrectomy was 6.76 ± 4.1 months after onset. Six (50%) eyes had a recurrence, two settled with medical treatment, and four underwent re-surgery. The mean follow-up was 2.7 years. At the last visit, 10 (83.3%) eyes had attached retina; the best-corrected visual acuity (BCVA) had reduced to 1.6 ± 0.7 logarithms of the minimum angle of resolution (logMAR) from 1.3 ± 0.7 at baseline. Conclusion: Vitrectomy in ERD can act as an adjuvant to conventional medical therapy and help maintain structural integrity. Early vitrectomy may help preserve visual function.

Purpose: To report the outcome of surgical intervention for inflammatory, exudative retinal detachment (ERD). Methods: A retrospective analysis of eyes with ERD that underwent vitrectomy. Results: Twelve eyes (10 patients) with ERD, non-responsive to medical therapy, underwent vitrectomy. The mean age was 35.7 ± 17.7 years. Five eyes (42%) had Vogt--Koyanagi--Harada disease, three (25%) had presumed tuberculosis (TB), two (17%) pars planitis, and one (8%) had sympathetic ophthalmia. The mean time of vitrectomy was 6.76 ± 4.1 months after onset. Six (50%) eyes had a recurrence, two settled with medical treatment, and four underwent re-surgery. The mean follow-up was 2.7 years. At the last visit, 10 (83.3%) eyes had attached retina; the best-corrected visual acuity (BCVA) had reduced to 1.6 ± 0.7 logarithms of the minimum angle of resolution (logMAR) from 1.3 ± 0.7 at baseline. Conclusion: Vitrectomy in ERD can act as an adjuvant to conventional medical therapy and help maintain structural integrity. Early vitrectomy may help preserve visual function. [ABSTRACT FROM AUTHOR]

Background: Vogt‒Koyanagi‒Harada (VKH) disease is a multifactorial systemic autoimmune disorder against melanocytes that is characterized by panuveitis. Familial occurrence of VKH disease is rare. Here, we report two cases of a father and his son with characteristic manifestations of VKH disease. Case presentation: A 53-year-old male with typical clinical symptoms of VKH disease was referred to Tangshan Eye Hospital. Examination showed the presence of ciliochoroidal effusion and exudative retinal detachment in both eyes. The patient was given intravenous methylprednisolone 120 mg for 2 days and intravenous methylprednisolone 80 mg for 1 day followed by 48 mg (1 mg/kg/day) oral methylprednisolone daily, accompanied by oral azathioprine 50 mg daily. Cycloplegic agent (0.5% tropicamide three times daily [TID]) was added. The patient was free of symptoms and recurrence within more than 1-year-follow-up period, the best corrected visual acuity (BVCA) was increased and maintained in both eyes with complete resolution of subretinal fluid. One year and nine months later, case 2 (his son) also presented with the typical clinical symptoms of VKH disease at 29 years of age. The son also recovered from VKH disease after routine and standard treatment. Conclusions: To the best of our knowledge, this is the first VKH disease case report of a father-son relationship. Although genetic factors have been demonstrated to be involved in the pathogenesis of VKH disease, the different inheritance modes of VKH patients need to be further explored and studied. [ABSTRACT FROM AUTHOR]

Case Summary: We present the case of a 50 year old male patient being treated for chronic myeloid leukemia by the tyrosine kinase inhibitor, Ponatinib. After 3 months of treatment, he developed a sight-threatening granulomatous panuveitis in both eyes, with choroidal effusions and neurosensory retinal detachments. Except for a positive interferon-gamma release assay suggesting previous Tuberculosis exposure, all uveitis investigations were normal. Discontinuation of the suspected causative drug led to resolution of signs and a consequent improvement in visual acuity. Conclusion: Ponatinib use may be associated with with a uveitic phenotype that is reminiscent of Harada's disease. We compare and contrast this rare ocular phenomenon with Vogt-Koyanagi-Harada syndrome and discuss a possible immunological basis. [ABSTRACT FROM AUTHOR]

This case demonstrates the dilemma in the diagnosis of ocular syphilis, as it can mimic many other ocular conditions where initial steroid therapy might complicate the disease course and can worsen the infection. Also, it represents an example of anchoring bias, where a provisional diagnosis resulted in unnecessary treatment that would have worsened her clinical outcomes. [ABSTRACT FROM AUTHOR]

Inflammation of sclera with involvement of uvea is an uncommon and underdiagnosed disease entity seen in an ophthalmological outpatient setting. A 34-year-old male presented with complaints of headache, redness, pain, photophobia, and blurring of vision in the left eye > right eye with associated tinnitus, decreased hearing, and recurrent severe headaches over 3 months. Detailed ophthalmological examination revealed signs of sclerouveitis. We thoroughly investigated the patient which revealed manifestations of incomplete VKH syndrome, along with strongly positive mantoux test hence, he was started on oral steroids under antituberculous treatment cover after physician consult. On follow-up, the patient was symptomatically better with resolution of inflammatory signs over a period of 3 months. The patient is being followed up by multispecialty approach to discern VKH manifestations of later disease.

Purpose: To report a case of systemic diffuse large B cell lymphoma presenting with ocular manifestations and neurologic findings resembling Vogt-Koyanagi-Harada syndrome. Observations: A 51-year-old Caucasian man presented with headache, ear pain, and blurry vision in both eyes. He was found to have bilateral exudative retinal detachments. After a short period of initial improvement with high dose systemic corticosteroid, his condition significantly worsened. An extensive work-up, including a kidney biopsy, led to a diagnosis of systemic diffuse large B cell lymphoma. He had excellent recovery following treatment with appropriate chemotherapy. Conclusions and Importance: Systemic malignancy may present with ocular manifestations and may masquerade as another diagnosis. An unexpected clinical course may suggest an alternative diagnosis. A broad systemic work-up including an evaluation for malignancy should be considered for patients presenting with unexplained exam or systemic findings.

Vogt-Koyanagi-Harada (VKH) syndrome is an immune-mediated granulomatous disease which affects melanin-rich organs like eyes, skin, nervous system, and ears. Neurological and auditory manifestations usually precede the involvement of other sites. Patients may manifest with “complete” or “incomplete” syndrome. We report two patients who presented with acute headache and impaired vision. Fundus examination revealed optic disc hyperemia and exudative retinal detachment which provided a clue for the diagnosis at the bedside. Fundus fluorescein angiogram (FFA) revealed abnormal dye leakage, whereas B scan showed choroid thickening. Cerebrospinal fluid (CSF) pleocytosis contrasted with unremarkable brain magnetic resonance imaging and lack of meningeal signs. Melanophagocytosis was evidenced by melanin-laden macrophages in CSF and skin biopsy. This finding is specific for VKH syndrome and helps to clinch the diagnosis even when the complete syndrome is not present cross-sectionally. VKH syndrome should be suspected in patients with aseptic meningitis if tests for common infectious and immune-mediated diseases are negative.

We aimed to describe a case of acute lymphoblastic leukemia (ALL) that initially presented and was managed as Vogt-Koyanagi-Harada syndrome (VKH). A 62-year-old man was referred for vision loss starting 1 week ago. There was no prior systemic or ocular history. Visual acuity was 20/200 in both eyes. Anterior segment exam was also normal in both eyes. On funduscopy, foveal reflex was significantly reduced. On optical coherence tomography, subretinal fluid was evident in both eyes. Fluorescein angiography showed a hypofluorescent area compatible with subretinal fluid and multiple pinpoint hyper- and hypofluorescent dots surrounding the detached retina. After 4 days, we were informed that the patient had been admitted to a general hospital due to spontaneous ecchymosis and melena. On complete blood count, there was a high white cell count, thrombocytopenia, and low hemoglobin concentration with a probable diagnosis of leukemia. On bone marrow biopsy, ALL with B-cell precursor was confirmed. In conclusion, exudative retinal detachment can be a primary presentation of leukemia and/or lymphoma. In atypical VKH or acute central serous chorioretinopathy cases with multiple pinpoint leakages distributed mostly in the periphery of the subretinal fluid area (not randomly scattered throughout the subretinal fluid), we should consider leukemia.

Abstract Background Vogt-Koyanagi-Harada syndrome is a rare disease characterized by skin and eyelash bleaching, chronic granulomatous iridocyclitis and exudative retinal detachment, and aseptic meningitis and encephalopathy. IgA nephropathy complicated by Vogt-Koyanagi-Harada syndrome is very rare, even though they might have similar pathogeneses. Ocular lesions often are not examined when patients are diagnosed with IgA nephropathy, which affects the prognosis. Case presentation We describe a 55-year-old male IgA nephropathy patient who was admitted with high fever and hematuria. Physical examination revealed impaired binocular vision with blurred vision, impaired hearing, and a congestive rash on the chest and back. Renal ultrasound examination showed no abnormalities. Laboratory examination showed that glomerulonephritis was complicated by infection, and anti-infection therapy was ineffective. Bilateral fluorescein angiography showed Vogt-Koyanagi-Harada syndrome. Further renal biopsy confirmed IgA nephropathy. Hormone shock therapy and cyclophosphamide adjuvant therapy were administered, and the patient’s symptoms improved. Conclusion For the first time, we reported the case of simultaneous onset of IgA nephropathy and Vogt-Koyanagi-Harada syndrome, which is very rare. The onset of Vogt-Koyanagi-Harada syndrome is rapid and serious, while that of IgA nephropathy is relatively milder, making it easy for specialized doctors to neglect this condition. Doctors should be highly alert to the clinical concomitant occurrence of the two diseases with similar mechanisms, especially in the case of neurological defects and ocular symptoms in IgA nephropathy patients, since timely immunosuppressive treatment may improve the outcome of ocular diseases.

Autoimmune uveitis is characterized by immune disorders of the eyes and the whole body and is often recurrent in young adults, but its pathogenesis is still unclear. IL-35 is an essential regulatory factor in many autoimmune diseases, which is produced by Breg cells and can induce Breg cells to regulate the immune response. The relationship between the expression and gene polymorphism of IL-35 and the immune status of patients with autoimmune uveitis has not been reported. The peripheral blood of the subjects was collected from patients with Behçet's Disease (BD) and those with Vogt–Koyanagi–Harada (VKH) syndrome. The percentage of immune cell subsets including B cells, DC, and T cells, and the expression of IL-35 in serum of these two kinds of disease were analyzed. And then, the associations between seven IL-35 single nucleotide polymorphism (SNP) sites and disease susceptibility, the immune status, the clinical characteristics, and the serum IL-35 levels were analyzed. Our results showed that the percentage of Breg cells was significantly decreased in the blood of patients with VKH syndrome compared to that of healthy controls. The levels of IL-35 in the serum of patients with VKH syndrome or BD patients were not changed significantly, compared to that of healthy controls. Furthermore, the associations between two subunits of IL-35 (IL-12p35 and EBI3) and BD or VKH patients were analyzed. We found that there was an association between the EBI3 rs428253 and the occurrence of BD. There was an association between the IL-12p35 rs2243131 and the low level of Breg cell of VKH patients. In addition, there were associations between the polymorphisms of EBI3 rs4740 and the occurrence of headache and tinnitus of VKH patients, respectively. And the genotype frequency of IL-12p35 rs2243115 was related to the concentration of serum IL-35 in patients with VKH syndrome. Thus, the specific SNP sites change of IL-35 were correlated to the immune disorders in uveitis. And they may also play a guiding role in the occurrence of clinical symptoms in patients with uveitis, especially for VKH syndrome. [ABSTRACT FROM AUTHOR]

Autoimmune uveitis is characterized by immune disorders of the eyes and the whole body and is often recurrent in young adults, but its pathogenesis is still unclear. IL-35 is an essential regulatory factor in many autoimmune diseases, which is produced by Breg cells and can induce Breg cells to regulate the immune response. The relationship between the expression and gene polymorphism of IL-35 and the immune status of patients with autoimmune uveitis has not been reported. The peripheral blood of the subjects was collected from patients with Behçet’s Disease (BD) and those with Vogt–Koyanagi–Harada (VKH) syndrome. The percentage of immune cell subsets including B cells, DC, and T cells, and the expression of IL-35 in serum of these two kinds of disease were analyzed. And then, the associations between seven IL-35 single nucleotide polymorphism (SNP) sites and disease susceptibility, the immune status, the clinical characteristics, and the serum IL-35 levels were analyzed. Our results showed that the percentage of Breg cells was significantly decreased in the blood of patients with VKH syndrome compared to that of healthy controls. The levels of IL-35 in the serum of patients with VKH syndrome or BD patients were not changed significantly, compared to that of healthy controls. Furthermore, the associations between two subunits of IL-35 (IL-12p35 and EBI3) and BD or VKH patients were analyzed. We found that there was an association between the EBI3 rs428253 and the occurrence of BD. There was an association between the IL-12p35 rs2243131 and the low level of Breg cell of VKH patients. In addition, there were associations between the polymorphisms of EBI3 rs4740 and the occurrence of headache and tinnitus of VKH patients, respectively. And the genotype frequency of IL-12p35 rs2243115 was related to the concentration of serum IL-35 in patients with VKH syndrome. Thus, the specific SNP sites change of IL-35 were correlated to the immune disorders in uveitis. And they may also play a guiding role in the occurrence of clinical symptoms in patients with uveitis, especially for VKH syndrome.

A splitting of the outer plexiform retinal layer in a saw-like hyporeflective pattern in addition to partially formed concentric circles centred at the foveola were observed using en-face OCT and OCT-angiography in a 27-year-old female patient with rhegmatogenous retinal detachment and a 50-year-old female patient with Vogt-Koyanagi-Harada chorioretinopathy.

AIM:To investigate the changes of Notch receptors and interleukin(IL)-22 expression in patients with Vogt-Koyanagi-Harada(VKH)syndrome, and to assess the regulatory activity of Notch signaling to IL-22 production by CD4+ T cells in patients with VKH syndrome.METHODS: Thirty-five patients with VKH syndrome(including fifteen active VKH and twenty inactive VKH)and twelve healthy controls were enrolled. Plasma was isolated, and CD4+T cells were purified. Notch receptors were investigated by qRT-PCR and Western blot. Plasma IL-22 expression was measured by ELISA. The percentage of Th17 and Th22 cells was investigated by flow cytometry. CD4+T cells, which were purified from active VKH patients, were stimulated with Notch signaling inhibitor DAPT. mRNA expression of transcription factor in CD4+T cells as well as IL-22 secretion by CD4+T cells was investigated.RESULTS: Notch1-Notch3 in CD4+T cells from active VKH syndrome patients was significantly elevated in comparison with inactive VKH and healthy controls. Plasma IL-22 expression and percentage of Th17 and Th22 was notably increased in active VKH syndrome in comparison with inactive VKH and controls. DAPT stimulation inhibited Notch signaling pathway in CD4+T cells, leading to the down-regulation of AhR mRNA and IL-22 secretion.CONCLUSION:Notch-AhR-IL-22 signaling pathway might take part in the pathogenesis of VKH syndrome.

ABSTRACT Vogt-Koyanagi-Harada (VKH) syndrome is an inflammatory condition of unknown etiology that can affect the eye. The most common ocular manifestation related to VKH is bilateral diffuse uveitis associated to exudative retinal detachment. Although these patients respond well to steroid pulse therapy, we report a case of a 44-year-old female patient presenting bilateral exudative retinal detachment and clinical diagnosis of VKH, who did not respond to the first cycle of 3-day pulse therapy with methylprednisolone. The exudation was reabsorbed only after a second cycle of steroid therapy.

Vogt-Koyanagi-Harada (VKH) syndrome is an inflammatory condition of unknown etiology that can affect the eye. The most common ocular manifestation related to VKH is bilateral diffuse uveitis associated to exudative retinal detachment. Although these patients respond well to steroid pulse therapy, we report a case of a 44-year-old female patient presenting bilateral exudative retinal detachment and clinical diagnosis of VKH, who did not respond to the first cycle of 3-day pulse therapy with methylprednisolone. The exudation was reabsorbed only after a second cycle of steroid therapy. [ABSTRACT FROM AUTHOR]

Vogt-Koyanagi-Harada (VKH) syndrome is an immune-mediated granulomatous disease which affects melanin-rich organs like eyes, skin, nervous system, and ears. Neurological and auditory manifestations usually precede the involvement of other sites. Patients may manifest with "complete" or "incomplete" syndrome. We report two patients who presented with acute headache and impaired vision. Fundus examination revealed optic disc hyperemia and exudative retinal detachment which provided a clue for the diagnosis at the bedside. Fundus fluorescein angiogram (FFA) revealed abnormal dye leakage, whereas B scan showed choroid thickening. Cerebrospinal fluid (CSF) pleocytosis contrasted with unremarkable brain magnetic resonance imaging and lack of meningeal signs. Melanophagocytosis was evidenced by melanin-laden macrophages in CSF and skin biopsy. This finding is specific for VKH syndrome and helps to clinch the diagnosis even when the complete syndrome is not present cross-sectionally. VKH syndrome should be suspected in patients with aseptic meningitis if tests for common infectious and immune-mediated diseases are negative. [ABSTRACT FROM AUTHOR]

Background. Vogt-Koyanagi-Harada Syndrome is rare in childhood and is usually seen between the 2nd and 5th decades. We present a 15-year-old girl with findings of incomplete Vogt-Koyanagi-Harada Syndrome. Case. In the first visit, anterior chamber inflammation, vitritis, serous retinal detachment and papillitis were observed in her both eyes. She also had neurological symptoms such as a headache. During the systemic treatment period, some of the side effects related to steroids emerged. Additionally, the symptoms and findings of the disease relapsed while the steroid dose was reduced. Conclusion. Early diagnosis and selection of an individualized appropriate treatment provided good clinical and visual results without any serious complications in our case. [ABSTRACT FROM AUTHOR]

Vogt-Koyanagi-Harada (VKH) syndrome is a systemic autoimmune disorder that targets tissues containing melanocytes such as the eye, inner ear, meninges and skin. Despite a common genetic susceptibility, the association between VKH syndrome and rheumatoid arthritis (RA) has been rarely reported. To report a rare case with RA who developed incomplete VKH syndrome. The case is described and a review of the literature on similar cases is presented. A 26 year-old Tunisian woman, with a medical history of Hashimoto's thyroiditis, was diagnosed on 2011 with seropositive and erosive RA treated with Leflunomide. She presented to the emergency department on June 2018 with bilateral blurred vision associated with photophobia, vomiting and severe headache that had gradually progressed over the preceding five days. Ophthalmological examinations showed typical findings of VKH syndrome. The patient received intravenous infusions of methylprednisolone at a daily dose of 1000 mg for 3 days that was followed orally with 2 mg/kg of prednisone equivalent. Given the lack of improvement in visual acuity after 3 weeks of treatment, azathioprine was added and VKH remission was achieved on September 2018 as confirmed on optical coherence tomography. However, the patient passed away on October 2018 due to infectious complications of the immunosuppressant agents. Treatments and outcomes of VKH are variable. Pharmacological management of such an association between RA and VKH may be challenging, so care must be taken to balance treatment escalation with adverse events in patients at risk. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of intravitreal methotrexate treatment and fluocinolone acetonide (Retisert®) implantation in a patient with Vogt-Koyanagi-Harada syndrome (VKH). Observations: A 34-year-old male was referred for worsening vision and bilateral panuveitis consistent with VKH. He was treated with prednisone, mycophenolate mofetil, prednisolone acetate eye drops, and injections of triamcinolone and adalimumab. He failed to improve with these therapies and developed multiple adverse effects, including hepatotoxicity, severe eye pain, cataracts, and cystoid macular edema. We treated him with intravitreal methotrexate injections in both eyes, which rapidly improved his eye pain, inflammation, and vision. He subsequently underwent fluocinolone acetonide (Retisert®) implantation, cataract extraction with intraocular lens insertion, and Ahmed tube placement for long-term intraocular pressure control. His vision improved from hand motions to 20/30, intraocular pressure remained stable at 17, there was complete resolution of his panuveitis and uveitic macular edema, and his systemic medications were able to be discontinued. Conclusions: /Importance: This case demonstrates intravitreal methotrexate may successfully treat intraocular inflammation, pain, and macular edema in VKH. Excellent long-term vision and reduction of adverse effects of systemic medications were also achieved with subsequent fluocinolone acetonide implantation. Combining these two targeted therapies may be an effective strategy in treating VKH in patients who have severe pain and cannot tolerate systemic therapy.

Objective: To describe the favorable clinical outcome in a case with bilateral choroidal neovascular membrane and quiescent Vogt-Koyanagi-Harada (VKH) syndrome by administering bilateral intravitreal aflibercept injections.Case report: A 30-year-old woman was diagnosed with VKH syndrome at another institution and had been in remission with oral mycophenolate mofetil for two years. However, nearly simultaneous right juxtafoveal and left subfoveal type 2 choroidal neovascular membrane was detected two years after the initial diagnosis. The right eye (OD) received three and the left eye (OS) received four aflibercept injections within a time span of eight months. Visual acuity was 20/30 in OD and 20/25 in OS at the last follow-up visit.Conclusion: Although suppression of inflammation is a must in eyes with inflammatory type choroidal neovascular membranes, anti-VEGF (vascular endothelial growth factor) therapy with agents, such as aflibercept in the present case, is a key therapeutic adjunct and may possibly help improve the visual prognosis.

Background: Vogt-Koyanagi-Harada syndrome is a rare disease characterized by skin and eyelash bleaching, chronic granulomatous iridocyclitis and exudative retinal detachment, and aseptic meningitis and encephalopathy. IgA nephropathy complicated by Vogt-Koyanagi-Harada syndrome is very rare, even though they might have similar pathogeneses. Ocular lesions often are not examined when patients are diagnosed with IgA nephropathy, which affects the prognosis.Case Presentation: We describe a 55-year-old male IgA nephropathy patient who was admitted with high fever and hematuria. Physical examination revealed impaired binocular vision with blurred vision, impaired hearing, and a congestive rash on the chest and back. Renal ultrasound examination showed no abnormalities. Laboratory examination showed that glomerulonephritis was complicated by infection, and anti-infection therapy was ineffective. Bilateral fluorescein angiography showed Vogt-Koyanagi-Harada syndrome. Further renal biopsy confirmed IgA nephropathy. Hormone shock therapy and cyclophosphamide adjuvant therapy were administered, and the patient's symptoms improved.Conclusion: For the first time, we reported the case of simultaneous onset of IgA nephropathy and Vogt-Koyanagi-Harada syndrome, which is very rare. The onset of Vogt-Koyanagi-Harada syndrome is rapid and serious, while that of IgA nephropathy is relatively milder, making it easy for specialized doctors to neglect this condition. Doctors should be highly alert to the clinical concomitant occurrence of the two diseases with similar mechanisms, especially in the case of neurological defects and ocular symptoms in IgA nephropathy patients, since timely immunosuppressive treatment may improve the outcome of ocular diseases. [ABSTRACT FROM AUTHOR]

Purpose To evaluate the characteristics of Vogt-Koyanagi-Harada (VKH) syndrome in Brazilian children.Methods Clinical data were obtained from the medical records of six children with VKH disease from March 2014 to June 2018 at the Federal University of São Paulo, Brazil.Results Six patients met the diagnostic criteria for VKH. The patients, who ranged in age from 5 to 8 years, all presented with chronic disease. The most common ocular finding was abnormal fundus pigmentation in five patients followed by band keratopathy in four. Two patients had glaucoma, cataract, and subretinal neovascularization. The final visual acuity was less than 20/40 in 50% of the eyes.Conclusion Visual outcomes were favorable in half of patients depending on the long-term sequelae. VKH is rarely reported in children and it may be sight-threatening, and requires careful attention, being an important differential diagnosis. [ABSTRACT FROM AUTHOR]

An 18-year-old female patient applied to our clinic owing to sudden visual loss which started 3 days ago on the right eye. There were +3 cells in the anterior chamber, +3 cells in the vitreous and multiple serous retinal detachment in the right eye. The anterior segment and fundus were normal in the left eye. Optic disc leakage in both eyes and hyperfluorescent areas due to subretinal fluid pooling in the phase in the right eye were detected in fundus fluoresceine angiography. Hypofluorescent foci were detected in the mid-late phase in both eyes in indocyanine green angiography. A marked thick choroid (690 μm) in optical coherence tomography and decreased choriocapillaris fl ow rate in right eye compared to left eye in optical coherence tomografiangiography were detected. Pleocytosis was detected in the lumbar puncture. Based on these findings, the patient was diagnosed as bilateral asymmetric onset acute Vogt-Koyanagi-Harada and treated with 1 g intravenous pulse steroid treatment. On the third day of the pulse steroid treatment, the amount of subretinal fl uid significantly decreased and choriocapillaris fl ow rate significantly increased in the right eye and slightly increased in the left eye. On the 7th day, 1 mg / kg / day oral steroid and 100 mg azothiopurine treatment were started after complete resolution of subretinal fl uid and increase of visual acuity up to 0.5. [ABSTRACT FROM AUTHOR]

Vogt-Koyanagi-Harada(VKH)syndrome is an autoimmune disease attacking against pigmented cells, resulting in blindness and usually affecting multiple organs including ears, meninges, hair and skin. Correct diagnosis and immediate treatment in the early stage is vital to visual prognosis. Currently, corticosteroids is first-line drug. In addition, VKH patients refractory to corticosteroids can choose other treatment such as immunosuppressive agents and biological agents.

A 43-year-old male with chronic Vogt–Koyanagi–Harada syndrome (VKH) presented with subfoveal choroidal neovascular membrane (CNVM) in the right eye with no evidence of active inflammation. He underwent intravitreal bevacizumab and dexamethasone injections. Postinjection he developed fresh keratic precipitates and exudative retinal detachment (RD). He received two more bevacizumab injections with oral corticosteroids and immunosuppressants causing resolution of exudative RD with scarred CNVM. We report this case to highlight that intravitreal injection may act as a trigger for rebound inflammation in VKH patients and may require anti-inflammatory drugs to be started even in the absence of an active inflammation.

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AIM:To discuss the diagnosis of Vogt-Koyanagi-Harada syndrome(VKHS)and evaluate the clinical effect of different corticosteroid therapies. METHODS: Forty-five patients(90 eyes)from January 2010 to February 2014 with VKHS were enrolled in this retrospective case study. The patients were divided into two groups. Twenty-five patients(50 eyes)in observation group were treated with methylprednisolone(1.0g)for 3 days. Then the methylprednisolone was reduced to 0.5g per day in the fourth and fifth day. Twenty patients(40 eyes)in control group were treated with dexamethasone(12mg)for 5 days. Then 60mg of prednisone were given to all the 45 patients and reduced according to the patients' inflammatory, taken off 5mg every time. When the dose was reduced to 15～20mg, the treatment was sustained for 6mo at least. Then the dose was reduced again till prednisone was taken off. The total course of treatment was more than 9mo. All patients underwent B type ultrasound scan, optical coherence tomography(OCT)and fundus fluorescein angiography(FFA)before and after treatments. The changes were analyzed. RESULTS: There was no statistical significance on vision between the two groups before treatments. Vision of all patients improved after treatments, and that in observation group(0.44±0.19)were significantly better than that in control group(0.55±0.29)at the fifth day(PP>0.05). Thirty days after treatment, vision of the two groups was almost back to normal. According to OCT, 84 eyes(93%)had macular neuroepithelial detachment or serous macular detachment, and height of detachment was 1009.67±319.40 μm in observation group, 1098.13±283.45μm in control group. Five days after treatments, the height of detachment in observation group was 307.79±71.35μm, significantly lower than control group(434.13±88.67μm)(PP>0.05). And 30 days after treatments, the morphology of fovea was almost back to normal. One month after treatments, the FFA results were almost normal, expect 8 eyes had slightly leakage.CONCLUSION: Large dose of methylprednisolone in early stage can improve the VKH patients' vision and promote the subretinal effusion absorption, which has better clinic effect and less adverse reaction than dexamethasone, is worth spread.

Introduction: Vogt-Koyanagi-Harada syndrome (VKHS) is an inflammatory systemic autoimmune disease principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Patients are generally women in the fourth decade of life. The prognosis is correlated mainly with the time between diagnosis and the start of treatment and number of recurrent episodes of inflammation. Most complications are mainly ocular. The purpose of this paper is to describe a clinical case of VKHS.Material and methods: A child with a challenging clinical presentation in which the dermatological symptoms occurred before ocular manifestations.Discussion and Conclusion: VKHS is rare in children and can be a diagnostic challenge. It seemed interesting to share this case as an opportunity to expand our knowledge of the clinical spectrum of diseases and reflect about current diagnostic criteria. [ABSTRACT FROM AUTHOR]

Purpose: To report the imaging characteristics of acute Vogt-Koyanagi-Harada (VKH) disease using optical coherence tomography angiography (OCTA).Methods: In this prospective study, patients with acute VKH (n = 10; mean age: 30.5 ± 13.43 years) underwent multimodal imaging (baseline and follow-up) using fundus photography, fluorescein angiography (FA), indocyanine green angiography (ICGA), OCT, and OCTA. The OCTA images were analyzed to assess the retinochoroidal vasculature and compared with other imaging techniques.Results: During the active stage, all eyes showed multiple foci of choriocapillaris flow void that correlated with ICGA. These foci decreased in number and size after initiation of therapy. In one patient, flow void areas reappeared after cessation of therapy without any detectable change on ICGA. This patient soon developed clinical recurrence requiring re-initiation of immunosuppression.Conclusions: OCTA allows high-resolution imaging of inflammatory foci suggestive of choriocapillaris hypoperfusion in acute VKH disease non-invasively. OCTA may be very helpful in the follow-up of such patients. [ABSTRACT FROM AUTHOR]

Purpose: We report a case of laryngeal edema associated with the Vogt-Koyanagi-Harada (VKH) syndrome. Patient and Methods: A 32-year-old African-American female presented with a 12-day prodrome, including headache, tinnitus and shortness of breath, which preceded sudden photophobia and bilateral visual loss. Examination and clinical testing were most consistent with VKH, and the patient improved with intravenous methylprednisolone therapy. Results: The patient had persistent dyspnea, which was out of proportion to chest CT findings and which was exacerbated during a recurrence of VKH. Flexible fiberoptic laryngoscopy with stroboscopy revealed diffuse laryngeal edema. Symptoms were alleviated with breathing exercises. Conclusions: Several autoimmune diseases may cause diffuse laryngeal edema. In this case, VKH was associated with the patient's glottic edema and dyspnea. We recommend that laryngeal edema be considered in the differential diagnosis for patients with dyspnea and VKH.

AIM: To analyze features of optical coherence tomography(OCT)in Vogt-Koyanagi-Harada syndrome before and after therapy.METHODS: Thirty-four eyes of 17 patients, which were identified as Vogt-Koyanagi-Harada syndrome, were examined with OCT and fundus photography, after the treatment of steroid therapy, the change of retinal structure were observed after 5, 15, 30d. RESULTS: OCT revealed retinal neuroepithelial edema and serous detachment of retinal neuroepithelia and subretinal septa that divided the subretinal space into several compartments all of cases. After steroid therapy, the retinal edema, serous detachment and subretinal septa were improved, even resolved completely. CONCLUSION: OCT can observe retinal structural change of Vogt-Koyanagi-Harada syndrome before and after therapy, it is vital for diagnosis and treatment evaluation of the disease.

Background: The aim of this study is to determine the differences in optical coherence tomography angiography (OCTA) features of acute Vogt-Koyanagi-Harada disease (VKH) and acute central serous chorioretinopathy (CSC). Clinical and imaging data of patients with acute CSC and VKH in a tertiary-care institute were analyzed. Multimodal imaging including fluorescein angiography, indocyanine green angiography (ICGA), and enhanced-depth imaging OCT were performed. OCTA images were analyzed for alterations in retinochoroidal microvasculature. Results: Thirty-four eyes (24 patients; 10 with VKH and 14 with CSC) were included. OCTA en face images showed apparent areas of choriocapillaris flow void due to shadowing effect from overlying subretinal fluid and pigment epithelial detachment in CSC. However, eyes with VKH showed presence of true choriocapillaris flow void on OCTA that corresponded to choriocapillaris ischemia on ICGA. Conclusions: OCTA is a useful tool to assess choriocapillaris ischemia in VKH and is helpful to differentiate it from CSC in the acute stage. [ABSTRACT FROM AUTHOR]

Purpose: To report the multimodal imaging findings and follow-up of a case of graftversus- host disease-induced bilateral panuveitis and serous retinal detachments after allogenic bone marrow transplant for acute myeloid leukemia. Methods: A 75-year-old black man presented with acute decreased vision in both eyes for 1 week. Clinical examination and multimodal imaging, including spectral domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, and swept-source optical coherence tomography angiography (Investigational Device; Carl Zeiss Meditec Inc) were performed. Results: Clinical examination of the patient revealed anterior and posterior inflammation and bilateral serous retinal detachments. Ultra-widefield fundus autofluorescence demonstrated hyperautofluorescence secondary to subretinal fluid; and fluorescein angiography revealed multiple areas of punctate hyperfluorescence, leakage, and staining of the optic discs. Spectral domain and enhanced depth imaging optical coherence tomography demonstrated subretinal fluid, a thickened, undulating retinal pigment epithelium layer, and a thickened choroid in both eyes. En-face swept-source optical coherence tomography angiography did not show any retinal vascular abnormalities but did demonstrate patchy areas of decreased choriocapillaris flow. An extensive systemic infectious and malignancy workup was negative and the patient was treated with high-dose oral prednisone immunosuppression. Subsequent 6-month follow-up demonstrated complete resolution of the inflammation and bilateral serous detachments after completion of the prednisone taper over a 3-month period. Conclusion: Graft-versus-host disease panuveitis and bilateral serous retinal detachments are rare complications of allogenic bone marrow transplant for acute myeloid leukemia and can be diagnosed with clinical and multimodal imaging analysis. This form of autoimmune inflammation may occur after the recovery of T-cell activity within the donor graft targeting the host. Infectious and recurrent malignancy must be ruled out before initiation of immunosuppression, which can affectively treat this form of graft-versus-host disease. [ABSTRACT FROM AUTHOR]

Ligneous conjunctivitis (LC) is a rare form of bilateral chronic recurrent disease in which thick membranes form on the palpebral conjunctiva and other mucosal sites. We report the clinical features and describe the management of two cases. Case 1 was an 8-month-old patient with bilateral membranous conjunctivitis. Case 2 was a 5-year-old patient with unilateral membranous conjunctivitis, esotropia, mechanical ptosis and complicated cataract, and had been treated with a number of medications. Histological investigation of the membrane in both cases showed LC. Treatments with amniotic membrane transplantation and institution of topical cyclosporine have shown good response. There has been complete resolution of the membranes with no recurrence at the end of 40- and 28-month follow-ups, respectively. No treatment related side effects were seen. Thus, it appears that amniotic membrane transplantation and topical cyclosporine are effective alternatives for the treatment of LC.

The purpose of this study was to measure peripapillary retinal nerve fiber layer thickness (RNFLT) using spectral domain optical coherence tomography (SD-OCT) in normal Indian eyes, for which, 210 normal volunteers were recruited. One eye of each subject underwent RNFL scanning at 3.4 mm circle diameter around optic nerve using SD OCT. The data were analyzed to determine RNFLT in the sample population and its variation with age and gender. The average peripapillary RNFLT was 114.03 ± 9.59 μm. There was no effect of gender on RNFLT parameters. Age had significant negative correlation with average (P = 0.005), superior (P = 0.04), temporal (P = 0.049), and nasal quadrants (P = 0.01) RNFLT. Inferior quadrant RNFLT also had a negative correlation with age, but it was not statistically significant (P = 0.15).

A 2-year-old, intact male Siberian Husky was presented with corneal opacity, eye lid swelling, depigmentation, alopecia and erythema predominantly on face. Bilateral uveitis preceded periocular and nasal planum depigmentation. Ancillary tests excluded other systemic diseases. Histopathology of the skin revealed lichenoid interface dermatitis with pigmentary incontinence. Diffuse pyogranulomatous inflammation of the globes was also noted. Based on the clinical signs and characteristic histopathological findings, the dog was diagnosed as uveodermatologic syndrome (UDS). Further treatment was not attempted in this case and the dog was euthanized. This case report describes typical clinical and histopathologic features of UDS in a Siberian Husky.

Introduction: Vogt-Koyanagi-Harada's disease is a rare syndrome that affects tissues with melanocytes like eyes, central nervous system, skin and inner ear. It affects primarily Asians, Indians and Latin Americans and also women. Objective: To review in the literature the several aspects of Vogt-Koyanagi-Harada's disease focusing on its cause and also to study its otolaryngology aspects through online databases Cochrane, LILACS, MEDLINE, OMIM e SciELO. Literature Review: This exact cause remains unknown, but there are evidences of an autoimmune process against an antigenic component on the melanocyte configuring an inflammatory T-cell-mediated immune response. The HLADRB1* 0405 allele is the one that is most associated to the disease. The clinic manifestations are divided in four: prodromal, uveitic, chronic and recurrence stages. The otolaryngology's aspects occur at the uveitic stage. Hearing loss is sensorineural bilateral and quickly progressive and could be associated with tinnitus. The vestibular component is less affected with vertigo, nystagmus and abnormal vestibular reflex. The otolaryngology's manifestations do not influence the recurrences or complications and they also have great prognosis with the treatment established. The diagnosis of this syndrome is based on clinical criteria, but audiological test could help. The therapy is corticosteroid-based. Final Comments: The most important factor for prognosis is the immediately treatment. The rarity of this syndrome makes its diagnosis a challenge and the presence of deafness, tinnitus and vertigo must consider its diagnosis.

This report describes a unique case of systemic diffuse large B-cell lymphoma (DLBCL) with initial ocular manifestations of bilateral optic disc edema and serous retinal detachment (SRD). A 29-year-old man presented with altered color vision in the left eye, mild fever, weakness, and headache, followed by bilaterally reduced visual acuity. Anterior segment and vitreous examinations showed no inflammation with sluggish response of light reflex. His fundus examination revealed bilateral multiple SRDs and optic disc swelling with choroidal thickening. On fluorescein angiography, pinpoint hyperfluorescence, associated dye pooling, and optic disc staining with leakage were found bilaterally. Laboratory studies revealed elevated C-reactive protein and mild leukocytosis with neutrophil predominance. He was provisionally diagnosed with probable Vogt-Koyanagi-Harada syndrome and received methylprednisolone pulse therapy. Five days later, his systemic condition deteriorated following initial ocular symptom improvement. Whole-body computerized tomography revealed clustered lymphadenopathies, which were interpreted as DLBCL after lymph node biopsy. His ocular condition improved after DLBCL chemotherapy. We hope to promote early recognition with appropriate workups through this case and literature review., Competing Interests: Dr. Ju-Chuan Yen and Dr. Shiow-Wen Liou, the editorial board members at Taiwan Journal of Ophthalmology, had no roles in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper., (Copyright: © 2023 Taiwan J Ophthalmol.)

Vogt–Koyanagi–Harada syndrome (VKH) is a bilateral, diffuse granulomatous uveitis associated with neurological, audiovestibular, and dermatological systems. The primary pathogenesis is T-cell-mediated autoimmune response directed towards melanocyte or melanocyte-associated antigens causing inflammation of the choroidal layer. This phenomenon usually leads to diffuse inflammatory conditions throughout most parts of eye before ocular complications ensue. The diagnosis is achieved mainly by clinical features according to the revised diagnostic criteria of VKH published in 2001, without confirmatory serologic tests as a requirement. However, ancillary tests, especially multimodal imaging, can reliably provide supportive evidence for the diagnosis of early cases, atypical presentations, and evaluation of management. Prompt treatment with systemic corticosteroids and early non-steroidal immunosuppressive drug therapy can lessen visually threatening ocular complications and bring about good visual recovery. Close monitoring warrants visual stabilization from disease recurrence and ocular complications. This article review aims not only to update comprehensive knowledge regarding VKH but also to emphasize three major perspectives of VKH: immunogenetics as the major pathogenesis of the disease, multimodal imaging, and therapeutic options. The role of anti-vascular endothelial growth factor therapy and drug-induced VKH is also provided. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this article is to 'determine' the scope of Goya's eyesight difficulties and assess the extent to which those difficulties might explain his style of painting in the last years of his life. Methods: We analyse the correspondence and late works of the Aragonese painter Francisco de Goya y Lucientes (1746–1828), who has been admired for his use of colour, his energetic loose brushstrokes, his disregard for details and his bold compositions, as well as for his different artistic styles throughout his life. Results: The evolution of Goya's style of painting in his later works seems to have been the consequence of an eyesight condition, probably age‐related cataracts at an advanced stage. The faded dark backgrounds, which become blurred with the silhouette of the person portrayed, could indicate a certain degree of eye strain. This can be traced in all these works, but is especially evident in the unfinished portrait of Pío de Molina (1827–1828), as well as in the portraits of Mariano Goya, the artist′s grandson (1827), and Jacques Galos (1826). Conclusion: It has been considered that the late and isolated Goya′s sight problems were a belated consequence of his severe illness of 1792. Nevertheless, in our opinion, this is a simplistic explanation and, given the painter's age, it is logical to presume that their cause could be age‐related lens opacities. This article argues that medicine may become a subsidiary science to art history, as it can provide empirical evidence of the way painters' illnesses may have a strong impact on their artworks. [ABSTRACT FROM AUTHOR]