Two case reports of acute zonal occult outer retinopathy (AZOOR): Importance of multimodal diagnosis.

A presentation is made of two cases of acute zonal occult outer retinopathy (AZOOR); one a young man of 19 years, and the other a 42-year-old woman. The young man complained of unilateral scotoma and photopsia. The woman presented with bilateral visual loss and photopsia. Multimodal imaging, including fundus photography, fluorescein angiography, fundus autofluorescence, spectral-domain optical coherence tomography, and visual field testing, supported the diagnosis of AZOOR. The differential diagnosis is complicated, since it has clinical features in common with other retinopathies. This means that it is essential to use modern imaging tests, especially those where the characteristic trizonal pattern is shown, such as in autofluorescence and OCT.

Chronic cytomegalovirus necrotizing retinitis in a patient with scleroderma and mixed connective tissue disease.

The case is presented of a 52-year-old woman with scleroderma, mixed connective tissue disease, and interstitial lung disease, who developed chronic cytomegalovirus necrotizing retinitis while on treatment with prednisone, mycophenolate, and hydroxychloroquine. Initially diagnosed as macular hole, the patient underwent a pars plana vitrectomy. Two months after surgery, due to progressive worsening, the diagnosis was made and treatment started (intravenous and intravitreal ganciclovir). The patient developed severe macular atrophy with final visual acuity of counting fingers. A chronic retinal necrosis can be caused by cytomegalovirus infection in non-HIV patients with partial immune dysfunction from other causes, characterized by a slowly progressive granular retinitis with occlusive vasculitis.

Multifocal presentation in 2 cases of unilateral acute idiopathic maculopathy. / Presentación multifocal en 2 casos de maculopatía unilateral aguda idiopática.

Two cases of multifocal unilateral acute idiopathic maculopathy are presented, one in a 24 year-old man, and another in a 37 year-old woman. Both of them presented with acute vision loss and clinical findings compatible with unilateral acute idiopathic maculopathy. As a relatively uncommon finding, they had multifocal lesions around a larger central lesion. They experienced a spontaneous improvement of their vision. Atypical presentations of unilateral acute idiopathic maculopathy like multifocal lesions are possible. Ophthalmologists should be aware of this rare form of presentation.

Immune recovery uveitis in a patient with herpes retinitis as a complication of hairy cell leukaemia. / Respuesta similar a la uveítis de recuperación inmune en un paciente con retinitis herpética como complicación de una leucemia de células peludas.

A 51 year-old man with hairy cell leukaemia was treated with pentostatin. While receiving the treatment, he was diagnosed with herpes retinitis in his right eye. After the last cycle of pentostatin the patient developed a mild vitritis and cystoid macular oedema. There were no signs of herpes retinitis reactivation. After excluding other possible causes of intraocular inflammation, a diagnosis of immune recovery uveitis was made. The patient was treated with 2-monthly retro-septal injections of triamcinolone, oral corticosteroids, intravitreal dexamethasone implants and, finally, pars plana vitrectomy. An immune recovery uveitis-like response is possible in HIV negative individuals. The immune reconstitution after the treatment of hairy cell leukaemia may have led to intraocular inflammation. Management of immune recovery uveitis is challenging and difficult. Pars plana vitrectomy may be necessary. Ophthalmologists should be alert to the possibility of immune recovery uveitis in HIV negative patients.

Diagnosis of Eales disease from a macular epiretinal membrane. / Diagnóstico de enfermedad de Eales a partir de una membrana epirretiniana.

Macular involvement is a common finding in patients with Eales disease. The purpose of this communication is to describe the diagnosis of Eales disease from the finding of a macular epiretinal membrane in a young patient. The case is presented of a 38-year-old man referred to this medical service unit with blurred vision developed over the past 3 months, and was associated with vitreoretinal traction and a macular epiretinal membrane. After an ophthalmological examination including the retinal periphery, optical coherence tomography, tuberculin test, interferon gamma release assay (IGRA), and a systemic study, the patient was diagnosed with Eales disease. Macular oedema or epiretinal membranes due to Eales disease are relatively common. Sd-OCT is recommended in all patients with Eales disease. On the other hand, the presence of epiretinal membranes in young patients usually suggests a non-idiopathic aetiology.

Bilateral acute iris depigmentation and bilateral acute iris transillumination syndrome. / Síndrome de despigmentación aguda bilateral de iris y transiluminación aguda bilateral de iris.

The case is presented of a 32 year-old male who arrived with acute bilateral symptoms with blurred vision, red eye, severe photophobia and severe ocular pain after suffering from a flu-like syndrome. The patient presented with a clinical picture of bilateral involvement characterised by pupils in mid-mydriasis, scarcely reactive to light, iris transillumination, diffuse depigmentation of the iridian stroma, pigment dispersion in the anterior chamber, and ocular hypertension. After the eye examination an inflammatory syndrome and pigmentary glaucoma were ruled out. The patient showed depigmentation characteristics as well as bilateral iris transillumination. Both conditions could form part of the spectrum of the same disease.

Invasive aspergillosis manifesting as retinal necrosis in a patient treated with ruxolitinib. / Necrosis retiniana como manifestación de aspergilosis invasiva en un paciente tratado con ruxolitinib.

A 30 year-old man with acute myeloblastic leukaemia and secondary myelodysplastic syndrome developed graft-versus-host disease. The patient was treated with ruxolitinib. After being treated for 3 months with ruxolitinib, an inhibitor of Janus kinase, he developed Aspergillus retinal necrosis resistant to common treatment. Treatment with Janus kinase inhibitors may lead to an increased incidence of opportunistic infections. Janus kinase inhibitor administration may result in poor treatment efficacy.

Uveitis and serous retinal detachment secondary to systemic dabrafenib and trametinib. / Uveítis y desprendimiento seroso de retina secundarios al tratamiento sistémico con dabrafenib y trametinib.

CASE REPORT: The case is presented of a 39-year-old woman with metastatic melanoma treated with dabrafenib and trametinib. She presented with a severe acute panuveitis with granulomatous anterior uveitis, vitritis, and multiple serous retinal detachments. Dabrafenib and trametinib were suspended, and treatment with a systemic and topical corticosteroid was started. A good response was obtained, with a recovery of visual acuity of 1.0 in both eyes within two weeks. DISCUSSION: Dabrafenib and trametinib can lead to severe uveitis. Treatment with corticosteroids and discontinuation of therapy with dabrafenib and trametinib led to an anatomical and functional improvement, and resolved the episode rapidly. Ophthalmologists must be aware of this toxicity, given the increasing use of those drugs.

Focal choroidal excavaction associated with idiopathic choroidal neovascularization. / Excavación focal coroidea asociada a membrana neovascular coroidea idiopática.

CASE REPORT: The case is presented of a 45 year-old man with a focal choroidal excavation associated with choroidal neovascularisation not included in the area of excavation. Clinical features were analysed using retinography, fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography. The patient was treated with 3 intravitreal injections of bevacizumab, with a good response. DISCUSSION: Focal choroidal excavation can be associated with choroidal neovascularization not included in the area of excavation. Multimodal imaging provides a complete description of clinical features, before and after treatment.

Tubulointerstitial nephritis and uveitis syndrome (TINU). Treatment with immunosuppressive therapy. / Síndrome de nefritis túbulo-intersticial y uveítis (TINU). Tratamiento con inmunosupresores.

CASE REPORT: Two cases of tubulointerstitial nephritis and uveitis are presented. Immunosuppressive therapy was required to control the uveitis. Contrary to that usually described, uveitis became chronic, which made immunosuppressive therapy necessary. Nephritis was successfully treated with steroids. DISCUSSION: Tubulointerstitial nephritis and uveitis syndrome is an under-diagnosed disorder and requires clinical suspicion due to there being no specific laboratory study available. Recurrences and relapses of ocular inflammation are common. Immunosuppressive therapy is not often needed.

Purtscher-like retinopathy preceding acute renal failure. / Retinopatía Purtscher-like previa a fallo renal agudo.

CASE REPORT: The case is reported of a 61 year-old woman with Purtscher-like retinopathy associated with acute renal failure. Ophthalmic examination, fluorescein-angiography, and optical coherence tomography were consistent with Purtscher-like retinopathy. Ophthalmic symptoms and signs preceded renal failure. Pancreatitis and other systemic diseases were ruled out. The patient developed a neovascular glaucoma. DISCUSSION: Purtscher-like retinopathy rarely precedes the associated systemic illness. Early diagnosis based on ophthalmic symptoms may help in the recognition and treatment of the disease, and prevent later complications.

Unilateral uveitis associated with IgA nephropathy. / Uveítis unilateral asociada a nefropatía IgA.

CASE REPORT: The case is presented of a 42 year-old man with episodes of unilateral uveitis in his right eye. Ophthalmic examination showed a granulomatous anterior uveitis with vitritis. Systemic investigations revealed non-nephrotic proteinuria and microhaematuria. A renal biopsy showed IgA nephropathy. DISCUSSION: Uveitis and glomerulonephritis may have common immunological pathogenesis. IgA nephropathy should be a differential diagnosis in patients with uveitis and nephropathy.

Bilateral exudative retinal detachment associated with central serous chorioretinopathy in a patient treated with corticosteroids. / Desprendimiento de retina exudativo bilateral asociado con coriorretinopatía serosa central en una paciente tratada con corticoesteroides.

CASE REPORT: The case is presented on a 54-year-old woman with a central serous chorioretinopathy, misdiagnosed as Vogt-Koyanagi-Harada disease, and treated with systemic corticosteroids. The patient presented with a bilateral bullous exudative retinal detachment. DISCUSSION: Discontinuation of corticosteroid therapy, surgical drainage of subretinal fluid, and photodynamic therapy, led to anatomical and functional improvement. The recognition of an atypical presentation of central serous chorioretinopathy may avoid complications of the inappropriate treatment with corticosteroids.