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

Presentamos 2 casos de maculopatía idiopática aguda unilateral en 2adultos jóvenes, un varón de 24 años y una mujer de 37 años. Ambos sufrieron una pérdida aguda de visión acompañada de manifestaciones clínicas compatibles con la maculopatía idiopática unilateral aguda. Como hallazgo excepcional, presentaron lesiones multifocales alrededor de una lesión central de mayor tamaño. Con el tiempo, se produjo la recuperación espontánea de la pérdida visual. La maculopatía idiopática aguda unilateral puede presentarse de formas distintas de la convencional, por lo que deberíamos considerar la posibilidad de encontrarnos ante estas formas atípicas (AU)

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 (AU)

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.

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

Paciente de 51 años de edad con leucemia de células peludas tratado con pentostatina. Mientras recibía el tratamiento, desarrolló una retinitis herpética en el ojo derecho. Tras finalizar el tratamiento con pentostatina, presentó un cuadro de vitritis y edema macular quístico. No había signos de reactivación de la retinitis herpética. Tras excluir otras causas de inflamación intraocular, se estableció el diagnóstico de uveítis de recuperación inmune. El paciente fue tratado con triamcinolona intravítrea, corticoides orales, implantes de dexametasona intravítreos y, finalmente, vitrectomía. La uveítis de recuperación inmune puede aparecer en pacientes VIH negativos. La reconstitución inmune tras el tratamiento podría dar lugar a una inflamación intraocular. El manejo de esta puede ser bastante complicado, pudiendo ser necesaria la realización de una vitrectomía. La posibilidad de una uveítis de recuperación inmune en pacientes VIH negativos debería ser tenida en cuenta

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

Coroidopatía, retinopatía y neuropatía óptica hipertensiva en un paciente con fracaso de trasplante renal / Hypertensive choroidopathy, retinopathy and optic neuropathy in renal transplantation failure

Varón de 32 años que presenta una elevación grave de la tensión arterial con cifras de 200/140 mmHg tras fracaso de trasplante renal. Se observaron múltiples desprendimientos serosos retinianos, dilatación venosa y atenuación de las arteriolas, hemorragias retinianas y edema del disco óptico. Ante la imposibilidad de realizar una angiografía fluoresceínica, la angiotomografía y las imágenes en face-tomografía de coherencia óptica permitieron identificar las alteraciones vasculares en retina, coroides y coriocapilar. La angiotomografía y las imágenes en modo en face-tomografía de coherencia óptica pueden ser de gran utilidad en la identificación de lesiones oftalmológicas vasculares relacionadas con la hipertensión maligna de aquellos casos en los que no sea posible realizar una angiografía fluoresceínica

A 32 year-old man who presented with severe elevation of blood pressure after failure of kidney transplant. The patient had multiple serous retinal detachments, venous dilation, arteriolar narrowing, retinal haemorrhages, as well as optic disc oedema. Due to the impossibility of performing a fluorescein angiography, an angiotomography and en-face optical coherence tomography images were used to identify the vascular alterations in the retina, choroid, and choriocapillaris. Angiotomography and en face-optical coherence tomography mode images are very useful in cases where it is not possible to perform fluorescein angiography

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

Presentamos el caso de un varón de 38 años, remitido a nuestro servicio por tracción vitreorretiniana y membrana epirretiniana asociada a pérdida de visión de 3 meses de evolución. Tras una exploración oftalmológica que incluyó el examen de la periferia retiniana, tomografía de coherencia óptica, prueba de tuberculina, interferon gamma release assay (IGRA) y estudio sistémico se llegó al diagnóstico de enfermedad de Eales. La afectación macular en pacientes con enfermedad de Eales es un hallazgo común, ya sea en forma de edema macular o membrana epirretiniana. Por ello, es aconsejable realizar un estudio macular mediante OCT. Por otra parte, el hallazgo de una membrana epirretiniana en un paciente joven debe hacernos pensar en una posible etiología no idiopática

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

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.

Hypertensive choroidopathy, retinopathy and optic neuropathy in renal transplantation failure. / Coroidopatía, retinopatía y neuropatía óptica hipertensiva en un paciente con fracaso de trasplante renal.

A 32 year-old man who presented with severe elevation of blood pressure after failure of kidney transplant. The patient had multiple serous retinal detachments, venous dilation, arteriolar narrowing, retinal haemorrhages, as well as optic disc oedema. Due to the impossibility of performing a fluorescein angiography, an angiotomography and en-face optical coherence tomography images were used to identify the vascular alterations in the retina, choroid, and choriocapillaris. Angiotomography and en face-optical coherence tomography mode images are very useful in cases where it is not possible to perform fluorescein angiography.

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.

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

Varón de 32 años que acude con un cuadro agudo bilateral caracterizado por visión borrosa, ojo rojo, fotofobia severa y dolor ocular tras un cuadro seudogripal. El paciente presentaba un cuadro con afectación bilateral caracterizado por pupilas en midriasis media, escasamente reactivas a la luz, transiluminación del iris, despigmentación difusa del estroma iridiano, dispersión de pigmento en la cámara anterior e hipertensión ocular. Tras el examen ocular se descartó un cuadro inflamatorio y un glaucoma pigmentario. El paciente presentaba características tanto de la despigmentación como de la transiluminación bilateral de iris. Ambas entidades podrían formar parte del espectro de la misma enfermedad

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

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

Varón de 30 años de edad con leucemia mieloblástica aguda y síndrome mielodisplásico secundario que desarrolló una enfermedad injerto contra huésped. El paciente fue tratado con ruxolitinib, un inhibidor de la Janus quinasa. A los 3 meses de haber iniciado el tratamiento se produjo una necrosis retiniana por Aspergillus, sin respuesta al tratamiento. El tratamiento con inhibidores de la Janus quinasa favorecería un aumento en la incidencia de infecciones oportunistas. El uso de estos fármacos podría dar lugar a una menor eficacia de los tratamientos empleados

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

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.

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

Caso clínico: Presentamos el caso de una mujer de 39 años de edad con un melanoma metastásico en tratamiento con dabrafenib y trametinib, que desarrolló una panuveítis severa aguda con uveítis anterior granulomatosa, asociada a vitritis y múltiples desprendimientos serosos de retina. El tratamiento con dabrafenib y trametinib fue suspendido iniciándose corticoterapia tópica y sistémica, apreciándose una buena respuesta y recuperando una agudeza visual de 1,0 en ambos ojos en 2 semanas. Discusión: El dabrafenib y el trametinib pueden producir una severa panuveítis. El tratamiento con corticoides y la suspensión de dabrafenib y trametinib consiguió controlar la uveítis, con recuperación de la agudeza visual, rápidamente. Los oftalmólogos debemos conocer este tipo de toxicidad debido al creciente uso de estos fármacos

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

Vasculitis retiniana y del disco óptico en el síndrome de Susac / Retinal and optic disc vasculitis in Susac's syndrome

Mujer de 42 años, sin antecedentes de interés que acude con cefalea severa, náuseas, vómitos, pérdida de la audición y alteraciones mentales con desorientación y confusión. El examen oftalmológico reveló la presencia de hiperemia del disco óptico derecho y de áreas focales de oclusión arteriolar en ambos ojos. La audiometría puso de manifiesto una hipoacusia bilateral neurosensorial. En la resonancia magnética cerebral se detectaron múltiples lesiones de pequeño tamaño hiperintensas localizadas en el esplenio, cuerpo calloso, ganglios basales y sustancia blanca

The case is presented of 42 year-old woman with no significant medical history, with severe headaches, nausea and vomiting, hearing loss, and alteration of mental status with disorientation and confusion. Ophthalmic examination showed optic disc hyperaemia in right eye, and focal areas of arteriolar occlusion in both eyes. Audiometry demonstrated bilateral neurosensory hypoacusis. Magnetic Resonance Imaging showed multiple small round hyperintense lesions located in the splenium, corpus callosum, basal ganglia, and white matter

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

CASO CLÍNICO: Presentamos el caso de una mujer de 61 años, con una retinopatía de tipo Purtscher asociada a un fallo renal agudo. La exploración oftalmológica, angiografía fluoresceínica y tomografía de coherencia óptica fueron compatibles con una retinopatía de Purtscher-like. Las manifestaciones oculares precedieron al fracaso renal. El estudio sistémico y de laboratorio descartó su asociación con pancreatitis y otras enfermedades sistémicas. Finalmente se complicó con un glaucoma neovascular. DISCUSIÓN: La retinopatía de Purtscher-like rara vez precede a la enfermedad sistémica asociada. El diagnóstico precoz basado en las manifestaciones oftálmicas, podría contribuir al diagnóstico y tratamiento de la enfermedad de base y a la prevención de las posibles complicaciones

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

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

CASO CLÍNICO: Presentamos el caso de una mujer de 61 años, con una retinopatía de tipo Purtscher asociada a un fallo renal agudo. La exploración oftalmológica, angiografía fluoresceínica y tomografía de coherencia óptica fueron compatibles con una retinopatía de Purtscher-like. Las manifestaciones oculares precedieron al fracaso renal. El estudio sistémico y de laboratorio descartó su asociación con pancreatitis y otras enfermedades sistémicas. Finalmente se complicó con un glaucoma neovascular. DISCUSIÓN: La retinopatía de Purtscher-like rara vez precede a la enfermedad sistémica asociada. El diagnóstico precoz basado en las manifestaciones oftálmicas, podría contribuir al diagnóstico y tratamiento de la enfermedad de base y a la prevención de las posibles complicaciones

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. DISSCUSIÓN: 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

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.

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

CASO CLÍNICO: Presentamos 2 casos de síndrome de nefritis túbulo-intersticial y uveítis que necesitaron tratamiento con inmunosupresores para lograr el control de la uveítis. Al contrario de lo habitualmente descrito, la uveítis adquirió un carácter crónico, siendo necesario el tratamiento inmunosupresor. La nefritis sí respondió al tratamiento con corticoides. DISCUSIÓN: El síndrome de nefritis túbulo intersticial y uveítis constituye una condición infradiagnosticada que requiere un alto índice de sospecha diagnóstica, ya que no se acompaña de alteraciones específicas. La inflamación ocular cursa con frecuentes recurrencias, siendo necesario, en raras ocasiones, el tratamiento inmunosupreso

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.