Perifoveal exudative vascular anomalous complex (PEVAC).

We present three cases of patients aged 66, 80 and 23, who presented unilateral vision loss. Optical coherence tomography (OCT) in all of them showed macular oedema and a rounded lesion with hyperreflective wall, and fluorescein angiography (FAG) in two of them showed hyperfluorescent perifoveal aneurysmal dilations with exudation. None of the cases showed response to treatment after one year of follow-up, finally being diagnosed with Perifoveal Exudative Vascular Anomalous Complex (PEVAC).

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.

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.

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.

Peripapillary pachychoroidopathy. / Paquicoroidopatía peripapilar.

A study was made on 8 eyes of 5 patients between 47 and 65 years of age with peripapillary pachychoroidopathy. They all presented with a serous detachment of the neuroepithelium or retinal pigment epithelium in the nasal macular region that extended to the optic disc, with an increase in choroidal thickness at that level. An analysis was made of the choroidal thickness using high penetration optical coherence tomography, as well as their outcome in a period between 12 and 48 months. Peripapillary pachychoroidopathy is a distinct variant of the pachychoroid disease spectrum. It must be distinguished from other disorders, such as inflammatory or neuro-ophthalmological conditions, that may manifest in the vicinity of the optic disc. When faced with a patient with suspected pachychoroidopathy, a specific study of the papillary area should be considered using high penetration optical coherence tomography.

Paediatric Horner syndrome. A case series of 14 patients in a tertiary hospital.

BACKGROUND: Horner syndrome (HS) is characterised by the triad of upper eyelid ptosis, miosis, and facial anhidrosis. Due to its wide variety of causes, it can occur at any age, and is uncommon in paediatrics. The aetiology and diagnostic approach of paediatric HS (PHS) is controversial. OBJECTIVE: The purpose of this study is to describe the clinical characteristics of a 14 case series, focusing on the aetiology of HS and the clinical evolution the patients presented. METHODS: A retrospective observational study was conducted on patients under 14 years-old (enrolled between 1st January 2009 and 30th April 2020). Depending on the age at diagnosis (before or after the first 5 months of life), the study cases were divided into two groups: congenital or acquired. RESULTS: Fourteen patients, with a mean age of 8.5 months, were enrolled. The most frequent cause of PHS were tumours (6/14), with the most representative neoplasm being neuroblastoma (4/14). Of the acquired cases (8/14), the most frequent cause was iatrogenic (5/8), mainly secondary to cervical or thoracic surgery. The main origin of congenital HS (6/14) was neuroblastoma (4/6), being the first manifestation of the disease in 50% of patients (2/4). CONCLUSION: HS may be the first sign of a major underlying disease, such as neuroblastoma. For this reason, children presenting with HS of unknown origin require imaging studies to exclude a life threatening disease. A thorough examination is essential for early diagnosis of these patients.

Fundoscopic characteristics in three cases of familial hyperchylomicronaemia. / Características fundoscópicas en tres casos de hiperquilomicronemia familiar.

CASES REPORT: Three cases are presented of patients with familial hyperchylomicronaemia and lipaemia retinalis, in whom an analysis is made of the fundoscopic characteristics of each of them. DISCUSSION: The typical appearance of the retinal fundus is pale salmon coloured and corresponds to levels of severe lipaemia retinalis. As regards the findings, the vascular tree tonality is probably the best exploratory evidence to help in the ophthalmological diagnosis.

Takayasu's arteritis: Ischaemic retinal arterial occlusion as a possible initial presentation. / Arteritis de Takayasu: obstrucción arterial retiniana isquémica como posible presentación inicial.

Ocular manifestations are observed in 25% of patients with Takayasu's arteritis. Its signs and symptoms can be very variable. The case is presented of a 41-year-old woman with progressive vision loss in her right eye secondary to ischaemic retinal arterial occlusion. After a systematic study, a protein purified derivative (PPD) skin test compatible with tuberculosis was found to be the only alteration. After ruling out other causes, and based on the initial suspicion of tuberculous retinal vasculitis, treatment was started with antimicrobial agents and systemic corticosteroids, without any therapeutic response. Eighteen months later, the patient developed acute kidney failure, secondary to right renal artery stenosis. The CT-angiography revealed a thickening of the aortic arch and its branches, and Takayasús arteritis was finally diagnosed. Therefore, emphasis is made on the importance of the ophthalmologist in the diagnosis of Takayasús arteritis, in which its ophthalmological manifestations can be an early sign of the 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.

Macular neurosensory retinal detachment associated with topiramate use. / Desprendimiento de retina neurosensorial macular asociado al uso de topiramato.

A 36 year-old woman with idiopathic intracranial hypertension was treated with topiramate and acetazolamide. The patient was followed-up for 2 years, with a relationship between neurosensory detachments and topiramate being established, with recurrences after the introduction of topiramato and improvement after its withdrawal. These findings point topiramate as a possible cause of the clinical picture. Topiramate may cause retinal and macular neurosensory detachments. Although the ciliochoroidal effusion cases caused by this drug are well-known, its retinal side effects are less common. As it is a widely used drug, neurologists and ophthalmologists should be aware of its possible ocular side effects.

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.

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.

Retinal toxicity after accidental intravitreal injection of mepivacaine and adrenaline. / Afectación retiniana tras inyección accidental intravítrea de mepivacaína y epinefrina.

CASE REPORT: The case is presented of a 32 year-old male with no medical history of interest who suffered a traffic accident with mild traumatic brain injury. He had a left supraciliary incised and contused wound that extended to the left upper eyelid, with no loss of vision. After palpebral anaesthetic injection, there was a sudden visual acuity decrease in the left eye and hyposphagma located between I-III at 4mm from the limbus, with increased intraocular pressure. A whitish lesion with a central haemorrhagic focus was observed in the ocular fundus, corresponding to the area where the hyposphagma was located. DISCUSSION: Anaesthetic injection during palpebral repair may be complicated by inadvertent penetration of the eyeball. Intravitreal mepivacaine and adrenaline could cause macular and retinal lesions.

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

CASE REPORT: 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. DISCUSSION: Besides branch retinal arteriolar occlusion, the angiopathy in Susac's syndrome may affect the optic disc vessels.

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.

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.