Multimodal imaging in multiple evanescent white dot syndrome and new insights in pathogenesis.

PURPOSE: To report the multimodal imaging in multiple evanescent white dot syndrome (MEWDS) during the acute and convalescent stages in order to better understand the focus of the inflammatory process. METHODS: Retrospective cohort study of 4 patients with MEWDS. Each patient underwent: enhanced depth imaging-optical coherence tomography (EDI-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and en-face OCT and OCT angiography (OCT-A). Choroidal subfoveal thickness (CST) was measured manually. All patients were studied in the acute stage and convalescent stage after disappearance of OCT abnormalities and resolution of visual symptoms. RESULTS: Four MEWDS patients with a mean age of 23.5years were studied (range: 16-33years). Two patients were women. Initial mean visual acuity (VA) was 80.25 ETDRS. Final mean VA was 84.25 ETDRS. OCT imaging showed disruption of the ellipsoid zone and a slightly elevated RPE layer with overlying hyperreflective material, all of which corresponded to hyperautofluorescent FAF lesions. FA revealed multiple hyperautofluorescent lesions, correlated with hypocyanescent spots on the late ICGA. OCT-A showed normal superficial and deep retinal capillary plexus as well as choriocapillaris. The disease was self-limited in all the cases, with a mean time of 9weeks to resolution (range: 4-16). CONCLUSION: The pathophysiology of MEWDS is still debated. We believe that there is still not enough evidence to implicate the outer retina as the primary cause. For now, we suggest that this transient disease is the consequence of choriocapillaris hypoperfusion, but further studies are required to elucidate this hypothesis.

Acute panendothelial retinal leakage in a patient with diabetes mellitus type 1 with a poor metabolic control.

Ophthalmological diabetic complications are one of the main causes of blindness worldwide, so careful diagnostic and management is important. The screening plans implemented in our population made possible an early diagnosis and treatment, trying to reduce the consequences. Diabetic retinopathy is widely known, however we present the case of a rare retinal condition, acute panedothelial retinal leakage. It typically affects patients with type 1 DM and poor glycemic control. Its diagnosis is important since a strict metabolic control is sufficient for its resolution, avoiding aggressive therapies.

Acute panendothelial retinal leakage in a patient with diabetes mellitus type 1 with a poor metabolic control. / Capilaropatía edematosa aguda en un paciente con diabetes mellitus tipo 1 con mal control metabólico.

Ophthalmological diabetic complications are one of the main causes of blindness worldwide, so careful diagnostic and management is important. The screening plans implemented in our population made possible an early diagnosis and treatment, trying to reduce the consequences. Diabetic retinopathy is widely known, however we present the case of a rare retinal condition, acute panedothelial retinal leakage. It typically affects patients with diabetes mellitus (DM) type 1 and poor glycemic control. Its diagnosis is important since a strict metabolic control is sufficient for its resolution, avoiding aggressive therapies.

Pseudoxanthoma elasticum in childhood in patient with ß-thalassaemia. / Pseudoxantoma elástico infantil en paciente con ß-talasemia.

Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterised by elastic tissue alterations and caused by mutations in a single gene, ABCC6, on chromosome 16p that includes manifestations that are predominantly cutaneous, ocular and cardiovascular. PXE-like lesions in association with ß-thalassemia have previously been reported in the literature in patients with ß-thalassaemia intermediate and major, being clinically indistinguishable from classic PXE. The case is presented of a 10-year-old boy with ß-thalassaemia minor and characteristic lesions of PXE. It is worth noting the benefit of multimodal imaging in the diagnosis and monitoring of the lesions.

Iatrogenic retinal central artery occlusion after a cerebral digital subtraction angiography. / Oclusión de la arteria central de la retina iatrogénica tras angiografía de sustracción digital cerebral.

Digital subtraction angiography (DSA) is a technique used in interventional radiology for the diagnosis of vascular cerebral conditions. Central retinal artery occlusion (CRAO) is a complication of endovascular procedures that involve atherosclerotic plaque removal with subsequent embolisation. The case is presented on a patient who noticed a sudden vision loss during a DSA. Funduscopy showed retinal pallor and a cherry red spot. The angiography showed a structure suggesting the presence of an embolus in the central retinal artery. The patient was diagnosed with a CRAO, and unsuccessfully treated with ocular massage, topical hypotensive drugs, oral acetazolamide, and anterior chamber paracentesis, with a final visual acuity of 0.05. Central retinal artery occlusion is a rare complication, but due to its poor prognosis, it must be considered when performing neuroradiological procedures such as DSA.