Título

Paracentral Acute Middle Maculopathy in a Patient with Impending Central Vein Occlusion evaluated with multimodal retinal exams: a case report

Introdução

Paracentral Acute Middle Maculopathy (PAMM) was firstly described in 2013 as a hyperreflective lesion at level of inner nuclear layer of the retina, observed at Optical Coherence Tomography (OCT). PAMM occurs most commonly in over-50 years-old male patients, as a result of intermediate and/or deep retinal capillary plexus ischemia and has been associated with retinal vascular diseases.
Optical Coherence Tomography Angiography (OCT-A) is a fast, noninvasive imaging exam that can evaluate retinal superficial, deep and choroidal vasculature. It has been suggested that OCT-A could provide a better visualization of the macular capillaries and foveal avascular zone when compared to fluorescein angiography (FA). Therefore, it has been used in studies of different retinal vascular diseases.
In this case, we reported a patient with Impending Central Vein Occlusion (ICVO) and Paracentral Acute Middle Maculopathy diagnosed by Swept Source OCT that showed vascular abnormal flow in OCTA, and his follow-up with multimodal retinal exams.

Métodos

Color fundus photography, fluorescein angiography, swept source optical coherence tomography and optical coherence tomography angiography were used to report a case of a previously healthy 24-year-old male patient that presented to our service with unilateral Impending Central Vein Occlusion associated with PAMM.

Resultados

We reported a case of PAMM associated with an ICVO. FA did not show areas of ischemia, which corroborates to impending occlusion diagnosis and PAMM diagnosis was made by initial SS-OCT that showed hyperreflective band-like lesions at the level of inner nuclear layers in the same eye. OCT-A showed vascular flow abnormalities in the deep capillary plexus. At 3 months of follow up exams, OCT did not show hyperreflectivity, but thinning on intermediate layers, as expected in later phases of PAMM, which explains the residual visual deficit. This case showed improvement of fundus exam without treatment, and all systemic exams were normal, with inconclusive systemic diagnosis so far.

Conclusões

This is the first report of an ICVO associated with PAMM evaluated with multimodal retinal exams. Multimodal retinal exams enable discovery of new diseases, such as PAMM, an intermediate plexus infarct diagnosed through OCT findings. Some systemic vascular diseases have already been associated with PAMM; in this case, it could be an initial sign of Systemic Erythematous Lupus, considering the patient’s family history.

Palavras Chave

impending occlusion, optical coherence tomography angiography, paracentral acute middle maculopathy, retinal vein occlusion

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