Paracentral acute middle maculopathy in central retinal vein occlusion complicating AL-amyloidosis

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Retin Cases Brief Rep. 2020 Nov 6. doi: 10.1097/ICB.0000000000001061. Online ahead of print.


PURPOSE: To describe a case of paracentral acute middle maculopathy (PAMM) associated with central retinal vein occlusion (CRVO) in a patient affected by amyloid light-chain (AL)-amyloidosis.

METHODS: One patient with confirmed diagnosis of AL-amyloidosis, displaying PAMM and CRVO, was recruited. The patient underwent complete ophthalmologic examination and multimodal imaging, including: fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), spectral-domain optical coherence tomography (OCT), and OCT angiography (OCTA).

RESULTS: FAF showed a ferning pattern, corresponding to linear hypofluorescence in late-phase ICGA and delayed venous filling, detected by FA. Structural OCT revealed a hyperreflective line located in the outer plexiform layer, corresponding to the prominent middle limiting membrane, along with several placoid lesions. OCTA found that the superficial capillary plexus was preserved, while vessel density was reduced in both the deep capillary plexus and the choriocapillaris. After one year of follow-up, the patient achieved an almost complete morphological recovery.

CONCLUSION: Multimodal imaging described in depth the morphological features of a case of combined PAMM and CRVO in a patient affected by AL-amyloidosis.

PMID:33181801 | DOI:10.1097/ICB.0000000000001061