The pseudohypopyon stage in adult-onset foveomacular vitelliform dystrophy

PurposeTo gain insight into the pathogenesis of adult-onset foveomacular vitelliform dystrophy (AFVD) via assessment of its pseudohypopyon stage (PHS).MethodsRetrospectively, data were collected in a tertiary center from established cohorts of a genetically evaluated AFVD and best vitelliform macular dystrophy (BVMD) eyes in the pseudohypopyon stage. Best-corrected visual acuity (BCVA, LogMAR), lesion characterization, including lesion dimensions, liquefaction areas and patterns (altitudinal or lateral), and ellipsoid zone integrity were analyzed from spectral-domain optical coherence tomography images.ResultsOut of 167 eyes of 90 AFVD patients and 56 eyes of 28 BVMD patients, 8 eyes of six AFVD patients and five eyes of four BVMD patients were at the PHS were included. The mean LogMAR BCVA +/- SD was 0.21 +/- 0.20 and 0.41 +/- 0.10 in AFVD and BVMD diseases, respectively (p = 0.13). Seven AFVD eyes (87.5%) demonstrated lateral liquefaction, while all BVMD eyes demonstrated an altitudinal pattern (p = 0.005). Maximal horizontal lesion diameters were 1.41 +/- 0.46 mm and 2.64 +/- 0.77 mm in AFVD and BVMD, respectively (p = 0.02). AFVD patients were older (69 +/- 14) than BVMD patients (22 +/- 13; p = 0.009).ConclusionThe pseudohypopyon stage in AFVD is often characterized by a lateral liquefaction pattern, unlike the altitudinal pattern characterizing BVMD. Age, lesion size, or pathogenesis pathways may underline the different pseudohypopyon stage patterns in AFVD and BVMD.

Automated summary

This study aimed to understand the pathogenesis of adult-onset foveomacular vitelliform dystrophy (AFVD) by assessing its pseudohypopyon stage (PHS). Results showed that the PHS in AFVD is often characterized by lateral liquefaction, unlike the altitudinal pattern seen in BVMD. Age, lesion size, or pathogenesis pathways may underlie the different PHS patterns in AFVD and BVMD.