Choroidal changes associated with Bruch membrane pathology in pseudoxanthoma elasticum.

Abstract:

PURPOSE:To investigate the impact of Bruch membrane pathology on the choroid in pseudoxanthoma elasticum (PXE). DESIGN:Monocenter cross-sectional prospective case series. METHODS:The study included 61 eyes of 51 patients with PXE and 54 eyes of 54 normal subjects. The diagnosis of PXE was based on skin biopsy, genetic analysis or both. Eyes with PXE were subdivided into 3 groups: eyes without choroidal neovascularization (CNV) or chorioretinal atrophy (Group 1); eyes with active or fibrotic CNV (Group 2); and eyes with chorioretinal atrophy only (Group 3). Choroidal thickness was measured using enhanced-depth imaging optical coherence tomography (EDI-OCT). RESULTS:Compared to controls (331 μm ± 24; mean ± 95% CI), mean subfoveal choroidal thickness in eyes of patients with PXE was significantly reduced within all 3 groups (Group 1: 243 μm ± 29; Group 2: 184 μm ± 28; Group 3: 104 μm ± 28; P < 0.001). Associated structural changes included apparent loss of small choroidal vessels. The difference of PXE compared to control eyes was largest close to the optic disc and approximated the level of controls toward the periphery. Within the PXE subgroups, eyes without CNV or chorioretinal atrophy (Group 1) showed the least reduction of choroidal thickness, while it was most pronounced in Group 3. CONCLUSIONS:The results indicate that changes of Bruch membrane can be associated with choroidal alterations, which are most pronounced in the presence of advanced disease. A role of Bruch membrane in choroidal homeostasis may reflect a possible contribution of Bruch membrane alterations to CNV and geographic atrophy development in age-related macular degeneration.

journal_name

Am J Ophthalmol

authors

Gliem M,Fimmers R,Müller PL,Brinkmann CK,Finger RP,Hendig D,Holz FG,Charbel Issa P

doi

10.1016/j.ajo.2014.04.005

subject

Has Abstract

pub_date

2014-07-01 00:00:00

pages

198-207.e3

issue

1

eissn

0002-9394

issn

1879-1891

pii

S0002-9394(14)00192-5

journal_volume

158

pub_type

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