title: Choroidal changes associated with Bruch membrane pathology in pseudoxanthoma elasticum.
abstract: OBJECTIVE
  To investigate the impact of Bruch membrane pathology on the choroid in pseudoxanthoma elasticum (PXE).
  
  
  METHODS
  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.
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Gliem
authors.affiliation: Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
authors.forename: Rolf
authors.initials: R
authors.lastname: Fimmers
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Philipp L
authors.initials: PL
authors.lastname: Müller
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Christian K
authors.initials: CK
authors.lastname: Brinkmann
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany; Centre for Eye Research Australia, University of Melbourne, Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
authors.forename: Robert P
authors.initials: RP
authors.lastname: Finger
authors.affiliation: Institute for Laboratory and Transfusion Medicine, Heart and Diabetes, Center North Rhine-Westphalia, University Hospital of the Ruhr University of Bochum, Bad Oeynhausen, Germany.
authors.forename: Doris
authors.initials: D
authors.lastname: Hendig
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Frank G
authors.initials: FG
authors.lastname: Holz
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany. Electronic address: peter.issa@ukb.uni-bonn.de.
authors.forename: Peter
authors.initials: P
authors.lastname: Charbel Issa
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016570:Bruch Membrane / Q000473:pathology*; D015862:Choroid Diseases / Q000175:diagnosis / Q000209:etiology*; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011561:Pseudoxanthoma Elasticum / Q000150:complications* / Q000175:diagnosis; D041623:Tomography, Optical Coherence; D055815:Young Adult