Multimodal imaging of retinal pigment epithelial detachments in patients with C3 glomerulopathy: case report and review of the literature

BMC Ophthalmol. 2017 Nov 22;17(1):207. doi: 10.1186/s12886-017-0602-4.

Abstract

Background: To describe the optical coherence tomography angiograhy (OCTA) of drusenoid pigment epithelial detachments (PEDs) in a woman affected by Complement 3 (C3) glomerulopathy, which represents a spectrum of glomerular diseases characterized on fluorescent microscopy by C3 accumulation with absent, or scanty, immunoglobulin deposits. It is due to acquired or genetically defective alternative pathway control and is generally associated with drusen-like deposits in Bruch's membrane, as well as choriocapillaris. These retinal lesions can be associated with choroidal neovascularization and central serous chorioretinopathy (CSCR). OCTA is useful to detect neovascularization without injecting a contrast product, particularly in these patients who may have renal insufficiency.

Case presentation: A 28-year-old woman affected by C3 glomerulpathy was diagnosed with asymptomatic multiple bilateral PEDs during a routine ophthalmologic consultation. To better characterize the lesions, multimodal imaging was performed and included: optic coherence tomography (OCT), en-face OCT, OCTA, fluorescence and indocyanine angiography. The OCTA clearly identified vascular network rarefaction with decreased choriocapillary vascularization. It confirmed that PEDs associated with C3 glomerulonephritis are not vascularized, but rather of serous type.

Conclusions: Patients affected by C3 glomerulopathy can develop neovascular membranes as retinal complications of pigment epithelial detachments. Optical coherence angiography may be indicated to identify this complication, without injecting any contrast product that could produce further kidney damage.

Keywords: Alternative pathway; Choroidal neovascularization; Complement 3 glomerulopathy; Drusenoid pigment epithelial detachments; Multimodal imaging; Optic coherence tomography angiography.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Complement C3 / metabolism
  • Female
  • Fluorescein Angiography
  • Glomerulonephritis, Membranoproliferative / complications*
  • Humans
  • Multimodal Imaging
  • Retinal Detachment / diagnostic imaging
  • Retinal Detachment / pathology*
  • Retinal Pigment Epithelium / diagnostic imaging
  • Retinal Pigment Epithelium / pathology*
  • Tomography, Optical Coherence

Substances

  • Complement C3