Acquired focal choroidal excavation associated with multiple evanescent white dot syndrome: observations at onset and a pathogenic hypothesis

BMC Ophthalmol. 2014 Nov 20:14:135. doi: 10.1186/1471-2415-14-135.

Abstract

Background: The mechanism underlying focal choroidal excavation (FCE) remains largely unknown. We evaluated the sequential progression of FCE generation using enhanced depth imaging optical coherence tomography (EDI-OCT) in a patient with multiple evanescent white dot syndrome (MEWDS).

Case presentation: A 37-year-old woman suffered MEWDS in the right eye. EDI-OCT showed the loss of photoreceptor inner segment/outer segment junction line, detachment between the retinal pigment epithelium (RPE) and Bruch's membrane, and dome-shaped, moderately reflective, focal photoreceptor-layer lesions corresponding to perifoveal white dots. The region with pigment epithelium detachment involved RPE/Bruch's membrane ruptures. After 1 month, almost all white dots spontaneously resolved together with improvements of the perifoveal OCT findings. Interestingly, perifoveal region developed a conforming-type FCE. An abnormal hyper-reflective lesion on OCT, regarded as fibrosis formation, simultaneously appeared within the choroid below the FCE and subsequently increased in size.

Conclusions: These results suggest that the RPE/Bruch's membrane disruption due to chorioretinal abnormalities and subsequent intrachoroidal scar formation play a role in the pathogenesis on an acquired FCE.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bruch Membrane / pathology*
  • Choroid Diseases / diagnosis
  • Choroid Diseases / etiology*
  • Female
  • Fluorescein Angiography
  • Humans
  • Retinal Diseases / complications*
  • Retinal Diseases / diagnosis
  • Retinal Photoreceptor Cell Inner Segment / pathology*
  • Retinal Photoreceptor Cell Outer Segment / pathology*
  • Retinal Pigment Epithelium / pathology*
  • Tomography, Optical Coherence
  • Visual Acuity