Fundus changes of Parry-Romberg syndrome: A report of three cases and literature review

Eur J Ophthalmol. 2022 Sep;32(5):NP13-NP19. doi: 10.1177/11206721211015560. Epub 2021 May 21.

Abstract

Purpose: To report three cases of Parry-Romberg syndrome (PRS) with progressive hemifacial atrophy and similar fundus changes.

Methods: Retrospective case series of three patients who presented to the ophthalmology department with blurred vision in one eye. All of them underwent three-dimensional reconstruction of head computed tomography (CT), related physical examinations, and also monthly ocular examinations of both eyes, including the slit-lamp microscope, fundus autofluorescence, optical coherence tomography (OCT), and fundus fluorescein angiography (FFA). We also reviewed the current literature about the fundus change in PRS from inception to March 2020 via PubMed.

Results: Coats-like fundus and exudative retinal detachment were found in all three eyes of three patients during the follow-up. No systematic changes were found except for hemifacial atrophy. The final diagnoses of these three patients were PRS.

Conclusion: Only a subset of PRS patients have concomitant ocular changes, but it still should be noticed that the diagnosis of PRS should be considered if adults presented with Coats-like retinopathy and hemifacial atrophy.

Keywords: Coats-like retinopathy; Parry-Romberg syndrome; fundus changes; hemi-facial atrophy; literature review.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Facial Hemiatrophy* / complications
  • Facial Hemiatrophy* / diagnosis
  • Facial Hemiatrophy* / surgery
  • Fluorescein Angiography
  • Fundus Oculi
  • Humans
  • Retrospective Studies
  • Tomography, Optical Coherence