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Case Reports
. 2021 May 21;6(5):409-411.
doi: 10.1177/24741264211015538. eCollection 2022 Sep-Oct.

Unilateral Retinal Vasculitis as the Presenting Manifestation of Parry-Romberg Syndrome (Progressive Hemifacial Atrophy)

Elizabeth A Atchison  1 , Aimee J Szewka  2   3 , Pauline T Merrill  2
Affiliations
Case Reports

Unilateral Retinal Vasculitis as the Presenting Manifestation of Parry-Romberg Syndrome (Progressive Hemifacial Atrophy)

Elizabeth A Atchison et al. J Vitreoretin Dis. .

Abstract

Purpose: This work aims to present a case of retinal vasculitis associated with Parry-Romberg syndrome.

Method: A case report is presented.

Results: A 17-year-old man with new floaters was found to have 20/40 vision with 1+ vitreous cell and retinal vasculitis in the right eye only. Workup for infectious etiologies did not reveal an explanation for the retinal vasculitis. However, magnetic resonance imaging of the head showed areas of linear band-like atrophy and scarring of the scalp and soft tissues as well as areas of gliosis and encephalomalacia in the subcortical white matter, all of which were consistent with Parry-Romberg syndrome. The patient was prescribed oral steroids and methotrexate, and the retinal vasculitis improved.

Conclusions: Parry-Romberg syndrome is a rarely reported cause of retinal vasculitis and should be kept in the differential for retinal vasculitis.

Keywords: Parry-Romberg syndrome; en coupe de sabre; progressive hemifacial atrophy; retinal vasculitis.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
A color fundus photograph of the right eye showing retinal vasculitis, edema, and hemorrhage..
Figure 2.
Figure 2.
Fluorescein angiogram confirming the retinal vasculitis and demonstrating areas of nonperfusion.
Figure 3.
Figure 3.
Optical coherence tomography (OCT) of the right eye showing macular edema and optic nerve edema.
Figure 4.
Figure 4.
T2-weighted coronal magnetic resonance imaging showing encephalomalacia and gliosis in the subcortical white matter of the right frontal lobe.
See this image and copyright information in PMC

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