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Presentation type	E-poster
Title	Ocular Involvement in Progressive Hemifacial Atrophy: a Case Report

Purpose	Progressive hemifacial atrophy (PHA, or Parry-Romberg syndrome) is a rare auto-immune disorder characterized by progressive unilateral craniofacial atrophy along with various extracutaneous manifestations including orbital and ocular manifestations. We report on a novel case of intraocular involvement in PHA.
Methods	Case report.
Results	A 6-year-old girl was referred because of left-sided craniofacial atrophy suggestive of PHA. Ophthalmologic examination revealed best-corrected visual acuity (BCVA) of 1.0 (HOTVX optotype) in the right eye (+1.75, -0.25 178°) and 0.7 in the left (+5.00, -1,00 148°). No strabismus or ocular motility impairment was detected. The left eye showed clear ocular media, 1+ cells in the anterior chamber and a binocular indirect ophthalmoscopy score of 1. The left fundus revealed a Coats-like vasculopathy nasal to the optic disc with marked retinal telangiectasia, macroaneurysms, and extensive lipoid exudation extending past the optic disc towards the fovea. Increased retinal thickness was observed in the nasal macular area. A similar lesion was evident in the inferotemporal periphery. Ophthalmologic examination of the right eye revealed no abnormalities. She was started on weekly subcutaneous injections of methotrexate and monthly pulse doses of methylprednisolone for a total of three doses. Occlusion therapy was commenced to treat amblyopia of the left eye. Extension of the retinal exudation was prevented by sectoral laser treatment. Follow-up showed a resolution of uveitis and stabilization of exudation after 6 months, with improvement of BCVA to 0.9.
Conclusion	Unilateral panuveitis with Coats-like disease is associated with PHA. Ocular screening is paramount in PHA given the potential sight-threatening manifestations.
Conflict of interest	No

Authors 1

Last name	VOLCKE
Initials	P
Department	UZGent
City	Gent

Authors 2

Last name	CALLENS
Initials	H
Department	UZGent
City	Gent

Authors 3

Last name	DE ZAEYTIJD
Initials	J
Department	UZGent
City	Gent

Authors 4

Last name	NICLAUS
Initials	V

Authors 5

Last name	ACCOU
Initials	G

Authors 6

Last name	RENSON
Initials	T

Authors 7

Last name	KREPS
Initials	E