Progressive hemifacial atrophy with ciliary body atrophy and ocular hypotony

Abstract

Progressive hemifacial atrophy (PHA) is a disease of unknown etiology affecting one-half of the face. Ocular involvement is uncommon. Atrophy of iris is rare, with only a few cases of partial atrophy being reported in the literature. We report a case of total atrophy of iris and ciliary body with associated ocular hypotony in a 16-year-old girl with PHA. We believe this is the first reported case of complete atrophy of iris and ciliary body in PHA. Ocular hypotony in PHA was thought to be due to intra-ocular inflammation. However in our case it appears to be secondary to severe atrophy of the ciliary body.

Keywords

• Cup to disc ratio
• disc area
• glaucoma probability score
• moorfields regression analysis
• optical coherence tomography
• Antioxidant supplementation
• diabetes mellitus
• free oxygen radicals test
• nonproliferative diabetic retinopathy
• reactive oxygen species
• In vivo confocal microscopy
• keratocyte density
• prostaglandin analogues
• Chronic glaucoma patients
• economic burden
• socioeconomic profile
• Bevacizumab
• macular edema
• refractory
• retinal vein occlusion
• triamcinolone acetonide
• Corneal thickness
• menstrual cycle
• pachymetry
• refractive surgery
• Cytokines
• diabetic retinopathy
• pigment epithelium-derived factor
• vascular endothelial growth factor
• Choroidal metastases
• intravitreal bevacizumab
• transpupillary thermotherapy
• Fundus examination
• halogen light
• indirect ophthalmoscope
• light emitting diode
• photo-stress test
• Algorithm
• cross-linking
• Intacs
• keratoconus
• normogram
• Bibliometrics
• biomedical research
• journal impact factor
• ophthalmic research
• surgical research
• Aortic insufficiency
• arrhythmia
• ocular pulse amplitude
• ventricular bigeminy
• Ciliary body atrophy
• iris atrophy
• ocular hypotony
• progressive hemifacial atrophy