title: 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.
authors: Department of Ophthalmology, Comtrust Super Speciality Eye Hospital, Calicut, Kerala, India.
authors: T Ashwini
authors: TA
authors: Kini
authors: V S
authors: VS
authors: Prakash
authors: Suresh
authors: S
authors: Puthalath
authors: P L
authors: PL
authors: Bhandari
mesh_terms: D000293:Adolescent; D001284:Atrophy; D002924:Ciliary Body / Q000000981:diagnostic imaging / Q000473:pathology*; D018450:Disease Progression; D005150:Facial Hemiatrophy / Q000150:complications* / Q000175:diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007498:Iris / Q000000981:diagnostic imaging / Q000473:pathology; D033401:Microscopy, Acoustic; D015814:Ocular Hypotension / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D014603:Uveal Diseases / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology