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Primary iris pigment epithelial hyperplasia (PIPH) is a rare condition characterised by the presence of iris pigment epithelium on the anterior surface of the iris stroma. There are few reports that are available in the literature1–11 and they generally refer to it as congenital ectropion uveae (CEU). CEU, however, is a misnomer, since the iris pigment layer is known to be separate from the uvea.7 Yet, the term CEU persists in clinical use.
Primary iris pigment epithelial hyperplasia is a congenital non-progressive condition that is easily differentiated from acquired progressive ectropion uveae, the latter resulting from tractional eversion of posterior pigment layer and sphincter muscle, secondary to glaucoma and/or uveitis.
The most common association is neurofibromatosis8, 9, 12 though other ocular7, 10 and systemic associations8, 9 have been described, including a chromosomal abnormality.6
This report describes two cases of primary iris pigment epithelial hyperplasia with glaucoma and reviews the available literature on this rare abnormality. In one case the presentation of glaucoma was in adolescence and in infancy in the other. Associated ocular features are described. In these two patients there were no systemic features of diagnostic significance.
Case 1
A 15 year boy presented with occasional watering in the left eye for 4 years. There was no history of any visual disturbance.
On examination his left eye was apparently proptosed and Hertel exophthalmometry readings were 19 mm in the left eye and 13 mm in the right with bar reading of 105. He had a ptosis of 2 mm (Fig 1, top right) with good levator function. The appearance of the left upper lid was suggestive of plexiform neurofibroma but no mass was palpable. The patient's vision was 20/20 right eye (emmetropic) and 20/25 left eye (cylinder of −1.50 D at 110°, keratometry left …