Congenital nonpigmented epithelial iris cyst after amniocentesis. Clinicopathologic report on two children

Congenital nonpigmented epithelial iris cysts are not common. They may arise spontaneously from developmental entrapment of surface ectodermal epithelium or from occult ocular trauma prenatally or at birth.

Between 1989 and 1991, an 8-month-old child and a 6-year-old child presented with large, progressive congenital epithelial iris cysts. Both children had a maternal history of diagnostic amniocentesis after an ultrasound scan, and there was no history of postnatal ocular trauma. The cysts were successfully removed by a modified block excision and tectonic corneoscleral grafting.

A dense adherence of the cyst wall to Descemet's membrane resembled old anterior synechiae after occult perforation of the globe in both patients. On histopathologic examination, the epithelial lining of the cysts consisted of non-keratinizing stratified squamous epithelium with goblet cells resembling conjunctival epithelium. A perforating limbal scar with a corresponding break in Descemet's membrane could be detected in one eye. The long-term visual acuity of both children was encouraging, and there was no evidence of recurrence of the iris cyst during the follow-up period (average, 23 months).

The authors conclude that the clinical and histopathologic features of these congenital iris cysts may be consistent with an occult intrauterine limbal perforation of the anterior chamber with a needle during amniocentesis. Amniocentesis, when not guided by a real-time ultrasound scan, may be a risk factor for prenatal ocular trauma, which should be considered in the differential diagnosis of congenital ocular disorders.