A 6-YEAR-OLD boy with Rieger syndrome was found to have abnormal pupils(Figure 1) and an angle closure of 360° OD (Figure 2) and approximately 90° OS. Elevated intraocular pressure in the right eye had been reasonably well controlled with medication until the patient was aged 14 years. At this time, cyclocryotherapy was performed on the right eye, followed by a trabeculectomy 2 years later resulting in good control of the intraocular pressure. Pathologic examination revealed a severely malformed angle of complete iridocorneal adhesion(Figure 3) as well as abnormal collagens and basement membranes (Figure 4).
Chan C, Datiles M, Kaiser-Kupfer MI, Kupfer C. Congenital Iridocorneal Malformation in Rieger Syndrome. Arch Ophthalmol. 2003;121(4):582-583. doi:10.1001/archopht.121.4.582