CASE REPORT OF A RIEGERS ANOMALY
M. Hacker, K. Vanselow, C. Klais, A. Zubcov-lwantscheff, N. Stärk
Background: Riegers anomaly as a rare congenital disorder is one aspect of the "anterior chamber cleavage syndrome" combining posterior embryotoxon, iris adhesions to the cornea and iris hypoplasia. Riegers anomaly is normally bilateral with variable expressivity and in 60% associated with glaucoma.
Case report: In October 1997 a five months old child was presented to us with a "harmless congenital anomaly of the pupil". Following findings were made during examination in general anesthesia: The right pupil was stroke-like shaped, the left one coloboma-like. In addition we found linear tears in Descemets membrane and megalocornea with diameters of 14 mm at the right and 13 mm at the left eye. The gonioscopy showed in both eyes broad trapezoid synechias. The funduscopy showed an increased cup/disc ratio of the optic nerve of 0,8 on the right hand side and 0,6 on the left side. In both eyes the intraocular pressure was increased (35 mmHg at the right side and 28 mmHg at the left side). In ultrasound to determine the axial length we measured 22,36 mm on the right and 21,26 on the left eye. First we performed a trabeculectomy at the right eye, which lowered the intraocular pressure down to 24 mmHg. The synechias were not cut through. In a second step bilateral goniotomy was performed with a Barkan-blade and Healon. Because intraocular pressure remained increased this procedure had to be repeated twice. The adhesions showed hereby an extraordinary toughness. Postoperatively the intraocular pressure was nomalized. In addition the child underwent a pediatric examination to find out Rieger syndrome associated disorders. Striking was the telecanthus and asymmetry of the facies.
Conclusions: Deformation of the pupil makes additional diagnostics in regard to a possible iridocorneal dysgenesis combined with glaucoma necessary. Surgical treatment has often to be repeated in cases with increased intraocular pressure in combination with goniodysgenesis because of tough synechias.
Department of pediatric ophthalmology and strabism. Johann Wolfgang Goethe Universität, Theodor-Stern-Kai 7, D-60590 Frankfurt/M