Primary cysts of the iris pigment epithelium (IPE) are uncommon and usually cause no ocular symptoms. We describe an infant with large congenital IPE cysts surrounding the entire pupillary area, which required surgical treatment. Based on an extensive MEDLINE search, we believe that this is the first report of a case of total posterior synechia causing secondary angle-closure glaucoma from birth.
A 2-month-old Japanese boy was first seen at our hospital for a corneal opacity in his right eye. It had been present since birth and seemed to be getting worse. The patient's birth was normal, and his mother reported no remarkable history for the infant or his family. He could not fix or follow with his right eye, but his left eye was normal. The horizontal corneal diameters were approximately 12.5 mm OD and 10.5 mm OS. Slitlamp examination of his right eye revealed a corneal stromal opacity (Figure 1A). The peripheral anterior chamber was collapsed, suggesting the existence of iris bombé. B-mode ultrasonography and computed tomography(CT) revealed no remarkable abnormalities in the posterior and periocular lesions in both eyes. In the 0.5-mm slice of the CT scan, the axial lengths were estimated to be 20 mm OD and 17 mm OS.
A, Slitlamp examination revealed a corneal opacity. B, A cross-sectional ultrasound biomicroscopy image of iris cysts at the 3-o'clock and 9-o'clock positions on the meridian. The cysts surround the whole pupillary area and appear to be thin walled, with no internal reflectivity. The margin of the anteriorly bowed iris adheres to the lens surface.
At age 2.5 months, the infant underwent examination and surgical intervention under general anesthesia. Intraocular pressure was 35 mmHg OD (by pneumatonometer). Ultrasound biomicroscopy revealed a large iris cyst surrounding the pupillary area (Figure 1B). The pupillary margin adhered posteriorly to the lens. After the examination, the cysts were surgically resected. A small incision was made at the corneal limbus, and peripheral iridotomy was performed to form the peripheral anterior chamber. Viscoelastics were introduced, and the iris was cut circumferentially around the posterior synechia with Zaldivar iridectomy scissors (American Surgical Instruments Corp, Westmont, Ill). Adherent cyst tissue was gently removed from the lens surface with microforceps. The pupil was reconstructed with a suture. Histopathological analysis of the removed tissue confirmed the presence of IPE cysts. (Figure 2)
Light microscopy of a part of the iris cysts resected. A, The cyst wall (arrows) consists of iris pigment epithelial cells (hematoxylin-eosin, original magnification ×40). B, The cyst wall (arrow) is pigmented and monolayered (hematoxylin-eosin, original magnification ×400).
Three months postoperatively, intraocular pressure was 20 mmHg OD without medication. The corneal opacity had diminished, and the angle was open with scattered peripheral anterior synechia. The corneal diameter was 12 mm OU. No remarkable abnormalities were observed in the lens, vitreous body, and fundus, including the optic nerve head. The patient could fix and follow with the involved eye. One year after normalization of intraocular pressure, the axial lengths of the eyes showed no apparent bilateral difference by estimation from a CT scan.
Among the primary iris cysts of childhood, stromal (nonpigmented epithelial) cysts are reported to be progressive and to cause complications. On the other hand, IPE cysts, which are more commonly found, rarely cause severe complications, 1,2 although Bron et al3 described a 28-year-old woman who suffered from acute angle-closure glaucoma caused by an IPE cyst around the pupillary margin. This is the first reported case of secondary angle-closure glaucoma present from birth and caused by primary IPE cysts.
The authors have no relevant financial interest in this article.
Corresponding author and reprints: Jiro Numaga, MD, PhD, 3-2-1-214, Nishigahara, Kita-ku, Tokyo 114-0024, Japan (e-mail: firstname.lastname@example.org).
Ryo Obata, Shigenobu Suzuki, Jiro Numaga, Hidetoshi Kawashima, Makoto Araie. Congenital Iris Bombé Induced by Large Iris Cysts. Arch Ophthalmol. 2003;121(6):906–907. doi:10.1001/archopht.121.6.906