Angle-Closure Glaucoma Due to Multiple Cysts of the Iris and Ciliary Body
Ian Conner, MD, PhD; Joel S. Schuman, MD, FACS; and David L. Epstein, MD, MMM
Intra-epithelial cysts1 (Figure 35-1) of the iris and ciliary body, if multiple, may gradually push the peripheral iris forward so as to close the angle and result in elevated intraocular pressure (IOP). Angle-closure glaucoma caused by cysts of the iris and ciliary body is rare compared with angle-closure glaucoma from other causes.
The onset of the glaucoma is sometimes rapid with the typical picture of acute angle-closure glaucoma. The diagnosis may be suspected by finding on gonioscopic examination a considerable difference in the width of the chamber angle in various parts of the circumference. In some areas, the angle may appear closed, but in other areas, the angle may be wide, with a broad ciliary body band visible. Such marked variation in angle width does not occur in primary angle-closure glaucoma and should alert the astute clinician as to the possibility of posterior iris pathology, either in the form of pigment epithelium cysts or tumor. There is often also a characteristic irregularly spaced bumpy contour to the peripheral iris. Occasional patients have been observed with 1 or 2 such areas in the total angle circumference. Presumably, occasional cysts may not be as rare as once thought. From a practical point of view, the presence of only a few such areas does not substantially affect aqueous outflow.
It is important to recognize this entity, especially from the standpoint of prognosis. In primary angle-closure glaucoma, laser iridotomy, if performed in a timely fashion, usually affects a cure of the glaucoma. If the angle closure is caused by cysts of the iris and ciliary body, the prognosis must be more guarded.
The true state of affairs may not be recognized until after there is an iridectomy or iridotomy. After surgical iridectomy (eg, in cataract or filtration surgery), one or more cysts of the ciliary processes may be seen in the iris opening. Even through typically small laser iridotomies, cysts may occasionally be seen through the iris opening by gonioscopy. Also, either with the miotic pupil or, more commonly, after dilation, an iris cyst can be seen presenting at or behind the pupillary border. These iris cysts are dark brown and may be mistaken for a melanoma. In the area of an iris cyst, the iris is usually lifted off the lens sufficiently so that, on gonioscopic examination, one can look under the iris on either side of the cyst and sometimes see smaller cysts of the ciliary processes. These cysts of the ciliary processes may be heavily pigmented like iris cysts, or they may be perfectly clear. Ultrasound biomicroscopy should be used to confirm the diagnosis in cases of suspected iris or ciliary body cysts.
Iris cysts can be punctured with an argon or neodymium:yttrium-aluminum-garnet (Nd:YAG) laser using a mirrored gonioscopy lens if they extend into or beyond the pupillary border and apparently show little tendency to recur. Puncture of an iris cyst results in considerable widening of the angle in the region of the cyst if peripheral anterior synechiae have not already formed. Ciliary body cysts likely could be similarly easily treated with the laser, if visualized. (Occasionally, one may see 2 or 3 ciliary body cysts on either side of an iris cyst.)
In one eye that we observed after intracapsular cataract extraction, we could easily see behind the iris during gonioscopy and could view a continuous row of cysts of the ciliary processes in the entire circumference. In cases in which cysts of the ciliary body appear to be present in the whole circumference, the angle may remain extremely narrow after iridectomy or iridotomy and may close again, causing another acute increase in IOP. In most cases, the glaucoma can be controlled medically after iridotomy, but in exceptional cases, a filtering operation may be necessary. Ciliary body cysts in particular may continue to grow and cause further closure of the angle, requiring a filtering operation.
Figure 35-1. Cysts of the iris and ciliary body may be visible only as an irregular iris surface contour gonioscopically but are visualized by ultrasound biomicroscopy.
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