Spark ImageWise – 70 Iridocorneal Syndrome – Case Report

Dr. Ravi Daberao, Dr. Sarang Lambat, Dr. Prabhat Nangia, Dr. Vinay NangiaSuraj Eye Institute, Nagpur

Case Description
A 52-year-old male presented to us for a glaucoma evaluation. His best corrected visual acuity was 6/6p in the right eye and 6/18 in the left eye. Anterior segment examination showed a mildly hazy cornea, diffuse iris stromal atrophy, and 360 degrees of peripheral anterior synechiae (PAS) in both eyes, with a clear lens. He had previously undergone a laser peripheral iridotomy in both eyes six years ago. His right eye’s intraocular pressure (IOP) was 18 mmHg, and 24 mmHg in his left eye on timolol 0.50% and brinzolamide 1% eye drops. A Gonioscopy of both eyes showed completely closed angles with 360 degrees broad multiple PAS. The axial length of his right eye was 23.88 mm, and his left eye was 23.57 mm. He was diagnosed with iridocorneal endothelial syndrome (essential iris atrophy). The patient had a history of left eye central retinal vein occlusion two years ago and had received four doses of intravitreal anti-vascular endothelial growth factor (anti-VEGF). We started him on brimonidine 0.2% and timolol 0.50% in both eyes. However, he was lost to follow-up and revisited after five and a half months, with an IOP of 24 mmHg in the right eye and 32 mmHg in the left eye on brimonidine 0.2% and timolol 0.50%. Therefore, we added half a tablet of oral acetazolamide twice a day and advised him to review after one month for IOP. On the one-month follow-up, the vision in his right eye was 6/6P and 6/9 in the left eye, but the IOP in the right eye was 48 mmHg and 18 mmHg in the left eye on timolol 0.50%, brinzolamide 1% eye drops, and oral acetazolamide tablets.

Fig. 1 Anterior segment photograph of the right eye showed iris atrophic patches (red arrow) with 360-degree peripheral anterior synechiae (yellow arrows). The pupil appeared mid-dilated, irregularly oval, sluggishly reacting to light. Conjunctival congestion was also noted.
Fig. 2 Anterior segment photograph of the left eye showed iris atrophic patches (red arrow) with almost 360 degrees of peripheral anterior synechiae inferiorly, nasally, and temporally (yellow arrows). Conjunctival congestion was also noted.

Fig. 9 An anterior segment photograph of the right eye showed iris atrophic patches (red arrow) and 360-degree peripheral anterior synechiae (yellow arrows) with pseudophakia. IOP was 13 mmHg at the time of the presentation. The tube of the glaucoma implant is not seen since it is behind the iris.
Fig. 10 Anterior segment photograph of the left eye showed iris atrophic patches (red arrow) with 270 degrees of peripheral anterior synechiae inferiorly, nasally, and temporally (yellow arrows) with pseudophakia. IOP was 14 mmHg at the time of presentation. The tube of the glaucoma implant is not seen since it is behind the iris.

Discussion:

Our patient demonstrated a volatile and unpredictable course of elevated intraocular pressure in both eyes over a short period. In view of the raised intraocular pressure (IOP) in the right eye, the patient underwent right eye lens extraction with an Aurolab aqueous drainage implant (AADI). On one-week follow-up, the IOP in the right eye decreased to 11 mmHg. However, in the left eye, the IOP increased to 58 mmHg on brimonidine 0.2%, timolol 0.50%, brinzolamide 1% eyedrops, and oral acetazolamide tablets. To reduce the IOP in the left eye and prevent damage to the optic disc, he was advised to undergo left-eye lens extraction surgery with a glaucoma drainage device. The patient underwent left eye surgery, and on a one-week follow-up post-surgery, the IOP in the left eye was 14 mmHg. However, the IOP in the right eye increased to 36 mmHg on three anti-glaucoma medications after four weeks. The intraluminal suture was removed from the glaucoma implant in the right eye. The left eye intraluminal suture was also removed after six weeks of left eye surgery. The IOP when last seen was 14 mmHg in both eyes.

Patients with significantly elevated IOP with iridocorneal endothelial syndromes, especially when associated with peripheral anterior synechiae as seen in our patient, tend not to do well unless the IOP is reduced. Trabeculectomy surgery tends not to work very well over long periods of time. Glaucoma implants are considered to do better. One of the challenges in such patients is the anterior drawing and movement of the iris towards the cornea because of the broad peripheral anterior synechiae, which tend to creep in an anterior direction besides being circumferential. This takes away the space required for placing the tube of the glaucoma implant in the anterior chamber since there is a higher possibility of coming in contact with the endothelium. However, the iris’s drawing forward creates significant space between the iris and the lens. While placing the tube behind the iris is an option, there may be the uncertainty of the tube touching the lens and the possible difficulty in the visualization of the resting plane and position of the tube. Therefore, the decision was taken to do a lens extraction along with the glaucoma implant. The tube is placed behind the iris, making use of the space between the iris plane and the lens implant

A word of caution in such patients is that the cornea is significantly more predisposed to undergoing decompensation following lens extraction. One may wish to do specular microscopy to look for pathognomic endothelial changes in such patients prior to surgery. The discussion is, therefore, whether one would like to do a lens extraction earlier in such patients so as to use less energy during surgery and reduce the possibility of corneal decompensation. There may be further challenges also in doing corneal surgery in this type of anterior chamber, where the iris plane is shifted anteriorly.

Read Wise

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