PMC4549539_01
Clinical and diagnostic images from case 1: initial presentation. (a) Slit-lamp photograph of the patient's right eye upon presentation two years after his initial penetrating ocular injury with a wire fence. An inferocentral corneal scar is seen (asterisk). The pupil is irregular with a large, opaque, iris cyst extending from 7 to 9 o'clock with two embedded cilia (arrows). The lens is clear with no signs of cataract formation. (b) Anterior segment optical coherence tomography (AS-OCT) images illustrating the healed corneal laceration (arrow) from the patient's prior penetrating ocular injury and the location and contours of the iris implantation cyst. The cyst abuts the posterior cornea and there is absence of normal angle architecture. High reflectivity within the cyst indicates that the lesion is unlikely to be fluid-filled. (c) Slit-lamp photograph of the patient's eye one week after debulking of the cyst and injection of 5-Fluorouracil (5-FU) within the cyst cavity. The cyst was approached through a temporal scleral tunnel incision to facilitate precise entry into the body of the cyst for drainage and prevent expulsion of debris into the anterior chamber. Following surgery, the cyst initially resolved and there is rounding of the pupil.
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Visual acuity was 20/150 in the right eye, intraocular pressure was 14 mmHg, and anterior segment exam revealed a 4 mm healed central corneal scar, quiet anterior chamber, and a large iris cyst from 7 to 9 o'clock with 2 cilia embedded in the iris (Figures 1(a) and 1(b)). One week postoperatively, the patient's eye remained quiet with the best spectacle-corrected visual acuity (BCVA) of 20/80 OD with no recurrence of the iris cyst (Figure 1(c)).
Clinical and diagnostic images from case 1: subsequent presentation. (a) Slit-lamp photograph illustrating recurrence of the iris cyst along the inferotemporal iris after the patient was lost to follow-up for 1.5 years. Posterior synechiae have developed and the pupil is largely obstructed by the iris cyst. A white cataract can be seen through the small pupillary opening. (b) Ultrasound biomicroscopic images demonstrating copious echogenic material within the iris cyst. Normal iris and angle structure are disrupted and the cyst abuts the ciliary body. (c) Slit-lamp photograph showing shrinkage of the iris cyst after repeat debulking of the cyst and intracystic injection of 5-FU. (d) Slit-lamp photograph four weeks after surgical excision of the cyst demonstrating a large sector iridectomy. The cataract was removed with a vitrector and the patient is aphakic. The temporal cornea demonstrates posterior opacity and vascularization in the area previously abutted by the cyst. (e) AS-OCT image four weeks after surgical excision of the cyst. The sector iridectomy is seen. There is increased reflectivity and thickening on the posterior cornea in the area previously adjacent to the cyst where neovascularization and scarring were identified by slit-lamp biomicroscopy (arrow).
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There was now 360 degrees of posterior synechiae and a white cataract had also developed in the interim (Figure 2(a)). Ultrasound biomicroscopic (UBM) images revealed a solid cyst filled with copious echogenic material that disrupted the normal iris and angle architecture and abutted the ciliary body (Figure 2(b)). The cyst reduced in size after this second procedure (Figure 2(c)), but the patient continued to experience severe pain and diminution of vision and opted to undergo complete resection of the iris cyst, lysis of synechiae, and cataract extraction three months later. However, three weeks later, his pain had decreased and spectacle-corrected visual acuity had improved to 20/400 with resolved hyphema and improving vitreous hemorrhage (Figure 2(d)). Postoperative AS-OCT showed the sector iridectomy and increased reflectivity and thickening on the posterior cornea in the area previously adjacent to the cyst, where neovascularization and scarring were identified by slit-lamp biomicroscopy (Figure 2(e)).
Steps of surgical procedure from case 1. (a) Intraoperative view of the iris implantation cyst along the inferotemporal iris. (b) A conjunctival peritomy and 180-degree scleral tunnel were fashioned along the temporal aspect of the cyst to facilitate access to and removal of the cyst. (c) The cyst was dissected from the posterior cornea using viscoelasticity. The cornea was then retracted to expose the cyst. Intraocular diathermy was applied along the iris adjacent to the cyst to reduce bleeding prior to performing a large sector iridocyclectomy. (d) The scleral incision was closed with nine 9-0 nylon sutures. After lysis of posterior synechiae, three iris hooks were used to retract the remaining iris tissue and the cataract was removed with an anterior vitrector. (e) Intraocular lens placement was deferred at the time of surgery until longer-term ocular stability could be demonstrated. The patient was left aphakic.
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After visual inspection of the cyst (Figure 3(a)), a conjunctival peritomy was performed along the temporal aspect of the cyst to expose bare sclera. A 180-degree scleral tunnel was fashioned from 5 to 11 o'clock (Figure 3(b)). The cornea was then retracted to expose the cyst (Figure 3(c)). The lens was removed with a vitrector as well as any vitreous that presented anteriorly (Figure 3(d)). Given the limited visualization and until longer term stabilization could be ensured, the patient was left aphakic (Figure 3(e)).
Histopathology images from case 1. (a) Iris implantation cyst lined by stratified squamous epithelium (arrow) with a large amount of keratin debris with the cyst cavity. Fibrosclerotic material is seen surrounding the cyst wall (asterisk). (b) Positive immunohistochemistry for pankeratin cocktail, confirming the cyst contents to be keratin debris.
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Pathological analysis of the resected iris tissue revealed an epithelial lined cyst with keratin, keratohyalin granules in the epithelium, and subepithelial pigmentation (Figures 4(a) and 4(b)).
PMC4549539_02
Clinical and diagnostic images from case 2. (a and b) Slit-lamp photographs illustrating multiple serous cysts emanating from the inferior iris, obscuring the pupillary opening, and apposing the corneal endothelium. Blood vessels and iris pigment can be seen along the cyst walls. The inferior cysts appear more opaque and homogenous as compared with the superior cysts. (c) AS-OCT showing 4 distinct cavities with low central reflectivity and highly reflective walls, confirming the fluid-filled nature of the iris cysts. The cysts can be seen abutting the corneal endothelium and normal iris architecture is disrupted. (d) B-scan ultrasound image confirming extension of the iris cysts into the vitreous cavity. (e) Slit-lamp photograph showing recurrence of multiple, serous iris cysts that once again appose the corneal endothelium and encroach upon the pupillary opening. (f) Slit-lamp photograph 1 year after cyst aspiration and second injection of 5-FU. There is complete regression of the cysts with residual iridocorneal adhesions.
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Visual acuity was 20/50 in the right eye, intraocular pressure was 19 mmHg, and anterior segment examination revealed multiple, vascular, fluid-filled cysts emanating from the inferior iris that were apposed to the corneal endothelium and obscuring the pupil (Figures 5(a) and 5(b)). AS-OCT confirmed four multilobulated, fluid-filled cysts that disrupted the normal iris architecture (Figure 5(c)). B-scan indicated extension of the cysts into the vitreous cavity (Figure 5(d)). However, three months later, the patient began to experience increasing pain and decreased vision and was found to have recurrence of multiple fluid-filled cysts (Figure 5(e)). One year postoperatively, his vision improved to 20/40 with no recurrence of the cysts to date (Figure 5(f)).