A, Absolute (100%) dehydrated alcohol (JCB Laboratories) is used for sclerosis and is drawn into a 1-mL syringe. B, Three-arm T-extension for 2 female attachments (syringes) and 1 male attachment to a 30-gauge needle. The female attachments are to a 3-mL aspiration syringe and to a connector tubing attached to an alcohol-filled 1-mL syringe. The alcohol is primed through to the distal margin of the connector tubing to minimize injection of air. C, After viscoelastic fill of the anterior chamber, the cyst is entered through its attachment to the endothelium. D, Aspiration to syringe 1 collapses the cyst. E, Infusion of alcohol through the primed connector tube causes the cyst wall to appear slightly gray. Occasionally, a bubble of air enters the cyst at this point. The alcohol remains in the cyst for 1 to 2 minutes and then is withdrawn. This cycle is repeated for 2 or 3 washes. F, Alcohol is removed from the cyst, and it appears collapsed against the endothelium, with viscoelastic material removed through paracentesis (anterior chamber deep) and wound hydration (to the right). The visual axis is unobstructed.
A and B, A 1-year-old girl with a primary congenital iris stromal cyst that failed simple aspiration (A) and manifested immediate cyst involution following therapy (B), which persisted at the 9-year follow-up visit, with visual acuity of 20/20. C and D, A 57-year-old woman with a slowly enlarging primary congenital iris stromal cyst (C), in whom a single treatment of aspiration with absolute alcohol manifested permanent sclerosis of the cyst at the 3-year follow-up visit (D); visual acuity was 20/400 because of progressive cataract. E and F, A 52-year-old man with an iris stromal cyst secondary to trauma and failure to control the cyst after 2 aspirations with laser elsewhere (E); 1 year after treatment with absolute alcohol, the collapsed cyst was stable, and visual acuity had increased to 20/20 (F). G and H, A 71-year-old woman with a primary acquired iris stromal cyst (G); after 3 aspirations with absolute alcohol, the cyst was sclerotic at the 3-year follow-up visit, and visual acuity was 20/40 (H).
Shields CL, Arepalli S, Lally EB, Lally SE, Shields JA. Iris Stromal Cyst Management With Absolute Alcohol–Induced Sclerosis in 16 Patients. JAMA Ophthalmol. 2014;132(6):703-708. doi:10.1001/jamaophthalmol.2014.160
The management of symptomatic iris stromal cyst is challenging using methods of aspiration with or without adjunctive cryotherapy, intracameral cautery, or photocoagulation. Failed cases often require surgical resection, with risks for epithelial downgrowth, glaucoma, blindness, and loss of the eye.
To study the safety and efficacy of the management of iris stromal cysts with aspiration and alcohol irrigation to induce cyst sclerosis.
Design, Setting, and Participants
Interventional case series at a tertiary referral center among 16 patients.
Microscopically monitored transcorneal aspiration of cysts was performed with a 30-gauge needle on a 3-way T-extension into a 3-mL syringe, followed by immediate infusion of absolute alcohol from a separate 1-mL syringe through the other arm of the T-extension. Subsequent alcohol aspiration and repetition of the cycle was performed until the collapsed cyst wall appeared gray.
Main Outcomes and Measures
Cyst involution, visual acuity, and treatment complications.
The iris stromal cysts were primary congenital (n = 6), primary acquired (n = 4), or secondary (n = 6). The cysts had a median basal diameter of 12 mm and a thickness of 4 mm, occupying 50% or more of the anterior or posterior chamber in each case. Treatment was administered following failure of simple aspiration (n = 16) and additional methods (n = 8). During a median follow-up period of 5 years, treatment was successful in 14 of 15 patients (1 patient was lost to follow-up). Cyst sclerosis with stabilization (n = 1) or involution (n = 13) was achieved following 1 (n = 10), 2 (n = 2), or 3 (n = 2) procedures. The single failure occurred in a 3-year-old child with cyst recurrence and severe photophobia requiring resection. Visual acuity remained stable or had improved in 14 patients and was reduced in 1 patient because of cataract. Complications included transient corneal edema (n = 4) and transient anterior chamber inflammation (n = 1), which resolved following topical corticosteroid therapy. No evidence was seen of treatment-related glaucoma, epithelial downgrowth, tissue necrosis, cataract, posterior segment toxic effects, or need for enucleation.
Conclusions and Relevance
Microscopically monitored aspiration and absolute alcohol–induced sclerosis of iris stromal cysts is safe and effective, with cyst involution obtained in 93% (14 of 15) of patients.
Tumors of the iris are classically divided into solid or cystic tumors.1- 4 In a clinical survey of 3680 iris tumors and pseudotumors from an ocular oncology service, 2912 (79%) were solid tumors, and 768 (21%) were cystic tumors.1 Cystic tumors were further divided into those originating from the iris pigment epithelium (n = 672) or those originating from the iris stroma (n = 96).1 The age at diagnosis correlated with cyst type, with congenital iris stromal cysts diagnosed most often in individuals younger than 20 years (n = 25), whereas acquired stromal cysts were found most often in middle-aged or older adults (n = 33).
The difficulty with iris stromal cysts stems from their tendency to enlarge, fill the anterior chamber, lead to angle occlusion and glaucoma, and occasionally require surgical resection or enucleation. In contrast, iris pigment epithelium cysts tend to remain quiescent, with little effect on the structure or function of the eye and rarely requiring therapy. Lois and coworkers5 evaluated 17 eyes with primary iris stromal cysts and found these cysts to be aggressive, necessitating multiple treatments, including resection, with risks for astigmatism, amblyopia, cataract, and loss of the eye. In this analysis, we explored a novel technique of absolute alcohol–induced cyst sclerosis initially described by Behrouzi and Khodadoust6 in 2003 and modified by our group. Herein, we report our experience with the technique in 16 cases.
Institutional review board approval from Wills Eye Hospital was obtained for this retrospective analysis. The potential risks and benefits of this technique were discussed with the patients and families, and signed informed consent was obtained. This retrospective interventional case series included patients with iris stromal cysts managed with a technique of aspiration and absolute alcohol–induced infusion and sclerosis at Wills Eye Hospital Ocular Oncology Service between September 1, 2003, and February 1, 2013. A total of 16 patients were included in this study.
Patient data were extracted from medical records and included the following: sex (male or female), race/ethnicity (white, African American, Asian, or Hispanic), and patient age at initial examination in our department (in years), as well as the presenting symptom, and history of previous trauma or ocular therapy. The ocular features were recorded as follows: the number of cysts per eye, corneal endothelial touch, pupil overhang by the cyst, best-corrected visual acuity (BCVA), clock-hour location of the cyst epicenter, intraocular pressure (in millimeters of mercury), and largest basal diameter and thickness (in millimeters). The cyst basal diameter and thickness were measured by slitlamp biomicroscopy and were confirmed with ultrasonographic biomicroscopy. All findings were documented with full-page color anterior segment drawings, anterior segment photography, and ultrasonographic biomicroscopy or anterior segment optical coherence tomography.
Using microscopic guidance, the procedure was performed under sterile conditions in the operating room, with the patient prepped and draped. General anesthesia was used for children, and retrobulbar anesthesia with intravenous sedation was used for adults. Viscoelastic material was injected through a limbal incision opposite the cyst with a 30-gauge needle for corneal endothelial protection. The equipment included a special setup with a 3-way T-extension (Alaris; CareFusion Corporation, allowing 2 female attachment arms and 1 male attachment arm (Figure 1). One of the female arms leads directly to an empty 3-mL syringe (syringe 1 for aspiration) and the other to a short connector tube with a 1-mL syringe of absolute alcohol (JCB Laboratories) (syringe 2 for irrigation). The single male arm of the T-extension was directly attached to a 30-gauge needle. The connector tubing to the filled alcohol syringe was primed with alcohol up to the precise attachment to the T-extension to avoid air entrapment.
The 30-gauge needle was selectively entered through the limbal cornea where the cyst was adherent to the endothelium to avoid penetration through the anterior chamber and possible risk for alcohol leakage into the aqueous. Using constant microscopic visualization, with the needle bevel facing up and centralized within the cyst, aspiration of the cyst (with syringe 1) to near-complete collapse was performed, with avoidance of perforation of the cyst by the sharp needle tip. With caution to avoid cyst rupture, alcohol infusion from syringe 2 was administered to subtotal reinflation. The alcohol remained within the cyst for 1 to 2 minutes, timed on a clock, until the cyst wall appeared slightly gray. Aspiration of alcohol into syringe 1 to near-complete deflation was performed. The cycle was repeated until a sufficient gray color of the cyst wall was achieved. When finalized, complete aspiration by syringe 1 was performed, and the needle was withdrawn. Viscoelastic material was irrigated out of the anterior chamber with corneal wound hydration for closure. The anterior chamber was infused with balanced salt solution so that the globe would be firm with cyst tamponade for cyst wall internal adherence. Topical antibiotic and corticosteroid ointment with single-day patching was provided, and daily topical antibiotic and corticosteroid ointment was prescribed for 3 weeks.
Follow-up examination was performed at 1 month, then every 4 months for 1 year, and twice yearly thereafter. At each visit, recorded data included BCVA, cyst basal diameter and thickness, ultrasonographic biomicroscopy findings, and status of the cyst (involution, unchanged, or recurrence), as well as any complications from treatment. The BCVA was compared with the initial BCVA and was categorized as stable, improved (increase in BCVA of ≥2 lines), or worsened (decrease in BCVA of <2 lines).
Iris stromal cyst aspiration and absolute alcohol–induced sclerosis was performed in 16 patients. Patient demographics are listed in Table 1. The mean age at presentation was 29 years (median, 18 years; age range, 9 months to 76 years). The 6 patients with primary congenital iris stromal cysts had a mean age of 2.6 years (median, 1.75 years; age range, 9 months to 7 years). The most common presenting symptom was a painless mass (n = 8). The BCVA was 20/20 to 20/30 in 4 patients, 20/70 to 20/100 in 2 patients, 20/150 to 20/400 in 4 patients, and hand motion to light perception in 2 patients. In preverbal children, the visual acuity was fix and follow in 3 patients and no fix or follow in 1 patient. Visual acuity decrease was related to lens tilt, amblyopia, corneal scar, or cyst-induced obstruction of the visual axis. Prior treatment of the cysts at our institution or elsewhere with simple aspiration had failed in all 16 patients. Seven patients had additional treatment with cryotherapy, laser photocoagulation, and absolute alcohol–induced sclerosis before referral for our management.
Cyst characteristics are listed in Table 2. In all cases, the cyst was unilateral and unifocal, appeared as a clear mass in the segment of the eye, and was adherent to the corneal endothelium. The mean cyst diameter was 10 mm (median, 12 mm; range, 6-15 mm), and the mean cyst thickness was 4 mm (median, 4 mm; range, 3-9 mm), occupying 50% or more of the anterior or posterior chamber in each case. The cyst most often involved the inferior (n = 8) or nasal (n = 7) quadrants.
Treatment details and outcomes are listed in Table 3. All patients had failed previous simple aspiration of their cyst, manifesting cyst recurrence. One patient was lost to follow-up immediately after treatment. Of 15 patients followed up, the initial procedure led to cyst involution or stabilization in 10. Of 5 patients with recurrence, 1 required urgent surgical resection; 4 were managed using a second procedure of absolute alcohol–induced sclerosis, with cyst involution in 2. Of 2 patients requiring a third procedure, both responded with cyst involution. All patients with primary congenital cysts were managed by 2 procedures and required no further therapy. In summary, of 15 patients followed up, the management included 1 procedure (n = 11), 2 procedures (n = 2), or 3 procedures (n = 2), with overall success in 14 of 15 patients (93%) (Figure 2). Cyst sclerosis with stabilization (n = 1) or involution (n = 13) was achieved. The single failure occurred in a 3-year-old child with cyst recurrence and severe photophobia requiring resection.
Complications following absolute alcohol–induced cyst sclerosis included transient corneal edema (n = 4) and transient anterior segment inflammation (n = 1), which resolved following topical corticosteroid therapy. All patients had cyst adherence to the endothelium, leading to endothelial compromise before treatment. In most cases, endothelial adherence persisted following cyst collapse. Corneal edema and anterior segment inflammation resolved with topical corticosteroid therapy. Two patients had pretreatment cataracts. At a median follow-up period of 5 years, the BCVA remained stable (n = 6), had improved (n = 8), or had decreased because of cataract (n = 1). Final visual acuity was 20/20 to 20/40 in 6 patients, 20/60 to 20/70 in 4 patients, and 20/200 to 20/400 in 2 patients. In preverbal children, the visual acuity was fix and follow in 2 patients and no fix or follow in 1 patient.
An iris stromal cyst appears as a translucent mass occupying the anterior lamella of the iris and expanding to conform to the anterior chamber.1- 5 The rate of expansion can be slow for many years, as is generally found in older patients, or rapid during a period of days, as is more common in infants, potentially progressing to glaucoma. Occasionally, the cyst can rupture, leading to anterior uveitis, secondary glaucoma, and risk for epithelial downgrowth. The management of an iris stromal cyst is based on the anticipated cyst complications to the eye and its effect on visual outcome.
Lois and coworkers5 reviewed 17 cases of iris stromal cysts and noted that cysts in children (n = 9) were larger, seemed more aggressive, and required more treatment compared with those in older patients (n = 8). In 9 children, the median cyst diameter was 8 mm, and findings of corneal touch (n = 5), glaucoma (n = 2), corneal scar (n = 1), and subluxed lens (n = 1) were noted. Treatment was necessary in 7 children, including aspiration (n = 5) and excision (n = 4) or both. In 8 teenagers and adults, the cysts were smaller, with a median diameter of 4 mm, and required therapy in only 1 patient (aspiration). Laser photocoagulation with cyst deflation has been used, with mixed results. Gupta and associates7 noted that laser therapy is noninvasive but is associated with a high recurrence rate. They further concluded that surgical outcomes are poor overall, especially in young patients.
Sclerosing agents have been used for the management of large cysts in the kidney, liver, and lung, as well as for cystic lymphangioma.8 Sclerosing agents for intraocular stromal cysts and epithelial downgrowth should be used with caution, and their use has been published as single case reports. In 1993, Capó and coworkers9 reported using trichloroacetic acid to manage a congenital iris stromal cyst in a 5-month-old girl, but subsequent secondary glaucoma and retinal detachment necessitated enucleation. No residual cyst was found on histopathological examination. In 2002, Shaikh and colleagues10 reported the use of 5-fluorouracil mixed with viscoelastic material to successfully treat a case of postsurgical epithelial downgrowth. In 2005, Yu and associates11 described an iris stromal cyst that responded to intralesional administration of mitomycin C. In 2013, Hong et al12 reported a case of posttraumatic iris stromal cyst that was managed by ethanol irrigation, with cyst collapse at the last follow-up visit at 16 months.
The largest series published to date on sclerosing agents for iris cysts was by Behrouzi and Khodadoust6 in 2003, in which they reviewed treatment of 99 eyes with intracystic ethanol irrigation. They achieved cyst involution after 1 procedure in 93 (94%), after 2 procedures in 96 (97%), and after 3 procedures in 98 (99%). Only one eye did not respond. Most cysts (n = 78) cysts were reduced within 1 day after ethanol irrigation. In that series, 99% of cysts were secondary acquired cysts following trauma, originating from an accident (n = 50) or from surgery (n = 51). Only one cyst was of unknown cause, in a 4-year-old boy (1%), presumed to represent a primary congenital iris stromal cyst. No infants were included in that series; the youngest patient was 4 years old. Our series is different in that 6 patients were 5 years or younger, 4 of whom were 2 years or younger, and the cysts were primary in 10 and secondary in 6. In our group, complete cyst control was achieved in 93% (14 of 15) of patients, most of whom had failed previous therapies.
Our technique of alcohol sclerosis is a modification of the technique by Behrouzi and Khodadoust.6 Instead of entering the cyst from 2 mm posterior to the limbus, we entered the cyst through the cornea at the site of adherence to allow constant observation of the needle and avoidance of the lens or other structures. In addition, most cases were performed using viscoelastic protection to the cornea. In our 16 patients, no needle-related or alcohol-related injury occurred to the lens. Transient corneal edema was observed in 4 patients, all of whom had cyst adherence to the corneal endothelium and were responsive to topical corticosteroid therapy. No evidence was seen of cataract, tissue necrosis, epithelial downgrowth, treatment-related glaucoma, posterior segment toxic effects, or need for enucleation.
We believe that the mechanism of absolute alcohol–induced cyst sclerosis is related to chemical toxic effects on the epithelial cyst lining. Often, we could microscopically visualize the sloughed epithelium free-floating within the aspirate. Removal of this debris with collapse of the cyst led to adherence of the cyst wall devoid of lining. The collapsed cyst most often resided quietly in the peripheral iris, allowing an open visual axis. It often required 2 sclerosis procedures separated by a few weeks or months to obtain complete cyst collapse. Our only failure was a child with severe photophobia several weeks following cyst sclerosis, who demonstrated cyst recurrence, and the parents preferred urgent surgical resection rather than repeat sclerosis.
Absolute alcohol–induced sclerosis of iris stromal cysts is safe and effective, particularly in infants. This technique spares patients the complications of glaucoma and loss of the eye and avoids the potential pitfalls of surgical resection. This procedure should be performed using exquisite care, with constant monitoring of the needle tip and injection to avoid alcohol leakage into the anterior chamber.
Submitted for Publication: August 3, 2013; final revision received November 20, 2013; accepted December 9, 2013.
Corresponding Author: Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 840 Walnut St, Ste 1440, Philadelphia, PA 19107 (firstname.lastname@example.org).
Published Online: April 10, 2014. doi:10.1001/jamaophthalmol.2014.160.
Author Contributions: Dr C. L. Shields had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Study concept and design: C. L. Shields, Arepalli.
Acquisition, analysis, and interpretation of data: All authors.
Drafting of the manuscript: C. L. Shields, Arepalli, E. B. Lally.
Critical revision of the manuscript for important intellectual content: C. L. Shields, S. E. Lally, J. A. Shields.
Obtained funding: C. L. Shields, E. B. Lally, J. A. Shields.
Administrative, technical, or material support: C. L. Shields, Arepalli.
Study supervision: C. L. Shields, J. A. Shields.
Conflict of Interest Disclosures: None reported.
Funding/Support: This study was supported by a Wills Eye Innovation Grant (Drs C. L. Shields and E. B. Lally), the Eye Tumor Research Foundation (Drs C. L. Shields and J. A. Shields), Lift for Cure (Drs C. L. Shields and J. A. Shields), and the Lucille Weidman Fund for Pediatric Eye Cancer (Drs C. L. Shields and J. A. Shields).
Role of the Sponsor: The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.