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Cavernous Hemangioma of the Iris Malformation

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Cavernous Hemangioma of the Iris Malformation LETTERS Iris capillary hemangioma can occur in children with RESEARCH LETTERS congenital periocular cutaneous capillary heman- gioma.1 It tends to regress spontaneously with regres- sion of the concurrent cutaneous hemangioma. Iris ra- cemose hemangioma is an anomalous arteriovenous Cavernous Hemangioma of the Iris malformation. In a recent report of 14 cases, these vas- cular malformations were divided into simple and com- ascular tumors and malformations of the iris are plex types.3 Unlike the retinal racemose hemangioma, it rare.1 We report an iris cavernous hemangioma is not associated with Wyburn-Mason syndrome. Iris varix V associated with recurrent hyphemas and el- occurs as a red-blue mass that lacks a distinct blood sup- evated intraocular pressure. ply and is generally hypofluorescent with fluorescein angiography.4 Report of a Case. A 55-year-old man was referred for an Iris cavernous hemangioma can have either of 2 clini- iris lesion in the right eye. Eighteen years earlier he had a cal variations.1 One is typically very small and located spontaneous hyphema that led to detection of the lesion. near the pupillary border. It often is difficult to visualize During the ensuing 2 decades, he had 30 spontaneous hy- and can cause spontaneous hyphema, particularly after phemas, each associated with transient elevated intraocu- cataract surgery.1 The other type, like the case reported lar pressure. One month prior to referral, a hyphema with here, appears as a clearly visible mass in the iris stroma. a pressure of 55 mm Hg was treated successfully with topi- Either type can rarely be seen as part of a syndrome with cal medication. Visual acuities were 20/40 OD and 20/20 similar lesions in brain, kidney, and skin.5 OS. Intraocular pressures were 12 mm Hg OD and 16 mm Hg OS. Slitlamp biomicroscopy showed a few scat- tered erythrocytes in the aqueous of the right eye but no hyphema. Mild pigment dispersion was present on the iris surface and transillumination revealed a circular pattern of radial slitlike defects in both irides. The main finding was a lobulated, reddish-blue tem- poral iris mass measuring 3 mm in diameter (Figure 1). Ultrasound biomicroscopy showed a multiloculated mass, and fluorescein angiography demonstrated early hypo- fluorescence of the lesion with mild hyperfluorescence in the superior aspects of the vascular spaces. The clini- cal diagnosis was iris cavernous hemangioma, but vas- cular iris melanoma could not be excluded. The mass was removed by sector iridectomy. Histopathologic exami- nation revealed a benign tumor composed of large blood- Figure 1. Clinical appearance of iris cavernous hemangioma showing a red-blue circumscribed iris stromal mass. filled vessels lined by thin endothelial cells, compatible with a cavernous hemangioma (Figure 2). Scattered pig- mented macrophages were present outside the vascular channels. The adjacent iris stroma near the pupil ap- peared focally fibrotic and hyalinized. The visual acuity remained 20/40 OD after surgery. Comment. There are several vascular malformations and tumors that occur in the retina and choroid, but such lesions in the iris are rare.1-5 Ferry1 reviewed sec- tions of cases submitted to the Armed Forces Institute of Pathology prior to 1972 and found that most “hem- angiomas” had been misdiagnosed histopathologically and proved to be highly vascular melanomas, juvenile xanthogranuloma, and other lesions. He suggested that iris vascular tumors were extremely rare, and he even questioned their existence. However, based on our clinical experience, there are convincing cases of Figure 2. Low-magnification photomicrograph showing cavernous venous iris capillary hemangioma, cavernous hemangioma, channels with intraluminal erythrocytes (hematoxylin-eosin, original racemose hemangioma, and varix.2-5 magnification ϫ20). (REPRINTED) ARCH OPHTHALMOL / VOL 126 (NO. 11), NOV 2008 WWW.ARCHOPHTHALMOL.COM 1602 ©2008 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Our patient had mild bilateral pigment dispersion syn- tients sustained permanent vision loss.4 Topiramate drome. We speculate that the unilateral episodes of el- (Topamax; Ortho-McNeil Neurologics, Titusville, New evated intraocular pressure were due to the recurrent hy- Jersey)5 was approved by the Food and Drug Adminis- phemas and not to the pigment dispersion. Some iris tration to prevent seizures in 1996 and migraine head- melanomas can also be highly vascular and simulate a aches in 2004, but it is also being used off-label for de- hemangioma. However, the diagnosis of cavernous he- pression and bipolar disorders, neuropathic pain, and mangioma should be suspected when the entire lesion weight reduction.4 In 2001, a warning was added to the is composed of large venous channels without a solid me- package insert describing a rare syndrome consisting of lanocytic component. ciliochoroidal effusion, forward displacement of the lens- iris diaphragm, marked anterior chamber shallowing, Jerry A. Shields, MD acute myopia, and secondary angle-closure glaucoma.1 Carol L. Shields, MD This process is usually reversible if topiramate is discon- Ralph C. Eagle Jr, MD tinued and ocular hypotensive therapy is instituted.4 In Correspondence: Dr J. A. Shields, Ocular Oncology Ser- eyes unresponsive to these measures, there are no de- vice, Ste 1440, Wills Eye Institute, 840 Walnut St, Phila- finitive treatment recommendations to avoid high-risk delphia, PA 19107 (jerry.shields@shieldsoncology fistulizing surgery. Because pupillary block is not pres- ent in topiramate-associated BAACG, peripheral iridec- .com). 4 Author Contributions: Dr J. A. Shields had full access to tomy is ineffective. all of the data in the study and takes responsibility for the We describe 4 patients (8 eyes) who were effectively integrity of the data and the accuracy of the data analysis. treated with argon laser peripheral iridoplasty (ALPI) for Financial Disclosure: None reported. topiramate-associated BAACG unresponsive to ocular hy- Funding/Support: This work was supported by a dona- potensive therapy and topiramate discontinuation. To our tion from the Eye Tumor Research Foundation, Phila- knowledge and according to a MEDLINE literature re- delphia, Pennsylvania (Drs C. L. Shields and J. A. Shields), view performed in March 2008, there are no published Mellon Charitable Giving from the Martha W. Rogers reports on the use of ALPI for this condition. Charitable Trust (Dr C. L. Shields), the Paul Kayser In- ternational Award of Merit in Retina Research, Hous- Report of Cases. Table1 and Table 2 contain patient ton, Texas (Dr J. A. Shields), the LuEsther Mertz Retina demographics, rationale and duration of topiramate treat- Research Foundation (Dr C. L. Shields), the Noel T. and ment, clinical parameters of topiramate-associated BAACG Sara L. Simmonds Endowment for Ophthalmic Pathol- before and after ocular hypotensive therapy, peripheral ogy (Dr Eagle), and a donation from Michael, Bruce, and iridoplasty treatment and effect, and clinical parameters Ellen Ratner, New York, New York (Drs C. L. Shields and of topiramate-associated BAACG after peripheral irido- J. A. Shields). plasty. Because bilateral complete angle closure devel- Role of the Sponsors: The sponsors had no role in the oped and persisted in 4 white women despite medical preparation of the manuscript. therapy and topiramate discontinuation, ALPI (300- or 500-µm spot size, 0.5-second duration, 200- to 400-mW 1. Ferry AP. Hemangiomas of the iris and ciliary body: do they exist? a search power range) was performed (by A.H.Z.) in all of the 8 for a histologically proven case. Int Ophthalmol Clin. 1972;12(1):177-194. Figure 2. Shields JA, Shields CL. Iris vascular tumors and malformations. In: Shields eyes ( ). Within 30 minutes of ALPI, the intra- JA, Shields CL. Intraocular Tumors: An Atlas and Textbook. 2nd ed. Philadel- ocular pressure was markedly reduced and the periph- phia, PA: Lippincott Williams & Wilkins; 2008:256-261. eral anterior chamber had significantly deepened in all 3. Shields JA, Streicher TFE, Spirkova JHJ, Stubna M, Shields CL. Arteriove- nous malformation of the iris in 14 cases. Arch Ophthalmol. 2006;124(3): of the eyes. In an effort to prevent a subsequent pupil- 370-375. lary block, all of the eyes underwent a laser iridotomy 4. Shields JA, Shields CL, Pulido J, Eagle RC Jr, Nothnagel AF. Iris varix simu- later the same day. Five months after iridoplasty, the in- lating an iris melanoma. Arch Ophthalmol. 2000;118(5):707-710. 5. Thangappan A, Shields CL, Gerontis CC, Dinowitz M, Shields JA. Iris cav- traocular pressure was normal in all of the eyes in the ernous hemangioma associated with multiple cavernous hemangiomas in the absence of ocular medications; only 4 eyes had mild, re- brain, kidney, and skin. Cornea. 2007;26(4):481-483. sidual peripheral anterior synechiae. Comment. Argon laser peripheral iridoplasty is a rela- tively easy, safe, and effective treatment for refractory topiramate-associated BAACG. To our knowledge, this Peripheral Iridoplasty Efficacy in Refractory case series represents the first report describing the Topiramate-Associated Bilateral Acute use of ALPI for this condition. The ALPI technique is a Angle-Closure Glaucoma well-recognized procedure used to treat angle closure from mechanisms other than pupillary block.6 imultaneous bilateral acute angle-closure glau- Although this report is limited by its small sample size coma (BAACG) is a rare1 and potentially blind-
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