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Jerry A. Shields, MD; Carol L. Shields, MD; Ralph C. Eagle Jr, MD

Arch Ophthalmol. 2008;126(11):1602-1603.

Vascular tumors and malformations of the iris are rare.¹ We report an iris cavernous hemangioma associated with recurrent hyphemas and elevated intraocular pressure.

Report of a Case
A 55-year-old man was referred for an iris lesion in the right eye. Eighteen years earlier he had a spontaneous hyphema that led to detection of the lesion. During the ensuing 2 decades, he had 30 spontaneous hyphemas, each associated with transient elevated intraocular pressure. One month prior to referral, a hyphema with a pressure of 55 mm Hg was treated successfully with topical medication. Visual acuities were 20/40 OD and 20/20 OS. Intraocular pressures were 12 mm Hg OD and 16 mm Hg OS. Slitlamp biomicroscopy showed a few scattered 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 temporal iris mass measuring 3 mm in diameter (Figure 1). Ultrasound biomicroscopy showed a multiloculated mass, and fluorescein angiography demonstrated early hypofluorescence of the lesion with mild hyperfluorescence in the superior aspects of the vascular spaces. The clinical diagnosis was iris cavernous hemangioma, but vascular iris melanoma could not be excluded. The mass was removed by sector iridectomy. Histopathologic examination revealed a benign tumor composed of large blood-filled vessels lined by thin endothelial cells, compatible with a cavernous hemangioma (Figure 2). Scattered pigmented macrophages were present outside the vascular channels. The adjacent iris stroma near the pupil appeared focally fibrotic and hyalinized. The visual acuity remained 20/40 OD after surgery.

View larger version (49K): [in this window] [in a new window] [as a PowerPoint slide]   Figure 1. Clinical appearance of iris cavernous hemangioma showing a red-blue circumscribed iris stromal mass.

View larger version (196K): [in this window] [in a new window] [as a PowerPoint slide]   Figure 2. Low-magnification photomicrograph showing cavernous venous channels with intraluminal erythrocytes (hematoxylin-eosin, original magnification x20).

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 sections of cases submitted to the Armed Forces Institute of Pathology prior to 1972 and found that most "hemangiomas" 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 iris capillary hemangioma, cavernous hemangioma, racemose hemangioma, and varix.^2-5

Iris capillary hemangioma can occur in children with congenital periocular cutaneous capillary hemangioma.¹ It tends to regress spontaneously with regression of the concurrent cutaneous hemangioma. Iris racemose hemangioma is an anomalous arteriovenous malformation. In a recent report of 14 cases, these vascular malformations were divided into simple and complex types.³ Unlike the retinal racemose hemangioma, it is not associated with Wyburn-Mason syndrome. Iris varix occurs as a red-blue mass that lacks a distinct blood supply and is generally hypofluorescent with fluorescein angiography.⁴

Iris cavernous hemangioma can have either of 2 clinical variations.¹ One is typically very small and located near the pupillary border. It often is difficult to visualize and can cause spontaneous hyphema, particularly after cataract surgery.¹ The other type, like the case reported here, appears as a clearly visible mass in the iris stroma. Either type can rarely be seen as part of a syndrome with similar lesions in brain, kidney, and skin.⁵

Our patient had mild bilateral pigment dispersion syndrome. We speculate that the unilateral episodes of elevated intraocular pressure were due to the recurrent hyphemas and not to the pigment dispersion. Some iris melanomas can also be highly vascular and simulate a hemangioma. However, the diagnosis of cavernous hemangioma should be suspected when the entire lesion is composed of large venous channels without a solid melanocytic component.

AUTHOR INFORMATION
Correspondence: Dr J. A. Shields, Ocular Oncology Service, Ste 1440, Wills Eye Institute, 840 Walnut St, Philadelphia, PA 19107 (jerry.shields{at}shieldsoncology.com).

Author Contributions: Dr J. A. Shields had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Financial Disclosure: None reported.

Funding/Support: This work was supported by a donation from the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (Drs C. L. Shields and J. A. Shields), Mellon Charitable Giving from the Martha W. Rogers Charitable Trust (Dr C. L. Shields), the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (Dr J. A. Shields), the LuEsther Mertz Retina Research Foundation (Dr C. L. Shields), the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology (Dr Eagle), and a donation from Michael, Bruce, and Ellen Ratner, New York, New York (Drs C. L. Shields and J. A. Shields).

Role of the Sponsors: The sponsors had no role in the preparation of the manuscript.

REFERENCES
1. Ferry AP. Hemangiomas of the iris and ciliary body: do they exist? a search for a histologically proven case. Int Ophthalmol Clin. 1972;12(1):177-194. FULL TEXT | PUBMED 2. Shields JA, Shields CL. Iris vascular tumors and malformations. In: Shields JA, Shields CL. Intraocular Tumors: An Atlas and Textbook. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:256-261.3. Shields JA, Streicher TFE, Spirkova JHJ, Stubna M, Shields CL. Arteriovenous malformation of the iris in 14 cases. Arch Ophthalmol. 2006;124(3):370-375. FREE FULL TEXT 4. Shields JA, Shields CL, Pulido J, Eagle RC Jr, Nothnagel AF. Iris varix simulating an iris melanoma. Arch Ophthalmol. 2000;118(5):707-710. FREE FULL TEXT 5. Thangappan A, Shields CL, Gerontis CC, Dinowitz M, Shields JA. Iris cavernous hemangioma associated with multiple cavernous hemangiomas in the brain, kidney, and skin. Cornea. 2007;26(4):481-483. FULL TEXT | WEB OF SCIENCE | PUBMED

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