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Arch Ophthalmol. 2002;120:984-985.

We present the unique case of a patient with a vascular iris lesion consistent with a cavernous hemangioma and central nervous system (CNS) cavernous hemangiomas demonstrated by magnetic resonance imaging (MRI). Clinical reports of iris vascular tumors are rare and cases with histopathologic abnormalities are even more uncommon.¹ Hemangiomas of the eye are most often associated with posterior segment structures. We could find no previously described association between vascular tumors of the iris and CNS in adults. Most of the modern reports of iris vascular tumors occur in isolation.^2-3 There is one case of diffuse congenital hemangiomatosis with a unilateral iris cavernous hemangioma; however, this syndrome appears to be uniformly fatal by age 1 year.⁴

Report of a Case
A 48-year-old white woman was referred for evaluation after her local ophthalmologist noted an unusual iris mass in her right eye. The patient's medical history was significant for schizophrenia for which she had been treated with thioridazine hydrochloride for many years. She was diagnosed with multiple cavernous hemangiomas of the brain and brainstem 7 years earlier after a seizurelike episode prompted brain imaging (Figure 1). The brain lesions have since been followed with serial MRI studies and found to be stable. Examinations by her neurosurgeon showed no neurologic deficit. Recent laboratory studies revealed normal liver and kidney function. Her other medications were lorazepam, carisoprodol, and famotadine. Her ocular history is significant for myopia with astigmatism.

View larger version (37K): [in this window] [in a new window] Figure 1. Magnetic resonance imaging of multiple cavernous hemangiomas of the brain and brainstem. A, Multiple hyperintense lesions with TI-weighted imaging; B, corresponding larger hypointense areas consistent with hemosiderin deposition with gradient echo imaging.

On examination, we found her best-corrected visual acuity was 20/25 OU. The right inferior iris had a lobulated blood-filled mass that appeared to be vascular in nature and did not extend into the angle on gonioscopy (Figure 2). Anterior segment echography showed this lesion to be 2.1 mm thick with an irregular internal structure and entirely contained within the iris. Iris angiography did not detect flow through the lesion. Golden brown deposits in the subepithelial layers and superficial stroma of her cornea and anterior stellate golden brown deposits in her lenses were thought to be secondary side effects of her thioridazine regimen. Her fundi were normal. She had no cutaneous vascular lesions.

View larger version (42K): [in this window] [in a new window] Figure 2. Inferior iris vascular tumor not involving the angle, right eye.

Comment
We describe a patient with a vascular iris lesion and CNS cavernous hemangiomas that may represent a single disease process. Although we have no histopathologic findings from her iris lesion, its clinical appearance seems consistent with past reports of histologically proven iris cavernous hemanigomas.³ Her CNS lesions have the MRI characteristic of benign cavernous hemangiomas^5-6: no mass effect, hyperintensity on T1 weighting (Figure 1A), and significant T2 shortening creating a larger black halo representing hemosiderin deposition most notable on gradient echo (Figure 1 Figure 1B). In addition, the MRI lesions have not changed for the past 7 years. Multiple CNS hemangiomas raise the possibility of an inherited condition; however, the patient had no knowledge of this problem in her family.

To our knowledge, the association between CNS and iris hemangiomas has not been previously described. Furthermore, this patient did not have any retinal vascular abnormalities. We propose that this clinical syndrome may represent a new type of disseminated hamartoma distinctly different from other phakomatoses.

AUTHOR INFORMATION
The authors have no proprietary or financial interest in any products mentioned herein.

Scott A. Larson, MD; Thomas A. Oetting, MD, MS
Iowa City, Iowa

Corresponding author: Thomas A. Oetting, MD, MS, UIHC, Deptartment of Ophthalmology and Visual Sciences, 200 Hawkins Dr, Iowa City, IA 52242-1091 (e-mail: thomas-oetting{at}uiowa.edu).

REFERENCES
1. Ferry AP. Hemangiomas of the iris and ciliary body: do they exist? a search for a histologically proved case. Int Ophthalmol Clin. 1972;12:177-194. FULL TEXT | PUBMED 2. Prost M. Cavernous hemangioma of the iris. Ophthalmologica. 1987;195:183-187. ISI | PUBMED 3. Lam S. Iris cavernous hemangioma in a patient with recurrent hyphema. Can J Ophthalmol. 1993;28:36-39. ISI | PUBMED 4. Naidoff MA, Kenyon KR, Green RW. Iris hemangioma and abnormal retinal vasculature in a case of diffuse congenital hemangiomatosis. Am J Ophthalmol. 1971;72:633-644. ISI | PUBMED 5. Gomori JM, Grossman RI, Goldberg HI, Hackney DO, Zimmerman RA, Bilaniuk LT. Occult cerebral vascular malformations: high field MR imaging. Radiology. 1986;158:707-713. FREE FULL TEXT 6. Osborn AG. Intracranial vascular malformations. In: Diagnostic Neurology. St Louis, Mo: Mosby-Year Book Inc; 1994:284-328.

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