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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
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.
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.
Inferior iris vascular tumor not
involving the angle, right eye.
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 hemangiomas5,6:
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
The authors have no proprietary or financial interest in any products
Corresponding author: Thomas A. Oetting, MD, MS, UIHC, Deptartment
of Ophthalmology and Visual Sciences, 200 Hawkins Dr, Iowa City, IA 52242-1091
Larson SA, Oetting TA. Presumed Iris Hemangioma Associated With Multiple Central Nervous System Cavernous Hemangiomas. Arch Ophthalmol. 2002;120(7):984-985. doi: