Incidental fundus lesions found during routine exam

The patient was recently diagnosed with hypertension.

Jeffrey Chang

Vivek Chaturvedi

A 29-year-old white man presented to the Lahey Clinic for a routine eye exam.

His medical and ocular histories were notable for a recent diagnosis of hypertension that was well-controlled with metoprolol, arthroscopic surgery on his right knee for a torn anterior cruciate ligament and myopia. He works in a software company, has no history of smoking or illicit drug use, and drinks alcohol socially. To his knowledge, there is no family history of ocular or systemic diseases.

Examination

On exam, his best corrected visual acuity was 20/25⁺² in the right eye and 20/20 in the left eye. His IOPs were 16 mm Hg in the right eye and 15 mm Hg in the left eye. Pupils were symmetric with no afferent pupillary defect. Extraocular motility, confrontational visual fields and anterior segment examination were within normal limits in both eyes.

The right fundus exam was normal. Fundus examination of the left eye demonstrated two well-circumscribed, yellowish-colored nodules measuring approximately one-quarter disc diameter located in the periphery at the 7 o’clock and 11 o’clock positions (Figures 1 and 2). Fluorescein angiography showed early hyperfluorescence of the entire lesion followed by late leakage (Figure 3). Optical coherence tomography of the lesion showed a well-circumscribed elevated lesion with no subretinal involvement (Figure 4). OCT of the macula was normal (Figure 5).

What is your diagnosis?

Fundus lesions

The differential diagnosis for a retinal lesion that demonstrates hyperfluorescence on fluorescein angiography includes arteriovenous malformations, retinal macroaneurysm, astrocytoma, retinal telangiectasias and hamartomas including retinal capillary hemangioma. Given the history, funduscopic appearance and fluorescein angiography, a diagnosis of retinal capillary hemangioma was made.

Discussion

Retinal capillary hemangiomas are benign vascular tumors that arise from the optic disc or retinal vasculature. They are characterized by dilated feeder arteries and draining veins, and intraretinal or subretinal exudates. Our patient had two peripheral retinal lesions with an afferent feeding and efferent draining vessel and no surrounding exudate or subretinal fluid. Fluorescein angiography showed early filling of the lesion with late leakage. These findings are most consistent with a retinal capillary hemangioma.

Retinal capillary hemangiomas can be sporadic or associated with von Hippel-Lindau (VHL) syndrome. They can occur anywhere in the fundus, but they most often present in the periphery. Solitary lesions found in only one eye in otherwise healthy individuals are more likely associated with the sporadic form. Retinal capillary hemangiomas associated with VHL are more frequently bilateral and multifocal.

VHL is characterized by retinal capillary hemangiomas, central nervous system hemangioblastomas, renal cell carcinomas, pheochromocytomas, pancreatic islet cell tumors, and renal, pancreatic and epididymal cysts. It is an autosomal dominant disease. Our patient had no family history of VHL; however, given his history of hypertension and our incidental finding of two retinal capillary hemangiomas, we proceeded with a systemic workup for VHL syndrome.

An MRI of the head showed multiple cerebellar lesions consistent with hemangioblastomas. A CT scan of the abdomen showed a few scattered pancreatic cysts, the largest measuring 1.5 cm, and innumerable cystic and solid lesions in the kidneys bilaterally that were highly suspicious of bilateral renal cell carcinoma. A CT scan of the pelvis showed small cysts on the epididymis bilaterally.

Treatment

Treatment of these lesions is focused on preserving or restoring vision. Treatment options include careful observation, cryotherapy, photodynamic therapy, laser photocoagulation, radiotherapy and transpupillary thermotherapy. The most common treatment for lesions up to 3 mm in diameter is photocoagulation.

References

• Atebara NH. Retinal capillary hemangioma treated with photodynamic therapy. Am J Ophthalmol. 2002;134(5):788-790.
• Moskowitz C, Edelstein C, Oh M, Burnier MN Jr. Retinal capillary hemangioma in von Hippel-Lindau disease. Can J Ophthalmol. 2005;40(2):192-194.
• Ridley M, Green J, Johnson G. Retinal angiomatosis: the ocular manifestations of von Hippel-Lindau disease. Can J Ophthalmol. 1986;21(7):276-283.
• Rodriguez-Coleman H, Spaide RF, Yannuzzi LA. Treatment of angiomatous lesions of the retina with photodynamic therapy. Retina. 2002;22(2):228-232.
• Singh A, Shields J, Shields C. Solitary retinal capillary hemangioma: hereditary (von Hippel-Lindau disease) or nonhereditary? Arch Ophthalmol. 2001;119(2):232-234.
• Singh AD, Nouri M, Shields CL, Shields JA, Smith AF. Retinal capillary hemangioma: a comparison of sporadic cases and cases associated with von Hippel-Lindau disease. Ophthalmology. 2001;108(10):1907-1911.

• Namrata Nandakumar, MD, can be reached at Tufts Medical Center, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
• Fina C. Barouch, MD, is affiliated with Tufts Medical Center and Lahey Clinic, and can be reached at Lahey Clinic, 1 Essex Center Drive, Peabody, MA 01960; 978-538-4400; Web site: www.lahey.org.

• Edited by Jeffrey Chang, MD, and Vivek Chaturvedi, MD. Drs. Chang and Chaturvedi can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Chang and Chaturvedi have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.