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From Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania (MP, CLS, SA, JAS); and the Department of Biomedical and Clinical Science "Luigi Saccoâ[euro]*, Eye Clinic, University of Milan, Luigi Sacco Hospital, Milan, Italy (MP).
Supported by the Retina Research Foundation of the Retina Society (CLS), Lift for a Cure, Morrisdale, Pennsylvania, and the Eye Tumor Research Foundation, Philadelphia, Pennsylvania.
The authors have no financial or proprietary interest in the materials presented herein.
Introduction
Persistent fetal vasculature (PFV) is classically a unilateral sporadic condition defined by the presence of one or more components of the hyaloid vascular system persistent at birth.1,2 Despite traditional classifications subdividing this condition into purely anterior or posterior forms, PFV typically presents with a combination of both forms involving the iris, lens, and vitreous with patent or sclerosed fetal vascularity to these structures.1 Management depends on the extensiveness of the vascular tissue and related complications of hemorrhage, retinal traction, and glaucoma. Importantly, it should be recognized that PFV can occur in approximately 20% of eyes displaying medulloepithelioma; thus, evaluation for a solid tumor is critical.3 Furthermore, medulloepithelioma (a potentially malignant tumor) often demonstrates a retrolenticular vascularized membrane, resembling PFV.4
We report a case of a patient initially suspected to have ciliary body medulloepithelioma and later, upon referral, confirmed to have PFV. Fluorescein angiography, ultrasonography, and ultrasound biomicroscopy were instrumental in establishing the lack of tumor. Specifically, fluorescein angiography depicted the direction of flow from the Mittendorf dot to the periphery of the lens, consistent with PFV and opposite to the flow related to the retrolenticular vascularized membrane from medulloepithelioma.5
Case Report
A 17-month-old girl developed esophoria in the right eye with leukocoria and was found to have a possible ciliary body medulloepithelioma. Previous ocular and medical history were unremarkable.
On referral, visual acuity was fix and follow bilaterally. Intraocular pressures were 18 mm Hg in the right eye and 15 mm Hg in the left eye. The left eye was normal. Axial length of the eyes with ultrasonography was 22.9 mm in the right eye and 21.4 mm in the left eye. The left eye was normal and findings were limited to the right eye.
On examination, the right eye demonstrated findings suggestive of PFV, including non-perfused persistent...