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RETINAL CAVERNOUS HEMANGIOMA: Fifty-Two Years of Clinical Follow-up With Clinicopathologic Correlation

Shields, Jerry A. MD*; Eagle, Ralph C. Jr MD; Ewing, Madeleine Q. MD; Lally, Sara E. MD*; Shields, Carol L. MD*

Retina:
doi: 10.1097/IAE.0000000000000232
Clinicopathologic Correlation
Abstract

Purpose: To report long-term follow-up and histopathology of a retinal cavernous hemangioma and to review the literature on this subject.

Methods: A newborn girl was noted immediately after birth to a have hyphema and vitreous hemorrhage in her left eye. The bleeding recurred throughout childhood and the etiology was not determined. Upon referral at age 22, a large retinal cavernous hemangioma was first recognized and recurrent hemorrhages continued, eventually leading to pain and secondary glaucoma. The patient declined treatment. At age 52, the hemangioma was stable in size, but ocular pain and blindness necessitated enucleation.

Results: During the 52-year course, the fundus mass did not enlarge, but numerous episodes of hyphema and vitreous hemorrhage led to chronic glaucoma and eventual blindness. The main histopathologic finding in the disorganized globe was a retinal mass composed of large endothelial-lined vascular channels with thin walls, typical of a retinal cavernous hemangioma. The tumor extended anteriorly into the ciliary body, explaining the recurrent hyphemas. Additional chronic features included extensive fibrosis of the entire anterior segment, iris, and retina with proliferative vitreoretinopathy and widespread intraocular hemosiderosis from chronic hemorrhage. The patient has been free of pain since enucleation.

Conclusion: Retinal cavernous hemangioma is a congenital stationary lesion that can cause recurrent intraocular hemorrhage, fibrosis, glaucoma, pain, and blindness, requiring enucleation. Retinal cavernous should be included in the differential diagnosis of childhood hyphema and vitreous hemorrhage.

Author Information

*Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania;

Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania; and

Department of Ophthalmology, Pennsylvania Hospital, Philadelphia, Pennsylvania.

Reprint requests: Jerry A. Shields, MD, Ocular Oncology Service, Suite 1440, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107; e-mail: jerryashields@gmail.com

Supported by the Eye Tumor Research Foundation, Philadelphia, PA (J.A.S., C.L.S.), the Ocular Oncology Fund, Wills Eye Hospital (J.A.S., C.L.S.), Mellon Charitable Giving from the Martha W. Rodgers Charitable Trust (C.L.S.), Lift for a Cure, Morrisdale, PA (C.L.S., J.A.S.), and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital (R.C.E.), the Lucille Wiedman Fund for Pediatric Eye Cancer, Philadelphia, PA (J.S., C.L.S.).

None of the authors have any conflicting interests to disclose.

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

© 2014 by Ophthalmic Communications Society, Inc.