Orbital and Ocular Venous Congestion: A Rare Manifestation of Hypervascular Paget Disease of the Bone.

Abstract

A 73-year-old male with a history of incidentally diagnosed Paget disease of bone affecting the skull and left orbit 2 years prior presented with 3 months of vision loss, proptosis, and periorbital swelling of the OS. Examination showed best-corrected Snellen visual acuity of 20/150 in the affected eye, intact motility, 7 mm of relative proptosis, significant dilated and tortuous "corkscrew" conjunctival vessels, serous choroidal and retinal detachments, optic nerve hyperemia, and venous tortuosity and dilation. Although the bony lesions in the left orbit were stable from 1 year prior on imaging, the diagnostic angiogram demonstrated osseous blush and hypervascularity of the lesion. It was thus determined that the abnormal bone of the left orbit with enhanced blood flow was adversely affecting the blood flow in the orbit and eye. The patient underwent coil embolization of the feeding vessels and external blood supply to the lateral orbital wall and hyperostotic Paget lesion via coil embolization of the left internal maxillary artery and left middle meningeal artery with improvement of the choroidal effusions and subretinal fluid, followed by bony orbital decompression with improvement in the proptosis. This is a novel case of orbital and ocular vascular congestion due to Paget disease.