A 48-year-old man presented with a longstanding left upper eyelid mass that increased in size when bending forward. The mass was associated with enophthalmos and slowly increased in size with Valsalva maneuver. Magnetic resonance imaging and arteriography demonstrated a vascular malformation without an abnormal arterial component. Because of his increasing pain, the vascular malformation was excised in a multidisciplinary approach involving intraoperative fluoroscopic mapping, cyanoacrylate embolization, and surgical excision. Histopathology was consistent with a benign vascular malformation. Clinically, this was a low-flow, distensible venous malformation—or orbital varix.
We report a patient with left eye positional transient visual obscurations, diplopia, a left afferent pupillary defect, and optic disk edema—all occurring during pregnancy. Noncontrast magnetic resonance imaging revealed an orbital mass encasing the optic nerve and extending through the superior orbital fissure into the cavernous sinus. An magnetic resonance imaging with gadolinium after parturition and strongly positive somatostatin receptor scintigraphy suggested the diagnosis of meningioma. Biopsy confirmed the diagnosis, and she was treated with CyberKnife stereotactic radiosurgery. In cases of atypical presentation, somatostatin receptor scintigraphy can help distinguish optic nerve sheath meningioma from alternative orbital masses.
Dermatofibromas are most frequently encountered in women on the lower extremities, often after minor trauma. A recurrent lesion of the right lower eyelid developed in a 64-year-old woman. It harbored “monster cells” that were large, with either multiple nuclei or a single, large, convoluted, and hyperchromatic nucleus. The presence of these cells does not signify a malignant transformation. The background cells were either histiocytoid (many were adipophilin positive), spindled cells, or dendritiform cells without mitoses. Factor XIIIa, CD68, and CD163 immunostaining was positive, and a subpopulation of CD1a+ Langerhans cells was intermixed. Facial and eyelid dermatofibromas are more likely to recur and deserve wider, tumor-free surgical margins. Their microscopic differential diagnosis includes a cellular scar, peripheral nerve tumor, atypical fibrous xanthoma, and dermatofibrosarcoma protuberans.
The cavernous venous malformation of the orbit, previously called cavernous hemangioma, is the most common primary orbital lesion of adults. Cavernous venous malformation occurs more often in women and typically presents in the fourth and fifth decades of life. It is a benign vascular malformation characterized by a well-defined capsule and numerous large vascular channels. The most common sign of cavernous venous malformation is progressive axial proptosis from the preferential involvement of the intraconal orbital space. Optic nerve damage and other signs of orbital pathology may be present, with a variable degree of visual impairment. The combination of ultrasound, computed tomography, and magnetic resonance imaging leads to an accurate diagnosis in the vast majority of cases. Surgical and nonsurgical treatments are required in case of symptomatic lesions, with a characteristic multidisciplinary management influencing optimal outcome. Orbitotomy represents the traditional surgical approach. Recently, the endoscopic transnasal approach to the orbital cavity has gained interest, representing a feasible and safe, less-invasive surgical technique for the management of cavernous venous malformation.