BACKGROUND Carotid-cavernous fistulas (CCFs) are acquired pathological shunting lesions between the carotid artery and the cavernous sinus leading to elevated intraocular pressure (IOP). CCFs are commonly treated via endovascular embolization, which theoretically restores physiological pressure differentials. OBJECTIVE To present our institutional data with CCF treated with embolization and discuss endovascular routes, recurrence rates, and dynamic IOP changes. METHODS Retrospective analysis of 42 CCF patients who underwent Onyx (Covidien, Irvine, California) embolization and pre- and postoperative IOP measurement at a single institution. RESULTS CCFs were 19.0% direct (type A) and 81.0% indirect (types B, C, or D). Onyx-18 liquid embolisate was used during all embolizations. Overall rate of total occlusion was 83.3% and was statistically similar between direct and indirect fistulas. Preoperative IOP was elevated in 37.5%, 100.0%, 75.0%, and 50% in type A, B, C, and D fistulas, respectively. Average ΔIOP was −7.3 ± 8.5 mmHg (range: −33 to +8). Follow-up time was 4.64 ± 7.62 mo. Full angiographic occlusion was a predictor of symptom resolution at 1 mo (P = .026) and 6 mo (P = .021). Partial occlusion was associated with persistent symptoms postoperatively at 1 mo (P = .038) and 6 mo follow-up (P = .012). Beyond 6 mo, negative ΔIOP was associated with continued symptom improvement. Recurrence occurred in 9.5% of patients, all of which were indirect CCFs. CONCLUSION Onyx embolization of CCF is an effective treatment for CCF and often results in the reversal of IOP elevation. Full occlusion predicts favorable clinical outcomes up to 6 mo. Postoperative IOP reduction may indicate favorable long-term clinical outcomes.
We report a rare case of Epstein-Barr virus (EBV)-positive polymorphic B-cell lymphoproliferative disorder (LPD) involving the lacrimal gland of a 28-year-old, apparently immunocompetent woman. She presented with a chief complaint of orbital swelling and tenderness and was found to have a lesion involving the right lacrimal gland and distal superior and lateral rectus muscles. Histology of the lesion revealed histiocytes with pleomorphic nuclei, reactive lymphocytes, and scattered cells that resembled the Reed-Sternberg (R-S) cells of classical Hodgkin lymphoma. The R-S-like cells were positive for PAX5 and CD30 and negative for CD15, supporting a diagnosis of polymorphic B-cell LPD. In situ hybridization for EBV-encoded RNA demonstrated the presence of EBV. Most EBV-positive polymorphic B-cell LPDs are associated with immunodeficiency. However, the patient described is HIV-negative and has no identifiable defects in immunoglobulin levels or cell-mediated immunity. This raises the question of whether she has an underlying immunodeficiency resulting from subtle changes in T-cell physiology, or whether chronic EBV infection contributed to her immune dysfunction through an unclear mechanism. The orbital mass partially regressed with chemotherapy, and the patient has done well clinically with no recurrence of this EBV-LPD for over 2 years.
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