AUTHOR=Sun Peng , Chai Yuan , Fang Wei , Chen Hu , Long Qianfa , Zhao Zhenwei , Zhang Tao TITLE=Case report: Spontaneous carotid-cavernous fistula associated with persistent primitive trigeminal artery aneurysm rupture JOURNAL=Frontiers in Neurology VOLUME=Volume 13 - 2022 YEAR=2022 URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2022.923186 DOI=10.3389/fneur.2022.923186 ISSN=1664-2295 ABSTRACT=Background The incidence of carotid cavernous fistula (CCF) associated with persistent primitive trigeminal artery (PPTA) aneurysm rupture is extremely rare. We presented a case about a spontaneous CCF secondary to a ruptured PPTA aneurysm, which was successfully embolized with coils and onyx-18 by a trans-arterial approach. Case Presentation A 55-year-old female suffered sudden onset of headache, left orbital pain and pulsatile exophthalmos for a month without any history of trauma. Angiography revealed a left-sided CCF associated with a ruptured PPTA aneurysm, with a major drainage to the ipsilateral superior ophthalmic vein. Through a trans-arterial approach, the fistula and ruptured PPTA aneurysm were embolized with coils and onyx-18, without excessively cavernous sinus sacrificing. The well preserved PPTA vanished at 4 month follow-up. The patient had no neurological deficit during hospitalization and 1 year follow-up period. Conclusion Trans-arterial approach was a reasonable choice for spontaneous CCF associated with ruptured PPTA aneurysm. The requirement for PPTA preservation depended on individual evaluation.