A Troublesome Case of Indirect Carotid-Cavernous Fistula Presenting with Proptosis without Pulsation

Introduction

Carotid-cavernous fistula (CCF) is a rare condition in which an abnormal communication develops between the cavernous sinus (CS) and the carotid arterial system. CCFs are divided into subgroups according to the main cause or anatomical differentiation [1]. CCFs can develop spontaneously or after major head trauma. Shunts between the intracavernous part of the internal carotid artery (ICA) and the CS are called direct fistulas (type A), which are high-flow lesions mostly caused by trauma. Indirect fistulas, on the other hand, present slow-flow lesions and are more likely to occur spontaneously. Their locations are between the meningeal branches of the ICA (type B), external carotid artery (ECA) (type C), or both (type D) and the CS [1], [2].

The main symptoms of proptosis, chemosis, and orbital bruit during auscultation and ophthalmoplegia are due to progressive congestion of the venous sinuses draining into the CS. The severity of the symptoms is determined by the blood flow velocity of the shunts [3], [4]. Hence, direct fistulas can be identified easily while indirect fistulas are frequently misdiagnosed. With the help of cerebral angiography, which is the gold standard diagnostic method for CCFs [5], an accurate diagnosis and treatment plan with endovascular flow converting stents can be achieved in order to improve the clinical findings in appropriate cases [3].

Publication History

Received: 13 October 2022

Accepted: 20 November 2022

Accepted Manuscript online:
22 November 2022

Article published online:
12 January 2023

• References

• 1 Khan S, Gibbon C, Johns S. A rare case of bilateral spontaneous indirect caroticocavernous fistula treated previously as a case of conjunctivitis. Ther Adv Ophthalmol 2018; 10: 251584141878830
• 2 Abecassis IJ, Morton RP, Kim LJ. et al. Combined direct and indirect traumatic carotid-cavernous fistula (CCF): Case report and review of the literature. J Clin Neurosci 2017; 44: 240-242
• 3 Yüce G, Doğan A. A case of indirect carotid cavernous fistula presenting with proptosis and pulsatile exophthalmos. Gulhane Med J 2021; 63: 160-162
• 4 DʼAngelo L, Paglia F, Caporlingua A. et al. Atypical Manifestation of Direct Low-Flow Carotid-Cavernous Fistula: Case Report and Review of the Literature. World Neurosurg 2019; 125: 456-460
• 5 Laurentino M, Afonso COM, Rocha TAF. et al. Indirect carotid cavernous fistula type c: a case report. J Neurol Sci 2015; 357: e180
• 6 Ellis JA, Goldstein H, Connolly jr. ES. et al. Carotid-cavernous fistulas. Neurosurg Focus 2012; 32: E9
• 7 Barrow DL, Spector RH, Braun IF. et al. Classification and treatment of spontaneous carotid cavernous sinus fistulas. J Neurosurg 1985; 62: 248-256
• 8 Chaudhry I, Elkhamry S, Al-Rashed W. et al. Carotid cavernous fistula: ophthalmological implications. Middle East Afr J Ophthalmol 2009; 16: 57-63
• 9 Henderson AD, Miller NR. Carotid-cavernous fistula: current concepts in aetiology, investigation, and management. Eye (Lond) 2018; 32: 164-172
• 10 Korkmazer B, Kocak B, Tureci E. et al. Endovascular treatment of carotid cavernous sinus fistula: A systematic review. World J Radiol 2013; 5: 143-155
• 11 Tjong FVY, de Ruijter UW, Beurskens NEG. et al. A comprehensive scoping review on transvenous temporary pacing therapy. Neth Hear J 2019; 27: 462-473