HISTORICAL VIGNETTE J Neurosurg 126:1995–2001, 2017 C arotid-Cavernous fstula (CCF) was one of the ear- liest recognized intracranial vascular lesions in his- tory due to obvious clinical features such as pulsat- ing exophthalmos. 22,27 However, it wasn’t until years after the frst report of this condition that the etiology of CCF was elucidated. CCFs are abnormal vascular shunts that allow blood to fow from the internal or external carotid artery directly to the cavernous sinus (Fig. 1). These fstu- las commonly lead to engorgement of draining veins and orbital venous congestion, resulting in clinical manifesta- tions, such as vision decline, pulsating proptosis, cranial nerve palsies, headaches, tinnitus, conjunctival chemosis, and cephalic bruit. Furthermore, it can also lead to cor- tical venous refux and cerebral venous hypertension. 17 During the journey to the development of better treatment for CCF, the feld of endovascular neurosurgery was born. Currently, MRI and catheter-based angiography are the methods of choice for diagnosis, and endovascular oblit- eration is the mainstay of treatment. Early Cases The condition of pulsating exophthalmos was frst de- scribed in 1809 by Benjamin Travers (Fig. 2; born on April 3, 1783, in Cheapside, London). 47 The patient presented with proptosis, chemosis, and an ocular bruit. When the ipsilateral carotid artery was compressed, the exophthal- mos improved and the bruit disappeared. Travers sug- gested that the condition was confned to the eye and was most likely due to aneurysm of anastomosis of the orbit or intraorbital aneurysm. 47 In 1812, Dalrymple (1772–1947) reported a second case of pulsating exophthalmos. 10 Trav- ers’ proposed etiology was further supported by Guthrie (1785–1856) when he performed the frst autopsy on a pa- tient with pulsating exophthalmos in 1923 and observed bilateral large nut–sized aneurysms of the ophthalmic artery. 20,23,33 In 1839, Busk (1807–1886) reported fndings similar to Guthrie’s, and the English school assumed that aneurysm of the ophthalmic artery was the underlying mechanism. 7,27 In France, however, an autopsy report from Baron in 1835, even prior to Busk’s report, showed an ab- normal connection between the internal carotid and the cavernous sinus in a patient with pulsating exophthalmos. 3 Unfortunately, his brief report largely escaped the attention of others. The idea of abnormal communication between the cavernous sinus and the carotid artery became recog- nized as the true source for the constellation of symptoms when Gendrin, Nelaton, and Hirschfeld reported similar autopsy fndings in 1841, 1856, and 1857, respectively. 3,27 Furthermore, the autopsy reports demonstrated that the fstula was located intracranially rather than intraorbitally. In 1870, Delens’ work on cadavers (Fig. 3) showed that ABBREVIATIONS CCF = carotid-cavernous fistula. SUBMITTED October 13, 2015. ACCEPTED May 24, 2016. INCLUDE WHEN CITING Published online September 16, 2016; DOI: 10.3171/2016.5.JNS152372. A brief history of carotid-cavernous fstula Min Lang, MS, 1 Ghaith Habboub, MD, 2 Jeffrey P. Mullin, MD, MBA, 2 and Peter A. Rasmussen, MD 1,2 1 Cerebrovascular Center and 2 Department of Neurosurgery, Cleveland Clinic, Cleveland, Ohio Carotid-cavernous fstula was one of the frst intracranial vascular lesions to be recognized. This paper focuses on the historical progression of our understanding of the condition and its symptomatology—from the initial hypothesis of oph- thalmic artery aneurysm as the cause of pulsating exophthalmos to the recognition and acceptance of fstulas between the carotid arterial system and cavernous sinus as the true etiology. The authors also discuss the advancements in treat- ment from Benjamin Travers’ early common carotid ligation and wooden compression methods to today’s endovascular approaches. https://thejns.org/doi/abs/10.3171/2016.5.JNS152372 KEY WORDS carotid–cavernous sinus; fistula; pulsating exophthalmos; carotid ligation; history ©AANS, 2017 J Neurosurg Volume 126 • June 2017 1995