Vascular Traumatic Arteriovenous Fistula of the Posterior Inferior Cerebellar Artery Treated with Endovascular Coil Embolization: Case Report James K. Liu, M.D.,* David Decker, B.S.,† Michael S. Tenner, M.D.,‡ William T. Couldwell, M.D., Ph.D.,* and Bennie W. Chiles, III, M.D.† *Department of Neurosurgery, University of Utah, Salt Lake City, Utah, and †Departments of Neurosurgery and ‡Radiology, New York Medical College, Valhalla, New York Liu JK, Decker D, Tenner MS, Couldwell WT, Chiles BW III. Traumatic arteriovenous fistula of the posterior inferior cerebellar artery treated with endovascular coil embolization: case report. Surg Neurol 2004;61:255– 61. KEY WORDS Arteriovenous fistula, posterior inferior cerebellar artery, Guglielmi detachable coil embolization, Wallenberg syndrome, skull base fracture. BACKGROUND High-flow intracranial arteriovenous (AV) fistulas associated with giant varices are rare lesions. These varices can present with symptoms from mass effect, spontaneous hemorrhage, and seizures to cardiac failure. Direct AV fistulas of the posterior inferior cerebellar artery (PICA) are extremely rare lesions, with only two cases reported in the literature. CASE DESCRIPTION The authors present an unusual case of a 25-year-old male with a direct AV fistula of the PICA that resulted from a fracture of the occipital condyle. This high-flow AV fistula drained into a giant varix of the vein of the lateral recess that compressed the brainstem, resulting in a Wallenberg syndrome. The patient underwent embolization of the proximal PICA feeding the fistula with a Guglielmi detachable coil (GDC), which resulted in thrombosis of the varix. A postembolization angiogram showed occlusion of the PICA AV fistula and draining varix. A computed tomography (CT) scan performed at a 10-month follow-up visit showed dramatic decompression of the brainstem. Although the patient continued to have some sensory changes secondary to Wallenberg syndrome, he was otherwise doing well neurologically. CONCLUSION The treatment of this lesion is difficult because of its location near the brainstem. Postocclusion edema or hemorrhage can result in mass effect and life-threatening brainstem compression. Our patient, whose AV fistula was caused by trauma, was treated effectively with GDC embolization. © 2004 Elsevier Inc. All rights reserved. Address reprint requests to: Bennie W. Chiles III, M.D., Department of Neurosurgery, New York Medical College, Munger Pavilion, Valhalla, NY 10595. Received May 8, 2002; accepted March 27, 2003. © 2004 Elsevier Inc. All rights reserved. 360 Park Avenue South, New York, NY 10010 –1710 ntracranial arteriovenous (AV) fistulas are rare lesions that are usually associated with a giant varix when the fistula is of high flow [2,4,13,25]. These varices can present with symptoms due to mass effect, spontaneous hemorrhage, seizures, or cardiac failure. AV fistulas of the vertebrobasilar circulation are extremely rare lesions, with only three cases reported in the literature [13,25,26]. Two cases occurred spontaneously in the posterior inferior cerebellar artery (PICA) and were associated with a giant varix. One was treated surgically [25] and the other by endovascular balloon occlusion [26]. The third case was a spontaneous intracranial vertebral artery fistula treated by surgical clipping via a far lateral approach [13]. The treatment of these lesions is difficult because of their location near the brainstem. Postocclusion edema or hemorrhage can result in mass effect and life-threatening brainstem compression [24 –26]. We report here a case of a direct AV fistula of the PICA secondary to trauma, which was occluded with GDC embolization. I Case Report HISTORY A 25-year-old male suffered multiple injuries in a motor vehicle accident, including pelvic fractures, 0090-3019/04/$–see front matter doi:10.1016/S0090-3019(00)00422-1 256 Surg Neurol 2004;61:255– 61 1 Liu et al CT scan of the base of the skull demonstrating a fracture through the left occipital condyle. lung contusions, a torn urethra, and a skull base fracture through the left occipital condyle (Figure 1). He was stabilized and treated at a local hospital and was transferred to a rehabilitation center. Four months after the accident, the patient was referred to our center for evaluation of the stability of the occipitocervical junction. A magnetic resonance image (MRI) of his cervical spine revealed a large flow void, presumably a pseudoaneurysm, compressing the brainstem (Figure 2A, B). The patient stated that he had occasional double vision, dizziness, and ringing in his left ear. He also experienced decreased sensation to heat on the left side of his face and on the right side of his body while showering. EXAMINATION The patient was alert and awake. The pupils were anisocoric (right, 4 mm; left, 3 mm) but reactive to light. There was noticeable ptosis and miosis on the left side. The extraocular muscles were intact. The patient exhibited decreased sensation to pain and temperature on the left side of the face and on the right side of the body. The uvula was deviated to the right, and the tongue was deviated to the left. A cerebral angiogram revealed an AV fistula arising from the left PICA (Figure 3A–C). The fistula arose approximately 1 cm from the origin of the PICA and drained into an extremely dilated varix of the vein of the lateral recess, which then coursed into the region of the petrosal and basilar venous plexus (Figure 3D, E). No distal vessels were seen beyond the fistulous connection from the PICA. TREATMENT WITH GDC EMBOLIZATION The patient underwent embolization of the AV fistula with a GDC. A microcatheter was advanced into the left vertebral artery and then into the left PICA. A single 2 mm ⫻ 2 cm GDC was deposited transversely to the long axis of the PICA, distal to its origin from the vertebral artery and proximal to the dilated fistulous vein. The fistula was thrombosed and occluded. A postembolization angiogram showed occlusion of the left PICA and AV fistula with collateral circulation coming from the left anterior inferior cerebellar artery (AICA) feeding the PICA region (Figure 3F). POSTEMBOLIZATION COURSE After occlusion of the fistula, the patient underwent close monitoring in the intensive care unit for 24 hours. Postembolization CT and MRI showed thrombosis of the dilated varix with continued compression of the lateral medulla (Figure 4A, B). The patient remained stable neurologically and was discharged on the third hospital day. At a 10-month Traumatic PICA AV Fistula 2 Surg Neurol 257 2004;61:255– 61 MRI of the cervical spine, sagittal (A) and axial (B) views, revealing the dilated varix, compressing the left lateral aspect of the brainstem. follow-up visit, the patient was functioning well; however, he continued to have a left 12th nerve palsy and loss of sensation in his ipsilateral face and contralateral body. A CT angiogram showed dramatic resolution of the giant draining varix with decompression of the lateral medulla without evidence of recurrent fistula (Figure 4C, D). Discussion AV fistulas are abnormal connections between the arterial and venous circulations and may occur anywhere in the body. They were first described in 1762 by Hunter, a Scottish anatomist, who noted that a fistulous connection developed after an artery and vein were simultaneously injured [15]. Their etiology may be spontaneous, iatrogenic, or posttraumatic. When they occur spontaneously, they are often associated with fibromuscular dysplasia [21], neurofibromatosis, or congenital abnormalities [11,12,17]. Most iatrogenic causes are from percutaneous angiography and rarely from intraoperative trauma to the vertebral artery during cervical spine surgery [5,20,28]. Traumatic causes include penetrating and nonpenetrating injuries [1,3,18]. Direct intracranial AV fistulas are very uncommon. The majority are carotid-cavernous fistulas that occur as a result of trauma, although some occur spontaneously as a result of an intracavernous aneurysmal rupture [6,10,27]. Most AV fistulas of the vertebrobasilar circulation are extracranial, occurring mostly in the high cervical vertebral artery [12,18,21]. Intracranial varices that are produced by a direct communication between an artery and a draining varix resulting in a high-flow AV shunt are very rare [25]. These AV fistulas have an angiographic appearance distinct from that of classic arteriovenous malformations or vein of Galen malformations. Vinuela et al [26] reported the first spontaneous AV fistula of the PICA associated with a large varix that was successfully treated by detachable balloon occlusion. The same authors also reported a similar case in which the fistula was surgically ligated [25]. Halliday et al [13] reported an intracranial vertebral AV fistula that was surgically clipped with a far lateral approach. Traumatic injury to the intracranial arteries, by either closed or penetrating head trauma, is an uncommon event that may result in arterial dissections, pseudoaneurysms, and fistulas [1,14]. Our patient suffered a fracture of the left occipital condyle, and the fistula developed 1 cm from the origin of the PICA. The anatomic location of the fracture suggests that it was probably involved in the formation of the AV fistula. 258 Surg Neurol 2004;61:255– 61 Liu et al Pre-embolization (A–E) and postembolization (F) angiograms. (A) Lateral view of early arterial phase shows filling of the large draining varix through a direct fistula that originates from the PICA. (B) and (C) Lateral (B) and oblique (C) views of the mid-arterial phase show progressive filling of the large draining varix. (D) and (E) Frontal (D) and lateral (E) venous phase left vertebral angiogram. The dilated vein of the lateral recess is seen to drain into the dilated petrosal venous system. (F) Postembolization angiogram shows obliteration of the PICA, fistula, and varix from the circulation. 3 The clinical presentation depends on the size and location of the fistula and draining varix and on the age of the patient. High-flow intracranial AV fistulas can cause neurologic symptoms by local mass effect of the expanding varix and/or by a vascular “steal” phenomenon due to shunting of arterial blood from neural structures [18,19,21,22]. The patient described here developed a Wallenberg syndrome that was likely caused by compression of the lateral medulla by the draining varix. The patient also had a left 12th nerve palsy, which was most likely caused by the initial fracture through the occipital condyle. Another possibility for the 12th nerve palsy, although less likely, may be the dilated varix compressing the neighboring hypoglossal nerve rootlets as they exit the medulla and enter the hypoglossal canal. This anatomic relationship has been well described [16]. The primary goal of therapy is to occlude the fistula while preserving distal flow to the artery [8,14], which has been successfully achieved with either surgical ligation or endovascular balloon occlusion [13,23,25,26]. In the present case, the PICA and the fistula were occluded by GDC embolization. Because of the high-flow fistula in our case, attempts at embolizing the fistula itself may have resulted in distal embolization into the lungs. Thus, the PICA was occluded just distal to its origin from the vertebral artery and before the fistulous connection. When the placement of the coil is confirmed, it is electrolytically detached, and clotting and occlusion of the fistula proceed. The draining varix eventually undergoes progressive thrombosis secondary to venous stasis. One major concern of sudden occlusion of a highflow AV fistula is the development of acute parenchymal edema and/or hemorrhage, explained by the normal pressure perfusion breakthrough theory [24]. By removing the sump effect of an AV fistula, the acute diversion of blood flow to areas of ischemic cerebral vasculature lacking regional autoregulation may produce substantial vasogenic edema Traumatic PICA AV Fistula Surg Neurol 259 2004;61:255– 61 Postembolization CT and MRI scans. Immediate post-embolization axial CT (A) and MRI (B) scans show thrombosis of the varix with continued compression of the lateral medulla. No evidence of edema or hemorrhage is seen in the parenchyma. At a 10-month follow-up visit, the CT angiogram (C) shows the previously thrombosed varix has dramatically diminished and the brainstem is adequately decompressed. Three-dimensional reconstruction (D) shows patency of the left vertebral artery without evidence of flow into the previous varix of the vein of the lateral recess. 4 260 Surg Neurol 2004;61:255– 61 and possible hemorrhage [9,12]. Another potential complication is the increase in mass effect secondary to swelling of the thrombosed varix, which may compress vital neural structures [26]. In some cases, acute obliteration of the fistula may lead to acute cardiac decompensation [7]. These complications may be minimized by performing staged procedures and controlling the blood pressure intraand postoperatively [12,25,26]. Vineula et al [25] described the “balloon-in-tandem” technique, where the proximal balloon functions to decrease local flow through the AV fistula while the distal balloon is fully inflated to occlude it. This technique minimizes the risk of embolizing the distal varix and the lungs by promoting adequate proximal control of the high-flow AV fistula. A postembolization angiogram showed that the PICA region was supplied by collateral feeders from the AICA (Figure 3F). The patient tolerated the procedure well without complications. Immediately after the procedure, his blood pressure was controlled under close monitoring to prevent a sudden increase in perfusion pressure. No signs of mass effect from potential swelling of the thrombosed varix were noted after embolization. REFERENCES 1. Amirjamshidi A, Rahmat H, Abbassioun K. Traumatic aneurysms and arteriovenous fistulas of intracranial vessels associated with penetrating head injuries occurring during war: principles and pitfalls in diagnosis and management. A survey of 31 cases and review of the literature. J Neurosurg 1996;84:769 –80. 2. Aoki N, Sakai T, Oikawa A. Intracranial arteriovenous fistula manifesting as progressive neurological deterioration in an infant: case report. Neurosurgery 1991; 28:619 –23. 3. Avellanosa AM, Glasauer FE, Oh YS. Traumatic vertebral arteriovenous fistula associated with cervical spine fracture. J Trauma 1977;17:885–8. 4. Barnwell SL, Ciricillo SF, Halbach VV, Edwards MS, Cogen PH. Intracerebral arteriovenous fistulas associated with intraparenchymal varix in childhood: case reports. Neurosurgery 1990;26:122–5. 5. Bergstrom K, Lodin H. Arteriovenous fistula as a complication of cerebral angiography. Br J Radiol 1966; 39:263–6. 6. Corradino G, Gellad F, Salcman M. Traumatic carotidcavernous fistula. South Med J 1988;81:660 –3. 7. Day AL, Friedman WA, Sypert GW, Mickle JP. Successful treatment of the normal perfusion pressure breakthrough syndrome. Neurosurgery 1982;11:625–30. 8. Debrun G, Legre J, Karbarian M, Tapias PL, Caron JP. Endovascular occlusion of vertebral fistulae by detachable balloon occlusion with conservation of the vertebral blood flow. Radiology 1979;13:141–7. 9. Drake CG. Cerebral arteriovenous malformations: considerations for and experience with surgical treatment in 166 cases. Clin Neurosurg 1979;26:145–208. 10. Fabian TS, Woody JD, Ciraulo DL, Lett ED, Phlegar RF, Liu et al Barker DE, Burns RP. Posttraumatic carotid cavernous fistula: frequency analysis of signs, symptoms, and disability outcomes after angiographic embolization. J Trauma 1999;47:275–81. 11. Gooddy W, Schechter MM. Spontaneous arteriovenous fistula of the vertebral artery. Br J Radiol 1960;33:709 –11. 12. Halbach VV, Higashida RT, Hieshima GB. Treatment of vertebral arteriovenous fistulas. AJR 1988;150:405– 12. 13. Halliday AL, Ogilvy CS, Crowell RM. Intracranial vertebral arteriovenous fistula. Case report. J Neurosurg 1993;79:589 –91. 14. Hemphill JC III, Gress DR, Halbach VV. Endovascular therapy of traumatic injuries of the intracranial cerebral arteries. Crit Care Clin 1999;15:811–29. 15. Hunter W. Further observations upon a particular species of aneurysm. Med Observ Inquiries 1762;2: 390 –414. 16. Lister JR, Rhoton AL Jr, Matsushima T, Peace DA. Microsurgical anatomy of the posterior inferior cerebellar artery. Neurosurgery 1982;10:170 –99. 17. Markham JW. Spontaneous arteriovenous fistula of the vertebral artery and vein. Case report. J Neurosurg 1969;31:220 –3. 18. Miller RE, Hieshima GB, Giannotta SL, Grinnell VS, Mehringer CM, Kerin DS. Acute traumatic vertebral arteriovenous fistula: balloon occlusion with the use of a contralateral approach. Neurosurgery 1984;14: 225–9. 19. Nagashima C, Iwasaki T, Kawanuma S, Sakaguchi A, Kamisasa A, Suzuki K. Traumatic arteriovenous fistula of the vertebral artery with spinal cord symptoms: case report. J Neurosurg 1977;46:681–7. 20. Olson RW, Baker HL Jr, Svien HJ. Arteriovenous fistula: a complication of vertebral angiography. Report of a case. J Neurosurg 1963;20:73–5. 21. Reddy SVR, Karnes WE, Earnest F IV, Sundt TM Jr. Spontaneous extracranial vertebral arteriovenous fistula with fibromuscular dysplasia. J Neurosurg 1981; 54:399 –402. 22. Reivich M, Holling HE, Roberts B, et al. Reversal of blood flow through the vertebral artery and its effect on cerebral circulation. N Engl J Med 1961;265:878 – 85. 23. Serbinenko FA. Balloon catheterization and occlusion of major cerebral vessels. J Neurosurg 1974;41:125– 45. 24. Spetzler RF, Wilson CB, Weinstein P, Mehdorn M, Townsend J, Telles D. Normal perfusion pressure breakthrough theory. Clin Neurosurg 1978;25:651–72. 25. Vinuela F, Drake CG, Fox AJ, Pelz DM. Giant intracranial varices secondary to high-flow arteriovenous fistulae. J Neurosurg 1987;66:198 –203. 26. Vinuela F, Fox AJ, Kan S, Drake CG. Balloon occlusion of a spontaneous fistula of the posterior inferior cerebellar artery. Case report. J Neurosurg 1983;58:287– 90. 27. Wadlington VR, Terry JB. Endovascular therapy of traumatic carotid-cavernous fistulas. Crit Care Clin 1999;15:831–54. 28. Weinberg PE, Flom RA. Traumatic vertebral arteriovenous fistula. Surg Neurol 1973;1:162–7.