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Global Arteriovenous Malformation of the Cervical Region

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Global Arteriovenous Malformation of the Cervical Region LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUES University of Toronto Neurosurgical Rounds No. 4 Global Arteriovenous Malformation of the Cervical Region R.C. HOLGATE and W.M. LOUGHEED INTRODUCTION tent low back pain and sciatica. The This case was presented as an physical findings were marked nuchal interesting example of a global arterio­ spasm, limitation of neck movements, lumbar muscle spasm, and straight leg venous malformation (AVM) involving raising on the right limited to 45 degrees. muscle, bone, extradural and intra­ Treatment consisted of bed rest and dural spaces of the cervical region. lumbar traction. Improvement occurred and he was discharged with his neck brace. CASE HISTORY Eight months later a third admission was necessary because of a sudden onset of A twenty-eight year old male in October, headache and vomiting. There were no 1976, fell at work sustaining a blow to his focal neurologic signs, but his neck was lower back. The following day he com­ stiff. A lumbar puncture revealed bloody plained of cervical pain. Physical examina­ spinal fluid. A CT scan showed slight tion was negative and he had a full range of ventricular enlargement. Cerebral angio­ movement of his cervical spine. grams were normal. Plain films and tomograms of the cervical spine showed a lytic honeycomb After much deliberation it was concluded lesion of C2 and C3, and a 4 mm. that in spite of the risks involved an subluxation of C3 on C4 (Fig. 1). The bone attempt at embolization should be made. scan showed increased flow and decreased Via a right transfemoral catheter the left static activity in the upper cervical region. ascending cervical artery was embolized The radiological diagnosis was vertebral with a mixture of gelfoam and pantopaque hemangioma; tumor, infection and histio­ (Fig. 7). This reduced considerably the cytosis were unlikely but important abnormal vasculature. The right occipital differential considerations. and ascending pharangeal arteries were The lesion was further evaluated by catheterized. There was a large aneurysm myelography which showed a circumferen­ arising off the right ascending pharangeal tial narrowing of the subarachnoid space artery. After embolization this aneurysm without actual cord compression from C4 was occluded and the vascular shunt much to the foramen magnum (Fig. 2). Angio­ reduced (Figs. 8 and 9). graphy revealed an extensive angiomatous After the procedure the patient com­ mass in the vertebral bodies and posterior plained of retrobulbar pain and that he elements of C2 and C3 with a large could not see properly. Examination extradural collection of abnormal vessels proved that with effort he could read and in the spinal canal. An intradural com­ that his vision was 20/30 in both eyes. He ponent was not detected and the anterior stated that the print was blurred. Fundi spinal artery was not identified. The lesion were normal and visual fields, although received blood supply from the vertebral difficult to assess, were probably normal. arteries, ascending and deep cervical He showed a loss of convergence and arteries, and the ascending pharyngeal paralysis (UMN) of the left arm and leg artery bilaterally. Muscular branches of with incoordination. Eight days later the these vessels, as well as the occipital arm and leg weakness had improved. There arteries, were linked by direct arterial was nystagmus in all direction of gaze, anastomosis to each other providing upward gaze was limited and pupillary alternate routes of supply (Figs. 3, 4, 5 and reaction only moderately brisk to light. He 6). had suffered a mid brain lesion resulting in Because of the complexity of the a Parinaud's syndrome. By the time of From the Department of Radiology. Toronto malformation it was decided to treat the discharge all findings had cleared. From General Hospital. University of Toronto, and the the above complication the emboli must Division of Neurosurgery. Toronto General Hospital, patient conservatively. He was fitted with a University of Toronto. neck brace and discharged. have reached the vertebral system from the Between his first admission in 1976 and muscular branches. Reprint requests to Dr. W.M. I.ougheed. Division of Neurosurgery. Toronto General Hospital. 101 College his second admission in March, 1978, the On February 26, 1980, he was carrying Street. Toronto. Ontario. Canada M5G 1 L7. patient continued to suffer from intermit­ his child upstairs on his shoulders when he Vol. 8 No. 1 FEBRUARY 1981 —41 Downloaded from https://www.cambridge.org/core. IP address: 170.106.35.76, on 29 Sep 2021 at 04:49:45, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0317167100042827 THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES in muscle, bone, extradural, intradural and intramedullary sites. The extradural AVM's form about 15-20% of all vascular spinal abnor­ malities according to Bischof (1967). Feeders to the extradural malforma­ tions usually do not have any com­ munications to the arteries feeding the spinal cord. However, Kendall and Logue (1977) reported one case draining to the intrathecal veins. The extradural AVM's are much less common in the cervical region, the most common site being mid thoracic. According to Pia (1978), 27% show an apoplectiform course, 42% a a b remitting course, and 31% a progressive course. Figure 3 — A and B — Right and left Extradural hemorrhage or mass vertebral angiogram. Subtracted views effect produces spinal cord compres­ of the vertebral artery injections show Figure 1 — Lateral view of the cervical extensive collections of abnormal vessels sion with serious consequences. When spine. There is a minor forward in the upper four vertebrae (right more the vertebral body is involved patho­ subluxation of C2 and C3. The bone than left). Lateral views confirmed the logical fracture may occur. texture of C2 and C3 is abnormal intraosseous position. Because of the partial block in the showing the typical honeycomb appear­ myelogram it would appear that in the ance of a vertebral hemangioma. On admission, examination was normal future this patient will suffer from except for limitation of neck movements. spinal cord compression. Pia (1978), While in hospital the patient stated he stated that the timing for extradural could no longer hear the bruit. We had AVM's to cause compression was 2-4 never heard a bruit, which had been years following diagnosis. He made a complained of on admission, and there was plea for early treatment before a fixed no bruit audible on this examination. The neurologic deficit. Decompression patient was again discharged having would require laminectomy and extra­ improved. dural cauterization of the feeding vessels to the extradural component. The difficulty in staging the procedures DISCUSSION becomes apparent as the muscular part Dr. W.M. Lougheed of the malformation feeds the extra­ The vascular supply to the nuchal dural component and hemostasis may muscles and vertebral bodies, extra­ be extremely difficult if the muscular dural space and spinal cord is outlined component is not occluded first. in Figure 11 (Pia and Djindjian, 1978). Unfortunately, we cannot surgically The ascending cervical and deep remove the muscular component due cervical arteries anastomose with both to the extent and the anatomy, and the spinal cord circulation and vertebral embolization is not possible without arteries. In turn, the vertebral artery first separating the extradural and contributed to the spinal cord circula­ intradural components. The only tion and with the external carotid certain way of accomplishing this would be to occlude all the radicular Figure 2 — Lateral view of the cervical circulation via the occipital artery. The occipital artery also anastomoses with arteries coming from the vertebral and myelogram. There is a circumferential ascending cervical branches. This, narrowing of the dural sac (seen best the deep cervical. Due to the extensive anteriorly) which extends from C4 to network of communicating arteries however, might compromise the normal the foramen magnum. each of these systems can therefore blood supply to the spinal cord. The take part in the supply to the AVM vertebral component might be elimi­ making total irradication a complex nated by ligation of both vertebrals sneezed. This resulted in a sudden severe intradurally. This would have to be pain in his neck. The patient had noted in problem. Figure 12 (Pia and Djindjian, 1978), staged, necessitating two exposures. the last few months that every time he The proximal portion of the vertebral sneezed, coughed or strained he had to nicely illustrates the complex arrange­ hold his head between his hands to prevent ments which may occur, especially arteries could be embolized with large severe neck pain. when the AVM is global and is located emboli. If the emboli were small there 42 — FEBRUARY 1981 Global A VM of Cervical Region Downloaded from https://www.cambridge.org/core. IP address: 170.106.35.76, on 29 Sep 2021 at 04:49:45, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0317167100042827 LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUES a Figure 4 — A and B — Deep cervical artery. Selective injections Figure 5 — A and B — Selective ascending cervical angiogram. into the deep cervical arteries show enlargement and tortuosity The ascending cervical arteries also show hypertrophy (more of the parent vessels and numerous abnormal vessels in the marked on the right) A.) and supply of abnormal vessels to both posterior elements and soft tissues. Of interest, an aneurysm can the vertebral bodies and surrounding soft tissues. be identified on the left (B) (arrowhead), and direct anastomoses to the vertebral artery (arrowheads) can be seen on the right (A). Figure 6 — Left external carotid injections. The left ascending Figure 7 — Embolization, left ascending cervical artery. The pharyngeal artery (A) and the left occipital artery (B) also give catheter is well seated in the ascending cervical artery (A- direct and anastomotic supply to the hemangioma.
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