ACTA NEUROCHIRURGICA 9 by Springer-Verlag 1978
Acta Neurochirurgica 45, 147--154 (1978)
Department of Surgery, Division of Neurological Surgery, University of Cincinnati College of Medicine and the Veterans Administration Hospital, Cincinnati, Ohio, U.S.A.
Atherosclerotic Occlusive Disease of the Vertebrobasilar System in Young Adults and Its Surgical Consideration By
G. Khodadad With 2 Figures
Summary A young diabetic man with recurrent attacks of headache, dizziness, and blurred vision who was originally thought to have episodes of hypoglycaemia, underwent investigation for the possibility of an occlusive cerebrovascular disease; and eventually was operated upon for vertebrobasilar insufficiency. The young age, the degree of occlusive disease, the clinical picture, and the surgical results in this patient on the one hand, and the scanty literature on the other prompted this report. Strokes due to atherosclerotic occlusive disease of the vertebrobasilar system are less frequent t h a n those due to disease o f the c a r o t i d arteries and, not considering a n y specific age group, it is recorded t h a t f o r every six or seven occlusive carotid strokes there is one occlusive vertebrobasilar stroke (Groch et al. 1966). This ratio seems to be almost the same in occlusive strokes in y o u n g adults u n d e r 40 years of age. Since atherosclerotic occlusive strokes are seen i n f r e q u e n t l y in y o u n g adults (Gurdjian and Webster 1953, Wells and Timberger 1961, Louis and M c D o w e l l 1967, a n d Irish 1939), one has o n l y rare chances to see and s t u d y y o u n g patients w i t h occlusive vertebrobasilar strokes.
Case Report A 26-year-old salesman was admitted to hospital because of juvenile diabetes and "spells". He had had diabetes mellitus for 20 years, and his treatment consisted of a 2,000 calories diet and 30-45 units of insulin daily. The urine was tested three times daily, and for about a quarter of the time it had been negative. 10"
0001-6268/78/0045/0147/$
01.60
148
G. Khodadad: Atheroscierotic Occlusive Disease
Arterial hypertension was discovered when he was 25 years old, and it was treated with antihypertensive medications. A few months later he was evaluated for the possibility of diabetic glomerulosclerosis. Three months before admission to hospital be began noticing daily "spells" which occurred in the early evening, immediately after supper. They were characterized by headaches, dizziness, blurred vision, double vision, and numbness around the mouth. He bad one or two
Fig. 1 a. An anteroposterior view of the arch aortogram showing a hypoplastic right vertebral artery (arrow) atta&s a day, and each lasted I0-15 minutes. Two months after these atta&s started he developed a sudden onset of numbness and weakness of the entire left side of the body, associated with headache and double vision. The symptoms cleared completely within an hour. In addition, the patient gave a four-year history of difficulty with erections, and a weight loss of 5-10 pounds. Fig. 1 b. An anteroposterior view of the left vertebral arteriogram showing 600/0 or more stenosis of the distal vertebral artery (arrow). The upper portion of the basilar artery and the posterior cerebral arteries are not visualized Fig. 1 c. A lateral view of the right carotid arteriogram demonstrating a retrograde filling of the upper basilar artery, indicating a low-pressure vertebrobasilar system (arrow)
Fig. 1 b
Fig. 1 r
150
G. Khodadad:
Fig. 2 a. A lateral view of the left external carotid arteriogram showing the occipital artery-caudal loop-posterior inferior cerebellar artery anastomosis (lower right arrow) and the visualization of basilar artery (upper arrow)
Fig. 2 b. A later phase of the same arteriogram shown in Fig. 2 a, further demonstrating the basilar artery (arrow) The general and neurological examinations were within normal limits except for mild bilateral retinopathy and diminution of pain sensation on the left side of the face. At the beginning, the patient's spells were thought to be due to hypoglycaemia. Subsequently, however, the possibility of vertebrobasilar insufficiency was brought up, and the patient underwent further neurological investigation. The electroencephalogram and brain scan showed no abnormality. Cerebral angiograms revealed
Atherosclerotic Occlusive Disease of the Vertebrobasilar System
151
Fig. 2 c. An anteroposterior view of the external carotid arteriogram demonstrating the occipital artery-caudal loop-posterior inferior cerebellar artery anastomosis (upper arrow). The lower right arrow points to the occipital artery
Fig. 2 d. A later phase of the same arteriogram shown in Fig. 2 c illustrating both the left and right vertebral and the posterior inferior cerebellar arteries as well as the basilar artery (arrow) a) hypoplasia of the right vertebral artery, which supplied only the right posterior inferior cerebellar artery; b) 60o/o or more stenosis of the left vertebral artery just proximal to the origin of the left posterior inferior cerebellar artery; c) slight to moderate stenosis and irregularities of the cavernous portion of the right internal carotid artery; d) filling of both posterior cerebral arteries and upper basilar artery in retrograde fashion through the right carotid injection, indicating a 10w
152
G. Khodadad:
pressure vertebrobasilar system; and e) lack of filling of the upper basilar and posterior cerebral arteries through the vertebral artery injections (Figs. 1 a-c). The diagnosis of vertebrobasilar insufficiency was confirmed, and on June 10, 1977, the patient underwent a left occipital artery-Caudal loop-posterior inferior cerebellar artery anastomosis (OCC art-Caudal loop-PICA anas.). The postoperative course was smooth, and he was discharged from hospital 10 days after the operation following postoperative left external carotid angiography. Eight months later he had no further spells and had no neurological abnormality. The angiogram obtained eight days after surgery showed a patent anastomosis. It was of particular note that both vertebral arteries and the basilar artery were filled through this anastomosis (Figs. 2 a-d). Discussion
Gurdjian and Webster (1953) analyzed 30 patients, aged 20-70 years or older, with strokes resulting from thrombosis of the cervical carotid artery. Of these 30 patients, 7 were 20-40 years of age, an incidence of 23~ Wells and Timberger (1961) studied cerebral thrombosis in 77 patients under 50 years of age. Of these, 22 were 40 years old or younger, an incidence of 30O/o. This slightly higher incidence seems to be due to the fact that the study was restricted to a selected group of patients under 50 years of age, and older patients were not included. Louis and McDowell (1967) studied 56 patients with cerebral infarction who were 50 years old or younger. Of these, 13 patients were under 40 years of age, giving an incidence of 23~ Of the entire group, 30 patients had cerebral angiography, and 3 of them showed involvement of the vertebrobasilar system. Two patients had narrowing of the basilar or vertebral arteries, and one patient had occlusion of the posterior cerebral artery. That is to say that 10~ of the infarctions occurred in the distribution of vertebrobasilar system. Irish (1939) reviewed 1,000 cerebrovascular lesions in a series of 12,000 autopsies and found that 12~ of the cerebrovascular accidents consisting of embolism, haemorrhage, and thrombosis occurred in patients between 20 and 39 years of age. The majority of these patients had either cerebral embolism or haemorrhage, and only 10~ of them (17 patients) suffered from cerebral thrombosis. The author did not divide these patients into carotid and vertebrobasilar thrombosis. Berlin and associates (1955) evaluated cerebral thrombosis in young adults, and discussed 17 patients between 18 and 37 years of age. Of these, two patients had vertebrobasilar strokes, an incidence of 15~ The first one (case 4), was a 29-year-old man with a clinical
Atherosclerotic Occlusive Disease of the Vertebrobasilar System
153
diagnosis of occlusion of the right posterior inferior cerebellar artery who gradually recovered. No cerebral angiography was performed in this patient. The second patient (case 13) was also a man, 28 years old, with a transient episode of loss of consciousness. On the following day he was found to be unconscious and having generalized convulsions, lie never regained consciousness, and died 80 days after the onset of his illness. Post-mortem examination revealed a white plaque at the terminal portion of the basilar artery and a narrowed lumen. Sections of the basilar artery at this site showed fibrous thickening of the intima with a haemorrhage dissecting the intima from the media. There was a large area of anaemic necrosis of the pons extending bilaterally from the floor of the fourth ventricle to the base. Review of the literature shows that between 10-23~ of the atherosclerotic occlusive strokes occur in patients 40 years old or younger and the occlusive vertebrobasilar strokes probably develop only in 10-15% of patients, an incidence similar to that seen in the older age group (Groch et al. 1966). Although no study was found in the literature of the surgical treatment of this young group of patients, treatment may not vary from that of the older group of vertebrobasilar stroke patients. The present patient who was only 26 years old, the youngest reported patient (and no other surgical approach seemed to be applicable) responded quite well to an occipital artery-Caudal loop-PICA anastomosis. This operation, which was described in 1976 (Khodadad 1967), has been used in 17 other patients, 49-68 years of age, with similar results. In the early stage of the illness, the patient's symptoms were errorously thought to be attacks of hypoglycaemia. Subsequently, however, cerebral angiography disclosed the true cause of symptoms. The atherosclerosis involved the distal vertebral artery which was not amenable to direct surgical treatment. Instead the new microvascular_procedure was used with satisfactory results. It was of particular note that the postoperative angiograms, performed through the left external carotid artery, demonstrated both vertebral arteries and the basilar artery, indicating that an occipital artery-Caudal-loop-PICA anastomosis is capable of supplying the vertebrobasilar system and thus relieving the brain stem ischaemia. References
Berlin, L., Tumarkin, B., Martin, H. L., Cerebral thrombosis in young adults. The New Eng. J. Med. 252 (1955), 162--166. Groch, S. N., McDevitt, E., Wright, I. S., A long term study of cerebral vascular disease. In: Cerebrovascular Disease. Baltimore, Maryland: The Williams & Wilkins Company. 1966.
154
G. Khodadad: Atherosclerotic Occlusive Disease
Gurdjian, E. S., Webster, J. E., Stroke resulting from internal carotid artery thrombosis in the neck. JAMA 151 (1953), 541--545. Irish, C. W., Cerebral vascular lesions in newborn infants and young children: With report of 40 among 1,000 necropsied cases with spontaneous vascular encephalopathy. J. Pediat. 15 (1939), 64--74. Khodadad, G., Occipital artery-posterior inferior cerebellar artery anastomosis. Surg. Neuro. 5 (1976), 225--227. Louis, S., and McDowell, F., Stroke in young adults. Ann. Int. Med. 66 (1967), 932--938. Wells, C. E., Timberger, R., Cerebral thrombosis in patients under fifty years of age. Arch. Neuro. 4 (1961), 268--271. Author's address, G. Khodadad, M.D., Department of Surgery, Division of Neurological Surgery, University of Cincinnati College of Medicine, 231 Bethesda Avenue, Cincinnati, OH 45267, U.S.A.
Acta Neurochirurgica 45, 147--154 (1978)
Department of Surgery, Division of Neurological Surgery, University of Cincinnati College of Medicine and the Veterans Administration Hospital, Cincinnati, Ohio, U.S.A.
Atherosclerotic Occlusive Disease of the Vertebrobasilar System in Young Adults and Its Surgical Consideration By
G. Khodadad With 2 Figures
Summary A young diabetic man with recurrent attacks of headache, dizziness, and blurred vision who was originally thought to have episodes of hypoglycaemia, underwent investigation for the possibility of an occlusive cerebrovascular disease; and eventually was operated upon for vertebrobasilar insufficiency. The young age, the degree of occlusive disease, the clinical picture, and the surgical results in this patient on the one hand, and the scanty literature on the other prompted this report. Strokes due to atherosclerotic occlusive disease of the vertebrobasilar system are less frequent t h a n those due to disease o f the c a r o t i d arteries and, not considering a n y specific age group, it is recorded t h a t f o r every six or seven occlusive carotid strokes there is one occlusive vertebrobasilar stroke (Groch et al. 1966). This ratio seems to be almost the same in occlusive strokes in y o u n g adults u n d e r 40 years of age. Since atherosclerotic occlusive strokes are seen i n f r e q u e n t l y in y o u n g adults (Gurdjian and Webster 1953, Wells and Timberger 1961, Louis and M c D o w e l l 1967, a n d Irish 1939), one has o n l y rare chances to see and s t u d y y o u n g patients w i t h occlusive vertebrobasilar strokes.
Case Report A 26-year-old salesman was admitted to hospital because of juvenile diabetes and "spells". He had had diabetes mellitus for 20 years, and his treatment consisted of a 2,000 calories diet and 30-45 units of insulin daily. The urine was tested three times daily, and for about a quarter of the time it had been negative. 10"
0001-6268/78/0045/0147/$
01.60
148
G. Khodadad: Atheroscierotic Occlusive Disease
Arterial hypertension was discovered when he was 25 years old, and it was treated with antihypertensive medications. A few months later he was evaluated for the possibility of diabetic glomerulosclerosis. Three months before admission to hospital be began noticing daily "spells" which occurred in the early evening, immediately after supper. They were characterized by headaches, dizziness, blurred vision, double vision, and numbness around the mouth. He bad one or two
Fig. 1 a. An anteroposterior view of the arch aortogram showing a hypoplastic right vertebral artery (arrow) atta&s a day, and each lasted I0-15 minutes. Two months after these atta&s started he developed a sudden onset of numbness and weakness of the entire left side of the body, associated with headache and double vision. The symptoms cleared completely within an hour. In addition, the patient gave a four-year history of difficulty with erections, and a weight loss of 5-10 pounds. Fig. 1 b. An anteroposterior view of the left vertebral arteriogram showing 600/0 or more stenosis of the distal vertebral artery (arrow). The upper portion of the basilar artery and the posterior cerebral arteries are not visualized Fig. 1 c. A lateral view of the right carotid arteriogram demonstrating a retrograde filling of the upper basilar artery, indicating a low-pressure vertebrobasilar system (arrow)
Fig. 1 b
Fig. 1 r
150
G. Khodadad:
Fig. 2 a. A lateral view of the left external carotid arteriogram showing the occipital artery-caudal loop-posterior inferior cerebellar artery anastomosis (lower right arrow) and the visualization of basilar artery (upper arrow)
Fig. 2 b. A later phase of the same arteriogram shown in Fig. 2 a, further demonstrating the basilar artery (arrow) The general and neurological examinations were within normal limits except for mild bilateral retinopathy and diminution of pain sensation on the left side of the face. At the beginning, the patient's spells were thought to be due to hypoglycaemia. Subsequently, however, the possibility of vertebrobasilar insufficiency was brought up, and the patient underwent further neurological investigation. The electroencephalogram and brain scan showed no abnormality. Cerebral angiograms revealed
Atherosclerotic Occlusive Disease of the Vertebrobasilar System
151
Fig. 2 c. An anteroposterior view of the external carotid arteriogram demonstrating the occipital artery-caudal loop-posterior inferior cerebellar artery anastomosis (upper arrow). The lower right arrow points to the occipital artery
Fig. 2 d. A later phase of the same arteriogram shown in Fig. 2 c illustrating both the left and right vertebral and the posterior inferior cerebellar arteries as well as the basilar artery (arrow) a) hypoplasia of the right vertebral artery, which supplied only the right posterior inferior cerebellar artery; b) 60o/o or more stenosis of the left vertebral artery just proximal to the origin of the left posterior inferior cerebellar artery; c) slight to moderate stenosis and irregularities of the cavernous portion of the right internal carotid artery; d) filling of both posterior cerebral arteries and upper basilar artery in retrograde fashion through the right carotid injection, indicating a 10w
152
G. Khodadad:
pressure vertebrobasilar system; and e) lack of filling of the upper basilar and posterior cerebral arteries through the vertebral artery injections (Figs. 1 a-c). The diagnosis of vertebrobasilar insufficiency was confirmed, and on June 10, 1977, the patient underwent a left occipital artery-Caudal loop-posterior inferior cerebellar artery anastomosis (OCC art-Caudal loop-PICA anas.). The postoperative course was smooth, and he was discharged from hospital 10 days after the operation following postoperative left external carotid angiography. Eight months later he had no further spells and had no neurological abnormality. The angiogram obtained eight days after surgery showed a patent anastomosis. It was of particular note that both vertebral arteries and the basilar artery were filled through this anastomosis (Figs. 2 a-d). Discussion
Gurdjian and Webster (1953) analyzed 30 patients, aged 20-70 years or older, with strokes resulting from thrombosis of the cervical carotid artery. Of these 30 patients, 7 were 20-40 years of age, an incidence of 23~ Wells and Timberger (1961) studied cerebral thrombosis in 77 patients under 50 years of age. Of these, 22 were 40 years old or younger, an incidence of 30O/o. This slightly higher incidence seems to be due to the fact that the study was restricted to a selected group of patients under 50 years of age, and older patients were not included. Louis and McDowell (1967) studied 56 patients with cerebral infarction who were 50 years old or younger. Of these, 13 patients were under 40 years of age, giving an incidence of 23~ Of the entire group, 30 patients had cerebral angiography, and 3 of them showed involvement of the vertebrobasilar system. Two patients had narrowing of the basilar or vertebral arteries, and one patient had occlusion of the posterior cerebral artery. That is to say that 10~ of the infarctions occurred in the distribution of vertebrobasilar system. Irish (1939) reviewed 1,000 cerebrovascular lesions in a series of 12,000 autopsies and found that 12~ of the cerebrovascular accidents consisting of embolism, haemorrhage, and thrombosis occurred in patients between 20 and 39 years of age. The majority of these patients had either cerebral embolism or haemorrhage, and only 10~ of them (17 patients) suffered from cerebral thrombosis. The author did not divide these patients into carotid and vertebrobasilar thrombosis. Berlin and associates (1955) evaluated cerebral thrombosis in young adults, and discussed 17 patients between 18 and 37 years of age. Of these, two patients had vertebrobasilar strokes, an incidence of 15~ The first one (case 4), was a 29-year-old man with a clinical
Atherosclerotic Occlusive Disease of the Vertebrobasilar System
153
diagnosis of occlusion of the right posterior inferior cerebellar artery who gradually recovered. No cerebral angiography was performed in this patient. The second patient (case 13) was also a man, 28 years old, with a transient episode of loss of consciousness. On the following day he was found to be unconscious and having generalized convulsions, lie never regained consciousness, and died 80 days after the onset of his illness. Post-mortem examination revealed a white plaque at the terminal portion of the basilar artery and a narrowed lumen. Sections of the basilar artery at this site showed fibrous thickening of the intima with a haemorrhage dissecting the intima from the media. There was a large area of anaemic necrosis of the pons extending bilaterally from the floor of the fourth ventricle to the base. Review of the literature shows that between 10-23~ of the atherosclerotic occlusive strokes occur in patients 40 years old or younger and the occlusive vertebrobasilar strokes probably develop only in 10-15% of patients, an incidence similar to that seen in the older age group (Groch et al. 1966). Although no study was found in the literature of the surgical treatment of this young group of patients, treatment may not vary from that of the older group of vertebrobasilar stroke patients. The present patient who was only 26 years old, the youngest reported patient (and no other surgical approach seemed to be applicable) responded quite well to an occipital artery-Caudal loop-PICA anastomosis. This operation, which was described in 1976 (Khodadad 1967), has been used in 17 other patients, 49-68 years of age, with similar results. In the early stage of the illness, the patient's symptoms were errorously thought to be attacks of hypoglycaemia. Subsequently, however, cerebral angiography disclosed the true cause of symptoms. The atherosclerosis involved the distal vertebral artery which was not amenable to direct surgical treatment. Instead the new microvascular_procedure was used with satisfactory results. It was of particular note that the postoperative angiograms, performed through the left external carotid artery, demonstrated both vertebral arteries and the basilar artery, indicating that an occipital artery-Caudal-loop-PICA anastomosis is capable of supplying the vertebrobasilar system and thus relieving the brain stem ischaemia. References
Berlin, L., Tumarkin, B., Martin, H. L., Cerebral thrombosis in young adults. The New Eng. J. Med. 252 (1955), 162--166. Groch, S. N., McDevitt, E., Wright, I. S., A long term study of cerebral vascular disease. In: Cerebrovascular Disease. Baltimore, Maryland: The Williams & Wilkins Company. 1966.
154
G. Khodadad: Atherosclerotic Occlusive Disease
Gurdjian, E. S., Webster, J. E., Stroke resulting from internal carotid artery thrombosis in the neck. JAMA 151 (1953), 541--545. Irish, C. W., Cerebral vascular lesions in newborn infants and young children: With report of 40 among 1,000 necropsied cases with spontaneous vascular encephalopathy. J. Pediat. 15 (1939), 64--74. Khodadad, G., Occipital artery-posterior inferior cerebellar artery anastomosis. Surg. Neuro. 5 (1976), 225--227. Louis, S., and McDowell, F., Stroke in young adults. Ann. Int. Med. 66 (1967), 932--938. Wells, C. E., Timberger, R., Cerebral thrombosis in patients under fifty years of age. Arch. Neuro. 4 (1961), 268--271. Author's address, G. Khodadad, M.D., Department of Surgery, Division of Neurological Surgery, University of Cincinnati College of Medicine, 231 Bethesda Avenue, Cincinnati, OH 45267, U.S.A.
