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Cerebral arteriovenous malformation: Papilledema as a presenting sign
524
Surg Neurol 1984;22:524-6
Cerebral Arteriovenous Malformation: PapiUedema as a Presenting Sign J. S c h i f f e r , M . D . , C. B i b i , M . D . , a n d D . A v i d a n , M . D . Department of Neurosurgery, Assaf Harofeh Medical Center, Zerifin, Israel
Schiffer J, Bibi C, Avidan D. Cerebral arteriovenous malformation: Papilledema as a presenting sign. Surg Neurol 1984;22:524-6.
Papilledema as a presenting sign of arteriovenous malformation without evidence of a space-occupying lesion or hydrocephalus is an unusual finding. The pathogenetic theory that seems to explain such cases is the increase of cerebral blood volume leading to intracranial hypertension, thus resulting in papilledema, We present the case of a 31-year-old woman complaining of headaches, with papilledema as the only objective finding. Computed tomography scan and right carotid angiography demonstrated a right mid rolandic arteriovenous malformation. The lesion was totally removed under intraoperative angiographic control. KEYWORDS: Cerebral arteriovenous malformation; Intracranial hypertension; Papilledema
Cerebral arteriovenous malformations are congenital lesions, characterized by a direct connection between arteries and veins, without the normal intermediary capillary system [5]. These malformations gradually enlarge. They also produce cerebral ischemia by a blood steal from the surrounding cerebral vessels. The well-known usual presenting symptomatology is hemorrhage, convulsions, headaches, progressive neurological deficit, and mental deterioration [5,7,9]. Papilledema, when it occurs, is generally associated with severe hemorrhage, hydrocephalus, or an intracranial space-occupying lesion produced by hematoma or giant arteriovenous malformation [5,7,9]. Papilledema not associated with hemorrhage or a space-occupying lesion is a very rare finding [5,7,9]. Case R e p o r t A 31-year-old woman suffered from general sporadic progressive headaches during the last 10 years before Address reprint requests to: Juana Schiffer, M.D., Chief, Neurosurgical Department, Assaf Harofeh Medical Center, Zerifin 70300, Israel.
© 1984 by Elsevier Science Publishing Co., Inc.
admission. These were treated by analgesic preparations. Two months before admission, the headaches worsened, and she complained of blurred vision. On examination, the only pathological finding was severe bilateral papilledema without hemorrhages. X-ray films o f the skull were normal. A 99mTc brain scan showed increased uptake in the right rolandic region. An unenhanced computed tomography (CT) scan was negative. After contrast medium injection a vascular lesion in the right rolandic region was found (Figure 1). A right carotid angiogram demonstrated a large right mid rolandic cerebral arteriovenous malformation. The feeding vessels were provided by the anterior and the middle cerebral arteries, and drainage was through the internal cerebral vein and the sagittal sinus (Figures 2A and 2B). On May 4, 1981, a right frontoparietal craniotomy was performed. The dura mater was tense. A 3 x 4 x 5-cm arteriovenous malformation situated in the mid rolandic region, extending from the subcortical area to the midline, was found and removed using microsurgical technique. An intraoperative right carotid angiogram demonstrated complete excision of the arteriovenous malformation (Figure 3). Histopathologic examination showed an arteriovenous malformation. Postoperatively, complete palsy of the left upper extremity without sensory disturbances was present. Ten days after the operation movements of the shoulder appeared and during the next 4 months the palsy of the left upper extremity disappeared. The patient recovered fully and returned to her former work. Discussion The usual presentation of cerebral arteriovenous malformation is intracranial hemorrhage due to rupture of the lesion, very severe or even untreatable headaches, convulsions, progressive neurological deficit, and mental deterioration due to cerebral damage caused by the arteriovenous malformation or by cerebral ischemia due to steal syndrome [5,7,9]. Intracranial hypertension as a presenting sign of cerebral arteriovenous malformation is usually produced by severe hemorrhage, a space0090-3019/84/S3.00
AVM with Papilledema
Surg Neurol 1984;22:524-6
525
Figure 1. Computed tomography scan after contrast medium injection showing a lesion in the rolandic region.
occupying lesion due to a huge arteriovenous malformation, or hydrocephalus [5,7,9]. Paterson and McKissock [5], in a series of 110 patients with arterioveno~Ls malformation, reported 17 cases with papilledema, 14 of which had recent intracerebral or subarachnoid hemorrhage. One patient had a large occipital arteriovenou~,~ malformation, and another suffered from obstructive hydrocephalus due to compression by a cerebellar an~gioma. Only one patient had papilledema without a specific cause. Papilledema was frequently reported in cases o f dural arteriovenous malformation [2,4,8]. Kulhner et al [3] reviewed 72 cases of dural arteriovenous malformation related to the transverse sinus. Papilledema was found in 10 of these cases. The pathogenesis of papilledema is discussed in the literature. Papilledema without hemorrhage, spaceoccupying lesion, or hydrocephalus may be the expression o f benign intracra:aial hypertension, similar to pseudotumor cerebri [1,6,8]. Van den Bergh [8] remarked that the probable cause of papilledema must be sought in the massive overload of the venous return, resulting in increased cerebral blood volume, impairment o f cerebrospinal fluid absorption, and increased cerebrospinal fluid production. Cerebrospinal fluid hypertension is responsible for the intracranial hypertension and papilledema. Lamas et al [4] reported a case of dural arteriovenous malformation with papilledema. They remarked that the blood arriving at the transverse sinus interfered with cerebrospinal fluid re~bsorption, producing intracranial
A
B
Figure 2. Angiogram of the right carotid artery, (A) frontal and (B) lateral views, demonstrating a mid rolandic arteriovenous malformation.
526
Surg Neurol 1984;22:524-6
Schiffer et al
the intracranial hypertension is related to increased intraluminar pressure in the sagittal sinus, which is due to shunting of blood from the arteriovenous malformation. He also quoted the theory of Johnston, which attributed the development of intracranial hypertension to the reduction of pressure across the arachnoid villi, causing relative obstruction of the absorption of cerebrospinal fluid. In our case, the huge arteriovenous malformation also produced a blood overload in the sagittal sinus. This could have been the cause of the development of intracranial hypertension and papilledema. The authors wish to thank Professor I. Bar-Itzjak for referring the patient and Professor M. Payewski for the roentgenologic investigations.
References
Figure 3. Intraoperative angiogram of the right carotid artery showing complete excision of the arteriovenous malformation.
hypertension. They also concluded that elevation of the sagittal sinus pressure could lead to hydrocephalus or a pseudotumor condition. In a review of the literature, we found only three cases of cerebral arteriovenous malformation with papilledema. The case reported by Paterson and McKissock [5] has been quoted, but not described. Weisberg et al [7] reported the case of a 40-year-old man with a parietooccipital arteriovenous malformation and papilledema that improved after lumboperitoneal shunt. Vassilouthis [9] presented the case of an 11-year-old boy with a parasagittal frontal arteriovenous malformation and papilledema. The boy recovered after the excision of the lesion. According to Vassilouthis, the cause of
1. Guidetti B, Giuffre R, Gambacorta D. Follow-up study of 100 cases of pseudotumor cerebri. Acta Neurochir 1968;18:259-67. 2. Kosnik EJ, Hunt WE, Miller CA. Dural arteriovenous malformations. J Neurosurg 1974;40:322-9. 3. Kuhner A, Krastel A, Stoll W. Arteriovenous malformations of the transverse dural sinus. J Neurosurg 1976;45:12-9. 4. Lamas E, Lobato RD, EsparzaJ, et al. Dural posterior fossa AVM producing raised sagittal sinus pressure. J Neurosurg 1977;46:804-10. 5. Paterson HJ, McKissock W. A clinical survey of intracranial angiomas with special reference to their mode of progression and surgical treatment: A report of 110 cases. Brain 1956;79:233-66. 6. Spallone A. Benign intracranial hypertension vs. intracranial arteriovenous malformations: A possible CT dilemma. Acta Neurochir 1981;58:75-84. 7. Weisberg LA, Pierce JF, Jabbari B. Intracranial hypertension resulting from a cerebrovascular malformation. South Med 1977;70:624-6. 8. Van den Bergh R. Pseudotumor cerebri due to intracranial arteriovenous malformation. Clin Neurol Neurosurg 1980;82:119-25. 9. Vassilouthis J. Cerebral arteriovenous malformation with intracranial hypertension. Surg Neurol 1979;11:402-4.
Surg Neurol 1984;22:524-6
Cerebral Arteriovenous Malformation: PapiUedema as a Presenting Sign J. S c h i f f e r , M . D . , C. B i b i , M . D . , a n d D . A v i d a n , M . D . Department of Neurosurgery, Assaf Harofeh Medical Center, Zerifin, Israel
Schiffer J, Bibi C, Avidan D. Cerebral arteriovenous malformation: Papilledema as a presenting sign. Surg Neurol 1984;22:524-6.
Papilledema as a presenting sign of arteriovenous malformation without evidence of a space-occupying lesion or hydrocephalus is an unusual finding. The pathogenetic theory that seems to explain such cases is the increase of cerebral blood volume leading to intracranial hypertension, thus resulting in papilledema, We present the case of a 31-year-old woman complaining of headaches, with papilledema as the only objective finding. Computed tomography scan and right carotid angiography demonstrated a right mid rolandic arteriovenous malformation. The lesion was totally removed under intraoperative angiographic control. KEYWORDS: Cerebral arteriovenous malformation; Intracranial hypertension; Papilledema
Cerebral arteriovenous malformations are congenital lesions, characterized by a direct connection between arteries and veins, without the normal intermediary capillary system [5]. These malformations gradually enlarge. They also produce cerebral ischemia by a blood steal from the surrounding cerebral vessels. The well-known usual presenting symptomatology is hemorrhage, convulsions, headaches, progressive neurological deficit, and mental deterioration [5,7,9]. Papilledema, when it occurs, is generally associated with severe hemorrhage, hydrocephalus, or an intracranial space-occupying lesion produced by hematoma or giant arteriovenous malformation [5,7,9]. Papilledema not associated with hemorrhage or a space-occupying lesion is a very rare finding [5,7,9]. Case R e p o r t A 31-year-old woman suffered from general sporadic progressive headaches during the last 10 years before Address reprint requests to: Juana Schiffer, M.D., Chief, Neurosurgical Department, Assaf Harofeh Medical Center, Zerifin 70300, Israel.
© 1984 by Elsevier Science Publishing Co., Inc.
admission. These were treated by analgesic preparations. Two months before admission, the headaches worsened, and she complained of blurred vision. On examination, the only pathological finding was severe bilateral papilledema without hemorrhages. X-ray films o f the skull were normal. A 99mTc brain scan showed increased uptake in the right rolandic region. An unenhanced computed tomography (CT) scan was negative. After contrast medium injection a vascular lesion in the right rolandic region was found (Figure 1). A right carotid angiogram demonstrated a large right mid rolandic cerebral arteriovenous malformation. The feeding vessels were provided by the anterior and the middle cerebral arteries, and drainage was through the internal cerebral vein and the sagittal sinus (Figures 2A and 2B). On May 4, 1981, a right frontoparietal craniotomy was performed. The dura mater was tense. A 3 x 4 x 5-cm arteriovenous malformation situated in the mid rolandic region, extending from the subcortical area to the midline, was found and removed using microsurgical technique. An intraoperative right carotid angiogram demonstrated complete excision of the arteriovenous malformation (Figure 3). Histopathologic examination showed an arteriovenous malformation. Postoperatively, complete palsy of the left upper extremity without sensory disturbances was present. Ten days after the operation movements of the shoulder appeared and during the next 4 months the palsy of the left upper extremity disappeared. The patient recovered fully and returned to her former work. Discussion The usual presentation of cerebral arteriovenous malformation is intracranial hemorrhage due to rupture of the lesion, very severe or even untreatable headaches, convulsions, progressive neurological deficit, and mental deterioration due to cerebral damage caused by the arteriovenous malformation or by cerebral ischemia due to steal syndrome [5,7,9]. Intracranial hypertension as a presenting sign of cerebral arteriovenous malformation is usually produced by severe hemorrhage, a space0090-3019/84/S3.00
AVM with Papilledema
Surg Neurol 1984;22:524-6
525
Figure 1. Computed tomography scan after contrast medium injection showing a lesion in the rolandic region.
occupying lesion due to a huge arteriovenous malformation, or hydrocephalus [5,7,9]. Paterson and McKissock [5], in a series of 110 patients with arterioveno~Ls malformation, reported 17 cases with papilledema, 14 of which had recent intracerebral or subarachnoid hemorrhage. One patient had a large occipital arteriovenou~,~ malformation, and another suffered from obstructive hydrocephalus due to compression by a cerebellar an~gioma. Only one patient had papilledema without a specific cause. Papilledema was frequently reported in cases o f dural arteriovenous malformation [2,4,8]. Kulhner et al [3] reviewed 72 cases of dural arteriovenous malformation related to the transverse sinus. Papilledema was found in 10 of these cases. The pathogenesis of papilledema is discussed in the literature. Papilledema without hemorrhage, spaceoccupying lesion, or hydrocephalus may be the expression o f benign intracra:aial hypertension, similar to pseudotumor cerebri [1,6,8]. Van den Bergh [8] remarked that the probable cause of papilledema must be sought in the massive overload of the venous return, resulting in increased cerebral blood volume, impairment o f cerebrospinal fluid absorption, and increased cerebrospinal fluid production. Cerebrospinal fluid hypertension is responsible for the intracranial hypertension and papilledema. Lamas et al [4] reported a case of dural arteriovenous malformation with papilledema. They remarked that the blood arriving at the transverse sinus interfered with cerebrospinal fluid re~bsorption, producing intracranial
A
B
Figure 2. Angiogram of the right carotid artery, (A) frontal and (B) lateral views, demonstrating a mid rolandic arteriovenous malformation.
526
Surg Neurol 1984;22:524-6
Schiffer et al
the intracranial hypertension is related to increased intraluminar pressure in the sagittal sinus, which is due to shunting of blood from the arteriovenous malformation. He also quoted the theory of Johnston, which attributed the development of intracranial hypertension to the reduction of pressure across the arachnoid villi, causing relative obstruction of the absorption of cerebrospinal fluid. In our case, the huge arteriovenous malformation also produced a blood overload in the sagittal sinus. This could have been the cause of the development of intracranial hypertension and papilledema. The authors wish to thank Professor I. Bar-Itzjak for referring the patient and Professor M. Payewski for the roentgenologic investigations.
References
Figure 3. Intraoperative angiogram of the right carotid artery showing complete excision of the arteriovenous malformation.
hypertension. They also concluded that elevation of the sagittal sinus pressure could lead to hydrocephalus or a pseudotumor condition. In a review of the literature, we found only three cases of cerebral arteriovenous malformation with papilledema. The case reported by Paterson and McKissock [5] has been quoted, but not described. Weisberg et al [7] reported the case of a 40-year-old man with a parietooccipital arteriovenous malformation and papilledema that improved after lumboperitoneal shunt. Vassilouthis [9] presented the case of an 11-year-old boy with a parasagittal frontal arteriovenous malformation and papilledema. The boy recovered after the excision of the lesion. According to Vassilouthis, the cause of
1. Guidetti B, Giuffre R, Gambacorta D. Follow-up study of 100 cases of pseudotumor cerebri. Acta Neurochir 1968;18:259-67. 2. Kosnik EJ, Hunt WE, Miller CA. Dural arteriovenous malformations. J Neurosurg 1974;40:322-9. 3. Kuhner A, Krastel A, Stoll W. Arteriovenous malformations of the transverse dural sinus. J Neurosurg 1976;45:12-9. 4. Lamas E, Lobato RD, EsparzaJ, et al. Dural posterior fossa AVM producing raised sagittal sinus pressure. J Neurosurg 1977;46:804-10. 5. Paterson HJ, McKissock W. A clinical survey of intracranial angiomas with special reference to their mode of progression and surgical treatment: A report of 110 cases. Brain 1956;79:233-66. 6. Spallone A. Benign intracranial hypertension vs. intracranial arteriovenous malformations: A possible CT dilemma. Acta Neurochir 1981;58:75-84. 7. Weisberg LA, Pierce JF, Jabbari B. Intracranial hypertension resulting from a cerebrovascular malformation. South Med 1977;70:624-6. 8. Van den Bergh R. Pseudotumor cerebri due to intracranial arteriovenous malformation. Clin Neurol Neurosurg 1980;82:119-25. 9. Vassilouthis J. Cerebral arteriovenous malformation with intracranial hypertension. Surg Neurol 1979;11:402-4.