J Neurosurg 75:461-464, 1991
Excision of cavernous angioma with preservation of coexisting venous angioma Case report OSAMU SASAKI, M.D., RYUICHI TANAKA, M.D., TL~rsuo KOIKE, M.D., AK1RA KOIDE, M.D., TAKAYUKIKOIZUMI, M.D., AND HIROSHI OGAWA, M.D. Departments of Neurosurgery and Neuropathology, Kuwana Hospital, and Department of Neurosurgery, Brain Research Institute, 3Higata University, Niigata City, Japan v" The case of a patient presenting with dysphasic seizures due to a cavernous angioma coexisting with a venous malformation is reported. The cavernous angioma was resected with preservation of the venous malformation, as confirmed by postoperative studies. The patient was seizure-free following surgery. KEY WORDS
9
cavernous angioma
9 venous angioma
9 arteriovenous malformation
9
seizure
AVERNOUSangiomas are a welt-known cause of seizures or hemorrhage. However, the clinical significance of venous malformations is not clear and the management of these lesions remains controversial. We recently encountered the case of a patient presenting with dysphasic seizures due to a cavernous angioma coexisting with a venous malformation in the same area. The patient was treated surgically and the cavernous angioma was resected with preservation of the venous malformation, resulting in relief of seizures. In this case, the cavernous angioma seemed to be closely related to the clinical presentation. If such lesions are managed surgically, it seems appropriate to remove only the cavernous angioma.
C
able. Electroencephalography showed focal spikes in the left temporal lobe. Computerized tomography (CT) scans demonstrated a slightly higher-density lesion in the left temporal lobe. After infusion of contrast medium, the density of the lesion increased slightly and a curvilinear enhancement was noted around the lesion (Fig. 1A). Magnetic resonance (MR) imaging depicted a core of mixed signal intensity, with a rim of decreasing signal intensity on the T2-weighted study (Fig. 1B). Angiography showed an appearance typical of a venous
Case Report
This 31-year-old right-handed woman came to our hospital on December 2, 1988, complaining of episodic speech dysfunction during the 4 years prior to presentation. During these episodes, which lasted a few minutes and occurred once or twice a day, she was temporally unable to comprehend spoken language. There were no other associated symptoms and her medical and family history was not significant. Examination. General, neurological, and neuropsychiatric examinations of the patient proved unremark-
J. Neurosurg. / Volume 75/September, 1991
FIG. 1. Preoperative studies. A: Postcontrast computerized tomography scan demonstrating a curvilinear enhancement. B: Magnetic resonance T2-weighted image depicting a core of mixed intensity with a rim of decreased intensity. 461
O. Sasaki, et al.
FIG. 2. Preoperative left internal carotid angiogram showing a typical venous malformation in the left temporal lobe.
FiG. 3. Schematic drawing of the intraoperative view. CA = cavernous angioma; MV = central medullary vein of the venous malformation; DV = draining vein.
malformation in the left temporal lobe (Fig. 2). A diagnosis was made of simple partial seizures associated with adjacent vascular malformations of the cavernous and venous types in the left temporal lobe. Initially, the patient was treated medically with 200 mg phenytoin and 50 mg phenobarbital administered daily for 1 month. When the frequency of the seizures did not decrease, 400 mg of carbamazepine was given for 1 month. This also showed no effect and was discontinued due to the development of side effects. Operation. To control the seizures and prevent future hemorrhage, the patient underwent a frontotemporal craniotomy on May 6, 1989. Electrocorticography revealed seizure discharges from the superior and middle temporal gyri around the vein of Labbe. The lesion was approached from the anterolateral aspect with splitting of the superior temporal sulcus. The cavernous angioma was found embedded in the markedly xanthochromic subcortical white matter of the superior temporal gyms, and was partially adherent to a hugely dilated, medially coursing vein of the venous malfor462
FIG. 4. Photomicrograph of the surgical specimen. The lesion is composed of sinusoidal vascular spaces of various sizes lined by a single layer of endothelium. Intervening neural tissue is absent in the intraparenchymal lesions, and the vessel wails are devoid of smooth-muscle and elastic tissue. These findings are typical of cavernous angiomas. Elastica Goldner stain, x I 15.
mation. Several small veins from the cavernous angioma drained to this central medullary vein (Fig. 3). After coagulation and cutting of these small veins, the cavernous angioma was carefully separated from the huge vein. Finally, the cavernous angioma was totally resected and the venous malformation was left untouched. Postoperative electrocorticography showed a significant reduction in seizure frequency. Pathological Examination. The lesion was composed of sinusoidal vascular spaces of various sizes lined by a single layer of endothelium (Fig. 4). There was no intervening neural tissue in the intraparenchymal lesions, and the vessel walls were devoid of smoothmuscle and elastic tissue. Several vascular spaces conmined old hyalinized thrombi, and deposits of calcium were seen in the vascular walls. The surrounding brain tissue showed gliosis with hemosiderin deposits. These were findings typical of a cavernous malformation. Postoperative Course. The patient's postoperative course was unremarkable. She developed dysphasic seizures only twice after the operation, on the 1 lth and 2lst postoperative days. She was discharged on May 30, 1989, without any neurological or neuropsychiatric deficit, and at her 189 follow-up review she remained seizure-free without anticonvulsant therapy. Postoperative CT scans and MR imaging revealed disappearance of the cavernous angioma (Fig. 5). Follow-up angiography (Fig. 6) performed on the 22nd day after surgery showed that the venous malformation was preserved; however, there were fewer small veins converging into the huge central medullary vein than preoperatively. This seemed to result from the obliteration of small draining veins from the cavernous angioma into the central medullary vein of the venous malformation.
J. Neurosurg. / Volume 75/September, 1991
Excision of cavernous angioma
FIG. 5. Postoperative magnetic resonance studies, enhanced T~-weighted image (A) and T2-weighted image (B), revealing disappearance of the cavernous angioma and preservation of the central medullary vein of the venous malformation.
Discussion
Venous malformations differ from cavernous angiomas in clinical, radiological, and histological features. In a clinical practice a clear distinction is made between these two entities, but the coexistence of two types of malformations in the same area seems to suggest that they are a continuum and have a similar pathogenesis. In our case, the cavernous and venous malformations adhered to each other and were connected by small medullary veins. This anomaly may represent a transitional form of cavernous and venous malformations. Cavernous angiomas are a well-known cause of seizures or hemorrhage, ~-5'~2but the clinical significance of venous malformations has not been clearly determined. Most authors have considered them to be generally silent) 9~' A large autopsy series reported by McCormick 6 showed that they were usually asymptomatic. On the other hand, some authors have stressed the tendency of venous malformations to bleed 2'4"7"9"~~ and have recommended surgical removal. 2,4 It seems true that at least a small number of patients with venous malformations present with symptoms; however, the characteristics of symptomatic malformations or the differences between symptomatic and asymptomatic malformations remain unexplained. Cavernous angiomas have no pathognomonic radiographic appearance. They cannot be visualized directly with angiography. 3~s'9 Although CT scanning is useful for detection (they characteristically appear as a nodular lesion), the findings are neither specific nor diagnostic. 5'8'9 A true diagnosis can only be made histologically? ,9 In recent years, MR imaging has proved sensitive in detecting these lesions. In most cases, a correct diagnosis can be made preoperatively, ~ although M R imaging cannot distinguish between a cavernous angioma and a thrombosed arteriovenous malformation. Venous malformations, in contrast, are diagnosed by angiography with ease because of their specific features. 2-35 Computerized tomography also aids in diag-
J. Neurosurg. / Volume 75/September, 1991
FIG. 6. Postoperative left internal carotid angiogram showing preservation of the venous malformation. There are slightly fewer small veins converging into the central medullary vein than preoperatively.
nosis if the classic linear or curvilinear enhancement is demonstrated. Numaguchi, et al.,7 reported that 11 of 44 venous malformations diagnosed angiographically showed nodular enhancement, which was more commonly associated with cavernous angiomas. Recently, Rigamonti and Spetzler 9 also stated that many symptomatic venous malformations showed global hyperdensity on CT scans. If high-field strength MR imaging is undertaken in such cases, a coexisting cavernous angioma could be demonstrated. Although such reports are very rare, the incidence of the association of these two vascular malformations appears to be higher than expected, 9 and the presence or absence of a coexisting cavernous angioma may explain the differences between symptomatic and asymptomatic venous malformations. In our case, the patient was treated surgically, resulting in resolution of her seizures. At surgery, only the cavernous angioma was excised since obliteration of the venous malformation in the left temporal lobe would have had disastrous consequences. This result suggests that the cavernous angioma was closely related to the seizures. Based on our experience, it would be appropriate to remove only the cavernous angioma if these lesions are managed surgically. References
1. Giombini S, Morello G: Cavernousangiomasofthe brain. Account of fourteen personal cases and review of the literature. Acta Neurochir 40:61-82, 1978 2. Handa H, Moritake K: Venous angiomas of the brain, in Fein JM, Flamm ES (eds): Cerebrovascular Surgery. Volume IV. New York: Springer-Verlag, 1985, pp 1139-1149 3. Huang YP, Robbins A, Patel SC, et at: Cerebral venous 463
O. Sasaki, et al.
4. 5.
6. 7. 8.
464
malformations (and a new classification of cerebral vascular malformations), in Kapp JP, Schmidek ttH (eds): The Cerebral Venous System and Its Disorders. Orlando: Grune & Stratton, 1984, pp 373-474 Malik GM, Morgan JK, Boulos RS, et al: Venous angiomas: an underestimated cause of intracranial hemorrhage. Surg Neurol 30:350-358, 1988 Martin NA, Wilson CB, Stein BM: Venous and cavernous malformations, in Wilson CB, Stein BM (eds): Intracranial Arteriovenous Malformations. Baltimore: Williams & Wilkins, 1984, pp 234-245 McCormick WF: The pathology of angiomas, in Fein JM, Flamm ES (eds): Cerebrovascular Surgery. Volume IV. New York: Springer-Verlag, 1985, pp 1074-1095 Numaguchi Y, Kitamura K, Fukui M, et al: Intracranial venous angiomas. Surg Neurol 18:193-202, 1982 Rigamonti D, Drayer BP, Johnson PC, et al: The MRI appearance of cavernous malformations (angiomas). J Neurosurg 67:518-524, 1987
9. Rigamonti D, Spetzler RF: The association of venous and cavernous malformations. Report of four cases and discussion of the pathophysiological, diagnostic, and therapeutic implications. Acta Neurochir 92:100-105, 1988 10. Rothfus WE, Albright AL, Casey KF, et al: Cerebellar venous angioma: "benign" entity? AJNR 5:61-66, 1984 11. Saito Y, Kobayashi N: Cerebral venous angiomas: clinical evaluation and possible etiology. Radiology 139:87-94, 1981 12. Simard JM, Garcia-Bengochea F, Ballinger WE Jr, et al: Cavernous angiomas: a review of 126 collected and 12 new clinical cases. Neurosurgery 18:162-172, 1986
Manuscript received August 28, 1990. Accepted in final form February 15, 1991. Address reprint requests to: Osamu Sasaki, M.D., Department of Neurosurgery, Kuwana Hospital, 6-4 Furukawacho, Niigata City 950, Japan.
J. Neurosurg. / Volume 75/September, 1991
Excision of cavernous angioma with preservation of coexisting venous angioma Case report OSAMU SASAKI, M.D., RYUICHI TANAKA, M.D., TL~rsuo KOIKE, M.D., AK1RA KOIDE, M.D., TAKAYUKIKOIZUMI, M.D., AND HIROSHI OGAWA, M.D. Departments of Neurosurgery and Neuropathology, Kuwana Hospital, and Department of Neurosurgery, Brain Research Institute, 3Higata University, Niigata City, Japan v" The case of a patient presenting with dysphasic seizures due to a cavernous angioma coexisting with a venous malformation is reported. The cavernous angioma was resected with preservation of the venous malformation, as confirmed by postoperative studies. The patient was seizure-free following surgery. KEY WORDS
9
cavernous angioma
9 venous angioma
9 arteriovenous malformation
9
seizure
AVERNOUSangiomas are a welt-known cause of seizures or hemorrhage. However, the clinical significance of venous malformations is not clear and the management of these lesions remains controversial. We recently encountered the case of a patient presenting with dysphasic seizures due to a cavernous angioma coexisting with a venous malformation in the same area. The patient was treated surgically and the cavernous angioma was resected with preservation of the venous malformation, resulting in relief of seizures. In this case, the cavernous angioma seemed to be closely related to the clinical presentation. If such lesions are managed surgically, it seems appropriate to remove only the cavernous angioma.
C
able. Electroencephalography showed focal spikes in the left temporal lobe. Computerized tomography (CT) scans demonstrated a slightly higher-density lesion in the left temporal lobe. After infusion of contrast medium, the density of the lesion increased slightly and a curvilinear enhancement was noted around the lesion (Fig. 1A). Magnetic resonance (MR) imaging depicted a core of mixed signal intensity, with a rim of decreasing signal intensity on the T2-weighted study (Fig. 1B). Angiography showed an appearance typical of a venous
Case Report
This 31-year-old right-handed woman came to our hospital on December 2, 1988, complaining of episodic speech dysfunction during the 4 years prior to presentation. During these episodes, which lasted a few minutes and occurred once or twice a day, she was temporally unable to comprehend spoken language. There were no other associated symptoms and her medical and family history was not significant. Examination. General, neurological, and neuropsychiatric examinations of the patient proved unremark-
J. Neurosurg. / Volume 75/September, 1991
FIG. 1. Preoperative studies. A: Postcontrast computerized tomography scan demonstrating a curvilinear enhancement. B: Magnetic resonance T2-weighted image depicting a core of mixed intensity with a rim of decreased intensity. 461
O. Sasaki, et al.
FIG. 2. Preoperative left internal carotid angiogram showing a typical venous malformation in the left temporal lobe.
FiG. 3. Schematic drawing of the intraoperative view. CA = cavernous angioma; MV = central medullary vein of the venous malformation; DV = draining vein.
malformation in the left temporal lobe (Fig. 2). A diagnosis was made of simple partial seizures associated with adjacent vascular malformations of the cavernous and venous types in the left temporal lobe. Initially, the patient was treated medically with 200 mg phenytoin and 50 mg phenobarbital administered daily for 1 month. When the frequency of the seizures did not decrease, 400 mg of carbamazepine was given for 1 month. This also showed no effect and was discontinued due to the development of side effects. Operation. To control the seizures and prevent future hemorrhage, the patient underwent a frontotemporal craniotomy on May 6, 1989. Electrocorticography revealed seizure discharges from the superior and middle temporal gyri around the vein of Labbe. The lesion was approached from the anterolateral aspect with splitting of the superior temporal sulcus. The cavernous angioma was found embedded in the markedly xanthochromic subcortical white matter of the superior temporal gyms, and was partially adherent to a hugely dilated, medially coursing vein of the venous malfor462
FIG. 4. Photomicrograph of the surgical specimen. The lesion is composed of sinusoidal vascular spaces of various sizes lined by a single layer of endothelium. Intervening neural tissue is absent in the intraparenchymal lesions, and the vessel wails are devoid of smooth-muscle and elastic tissue. These findings are typical of cavernous angiomas. Elastica Goldner stain, x I 15.
mation. Several small veins from the cavernous angioma drained to this central medullary vein (Fig. 3). After coagulation and cutting of these small veins, the cavernous angioma was carefully separated from the huge vein. Finally, the cavernous angioma was totally resected and the venous malformation was left untouched. Postoperative electrocorticography showed a significant reduction in seizure frequency. Pathological Examination. The lesion was composed of sinusoidal vascular spaces of various sizes lined by a single layer of endothelium (Fig. 4). There was no intervening neural tissue in the intraparenchymal lesions, and the vessel walls were devoid of smoothmuscle and elastic tissue. Several vascular spaces conmined old hyalinized thrombi, and deposits of calcium were seen in the vascular walls. The surrounding brain tissue showed gliosis with hemosiderin deposits. These were findings typical of a cavernous malformation. Postoperative Course. The patient's postoperative course was unremarkable. She developed dysphasic seizures only twice after the operation, on the 1 lth and 2lst postoperative days. She was discharged on May 30, 1989, without any neurological or neuropsychiatric deficit, and at her 189 follow-up review she remained seizure-free without anticonvulsant therapy. Postoperative CT scans and MR imaging revealed disappearance of the cavernous angioma (Fig. 5). Follow-up angiography (Fig. 6) performed on the 22nd day after surgery showed that the venous malformation was preserved; however, there were fewer small veins converging into the huge central medullary vein than preoperatively. This seemed to result from the obliteration of small draining veins from the cavernous angioma into the central medullary vein of the venous malformation.
J. Neurosurg. / Volume 75/September, 1991
Excision of cavernous angioma
FIG. 5. Postoperative magnetic resonance studies, enhanced T~-weighted image (A) and T2-weighted image (B), revealing disappearance of the cavernous angioma and preservation of the central medullary vein of the venous malformation.
Discussion
Venous malformations differ from cavernous angiomas in clinical, radiological, and histological features. In a clinical practice a clear distinction is made between these two entities, but the coexistence of two types of malformations in the same area seems to suggest that they are a continuum and have a similar pathogenesis. In our case, the cavernous and venous malformations adhered to each other and were connected by small medullary veins. This anomaly may represent a transitional form of cavernous and venous malformations. Cavernous angiomas are a well-known cause of seizures or hemorrhage, ~-5'~2but the clinical significance of venous malformations has not been clearly determined. Most authors have considered them to be generally silent) 9~' A large autopsy series reported by McCormick 6 showed that they were usually asymptomatic. On the other hand, some authors have stressed the tendency of venous malformations to bleed 2'4"7"9"~~ and have recommended surgical removal. 2,4 It seems true that at least a small number of patients with venous malformations present with symptoms; however, the characteristics of symptomatic malformations or the differences between symptomatic and asymptomatic malformations remain unexplained. Cavernous angiomas have no pathognomonic radiographic appearance. They cannot be visualized directly with angiography. 3~s'9 Although CT scanning is useful for detection (they characteristically appear as a nodular lesion), the findings are neither specific nor diagnostic. 5'8'9 A true diagnosis can only be made histologically? ,9 In recent years, MR imaging has proved sensitive in detecting these lesions. In most cases, a correct diagnosis can be made preoperatively, ~ although M R imaging cannot distinguish between a cavernous angioma and a thrombosed arteriovenous malformation. Venous malformations, in contrast, are diagnosed by angiography with ease because of their specific features. 2-35 Computerized tomography also aids in diag-
J. Neurosurg. / Volume 75/September, 1991
FIG. 6. Postoperative left internal carotid angiogram showing preservation of the venous malformation. There are slightly fewer small veins converging into the central medullary vein than preoperatively.
nosis if the classic linear or curvilinear enhancement is demonstrated. Numaguchi, et al.,7 reported that 11 of 44 venous malformations diagnosed angiographically showed nodular enhancement, which was more commonly associated with cavernous angiomas. Recently, Rigamonti and Spetzler 9 also stated that many symptomatic venous malformations showed global hyperdensity on CT scans. If high-field strength MR imaging is undertaken in such cases, a coexisting cavernous angioma could be demonstrated. Although such reports are very rare, the incidence of the association of these two vascular malformations appears to be higher than expected, 9 and the presence or absence of a coexisting cavernous angioma may explain the differences between symptomatic and asymptomatic venous malformations. In our case, the patient was treated surgically, resulting in resolution of her seizures. At surgery, only the cavernous angioma was excised since obliteration of the venous malformation in the left temporal lobe would have had disastrous consequences. This result suggests that the cavernous angioma was closely related to the seizures. Based on our experience, it would be appropriate to remove only the cavernous angioma if these lesions are managed surgically. References
1. Giombini S, Morello G: Cavernousangiomasofthe brain. Account of fourteen personal cases and review of the literature. Acta Neurochir 40:61-82, 1978 2. Handa H, Moritake K: Venous angiomas of the brain, in Fein JM, Flamm ES (eds): Cerebrovascular Surgery. Volume IV. New York: Springer-Verlag, 1985, pp 1139-1149 3. Huang YP, Robbins A, Patel SC, et at: Cerebral venous 463
O. Sasaki, et al.
4. 5.
6. 7. 8.
464
malformations (and a new classification of cerebral vascular malformations), in Kapp JP, Schmidek ttH (eds): The Cerebral Venous System and Its Disorders. Orlando: Grune & Stratton, 1984, pp 373-474 Malik GM, Morgan JK, Boulos RS, et al: Venous angiomas: an underestimated cause of intracranial hemorrhage. Surg Neurol 30:350-358, 1988 Martin NA, Wilson CB, Stein BM: Venous and cavernous malformations, in Wilson CB, Stein BM (eds): Intracranial Arteriovenous Malformations. Baltimore: Williams & Wilkins, 1984, pp 234-245 McCormick WF: The pathology of angiomas, in Fein JM, Flamm ES (eds): Cerebrovascular Surgery. Volume IV. New York: Springer-Verlag, 1985, pp 1074-1095 Numaguchi Y, Kitamura K, Fukui M, et al: Intracranial venous angiomas. Surg Neurol 18:193-202, 1982 Rigamonti D, Drayer BP, Johnson PC, et al: The MRI appearance of cavernous malformations (angiomas). J Neurosurg 67:518-524, 1987
9. Rigamonti D, Spetzler RF: The association of venous and cavernous malformations. Report of four cases and discussion of the pathophysiological, diagnostic, and therapeutic implications. Acta Neurochir 92:100-105, 1988 10. Rothfus WE, Albright AL, Casey KF, et al: Cerebellar venous angioma: "benign" entity? AJNR 5:61-66, 1984 11. Saito Y, Kobayashi N: Cerebral venous angiomas: clinical evaluation and possible etiology. Radiology 139:87-94, 1981 12. Simard JM, Garcia-Bengochea F, Ballinger WE Jr, et al: Cavernous angiomas: a review of 126 collected and 12 new clinical cases. Neurosurgery 18:162-172, 1986
Manuscript received August 28, 1990. Accepted in final form February 15, 1991. Address reprint requests to: Osamu Sasaki, M.D., Department of Neurosurgery, Kuwana Hospital, 6-4 Furukawacho, Niigata City 950, Japan.
J. Neurosurg. / Volume 75/September, 1991
