Neuroradiology 9, 125-- 128 (1975) © by Springer-Verlag 1975
Cerebral Venous Angioma L. N. Scotti, R. L. G o l d m a n , G. R. R a o * a n d E. R. H e i n z From the Departments of Radiology and Pathology*, University of Pittsburgh, School of Medicine, Presbyterian-University Hospital, Pittsburgh, Pennsylvania, U.S.A. Received: March 6, 1975 Summary. A case of venous angioma involving the frontal lobe is presented with surgical and histological confirmation. Only two other angiographically diagnosed cases have been reported previously in the literature. This case tends to support the entity of cerebral venous angioma as a distinct, though rare, type of cerebral vascular malformation. Angiome veineux cdrdbral Rdsumd. Les auteurs pr6sentent un cas d ' a n g i o m e veineux du lobe frontal avec confirmation chirurgicale et histologique. La litt6rature ne mentionne que 2 autres cas diagnostiqu6s
Cerebral Venous Angioma
par l'angiographie. Cette observation est en faveur de l'hypoth~se selon laquelle l'angiome veineux c6r6bral, bien que rare, est ~ consid~rer comme une malformation vasculaire c6r6brale. Cerebrale venOse Angiome Zusammenfassung. Fallbeschreibung eines 19jiihrigen Mannes mit einem ven6sen Angiom im Bereich des Frontallappens. Bisher wurden nur 2 weitere angiographisch untersuchte Patienten in der Literatur beschrieben. Es ergibt sich daraus die Seltenheit yon cerebralen ven6sen Angiomen.
Physical e x a m i n a t i o n r e v e a l e d a well d e v e l o p e d and well n o u r i s h e d m a l e with nuchal rigidity. T h e
D o strictly v e n o u s m a l f o r m a t i o n s (venous angiomas) exist? T h e y were said to b e "the c o m m o n e s t vasc u l a r a b n o r m a l i t y of the spinal c o r d [14]. H o w e v e r , spinal c o r d a n g i o g r a p h y has n o t b o r n e this o u t and m o s t v a s c u l a r m a l f o r m a t i o n s of ~:he c o r d are of the a r t e r i o v e n o u s type [5]. S o m e authors [8] w o u l d disp u t e the n e e d to distinguish v e n o u s a n g i o m a as a s e p a r a t e t y p e of v a s c u l a r m a l f o r m a t i o n . W h i l e a d m i t t e d l y r a r e [4], the a n g i o g r a p h i c r e c o g n i t i o n of a p u r e l y v e n o u s m a l f o r m a t i o n will a v o i d c o n f u s i o n in the differential diagnosis of a b n o r m a l v e n o u s channels [13] a n d will aid in the p r e o p e r a t i v e e v a l u a t i o n of such lesions. T h e following case r e p o r t helps to verify the existence of a venous a n g i o m a involving the c e r e b r u m . T o o u r k n o w l e d g e , only two o t h e r cases of a n g i o g r a p h i c a l l y d i a g n o s e d a n d surgically p r o v e n c e r e b r a l v e n o u s a n g i o m a s have b e e n r e p o r t e d in the l i t e r a t u r e [3].
Case Report A 19 y e a r o l d m a l e s t u d e n t gave a t h r e e d a y hist o r y of m i l d f r o n t a l h e a d a c h e a n d a sensation of fullness in his head. O n the night of a d m i s s i o n he exp e r i e n c e d a severe f r o n t a l h e a d a c h e a n d m a r k e d l e t h a r g y a s s o c i a t e d with nausea, vomiting, a n d p h o t o p h o b i a . N o h i s t o r y of t r a u m a was o b t a i n e d .
Fig. 1. Left lateral carotid arterial angiogram. Normal arterial structures are demonstrated without evidence of enlarged or tortuous vessels
126
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et al.:
Cerebral Venous Angioma
a
b
Fig. 2. a) Left AP and b) lateral venous angiograms show multiple enlarged medullary veins (open arrow) which drain into a large cortical vein (closed arrow) examination was otherwise normal. A lumbar puncture was performed and the cerebrospinal fluid was frankly bloody. The opening pressure was 200 m m of water. A brain scan revealed an abnormal uptake in the left frontal region. A cerebral angiogram was performed which revealed a normal circulation time with a normal arterial phase (Fig. 1). In the venous phase, a collection of multiple enlarged and tortuous medullary veins were demonstrated in the left frontal lobe which drained into a large cortical vein that subsequently drained into the anterior part of the sagittal sinus (Fig. 2). A left frontal craniotomy was performed and the vascular malformation in the left frontal lobe was excised. At this time, slight local staining was noted in the region of the malformation, but no intracerebral h e m a t o m a was present. No enlarged feeding arteries or capillaries were identified. The vascular structures were entirely venous in nature and no evidence of increased pressure or flow within the malformation was noted. The postoperative course was uneventful and the patient made a complete recovery. A postoperative angiogram showed successful removal of the venous malformation (Fig. 3). The tissue removed measured 3 X 2 X 1 cm and consisted of a portion of cerebral cortex with leptomeninges containing dilated vein with several vascular channels. Microscopic examination showed a large dilated vein with hyaline collagenous thickening (Fig. 4) and numerous venous channels of varying sizes in
the leptomeninges. A few vessels of venous character are seen extending into the cortex and subcortical white matter separated by brain parenchyma. Two leptomeningeal arteries are identified; however the
Fig. 3. A postoperative left lateral venous angiogram reveals complete removal of the venous angioma
L. N. Scotti et al.: Cerebral Venous Angioma
~27
Fig. 4. Markedly dilated and thickened vein (arrow) with overlying plexus of venous channels (Verhoeff's elastic stain)
Fig. 5. The malformation consisting of large dilated veins (arrows) with thickened walls principally involving the leptomeninges (Verhoeff's elastic stain)
main malformation is formed of vessels of venous character (Fig. 5).
cluster of vessels which are venous in character and which are separated from each other by thin layers of cerebral tissue. This latter finding distinguishes a venous angioma from a cavernous hemangioma which does not contain any intervening neural parenchyma. While this may be a means of histologically differentiating these two lesions, the angiographic appearance may be quite similar. Roberson et al., [10] have recently reported a case with both telangiectases and cavernous angioma producing a brainstem hematoma. The pontine cavernous angioma was not demonstrated on the angiogram but dilated venous channels arranged in a radial pattern were seen draining into a brachial tributary of a distended precentral cerebellar vein. The angiographic appearance was similar to the medullary veins noted in our case and suggested varices or a venous angioma but the channels histologically proved to be telangiectases. Bogren et al., [2] reported five cases of intracranial cavernous hemangiomas; Case 4 (histologically unconfirmed) bears a striking resemblance to our own and may
Discussion Vascular malformations are usually classified according to the following groups [7, 11, 12]: 1. Telangiectasia (capillary angioma) 2. Cavernous malformation (angioma, hemangioma) 3. Venous angioma (Racemose angioma) 4. Arteriovenous malformation 5. Varix Venous angioma or the older term, venous racemose angioma, is a malformation comprised only of venous structures. They most often arise in the region of the middle cerebral artery but can also occur in the anterior cerebral artery distribution, the cerebellum and near the vein of Galen [9]. Pathologically, the malformation presents as a conical or wedged-shaped lesion with its base at the meninges and its apex toward the ventricles [12]. Within the brain there is a Neuroradiology,Vol. 9
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L.N. Scotti et al.: Cerebral Venous Angioma
represent a venous angioma rather than a cavernous malformation. Constans et al., [3] reported two cases of cerebral venous angiomas. The angiographic and histological findings are identical with those f o u n d in this case. The most important and striking angiographic feature consist of enlarged draining veins (medullary or cortical). T h e arterial phase is normal, without enlarged feeding arteries. Since there is no arteriovenous shunting, the venous structures do not opacify early and the circulation time is normal. Unless an intracerebral h e m a t o m a occurs, no mass is present. The presenting symptoms are usually those related to subarachnoid hemorrhage, (as in our case), or seizures. The brain scan m a y be positive. Enlarged and tortuous veins m a y be seen in a n u m b e r of conditions which must be differentiated f r o m a venous angioma. As mentioned previously, cavernous hemangiomas, with or without associated telangiectases, m a y present with similar angiographic findings [6]. Early venous filling or delayed emptying associated with a space-occupying lesion would be more suggestive of cavernous h e m a n g i o m a [2, 10]. Enlarged veins m a y be seen in Sturge-Weber Syndrome [1]. The relative absence of cortical veins, the enlargement of the deep venous system, typical calcifications and clinical presentation help to exclude this diagnosis. Tumors, healing infarcts and traumatic lesions m a y also present angiographically with abnormally draining veins. The history, clinical findings and associated arterial abnormalities should help to differentiate these conditions f r o m a venous angioma.
References 1. Bentson, J. R., Wilson, G. H., Newton, T. H.: Cerebral venous drainage pattern of the Sturge-Weber syndrome. Radiol. 101, 111-118 (1971)
2. Bogren, C., Svalander, C., Wickbom, I.: Angiography in intracranial cavernous hemangiomas. Acta radiol. 10, 81--89 (1970) 3. Constans, J, P., Dilenge, D., Vedrenne, CI.: Angiomas veineux cerebraux. Neuro-Chirurgie 14, (1968) 4. Courville, C. B.: Pathology of the central nervous system. ed. 2. Mountain View, California: Pacific Press 1945 5. Djindjian, R., Hurth, M., Houdant, R.: Angiography of the spinal cord. Baltimore: University Park Press 1970 6. Jonutis, A. J., Sondheimer, F. K., Klein, H. Z., Wise, B. L.: Intracerebral cavernous hemangioma with angiographically demonstrated pathological vasculature. Neuroradiology 3, 57--63 (1971) 7. McCormick, W. F.: The pathology of vascular ("Arteriovenous") malformations. J. Neurosurg. 24, 807 (1966) 8. Aronson, S. M.: "Vascular malformations" in Minckler, J. Pathology of the nervous system. Vol. II. New York: McGraw-Hill Book Company 1968 9. Noran, H. H.: Intracranial vascular tumors and malformations. Arch. Path. 39, 393--416 (1945) 10. Roberson, G. H., Kase, C. S., Wolpow, E. R.: Telangiectases and cavernous angiomas of the brainstem: "Cryptic" vascular malformations. Neuroradiology 8, 83-- 89 (1974) 11. Russell, D. S., Rubinstein, L. J.: Pathology of tumours of the nervous system. London: Edward Arnold Ltd. 2nd ed. 1963 12. Stephbens, W. E.: Pathology of cerebral blood vessels. St. Louis: Mosby 1972 13. Wolf, P. A., Rosman, N. P., New, P. F. J.: Multiple small cryptic venous angiomas of the brain mimicking cerebral metastases. Neurology 17, 491--501 (1967) 14. Wyburn-Mason, R.: The vascular abnormalities and tumors of the spinal cord and its membranes. London: Kimpton 1943 Louis N. Scotti, M.D. Department of Radiology University of Pittsburgh School of Medicine Presbyterian-University Hospital 230 Lothrop Street Pittsburgh, Pennsylvania 15261 U.S.A.
Cerebral Venous Angioma L. N. Scotti, R. L. G o l d m a n , G. R. R a o * a n d E. R. H e i n z From the Departments of Radiology and Pathology*, University of Pittsburgh, School of Medicine, Presbyterian-University Hospital, Pittsburgh, Pennsylvania, U.S.A. Received: March 6, 1975 Summary. A case of venous angioma involving the frontal lobe is presented with surgical and histological confirmation. Only two other angiographically diagnosed cases have been reported previously in the literature. This case tends to support the entity of cerebral venous angioma as a distinct, though rare, type of cerebral vascular malformation. Angiome veineux cdrdbral Rdsumd. Les auteurs pr6sentent un cas d ' a n g i o m e veineux du lobe frontal avec confirmation chirurgicale et histologique. La litt6rature ne mentionne que 2 autres cas diagnostiqu6s
Cerebral Venous Angioma
par l'angiographie. Cette observation est en faveur de l'hypoth~se selon laquelle l'angiome veineux c6r6bral, bien que rare, est ~ consid~rer comme une malformation vasculaire c6r6brale. Cerebrale venOse Angiome Zusammenfassung. Fallbeschreibung eines 19jiihrigen Mannes mit einem ven6sen Angiom im Bereich des Frontallappens. Bisher wurden nur 2 weitere angiographisch untersuchte Patienten in der Literatur beschrieben. Es ergibt sich daraus die Seltenheit yon cerebralen ven6sen Angiomen.
Physical e x a m i n a t i o n r e v e a l e d a well d e v e l o p e d and well n o u r i s h e d m a l e with nuchal rigidity. T h e
D o strictly v e n o u s m a l f o r m a t i o n s (venous angiomas) exist? T h e y were said to b e "the c o m m o n e s t vasc u l a r a b n o r m a l i t y of the spinal c o r d [14]. H o w e v e r , spinal c o r d a n g i o g r a p h y has n o t b o r n e this o u t and m o s t v a s c u l a r m a l f o r m a t i o n s of ~:he c o r d are of the a r t e r i o v e n o u s type [5]. S o m e authors [8] w o u l d disp u t e the n e e d to distinguish v e n o u s a n g i o m a as a s e p a r a t e t y p e of v a s c u l a r m a l f o r m a t i o n . W h i l e a d m i t t e d l y r a r e [4], the a n g i o g r a p h i c r e c o g n i t i o n of a p u r e l y v e n o u s m a l f o r m a t i o n will a v o i d c o n f u s i o n in the differential diagnosis of a b n o r m a l v e n o u s channels [13] a n d will aid in the p r e o p e r a t i v e e v a l u a t i o n of such lesions. T h e following case r e p o r t helps to verify the existence of a venous a n g i o m a involving the c e r e b r u m . T o o u r k n o w l e d g e , only two o t h e r cases of a n g i o g r a p h i c a l l y d i a g n o s e d a n d surgically p r o v e n c e r e b r a l v e n o u s a n g i o m a s have b e e n r e p o r t e d in the l i t e r a t u r e [3].
Case Report A 19 y e a r o l d m a l e s t u d e n t gave a t h r e e d a y hist o r y of m i l d f r o n t a l h e a d a c h e a n d a sensation of fullness in his head. O n the night of a d m i s s i o n he exp e r i e n c e d a severe f r o n t a l h e a d a c h e a n d m a r k e d l e t h a r g y a s s o c i a t e d with nausea, vomiting, a n d p h o t o p h o b i a . N o h i s t o r y of t r a u m a was o b t a i n e d .
Fig. 1. Left lateral carotid arterial angiogram. Normal arterial structures are demonstrated without evidence of enlarged or tortuous vessels
126
L.N. Scotti
et al.:
Cerebral Venous Angioma
a
b
Fig. 2. a) Left AP and b) lateral venous angiograms show multiple enlarged medullary veins (open arrow) which drain into a large cortical vein (closed arrow) examination was otherwise normal. A lumbar puncture was performed and the cerebrospinal fluid was frankly bloody. The opening pressure was 200 m m of water. A brain scan revealed an abnormal uptake in the left frontal region. A cerebral angiogram was performed which revealed a normal circulation time with a normal arterial phase (Fig. 1). In the venous phase, a collection of multiple enlarged and tortuous medullary veins were demonstrated in the left frontal lobe which drained into a large cortical vein that subsequently drained into the anterior part of the sagittal sinus (Fig. 2). A left frontal craniotomy was performed and the vascular malformation in the left frontal lobe was excised. At this time, slight local staining was noted in the region of the malformation, but no intracerebral h e m a t o m a was present. No enlarged feeding arteries or capillaries were identified. The vascular structures were entirely venous in nature and no evidence of increased pressure or flow within the malformation was noted. The postoperative course was uneventful and the patient made a complete recovery. A postoperative angiogram showed successful removal of the venous malformation (Fig. 3). The tissue removed measured 3 X 2 X 1 cm and consisted of a portion of cerebral cortex with leptomeninges containing dilated vein with several vascular channels. Microscopic examination showed a large dilated vein with hyaline collagenous thickening (Fig. 4) and numerous venous channels of varying sizes in
the leptomeninges. A few vessels of venous character are seen extending into the cortex and subcortical white matter separated by brain parenchyma. Two leptomeningeal arteries are identified; however the
Fig. 3. A postoperative left lateral venous angiogram reveals complete removal of the venous angioma
L. N. Scotti et al.: Cerebral Venous Angioma
~27
Fig. 4. Markedly dilated and thickened vein (arrow) with overlying plexus of venous channels (Verhoeff's elastic stain)
Fig. 5. The malformation consisting of large dilated veins (arrows) with thickened walls principally involving the leptomeninges (Verhoeff's elastic stain)
main malformation is formed of vessels of venous character (Fig. 5).
cluster of vessels which are venous in character and which are separated from each other by thin layers of cerebral tissue. This latter finding distinguishes a venous angioma from a cavernous hemangioma which does not contain any intervening neural parenchyma. While this may be a means of histologically differentiating these two lesions, the angiographic appearance may be quite similar. Roberson et al., [10] have recently reported a case with both telangiectases and cavernous angioma producing a brainstem hematoma. The pontine cavernous angioma was not demonstrated on the angiogram but dilated venous channels arranged in a radial pattern were seen draining into a brachial tributary of a distended precentral cerebellar vein. The angiographic appearance was similar to the medullary veins noted in our case and suggested varices or a venous angioma but the channels histologically proved to be telangiectases. Bogren et al., [2] reported five cases of intracranial cavernous hemangiomas; Case 4 (histologically unconfirmed) bears a striking resemblance to our own and may
Discussion Vascular malformations are usually classified according to the following groups [7, 11, 12]: 1. Telangiectasia (capillary angioma) 2. Cavernous malformation (angioma, hemangioma) 3. Venous angioma (Racemose angioma) 4. Arteriovenous malformation 5. Varix Venous angioma or the older term, venous racemose angioma, is a malformation comprised only of venous structures. They most often arise in the region of the middle cerebral artery but can also occur in the anterior cerebral artery distribution, the cerebellum and near the vein of Galen [9]. Pathologically, the malformation presents as a conical or wedged-shaped lesion with its base at the meninges and its apex toward the ventricles [12]. Within the brain there is a Neuroradiology,Vol. 9
i0
128
L.N. Scotti et al.: Cerebral Venous Angioma
represent a venous angioma rather than a cavernous malformation. Constans et al., [3] reported two cases of cerebral venous angiomas. The angiographic and histological findings are identical with those f o u n d in this case. The most important and striking angiographic feature consist of enlarged draining veins (medullary or cortical). T h e arterial phase is normal, without enlarged feeding arteries. Since there is no arteriovenous shunting, the venous structures do not opacify early and the circulation time is normal. Unless an intracerebral h e m a t o m a occurs, no mass is present. The presenting symptoms are usually those related to subarachnoid hemorrhage, (as in our case), or seizures. The brain scan m a y be positive. Enlarged and tortuous veins m a y be seen in a n u m b e r of conditions which must be differentiated f r o m a venous angioma. As mentioned previously, cavernous hemangiomas, with or without associated telangiectases, m a y present with similar angiographic findings [6]. Early venous filling or delayed emptying associated with a space-occupying lesion would be more suggestive of cavernous h e m a n g i o m a [2, 10]. Enlarged veins m a y be seen in Sturge-Weber Syndrome [1]. The relative absence of cortical veins, the enlargement of the deep venous system, typical calcifications and clinical presentation help to exclude this diagnosis. Tumors, healing infarcts and traumatic lesions m a y also present angiographically with abnormally draining veins. The history, clinical findings and associated arterial abnormalities should help to differentiate these conditions f r o m a venous angioma.
References 1. Bentson, J. R., Wilson, G. H., Newton, T. H.: Cerebral venous drainage pattern of the Sturge-Weber syndrome. Radiol. 101, 111-118 (1971)
2. Bogren, C., Svalander, C., Wickbom, I.: Angiography in intracranial cavernous hemangiomas. Acta radiol. 10, 81--89 (1970) 3. Constans, J, P., Dilenge, D., Vedrenne, CI.: Angiomas veineux cerebraux. Neuro-Chirurgie 14, (1968) 4. Courville, C. B.: Pathology of the central nervous system. ed. 2. Mountain View, California: Pacific Press 1945 5. Djindjian, R., Hurth, M., Houdant, R.: Angiography of the spinal cord. Baltimore: University Park Press 1970 6. Jonutis, A. J., Sondheimer, F. K., Klein, H. Z., Wise, B. L.: Intracerebral cavernous hemangioma with angiographically demonstrated pathological vasculature. Neuroradiology 3, 57--63 (1971) 7. McCormick, W. F.: The pathology of vascular ("Arteriovenous") malformations. J. Neurosurg. 24, 807 (1966) 8. Aronson, S. M.: "Vascular malformations" in Minckler, J. Pathology of the nervous system. Vol. II. New York: McGraw-Hill Book Company 1968 9. Noran, H. H.: Intracranial vascular tumors and malformations. Arch. Path. 39, 393--416 (1945) 10. Roberson, G. H., Kase, C. S., Wolpow, E. R.: Telangiectases and cavernous angiomas of the brainstem: "Cryptic" vascular malformations. Neuroradiology 8, 83-- 89 (1974) 11. Russell, D. S., Rubinstein, L. J.: Pathology of tumours of the nervous system. London: Edward Arnold Ltd. 2nd ed. 1963 12. Stephbens, W. E.: Pathology of cerebral blood vessels. St. Louis: Mosby 1972 13. Wolf, P. A., Rosman, N. P., New, P. F. J.: Multiple small cryptic venous angiomas of the brain mimicking cerebral metastases. Neurology 17, 491--501 (1967) 14. Wyburn-Mason, R.: The vascular abnormalities and tumors of the spinal cord and its membranes. London: Kimpton 1943 Louis N. Scotti, M.D. Department of Radiology University of Pittsburgh School of Medicine Presbyterian-University Hospital 230 Lothrop Street Pittsburgh, Pennsylvania 15261 U.S.A.
