Neuroradiology (2000) 42: 455±457 Ó Springer-Verlag 2000
F. G. Aksoy J. M. Gomori Z. Tuchner
Received: 26 July 1999 Accepted: 11 August 1999 F. G. Aksoy Department of Radiology, Ankara Training and Teaching Hospital, Ankara, Turkey
)
J. M. Gomori ( ) Department of Radiology, Hadassah University Hospital, P. O. Box 12000, Jerusalem, Israel e-mail:
[email protected] Tel.: + 9 72-2-6 77 75 80 Fax: + 9 72-2-6 77 75 73
D IA G N O S T I C NE UR OR A DI O LO G Y
Association of intracerebral venous angioma and true arteriovenous malformation: a rare, distinct entity
Abstract We report a mixed cerebrovascular malformation in which a true arteriovenous malformation drained into associated venous angiomas. We describe the MRI and angiographic appearances and review the literature on mixed vascular malformations. Key words Angioma venous × Malformation arteriovenous
Z. Tuchner Department of Radiotherapy, Hadassah University Hospital, Jerusalem, Israel
Introduction
Case report
The classification of cerebrovascular malformations (CVM) into capillary, cavernous, venous and arteriovenous is widely accepted; each has distinct radiological features. Only recently have mixed CVM been appreciated as a distinct entity [1±5]. Their pathogenesis, incidence, natural history, clinical relevance and treatment remain controversial. The association of venous angioma (VA) and arteriovenous malformation (AVM) seems to be the least common; the association of cavernous angioma and VA seems more frequent [2]. There have been a few reports of ªarterialisedº VA shunts with [3] or without a typical nidus [1, 2, 6]. We report the MRI and angiographic findings of a combined VA and AVM.
A previously healthy 11-year-old-boy presented with episodes of headache and new-onset temporal lobe seizures. Neurological examination was normal. MRI (Fig. 1) revealed a vascular abnormality in the medial and anterior right temporal lobe. Two adjacent radiating spokewheet patterns (caput Medusae) of contrast-enhancing draining veins, characteristic of VA, were seen. Angiography (Fig. 2) showed an AVM of the right temporal lobe with enlarged feeding arteries and a nidus draining into two adjacent venous angiomas. There was no sign of intracerebral or intraventricular hemorrhage on MRI. The patient was treated with gamma-knife radiosurgery guided by stereotactic angiography. The radiation field covered both the AVM and the VA. The target dose was 2000 cGy. He tolerated the treatment well with no side effects or complications. He is asymptomatic 6 months after treatment and will be followed with MRI and angiography.
456
Fig. 1 Coronal contrast enhanced T1-weighted image demonstrates a radiating spokewheel pattern of draining veins, characteristic of venous angioma Fig. 2 a, b Arterial and late venous phases of right carotid angiogram, lateral projection, demonstrate the right temporal AVM and the large early draining vein with a spokewheel pattern (arrow) of the draining veins characteristic of a venous angioma. c Frontal projection, late venous phase, demonstrates the spokewheel pattern of the second venous angioma (arrow)
1
2a
2b
2c
Discussion In 1967, Wolf et al. [7] presented the first case of an intracerebral VA with angiographic arterial involvement. A hypertrophied posterior parietal branch of the middle cerebral artery was shown to supply one of multiple small VA. Our search of the literature revealed no other report of multiple venous angiomas, although Truwit [8], mentioned, without reference, that it was not rare to find two or more immediately adjacent to each other. In our case, two adjacent VA were associated with an AVM. Descriptions of combinations of AVM and VA have been restricted to VA with arterial components that correspond to arteriovenous (AV) shunting and lack the typical AVM nidus [1±3, 5, 7, 9±11]. We found one case of a true AVM with a nidus that drained into a VA [12]. The terminology used in the literature is not uniform. This association is usually described as mixed angiomas, arterialised VA, atypical AVM or as mixed AVM-venous malformations. The lesions are considered to be transitional forms between venous malformations and AVMs, or true mixed malformations of atypical AVM and VA, or small or atypical AVM with venous predominance [2, 3, 5, 12, 13].
In a retrospective review of 34 patients with angiographically occult venous malformations (AOVM), Robinson et al. [14] suggested that cavernous malformation, AOVM and the mixed lesions have similar nonspecific presentations (seizures, headache, focal neurological deficits) and haemorrhagic tendencies. In their series, 5 lesions previously thought to be solely AV, cavernous, or other vascular malformations were actually mixed vascular malformations with heterogeneous pathological features in the same lesion. Our patient had headache and temporal lobe seizures. VAs can be associated with cavernous malformations that have a bleeding tendency [1, 4, 12]. They also coexist with AV shunts, or capillary telangiectasia [2, 4, 13, 15]. These lesions have subtle angiographic findings or are angiographically occult [1, 2, 13±16]. To treat an AVM associated with a venous angioma could be a challenge because resection will necessarily lead to an unfavourable alteration in the haemodynamic situation, with removal of the venous drainage of nearby normal parenchyma. The controversy in the description of this rare entity also extends to its treatment [1, 6, 15, 17±21]. All recent reports show a low risk of intracerebral haemorrhage from pure VA [4, 6, 21]. Cere-
457
bral VA have also proved to be a common incidental finding on imaging [13, 15, 16, 21]. By using stereotactic radiosurgery in cases of combined AVM and VA, radiation can be directed only to the AVM component, while avoiding irradiation of the VA [19, 21]. Some workers doubtful of the benign history of VA favour surgical intervention in selected cases [18].
The association of a true AVM and VA is thus a distinct entity, less well known than the association of cavernous haemangioma and VA. The potential risk of a venous malformation appears to be due to its frequent association with other vascular lesions, which are underrecognised [1, 2, 4, 5, 13, 14, 16, 18]. The combination of MRI and angiography facilitates recognition of these previously occult combined vascular malformations.
References 1. Awad IA (1994) Radiosurgery and venous malformations (letter). J Neurosurg 80: 171±173 2. Awad IA, Robinson JR Jr, Mohanty S, Estes ML (1993) Mixed vascular malformations of the brain: clinical and pathogenetic considerations. Neurosurgery 33: 179±188 3. Fierstein SB, Pribram HW, Hieshima G (1979) Angiography and computed tomography in the evaluation of cerebral venous malformations. Neuroradiology 17: 137±148 4. Golfinos JG, Wascher TM, Zabramski JM (1991) The management of intracranial malformations. BNI Q 8: 2±11 5. Hirata Y, Matsukado Y, Nagahiro S, Kuratsu J (1986) Intracerebral venous angioma with arterial blood supply: a mixed angioma. Surg Neurol 25: 227±232 6. Kondziolka D, Dempsey PK, Lundsford L (1991) The case for conservative management of venous angiomas. Can J Neurol Sci 18: 295±299 7. Wolf PA, Rosman NP, New PF (1967) Multiple small cryptic venous angiomas of the brain mimicking cerebral metastases. Neuroradiology 17: 491±501
8. Truwit CL (1992) Venous angioma of the brain: history, significance, and imaging findings. AJR 159: 1299±1307 9. Kramer RA, Wing S (1977) Computed tomography of angiographically occult cerebral malformations. Radiology 123: 649±652 10. Preissig RS, Preissig SH, Goree JA (1976) Angiographic demonstration of a cerebral venous angioma. J Neurosurg 44: 628±631, 1976 11. Wendling LR, Moore JS, Kieffer SA, Goldberg HI, Latchaw RE (1976) Intracerebral venous angioma. Radiology 119: 141±147 12. Meyer B, Stangl AP, Schramm J (1995) Association of venous and true arteriovenous malformation: a rare entity among mixed vascular malformations of the brain. Case report. J Neurosurg 83: 141±144 13. Spetzler RF (1989) Letter to the editor. Surg Neurol 31: 412±413 14. Robinson JR Jr, Awad IA, Masaryk TJ, Estes ML (1993) Pathological heterogeneity of angiographically occult malformations of the brain. Neurosurgery 33: 547±555
15. Spetzler RF, Hamilton MG (1994) Radiosurgery and venous malformations (letter). J Neurosurg 80: 173±174 16. Rigamonti D, Spetzler RF (1988) 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 17. Senagor M, Dohrmann GJ, Wollmann RL (1983) Venous angiomas of the posterior fossa should be considered as anomalous venous drainage. Surg Neurol 19: 26±32 18. Lupret V, Negovetic L, Smiljanic D (1993) Cerebral venous angiomas: surgery as a mode of treatment for selected cases. Acta Neurochir 120: 33±39 19. Kurita H, Sasaki T, Tago M, Kaneko Y, Kirino T (1999) Successful radiosurgical treatment of arteriovenous malformation accompanied by venous malformation. AJNR 20: 482±485 20. Rigamonti D, Spetzler RF, Medina M, et al (1990) Cerebral venous malformations. J Neurosurg 73: 560±564 21. Lindquisit C, Guo W, Karlsson B, Steiner L (1993) Radiosurgery for venous angiomas. J Neurosurg 78: 531±536
