V ENOUS malformations, cavernous angiomas, capillary. Cerebral venous malformations

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1 J Neurosurg 73: , 1990 Cerebral venous malformations DANIELE RIGAMONTI, M.D., ROBERT F. SPETZLER, M.D., MARJORIE MEDINA, R.N., KAREN RIGAMONTI, M.D., DAVID S. GECKLE, M.D., AND CONRAD PAPPAS, M.D. Division of Neurological Surgery, University of Maryland School of Medicine, Baltimore, Maryland, and Division of Neurological Surgery, Barrow Neurological Institute, Phoenix, Arizona v, Although cerebral venous malformations have been reported to cause epilepsy, progressive neurological deficits, and hemorrhage, their clinical significance remains controversial. In an attempt to clarify the natural history of the lesion and suggest an appropriate management strategy, the authors review their experience with 30 patients. In four patients with cerebellar venous angioma, an acute episode of ataxia was documented. The coexistence of a cavernous malformation was pathologically confirmed in the two patients who underwent surgery for bleeding presumed caused by the venous angioma. Infarction was shown in two patients and a tumor in two others. Follow-up periods ranged between 18 and 104 months, with only five patients symptomatic at the time of this report. Rebleeding had not occurred, nor had acute episodes of neurological dysfunction been documented. This clinical experience suggests that a venous malformation is frequently associated with other, more symptomatic conditions and is often erroneously identified as the source of the symptoms. Because the nature of the relationship between the venous malformation and the allied conditions remains ambiguous, it is recommended that patients harboring a "symptomatic" venous malformation undergo high-field magnetic resonance imaging to rule out underlying pathology, and that any such pathology be treated independently of the venous malformation. KEY WORDS 9 venous malformation 9 angioma 9 hemorrhage 9 cavernous angioma 9 natural history V ENOUS malformations, cavernous angiomas, capillary telangiectasias, and arteriovenous malformations are four types of commonly described cerebrovascular malformations.11-13, ,63 Composed of radially arranged anomalous medullary veins that converge in a centrally located dilated trunk, the venous malformation is surrounded by normal neural parenchyma. 15'1s'29"46'6~ The characteristic angiographic appearance of a venous malformation has been described as "caput medusae" because of its resemblance to the snake-covered head of the mythical Gorgon Medusa.2,4-s, 10,14,17-19,22,23,25,27,32,34,37-41,45,48-54,57,61,62 The clinical relevance of this common vascular malformation is controversial. Therefore, we have reviewed the course of 30 patients diagnosed as having venous malformations in order to clarify the natural history of the lesion in an effort to suggest an appropriate management strategy. Clinical Material and Methods Physicians' and hospital records of 30 patients with cerebral venous malformations between January, 1980, and December, 1987, were reviewed at the Barrow Neurological Institute and at the University of Maryland. Each patient's chart was examined for demographic information, clinical presentation, neuroradiological findings, therapy, and follow-up data. There were 14 males and 16 females, ranging in age from 7 to 73 years (mean 37 years). Eighteen patients harbored the venous malformations in the supratentorial compartment, 12 lesions involved the cerebellum, and one patient had both a cerebellar and a pontine malformation. A familial incidence of venous malformation could not be established in any of our patients. Symptomatology Results The most common clinical finding was headache. Twenty patients (67%) complained of headache, and in 13 patients (43%) it was the only complaint. In one case, headache was caused by the rupture of an associated aneurysm. Four patients ( 13 %) experienced nausea and vomiting in addition to headache. The following 560 J. Neurosurg. / Volume 73/October, 1990

2 Cerebral venous malformations FIG. 2. Computerized tomography scans, without contrast enhancement (left) and after administration of contrast material (right), showing the linear enhancement characteristic of a venous malformation (arrows). FIG. 1. Angiogram, anteroposterior view, showing a large venous malformation of the left cerebellum. Multiple small medullary veins can be seen converging on the large venous trunk. symptoms were each reported in two patients (7%): seizures, psychiatric disturbances, amenorrhea, and ischemic symptoms. Among the 12 patients with cerebellar lesions, an acute episode of gait ataxia was reported in four ( 33 %), diplopia in three (25 %), numbness in two (17%), dysphagia and decreased hearing in one (8%), ischemic symptoms in one (8%), and epilepsy in one (8 %). Seven patients had multiple symptoms; however, four (33%) of the 12 cerebellar angiomas were associated with headache only. Radiological Findings Angiography, performed in 25 patients, always showed the typical caput medusae appearance of the lesion (Fig. 1). Computerized tomography (CT) studies were obtained in 24 patients (Fig. 2). Eighteen patients underwent CT without contrast enhancement; of these, seven scans (39%) were negative, four (22%) showed a round area of density, one (5%) showed both globular and linear regions of density, and one (5%) showed an area of low density. Of 22 CT scans with contrast medium administration, linear enhancement was present in 19 (86%), globular enhancement in one (4%), and both linear and globular enhancement in one (4%). One scan (4%) was negative. Magnetic resonance (MR) imaging was performed in 19 patients. A linear signal-void area was seen in each case (Fig. 3 left). The intravenous infusion of gadolinium further characterized the lesion (Fig. 3 right). In four cases (21%), a core of mixed increased and decreased signal intensity was depicted, corresponding to the globular density on the CT scans. Areas of increased FIG. 3. Left: Parasagittal T~-weighted magnetic resonance (MR) image depicting a cerebellar venous angioma characterized by a tubular-shaped signal-void area (arrow). Right: Gadolinium-enhanced MR image depicting the same venous angioma (arrowheads). J. Neurosurg. / Volume 73/October,

3 D. Rigamonti, et al. signal intensity, consistent with stroke, were seen in one patient. An area of increased signal intensity that proved to be a tumor was depicted in another case. Follow-Up Results Follow-up data were available for all but one patient and ranged from 18 to 104 months (mean 45 months, median 38 months). One elderly man was well until last examined 4 89 years after presentation, but he has since been lost to follow-up review. Of the remaining patients, 23 are well and asymptomatic, two are still having seizures, and one remains ataxic and complains of dizziness. One 14-year-old boy still experiences a severe headache at least once a month; on several occasions he has suddenly blacked out and, according to his mother, he is clumsy. One patient has developed a carcinoma of the neck. Discussion According to a large prospective autopsy series, 49 venous malformations are the most commonly encountered type of cerebrovascular malformation. Nonetheless, the number of symptomatic venous malformations described in the literature is limited, and their clinical relevance is still controversial. 59 A familial incidence of venous malformation has not yet been reported, although the familial occurrence of multiple vascular malformations has been demonstrated. 56 Diagnostic Studies The angiographic appearance of venous malformation is pathognomonic; enlarged medullary veins converge into a central draining trunk that reaches either the superficial system or, less commonly, the deep venous system. The arterial phase is normal. In our series, angiography always showed the caput medusae appearance with normal arterial and capillary phases. An early draining vein should always raise one's suspicion of an arteriovenous malformation. The CT appearance of venous malformation has been described extensively in the literature, l-6,8,9,14,16,17,19, 21-23,25,27,32,34, ,47,48,50,53,57,62 The most common finding is linear or curvilinear enhancement after contrast medium administration. 4'6'17'19'23'25'32'34"37'39'4~ A nodular hyperdense area has been noted on the nonenhanced study with faint enhancement after contrast administration, 25'27'34'37"45 and a round lesion seen only after administration of contrast material has also been described? 4 There is no mass effect unless a hematoma is nearby. A precontrast CT scan suggested the presence of an abnormal structure in 55 % of our cases. Contrastenhanced CT scanning depicted the typical linear enhancement in 86% of cases, and globular enhancement was present in 8%. Recently, MR imaging has been used to diagnose cerebral venous malformations. 2'8"26'44'5~ Magnetic resonance imaging may be equivalent to intravenously enhanced CT for detecting venous angioma and does not entail the risk of contrast medium infusion. The typical appearance of a venous malformation on T~- and T2- weighted images is a linear area of hypointensity, 32 which was visible in each of our cases. An area of increased signal intensity was seen in a patient who died of glioblastoma and in another who presented with stroke. In contrast, Augustyn, et al., 2 reported that 50% of venous malformations appeared as a linear region of hypointensity surrounded by an area of increased signal intensity. In four of our patients, a region of mixed increased and decreased signal intensity corresponded to the globular density seen on CT images. This finding strongly suggests some association, whether pathogenetic or coincidental, between venous and cavernous malformations. 42'43 This unusual combination, noted before the availability of MR imaging, was responsible for the statement that some cavernous malformations must have been diagnosed erroneously as venous malformations. 62 Symptomatology Although cerebral venous malformations have been reported to cause epilepsy, progressive neurological deficits, and hemorrhage, 3'5'35"4~ other authors have considered them to be clinically insignificant. 2~ Convulsions have frequently been associated with venous angiomas; ~,24,25'35'55 however, a positive correlation between the location of the angioma and the electroencephalographic focus is unusual. 48'55 A positive correlation cannot be ruled out in one of our patients who presented with seizures; in another patient, the cerebellar angioma was incidental. No correlation of venous angioma to psychiatric disorders was evident in this series. The venous angioma seemed incidental in two young women who presented with amenorrhea; in two patients aged in their 60's, who presented with ischemic symptoms, one had angiographically confirmed cerebral atherosclerosis in the area of occlusion and a spatially unrelated angioma while in the other patient the ischemic symptoms were referable to a territory in close proximity to the angioma. In the latter case, the possibility of an infarction resulting from occlusion of the angioma cannot be ruled out. Venous angiomas in the cerebellum are frequently associated with symptoms such as gait ataxia, diplopia, and dizziness, even in the absence of frank hemorrhage. 3'24'36'40 Cerebellar lesions are less commonly associated with numbness of the limbs. 36 Our experience confirms earlier reports that cerebellar venous angioma can be symptomatic. 33 Of 12 patients with cerebellar venous angiomas, an acute episode of gait ataxia was noted in four (33%), diplopia in three (25%), numbness in two (17%), and dysphagia and decreased hearing in one (8%). However, four cerebellar angiomas were associated with headache only. The true incidence of hemorrhage in cerebellar lesions is unclear, but more than two dozen cases of venous malformations located in the cerebellum have bled. 19'27'31'33"36A5'58"61 Of the reported cerebellar angio- fi62 J. Neurosurg. / Volume 73 / October, 1990

4 Cerebral venous malformations mas, 15% have bled acutely and another 20% have bled subacutely. 33 Operative intervention has therefore been recommended in the management of cerebellar venous angiomas. 28,33 Hemorrhage associated with venous angioma was noted in only two of our four patients with acute cerebellar dysfunction. At surgery, a hematoma was discovered close to an unsuspected cavernous malformation in one of these patients. In the second patient, MR imaging suggested the presence of a coexisting and spatially separate cavernous malformation. In the remaining two patients with acute cerebellar dysfunction, no evidence of hemorrhage could be found. In all 30 patients in this series, no episode of bleeding, new or recurrent, has been documented to date during a mean follow-up period of 45 months. Venous malformations have been surgically removed in less than 15% of the reported cases. 4'7'10'27'41'45'51 Although catastrophic infarction can ensue if the venous drainage of normal tissue is interrupted abruptly by the resection of the malformation, 4~ results have been satisfactory when surgery has been limited to evacuation of the hematoma. Only two of our patients underwent surgery; in both cases, the association of a venous and cavernous malformation was confirmed pathologically and it was believed that in the case of the hematoma, bleeding originated from the cavernous malformation. In one of these cases, the cerebellar venous malformation was partially resected. The patient's postoperative course was characterized by symptoms and signs of posterior fossa dysfunction related to acute swelling that developed by the time the dura was closed. Summary The association of venous malformations with cavernous malformations, tumors, or atherosclerosis may be easily overlooked; consequently, symptoms such as hemorrhage or focal neurological deficits can be attributed erroneously to a venous malformation. Patients harboring a "symptomatic" venous malformation should therefore undergo high-field MR imaging to rule out underlying pathology, which should be treated independently of the venous malformation. If no underlying pathology is found, we do not recommend surgery even when the malformation is located in the cerebellum. If a significant hematoma is found in association with the venous malformation, we recommend evacuation of the hematoma and careful pathological analysis of the specimen in search of a cavernous malformation or other angiographically occult lesions. Although resection of the venous structure is an option, our experience seems to suggest that sparing the venous malformation is wise and is not associated with rebleeding. References 1. Agnoli AL, Hildebrandt G: Cerebral venous angiomas. Acta Neurochir 78:4-12, Augustyn GT, Scott JA, Olson E, et al: Cerebral venous angiomas: MR imaging. Radiology 156: , Beatty RM, Zervas NT: Stereotactic aspiration of a brainstem hematoma. Neurosurgery 13: , Biller J, Toffol GL, Shea JF, et al: Cerebellar venous angiomas. A continuing controversy. Arch Neurol 42: , Bouchacourt E, Carpena JP, Bories J, et al: Accident ischrmique par thrombose d'un angiome veineux. A propos d'un cas. J Radiol 67: , Burke L, Berenberg RA, Kim KS: Choreoballismus: a nonhemorrhagic complication of venous angiomas. Surg Neurol 21: , Cabanes J, Blasco R, Garcia M, et al: Cerebral venous angiomas. Surg Neurol 11: , Cammarata C, Hans JS, Haaga JR, et al: Cerebral venous angiomas imaged by MR. Radiology 155: , Carter JE, Wymore J, Ansbacher L, et al: Sudden visual loss and a chiasmal syndrome due to an intrachiasmatic vascular malformation. J Clin Neuro Ophthalmol 2: , Constans JP, Dilenge D, Vrdrenne CL: Angiomes veineux crrrbraux. Neurochirurgie 14: , Courville CB: Pathology of the Central Nervous System. A Study Based Upon a Survey of Lesions Found in a Series of 30,000 Autopsies. Mountain View, Calif." Pacific Press, 1945, p Cushing H, Bailey P: Tumors Arising From the Blood Vessels of the Brain: Angiomatous Malformations and Hemangioblastomas. Springfield, II1: Charles C Thomas, 1928, pp Dandy WE: Venous abnormalities and angiomas of the brain. Arch Surg 17: , de Slegte RGM, Kaiser M, Valk J, et al: Cerebral venous angiomas: a review of clinical and radiological features of the literature and six additional cases. J Med Imaging 1: 15-25, Esser A: Ueber einen fall von gehirnvarizen. Pathol Gesellsch 20: , Fermaglich J, Kattah J, Manz H: Venous angiomas of the optic chiasm. Ann Neurol 4: , Fierstien SB, Pribram HW, Hieshima G: Angiography and computed tomography in the evaluation of cerebral venous malformations. Neuroradiology 17: , Guldenarm JA, Winkler C: Bijdragen tot de hersenchirurgie. Ned Tijdschr Geneeskd 27: , Hacker DA, Latchaw RE, Chou SN, et al: Case report. Bilateral cerebellar venous angioma. J Comput Assist Tomogr 5: , Handa J, Moritake K: Venous angiomas of the brain, in Fein JM, Flamm ES (eds): Cerebrovascular Surgery. New York: Springer-Verlag, 1985, pp Handa J, Suda K, Sato M: Cerebral venous angioma associated with varix. Surg Neurol 21: , Huang YP, Robbins A, Patel SC, et al: Cerebral venous malformations (and a new classification of cerebral vascular malformations), in Kapp JP, Schmidek HH (eds): The Cerebral Venous System and Its Disorders. New York: Grune & Stratton, 1984, pp Inagawa T, Taguchi H, Yamada T: Surgical intervention in ruptured venous angioma -- case report. Neurol Med Chit 25: , Jellinger K: The morphology of centrally-situated angiomas, in Pia HW, Gleave JRW, Grote E, et al (eds): Cerebral Angiomas: Advances in Diagnosis and Therapy. New York: Springer-Verlag, 1975, pp Koussa A, Chiras J, Poirier B, et al: Aspect tomodensito- J. Neurosurg. / Volume 73/October,

5 D. Rigamonti, et al. m6trique et angiographique des angiomas veineux du cerveau. A propos de 15 cas. Neurochirurgie 31: , Lee BCP, Herzberg L, Zimmerman RD, et al: MR imaging of cerebral vascular malformations. AJNR 6: , Maehara T, Tasaka A: Cerebral venous angioma: computed tomography and angiographic diagnosis. Neuroradiology 16: , Malik GM, Morgan JK, Boulos RS, et al" Venous angiomas: an underestimated cause of intracranial hemorrhage. Surg Neuroi 30: , McCormick WF: The pathology of vascular ("arteriovenous') malformations. J Neurosurg 24: , McCormick WF: Pathology of vascular malformations of the brain, in Wilson CB, Stein BM (eds): Intracranial Arteriovenous Malformations. Baltimore: Williams & Wilkins, 1984, pp I. McCormick WF, Hardman JM, Boutler TR: Vascular malformations (angiomas) of the brain with special reference to those occurring in the posterior fossa. J Neurosurg 28: , Michels LG, Bentson JR, Winter J: Computed tomography of cerebral venous angiomas. J Comput Assist Tomogr 1: , Moritake K, Handa H, Mori K, et al: Venous angiomas of the brain. Surg Neurol 14:95-105, Nagata K, Kubo T, Fukushima T: [Four cases of cerebral venous angioma -- with particular reference to the operative indication and the importance of CT diagnosis.] No Shinkei Geka 11: , 1983 (Jpn) 35. Nishikawa J, Maehara T: CT scan. Cerebral vascular disease, venous angioma. Igaku No Ayumi 104: , Numaguchi Y, Kitamura K, Fukui M, et al: Intracranial venous angiomas. Surg Neurol 18: , Olson E, Gilmor RL, Richmond B: Cerebral venous angiomas. Radiology 151:97-104, Pak H, Patel SC, Malik GM, et al: Successful evacuation of a pontine hematoma secondary to rupture of a venous angioma. Surg Neurol 15: , Pardatscher K, Fiore DL, Galligioni F, et al: Diagnosis of cerebral venous angioma by rapidly enhanced CT scan. Surg Neurol 14:l , Partain CL, Guinto FC, Scatliff JH, et al: Cerebral venous angioma: correlation of radionuclide brain scan, transmission computed tomography and angiography. J Nucl Med 20: , Preissig RS, Preissig SH, Goree JA: Angiographic demonstration of a cerebral venous angioma. Case report. J Neurosurg 44: , Rigamonti D, Drayer BP, Johnson PC, et al: The MRI appearance of cavernous malformations (angiomas). J Neurosurg 67: , 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: , Rigamonti D, Spetzler RF, Drayer BP, et al: Appearance of venous malformations on magnetic resonance imaging. J Neurosurg 69: , Rothfus WE, Albright LA, Casey KF, et al: Cerebellar venous angioma: "benign" entity? AJNR 5:61-66, Russell DS, Rubinstein LJ: Pathology of Tumours of the Nervous System, ed 4. Baltimore: Williams & Wilkins, 1977, pp Sadeh M, Shacked I, Rappaport ZH, et al: Surgical extirpation of a venous angioma of the medulla oblongata simulating multiple sclerosis. Surg Neurol 17: , Saito Y, Kobayashi N: Cerebral venous angiomas: clinical evaluation and possible etiology. Radiology 139:87-94, Sarwar M, McCormick WF: Intracerebral venous angioma: case report and review. Arch Neurol 35: , Scott JA, Augustyn GT, Gilmor RL, et al: Magnetic resonance imaging of a venous angioma. AJNR 6: , Scotti LN, Goldman RL, Rao GR, et al: Cerebral venous angioma. Neuroradiology 9: , Senegor M, Dohrmann G J, Wollman RL: Venous angiomas of the posterior fossa should be considered as anomalous venous drainage. Surg Neurol 19:26-32, Solomon EH, Bonstelle CT, Modic MT, et al: Angiographic and computed tomographic correlation in cerebral venous angiomas. CT 4: , Sordet D, Beroud P, Pharaboz CH, et al: Angiome veineux c6r6bral. Aspects angiographique et tomodensitom&- rique. Apropos d'une observation. J Radiol 67: , Suganuma Y, Oie K, Tanigawa K, et al: [A case of cerebral venous angioma.] Neurol Surg 6:77-83, 1978 (Jpn) 56. Takamiyay H, Kobayashi K, Mine T, et al: Familial occurrence of multiple vascular malformations of the brain. Neurol Med Chir 24: , Valavanis A, Wellauer J, Ya~,argil MG: The radiological diagnosis of cerebral venous angioma: cerebral angiography and computed tomography. Neuroradioiogy 24: , Wendling LR, Moore JS Jr, Kieffer SA, et al" Intracerebral venous angioma. Radiology 119: , Wilkins RH: Natural history of intracranial vascular malformations: a review. Neurosurgery 16: , Wolf A, Brock S: The pathology of cerebral angiomas. A study of nine cases. Bull Neurol Instit NY 4: , Wolf PA, Rosman NP, New PFJ: Multiple small cryptic venous angiomas of the brain mimicking cerebral metastasis. A clinical, pathological, and angiographic study. Neurology 17: , Yamasaki T, Handa H, Yamashita J, et al: Intracranial cavernous angioma angiographicauy mimicking venous angioma in an infant. Surg Neurol 22: , Zfilch K J: Brain Tumors: Their Biology and Pathology, ed 2. New York: Springer-Verlag, 1965 Manuscript received November 15, Accepted in final form March 5, Address reprint requests to: Daniele Rigamonti, M.D., Division of Neurosurgery, University of Maryland Hospital, 22 South Greene Street, Baltimore, Maryland J. Neurosurg. / Volume 73 / October, 1990