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 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  References

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Year : 2002  |  Volume : 50  |  Issue : 2  |  Page : 207-9

Midbrain venous angioma with obstructive hydrocephalus.


Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, 632 004, India.

Correspondence Address:
Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, 632 004, India.

  »  Abstract

A rare case of a mid brain venous angioma with obstructive hydrocephalus is described. A dilated draining vein from the lesion in the aqueduct as the cause of the hydrocephalus is highlighted, and interesting features of the pathology of venous angiomas and associated cavernous hemangioma are described. The management of this interesting condition is discussed.

How to cite this article:
Bannur U, Korah I, Chandy M J. Midbrain venous angioma with obstructive hydrocephalus. Neurol India 2002;50:207


How to cite this URL:
Bannur U, Korah I, Chandy M J. Midbrain venous angioma with obstructive hydrocephalus. Neurol India [serial online] 2002 [cited 2019 Aug 17];50:207. Available from: http://www.neurologyindia.com/text.asp?2002/50/2/207/1391




   »   Introduction Top

Although there have been a few references to venous angiomas in the brain stem, their occurrence in the midbrain with obstruction of the CSF pathway by a draining vein in the aqueduct is indeed rare. The pathology of these lesions make them unsuitable for surgical excision and therefore a CSF diversion procedure was carried out in the reported case with satisfactory result.


   »   Case report Top

An eleven year old boy was admitted to the neurosurgery ward with occipital headache of five months duration. Although the pain was dull and aching, there were two episodes of acute exacerbations. These exacerbations were associated with vomiting, vertigo and gait ataxia but they resolved on their own. There was no family history of neurological illnesses. On examination, the patient was normal on general physical review and neurological examination showed bilateral papilledema with no other positive findings.
Image studies : CT scan, plain and contrast examination showed a 'spoke wheel' shaped lesion with one large vessel running ventrally into the posterior third ventricular region. There was no evidence of hemorrhage within or around the lesion. The lateral and third ventricles were dilated and the fourth ventricle was normal.
MRI showed the 'caput medusa' within the mid brain, with multiple draining veins converging into a single vein, traversing the region of the aqueduct. This vein drained into subependymal veins and eventually into a dilated vein of Galen [Figure - 1] and [Figure - 2].
Absence of CSF dynamic flow signals across the aqueduct confirmed the site of obstruction due to the dilated vein. The above findings were diagnostic of a venous angioma of the mid brain. Interestingly, there was no associated cavernous hemangioma. A CSF diversion procedure was then performed successfully.


   »   Discussion Top

A venous angioma in the mid brain is rare and review of literature showed that the majority of these lesions were in other areas of the brain stem [Table I]. Before the advent of CT and MRI, there have been postmortem reports of venous angiomas and a high incidence of vascular malformation upto 63%,[1] and in a series of 168 posterior fossa vascular malformations, 11 venous angiomas were found in the brain stem.[2] Earlier workers have made special mention of the angiographic features, especially of the venous system, in patients with venous angiomas.
Venous angiomas were considered to be hamartomas by some some workers.[3] Angiography, in patients with posterior fossa venous angiomas, showed absence of the normal venous anatomy. The drainage territories of the 'missing veins' were in fact drained by the radial veins of the angioma itself.[4],[5],[1] The concept of anomalous venous drainage in these cases was however learnt after catastropic results following excision of a cerebellar venous angioma.[1] This concept developed the hypothesis that these angiomas are part of an anomalous venous drainage of otherwise normal brain tissue and that these lesions are best left alone since attempted excision may lead to venous infarction and or brain edema.
The spectrum of clinical presentation in patients with venous angiomas are varied even though the majority are incidentally found at autopsy or frequently seen on an MR study, more often than not in association with cavernous hemangioma. They rarely present as an ictus with subarachnoid or parenchymal hemorrhage, unless associated with cavernous hemangioma or as a progressive or fluctuating neurologic deterioration. The fluctuating course might simulate multiple sclerosis.[6] The features of raised intracranial pressure and hydrocephalus have been described and have been ascribed to the marked dilation of the subependymal veins and consequent obstruction of the aqueduct.[7],[8],[9] Craig and coworkers described a venous angioma in the basal ganglia with a draining vein adjacent to the aqueduct causing compression and obstruction to the CSF pathways.[10] MR studies in the present case demonstrated CSF obstruction at the aqueduct due to a dilated subependymal vein. This is similar to the earlier observations of dilated subependymal veins providing an alternative venous channel in cerebellar venous angiomas.[11]
MR scanning is crucial to the diagnosis and subsequent management of venous angiomas. It is also useful in the assessment of hydrocepahalus and in the evaluation of other associated anomalies such as cavernomas and occasionally arteriovenous malformations.[12] The role of digital substraction angiography is limited and can at best, document the associated anomalous venous drainage. Obstructive hydrocephalus due to a mid brain venous angioma is indeed rare and direct operative intervention is best avoided since it may lead to disastrous consequences.

 

  »   References Top

1.Senegor M, Dohrman GJ, Wollman RL : Venous angiomas of the posterior fossa should be considered as anomalous venous drainage. Surg Neurol 1983; 19 : 26-32.   Back to cited text no. 1    
2.McCormick WF, Hardman JM, Boulter TR : Vascular malformations ('angiomas') of the brain, with special reference to those occurring in the posterior fossa. J Neurosurg 1968; 28 : 241-251.   Back to cited text no. 2    
3.Lasjaunias P, Burrows P, Planet C : Development of venous anomalies(DVA), the so called venous angioma. Neurosurg Rev 1986; 9 : 233-244.   Back to cited text no. 3    
4.Courville CB : Morphology of small vascular malformations of the brain with particular reference to the mechanism of their drainage. J Neuropathology Exp Neurol 1963; 22 : 274-284.   Back to cited text no. 4    
5.Saito Y, Kobayashi N : Cerebral venous angiomas. Radiology 1981; 139 : 87-94.   Back to cited text no. 5    
6.Sadeh M, Shaked I, Rappaport ZH et al : Surgical extirpation of a venous angioma of the medulla oblongata simulating multiple sclerosis. Surg Neurol 1982; 17 : 334-337.   Back to cited text no. 6    
7.Askenasy HM, Herzberger EE, Wijsenbeek HS : Hydrocephalus with vascular malformations of Brain. A preliminary report. Neurology, Minneap 1953; 3 : 213-220.   Back to cited text no. 7    
8.Avman N, Dincer C : Venous malformation of the aqueduct of Sylvius treated by interventriculostomy : 15 years follow up. Acta Neurochir 1980; 52 : 219-224.   Back to cited text no. 8    
9.Damiano TR, Truwit CL, Dowd CF et al : Posterior fossa venous angiomas with drainage through the brain stem. AJNR 1994; 15 : 643-652.   Back to cited text no. 9    
10.Craig B, Alexander M : Aqueduct compression from venous angioma - MR findings. AJNR 1996;17 : 458-460.   Back to cited text no. 10    
11.Gaulao A, Alverez H, Monaco RG : Venous anomalies and abnormalities of posterior fossa. Neuroradiology 1990; 31 : 476-482.   Back to cited text no. 11    
12.Rigamonti D, Spetzler RF : The association of venous and cavernous malformations. Report of four cases and discussion of pathophysiological,   Back to cited text no. 12    

 

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