Hydrocephalus in unruptured brain arteriovenous malformations: pathomechanical considerations, therapeutic implications, and clinical course

Clinical article

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Object

The goal in this study was to present possible pathological mechanisms, clinical and imaging findings, and to describe the management and outcome in patients with hydrocephalus due to unruptured pial brain arteriovenous malformations (AVMs).

Methods

Medical records and imaging findings in 8 consecutive patients with hydrocephalus caused by AVMs and treated between June 2000 and September 2007 were retrospectively reviewed to determine clinical symptoms, AVM location, venous drainage, level/cause of obstruction, and degree of hydrocephalus. Management of hydrocephalus, AVM treatment, complications, and follow-up results were evaluated.

Results

Headaches were the most common clinical symptom (7 of 8 patients). Deep venous drainage was identified in all patients. Mechanical obstruction by the draining vein or the AVM nidus was seen in 6 patients, in whom obstruction occurred at the interventricular foramen (2 patients) or the aqueduct (4 patients). Hydrodynamic disorders following venous outflow obstruction and venous congestion of the posterior fossa led to hydrocephalus in the remaining 2 patients. Ventriculoperitoneal (VP) shunts were placed in 6 of 8 patients with a moderate to severe degree of hydrocephalus. Regression of hydrocephalus was noted in 4 patients, whereas in 2 the imaging findings were stable, 1 of whom had decreased hydrocephalus only after AVM size reduction. In 2 patients with mild hydrocephalus who were not treated with shunt insertion, 1 improved and 1 was clinically stable after AVM treatment.

Conclusions

The most common cause of hydrocephalus in unruptured brain AVMs is mechanical obstruction by the draining vein if it is located in a strategic position. Management should be aimed at treatment of the AVM; however, VP shunts may be necessary in acute and severe cases of hydrocephalus.

Abbreviations used in this paper:AVM = arteriovenous malformation; CSF = cerebrospinal fluid; ICV = internal cerebral vein; SDH = subdural hematoma; VP = ventriculoperitoneal.

Article Information

Address correspondence to: Prof. Dr. T. Krings, Department of Neuroradiology, University Hospital Aachen, Pauwelsstrasse 30, 52057 Aachen, Germany. email: tkrings@ukaachen.de.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. This 16-year-old girl presented with headaches that had been slowly progressive for 7 months. Plain axial CT scans obtained before (A and B) and after (C and D) VP shunt insertion revealed severe hydrocephalus with periventricular CSF resorption, which remained unchanged after VP shunt placement. Abnormal vessels were noted in the right thalamic region, with an enlarged right lateral perimesencephalic vein draining into a dilated vein of Galen that caused obstruction of the aqueduct due to pressure on the tectal plate (left vertebral artery angiogram, anteroposterior view [E] showing the AVM nidus, which is supplied by thalamoperforating and right posterior choroidal arteries). Embolization of the posterior choroidal feeding vessels (including the venous pouch—seen disappearing on the postembolization angiogram [F]) was performed to reduce the shunt and the size of the AVM, to make the AVM suitable for radiosurgery. Following embolization the patient had clinical improvement of her headaches at follow-up. She is scheduled for radiosurgery.

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    Case 3. This 35-year-old man presented with severe progressive headaches over the course of the preceding 2 months. Neuroimaging revealed a large right temporoparietal AVM that primarily drained into the right ICV. A VP shunt was placed, after which the hydrocephalus did not improve. In the upper row, images obtained 5 months after shunt placement and prior to radiotherapy are shown, whereas in the lower row, images obtained after radiotherapy of the AVM demonstrate reduction of the lesion's nidus, decreased size of the draining vein, reduction of the size of the ventricles, reduction of the transependymal CSF reabsorption, and a decrease of the ballooning of the third ventricle.

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    Case 4. This 39-year-old woman presented with headaches that had been gradually progressive for 4 months, with new onset of seizures since 3 months before admission. On a T2-weighted MR image (A), abnormal vessels causing obstruction of the right foramen of Monro with unilateral ventricular dilation were demonstrated. An internal carotid artery angiogram (B) revealed an AVM at the right frontocallosal region with dominant deep venous drainage toward the right thalamostriate vein, with a venous pouch at the junction to the ICV. After transarterial glue embolization and partial obliteration of the shunt along with diminution of the AVM size, clinical and imaging improvement of the hydrocephalus was noted, with the patient being free of headaches. The CT scans obtained after embolization (C and D) showed resolution of the hydrocephalus without placement of a VP shunt. The patient underwent further radiosurgery for her residual AVM.

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    Case 7. This 55-year-old man presented with headaches that had been progressive for 3 months, and sudden worsening of the symptoms, along with vomiting. A contrast-enhanced CT scan (A) showed abnormal vessels at the left cerebellum, with moderate hydrocephalus and generalized brain swelling. Prior to further diagnostic workup, a VP shunt was placed at another institution. After referral to our institution 2 weeks later, the hydrocephalus was still present. Left vertebral artery angiograms in anteroposterior view (B) with a late venous phase in lateral view (C) confirmed the diagnosis of an AVM at the left cerebellum supplied by branches of the left anterior and posterior inferior cerebellar arteries, and draining into the cerebellar veins, with marked stagnation and posterior fossa venous congestion. Nonopacification of the straight sinus is noted, with filling of collateral pathways into the basal vein of Rosenthal. After 2 sessions of transarterial glue embolization that decreased the AVM size by 80%, the patient's symptoms improved, and he is scheduled for further treatment of the remainder of the AVM. An MR image (D) obtained after the last embolization confirmed reduction of the hydrocephalus, and angiograms (E and F) confirmed the reduction in venous congestion of the posterior fossa.

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