Associated Cerebral And Spinal AVM in Infant and Adult

Summary

We report two cases of a rare association of brain and spinal arteriovenous malformation (AVM) in an infant and in an adult. Both patients were embolised with glue with good clinical and morphological results.

The first patient is an infant with a suspected family history of Rendu Osier Weber disease with multiple AVMs : two hemispheric cerebellar lesions (one of them revealed by a ventricular haemorrhage) and one cervical spinal cord AVM (SCAVM). The associated SCAVM fortuitously bled one month after treatment of the symptomatic cerebellar AV shunt.

In the adult case, the management first involved a symptomatic SCAVM with favourable outcome; the cerebral AVM bled secondarily and was then excluded by endovascular approach.

Introduction

Multiple cerebral arteriovenous malformations (MAVM) are rare both in pathological and clinical series ¹. The co-existence of brain AVM (BAVM) with spinal cord AVM (SCAVM) is even rarer^2-11. A high incidence of multifocal arteriovenous malformations of the central nervous system has been reported in patients with Rendu-Osler-Weber disease (ROW)¹² and yet their association at the cord and the brain is exceptional^3,7. The association of multiple related lesions results in complex therapeutic decision-making, which should be handled on a case by case basis. In addition, the treatment of symptomatic lesions often remains challenging if presenting during infancy. Moreover, the sequence of treatment is difficult, often partial, or palliative and the therapeutic strategy usually targets the symptomatic lesion¹³.

We report two cases of BAVM associated with SCAVM; our purpose is to illustrate the clinical situations and therapeutic challenges encountered in infant and adult cases.

Case Report

Case 1

A 1 year old boy, weighing 8 kg, with a suspected family history of ROW disease (recurrent epistaxis) was admitted in March 1999 for abrupt loss of consciousness. CT showed right hemispheric cerebellar and intraventricular haemorrhage (figure 1A). MR confirmed the diagnosis but also disclosed flow-void structures in the right cerebellum and in the upper cervical spinal cord. Angiography diagnosed three associated AVMs: two in the right cerebellar hemisphere and one SCAVM at the C4 level fed by a pial coronal branch of the anterior spinal artery. The main cerebellar arteriovenous shunt (AVS) was supplied by distal hemispheric branches of the ipsilateral anteroinferior cerebellar artery (AICA). It harbouredcongestive venous drainage in the posterior fossa with ectasias, one of them corresponding to a false aneurysm (figure 1B). We thought that this lesion had bled because of the angioarchitectural data and the location of haemorrhage. Because of concern about the risk of rebleeding in relation to the false aneurysm in the posterior fossa, this lesion was embolised with n-butyl cyanoacrylate (NBCA, Histoacryl*) during the same session. Satisfactory disconnection was achieved with eradication of the weakness factors and venous decongestion.

Figure 1.

Patient 1. A) Non enhanced axial CT scan showing a small cerebellar haematoma and intraventricular haemorrhage in the fourth ventricle. B) Right vertebral angiography, frontal view, disclosing multiple AVMs located in the right cerebellar hemisphere and at C2-C3 level. C) MR T2 WI, sagittal view. Note the haematomyelia at C4-C5 level. The cervical lesion was approached through a contributor to the anterior spinal axis originating from the ascending cervical artery, and the pial feeder to the lesion was superselectively catheterized (D). The AVM was then embolised with glue (E) and totally disconnected, as seen on the lateral view of the ascending cervical artery and anterior spinal axis (F).

The procedure was then stopped because we had reached the limits of the femoral artery puncture time and the amount of contrast allowed (6 cc/kg) in such an infant. A new appointment was scheduled for May 1999; at the time of discharge, the baby had recovered clinically.

One month later, however, the child presented with acute tetraplegia. MR (figure 1C) revealed cervical haematomyelia. Angiography confirmed a modification in the architecture of the SCAVM and showed an associated pseudoaneurysm. Because of this abrupt episode and this weak architectural point, an attempt to control the lesion by endovascular approach was decided. Surgical evacuation of the haematoma was not considered.

The coronal branch of the anterior spinal artery was selectively catheterized and the lesion embolised with NBCA and excluded (figures 1D, 1E). During the same session, the residual part of the previously described cerebellar AVM was embolised.

Following endovascular treatment and subsequent administration of steroids the paresis improved within one month, with almost normal strength on the upper limbs but persistent sphincter dysfunction and paraparesis. The 12-month follow-up showed minimal clinical changes with only slight improvement of the neurological deficits.

Case 2

A young patient developed old a progressive right Brown-Sequard syndrome with associated right lower limb atrophy when she was two years. Angiography had confirmed a SCAVM supplied by bilateral radiculopial arteries at LI and a right radiculopial artery at L3. Two sessions of embolisation with particles had been performed in an another institution, without any clinical changes or disconnection of the lesion.

In 1991, at the age of 11, orthopedic corrective surgery of the atrophic right lower limb was undertaken. During the physiotherapy that followed the procedure, she developed headaches, disorientation, and a mild left-sided deficit. CT showed a right parietal haematoma. Angiography revealed an AVM of the right post central gyrus supplied by distal parietal branches of the ipsilateral pericallosal artery. The patient recovered clinically with conservative treatment. No treatment was attempted at that time as the lesion was considered to be located in an eloquent area.

In March 1999, the patient, being 20 years old, was referred to our group for re-evaluation of her malformations. It was felt at that time that both lesions could be safely embolised : total eradication of the BAVM with NBCA was performed in two sessions without complications (figures 2A, 2B). The SCAVM was also cured during one additional therapeutic procedure (figures 2C-E). The clinical outcome was good without complications, the patient remaining neurologically stable.

Figure 2.

Patient 2. A) Right carotid angiogram, frontal view, before treatment of the right post-central gyrus AVM. B) After completion of endovascular treatment, no residual AVM is detected. C) Right L3 injection demonstrates the SCAVM of fistula type. Other radiculopial arteries were detected, all converging towards the same fistulous point (not shown). D) Angiogram after embolisation with NBCA through one feeder, occluding the fistula, showing the cure of the lesion.

Discussion

There are classically significant angio-architectural and topographical differences in AVMs between adults and young children (e.g. multifocal lesions, high flow lesions). Thus, infants often require more risk/ benefit evaluation, a different therapeutic decision process and frequently urgent management ¹⁴. In MAVM, endovascular approach is a good therapeutic modality ¹⁵, and its combination with surgery may also a yield satisfactory outcome ¹⁶. In both cases presented here, the embolisations achieved good clinical and morphological results. In the infant patient, we obtained a favorable outcome for the cerebellar AVM, unlike those described in the literature¹⁷. In multiple lesions, targetted therapy towards the symptomatic AVM and towards the architectural features of that shunt, deemed responsible for the symptoms, is mandatory. This is why the main cerebellar AVM was treated first, due to the initial clinical presentation and angioarchitectural weakness factors (ectatic venous drainage and venous-pseudo aneurysm). Venous drainage abnormalities are mainly responsible for haemorrhage, which is the prevalent clinical onset of the different AVMs of the patients^14,18 and has a poor prognosis in a cerebellar location.

The haematomyelia associated with acute tetraplegia observed in the same patient is a potential complication of any SCAVM. In this infant, surgical management was considered to have a high morbidity. Endovascular treatment was decided to exclude the pseudo-aneurysm that may have been responsible for recurrent haemorrhage ¹¹. The superselectivity achieved by catheterization showed that the lesion was in fact a fistula of simple architecture, draining into congested extrinsic and intrinsic veins. Cure of the lesion could therefore be easily obtained. Improvement of the symptomatology noted in our case and also described in other patients ¹⁹ allowed us to avoid surgical treatment.

In the adult patient, a stepwise progressive treatment was decided because of the eloquent territory in which the brain AVM was located and the absence of any angioarchitectural weakness factor. On the other hand, the cord AVM had already created a fixed deficit and emergent aggressive treatment was not warranted in this case. Treatment had to be balanced between the possible complications of embolisation² and its advantages with stabilization and /or improvement of the clinical situation. We preferentially use NBCA for embolisation of any arteriovenous malformation, including SCAVMs. Particles usually fail to exclude the lesion (as occured in our case). Moreover, it is our experience that particles in most cases often make future embolisation technically impossible or hazardous²; they allow recanalization and collateralization and may transform a reachable lesion into an unreachable one.

The co-existence of BAVM and SCAVM is extremely rare ^3-11. Hasegawa reported only eight cases from the literature^3-8,11 of association of these two entities. However, as far as multiple brain AVMs are concerned, Iizuka¹³ reported in the pediatric population that 20% of patients had multiple AVMs. In this situation, ROW should be suspected a priori, although arteriovenous shunts in this population associated with ROW are more often high flow macrofistulas with giant venous ectasias. Epistaxis and mucocutaneous telangectasias are unusual in children and the diagnosis of ROW has to be based on the family history and on the angiographical aspect of the shunts. Furthermore, multiple lesions seen in ROW involve various metameric territories. If one estimates to 10% the ratio SCAVM / BAVM the frequency of multiple BAVM-SCAVM is 1.3% in our SCAVM series and 0.13% in BAVMs. In none of these cases was ROW found.

Deciding the timing of treatment in the presence of MAVMs is a major challenge. The goal of the therapeutic procedure (total or partial targetted treatment) remains to prevent secondary morbidity from the disease. In any isolated AVM, the therapeutic decision is based on a postulated natural history and a careful assessment of the technical related morbidity. The difficult prediction of the natural history of MAVMs, whether located in the brain, spinal cord or in both territories, makes the decision more difficult. In the first case, the rapid consecutive bleeding of the associated SCAVM (the bleeding occurred one month before the planned session) is very rare and unpredictable. We had never faced before an early haemorrhage from another lesion during our management of any MAVM case. Tsurushima had described a similar case of an embolised SCAVM followed by bleeding of an associated BAVM ¹¹. The biological events leading to rupture may have applied to both lesions with different timing response, as may hypothetically occur in MAVMs, a specific group of AVMs described to have usually a metameric distribution²⁰. One could speculate that endothelial cells of MAVMs have often been involved in a metameric organization, and that they then can be submitted, because of their common origin, to a metameric biology leading to rupture.