Georges Rodesch

Cerebral arteriovenous malformations in children - Management of 179 consecutive cases and review of the literature

Over the past 10 years (1982–1992), we have been actively involved in the management of 179 cerebral arteriovenous malformations (CAVMs) in children and infants. Seventy-seven were true vein of Galen malformations (VGAMs) and 102 were pial AVMs (PAVMs), i.e., developed in the subpial space. Hemorrhage occurred as the first symptom in 50% of the children with pial AVMs, but was present in none of the VGAM cases. Only 31 children were found to be unsuitable for endovascular treatment, and in 124 cases embolization was indicated as the primary treatment (104 embolization performed). Only 21 children underwent a direct surgical approach (none in the VGAM group). In the embolized group in whom treatment has been completed (n = 56), 8 children died, 39 have an anatomical cure, and 34 are clinically normal. In the group under treatment (n = 56), 16 are not normal. The problems are timing and the aims (total or partial treatment) of the therapeutic procedures. In the nonembolized group (n = 31), 8/13 of the pial lesions were operated on (no mortality, 2 patients with moderate neurological deficits). In the VGAM group 13/18 died and 4 had spontaneous thrombosis (only 1 is neurologically normal). In the nonembolized group 13 lesions have been completely excluded, but only 5 patients are neurologically normal. This fact again stresses the need for prognostic evaluation before treatment and a clear definition of the treatment aims. Analysis of a large number of published series on the management of children with AVMs (1017 cases) reveals inconsistencies that hamper proper evaluation and comparison. In our experience, endovascular treatment always seems to be the best primary treatment in both VGAMs and PAVMs. However, management of children with these lesions requires a large multidisciplinary team, which is the only way of offering the most suitable and effective treatment, the sole guarantee of a good result.

Classification of spinal cord arteriovenous shunts: Proposal for a reappraisal - The Bicêtre experience with 155 consecutive patients treated between 1981 and 1999

OBJECTIVE Spinal cord arteriovenous shunts (SCAVSs) are currently classified according to their morphological features. Certain shunts cannot be fully integrated into the predetermined categories that are usually described, however. Can these classifications be reevaluated on the basis of recent anatomic, biological, and genetic advances? METHODS We reviewed the clinical and radiological files for 155 SCAVSs that were treated at Hôpital Bicêtre between 1981 and 1999. The lesions were examined with respect to their number (single or multiple), their primary architectural type (nidus or fistula), and their possible links with associated metameric lesions. RESULTS All SCAVSs were either arteriovenous malformations or fistulae, with the latter being either micro- or macrofistulae. All SCAVSs corresponded to three categories, i.e., genetic hereditary lesions (macrofistulae and hereditary hemorrhagic telangiectasia), genetic nonhereditary lesions (all of which were multiple lesions with metameric or myelomeric associations), and single lesions (which could represent incomplete presentations of one of the previous groups). Of the SCAVSs in our series, 81% were single lesions and 19% were multiple; among these, 59% were true intradural shunts with metameric features. Ten cases of Cobb syndrome, three cases of Klippel-Trenaunay syndrome, and two cases of Parkes-Weber syndrome, all with associated cord lesions, were observed. Nineteen percent of SCAVSs were fistulae; 23% of those were macrofistulae, of which 83% were related to Rendu-Osler-Weber disease. CONCLUSION It seems legitimate to propose a categorization that takes into consideration a primary malformation (nidus or fistula) that evolves with time and in which angioarchitectural changes occur. Recognition of the factors originally responsible for the shunt (e.g., genetic hereditary or genetic nonhereditary) allows a different classification of SCAVSs.

Transient Cerebral Arteriopathy: A Disorder Recognized by Serial Angiograms in Children With Stroke

Repeated clinical evaluation and cerebral arteriography during the evolution of ischemic strokes of idiopathic origin allowed us to characterize a transient cerebral arteriopathy. We retrospectively studied the clinical characteristics, course, and neuroimaging features of this disorder in nine children. Of 34 children with ischemic strokes seen consecutively between 1984 and 1995, 9 (26%) were diagnosed as having transient attack of the cerebral arterial wall, termed transient cerebral arteriopathy. All of these patients had previously been in good health. The mean age at the time of the first stroke was 6 years (range, 29/12 years to 134/12 years). All children presented with acute hemiplegia. A recurrence of the stroke took place 3 months at the latest after the initial infarct in three children (mean clinical follow-up 27/12 years). Cerebral imaging in all the patients showed small subcortical infarcts located in basal ganglia or internal capsule. Arteriography revealed multifocal lesions of the arterial wall (focal stenosis or segmental narrowing), mostly located in the initial parts of basal arteries of the carotid system. Longitudinal arteriographic follow-up showed initial worsening of these arterial lesions (n = 5) for a maximum duration of 7 months followed by complete regression (n = 2), improvement (n = 5), or stabilization of the lesions (n = 2). Five patients had a complete clinical recovery. Further studies are necessary to confirm a presumed inflammatory cause of this arteriopathy. (J Child Neurol 1998; 13:27-32).

Effect of partial targeted N-butyl-cyano-acrylate embolization in brain AVM.

Summary. Background: The management of cerebral arteriovenous malformations needs effective treatments. So far, no study has shown that partial targeted embolization treatment (PTET) reduces the risk of intracranial hemorrhage with respect to the natural history of the malformation. Methods: The pre-treatment and post-treatment-initialization hemorrhage incidences of neuro-interventional patients were compared. Two hundred fifteen patient years from 519 patients were used to observe the short term course of the untreated disease. Five hundred patient years from 326 patients were used to observe hemorrhage after the start of treatment. The Kaplan-Meier estimator of hemorrhage free time under treatment was compared with results in the literature. Confounding influences resulting from selection processes or the disease parameters were studied. Results: The yearly hemorrhage incidence rate of all untreated patients was observed as 0.089 (95% CI [0.053, 0.138]). This rate was 0.052 (95% CI [0.019, 0.114]) in the subgroup of patients who underwent PTET later. In the same group the observed annual rate after the start of PTET was 0.036 (95% CI [0.021, 0.057]). Crawfords results about intracranial hemorrhage during the natural course show the lowest risk values compared to other published studies [3]. There was a significant difference between the Crawfords reference data and the ICH incidence after the start of PTET in the neuro-interventional population (p=0.037). The morbidity risk in treated patients was 5.3% for a transitory and 2% for a persisting neurological deficit. Mortality results were compared with those of Crawford. Conclusion: The neuro-interventional patients under study show a lower hemorrhage risk than the population studied by Crawford. A significant superiority with respect to hemorrhage risk is established two years after the start of the PTET treatment.

Spinal cord intradural arteriovenous fistulae: Anatomic, clinical, and therapeutic considerations in a series of 32 consecutive patients seen between 1981 and 2000 with emphasis on endovascular therapy

OBJECTIVE:To review our series of intradural spinal cord arteriovenous fistulas (SCAVFs), analyzing symptoms and related angioarchitecture, and to study the morphological and clinical results of embolization. METHODS:Thirty-two SCAVFs (in 22 adults and 10 children) were treated between 1981 and 2000. These lesions were classified as microarteriovenous fistulas (mAVFs) or macroarteriovenous fistulas (MAVFs) according to shunt morphology. Location, architecture, presenting symptoms, and age group were detailed. The selection of patients for endovascular versus surgical treatments was analyzed, as were the anatomic and clinical results obtained by embolization with n-butylcyanoacrylate. Clinical status was evaluated according to the Karnofsky Performance Scale score. RESULTS:Ten SCAVFs were found in the pediatric population (four mAVFs and six MAVFs). All four mAVFs presented with acute symptoms. Three mAVFs (two cervical and one thoracic) presented hematomyelia; in one patient with a thoracic AVF, subarachnoid hemorrhage was suspected. All six MAVFs were located in the thoracolumbar cord (five associated with hereditary hemorrhagic telangiectasias). Four of the six MAVFs presented with hemorrhage. In the adult population, there were 21 mAVFS (95%) and one MAVF (5%). Only two mAVFs were found in the cervical cord, all other shunts affecting the thoracolumbar region. Hemorrhage was present in 6 of the 22 cases seen in adults (27%). The symptoms of SCAVFs did not differ from those found in spinal cord arteriovenous shunts of nidus type. Pial venous reflux and congestion were the most frequently encountered features in both the adult and pediatric groups. Arterial aneurysms (different from false aneurysms) were not found in association with hemorrhagic presentation of SCAVFs. Mean follow-up in our series was 3.3 years. Of the MAVFs, 86% were embolized, with 67% cured. The others had more than 75% occlusion. All patients followed up improved significantly. Of the mAVFs, 48% were treated endovascularly. Successful embolization was performed in 75% of patients. One patient was not embolized because of vasospasm, whereas 67% percent of mAVFs were completely occluded, 22% were more than 90% occluded, and 11% were 75% occluded. Complementary surgery was deemed unnecessary. All patients with mAVFs improved significantly at follow-up. Transient complications occurred in 22% of all patients, with no permanent morbidity or mortality. No patient bled or rebled after embolization. Thirty-six percent of mAVFs were operated on because of anticipated technical difficulties for endovascular approach or distal localization of the shunt. CONCLUSION:Endovascular treatment of SCAVFs stabilizes, normalizes, or improves neurological symptoms in all patients at long-term follow-up, with no bleeds or rebleeds. Embolization of SCAVFs with glue is a safe treatment that compares favorably with other approaches and significantly improves the poor natural history of the disease.

Prognosis of antenatally diagnosed vein of Galen aneurysmal malformations

In our experience of 168 consecutive cerebral arteriovenous shunts, all antenatally diagnosed lesions were vein of Galen aneurysmal malformations (VGAMs). This series consists of 18 cases of VGAMs detected by ultrasound during the third trimester of pregnancy. There were 12 normal vaginal deliveries, 5 deliveries by cesarean section, and 1 induced abortion. Sixteen newborns (94%) presented with systemic cardiac manifestations as the first clinical symptoms; 12/16 were managed effectively by digitalo-diuretic treatment, while 4 (25%) died shortly after birth form acute heart and/or multiorgan failure with extensive brain damage. Twelve babies underwent embolization via the arterial route in infancy (2 at 2 months of age). Total exclusion was obtained in 8 babies (67%, 3 with 6 months follow-up). Furthermore, 67% of the newborns managed by our team are neurologically normal (Denver and Brunet-Leizine tests). These results emphasize that the pessimism that follows antenatal discovery of these lesions and the previous assumption of a bad prognosis for VGAMs can nowadays be reviewed in the light of transarterial endovascular therapy applied according to a strict clinicoradiological protocol. Interventions in the neonatal period are rarely required. Generally, poorly timed mechanical therapy should be discouraged.

Hydrodynamics in vein of Galen malformations

Forty-three patients with vein of Galen aneurysmal malformations (VGAM) referred to us for endovascular treatment between 1985 and 1990 and 335 additional cases published in the literature were reviewed with particular attention to the presence of ventricular enlargement and outcome after shunting. Hydrocephalus was the second most frequent symptom (46.8%); it is more frequent in infants (73%) than in children, adults (30%) or neonates (15%). Of the patients reported in the literature, 17.9% had undergone shunting. Within the shunted population there was an overall morbidity of 41% and a mortality of 10% (especially in the infant group). In our series 17 patients (39.5%) were shunted and a significant difference in the clinical outcome was noted between the shunted and the nonshunted group. Of the nonshunted patients, 66.6% were free of any neurological deficit or mental retardation and fewer than 5% presented with significant mental retardation. On the other hand, only 33.3% of the shunted patients had a favorable outcome and more than 15% developed significant mental retardation. Among the various causes of hydrocephalus in patients with VGAMs, such as obstruction of the aqueduct, subarachnoid hemorrhage, or ex vacuo hydrocephalus, high venous pressure may be of particular importance. In this article a physiopathological interpretation of the hydrodynamics in VGAMs is developed and a speculative explanation for CSF disorders related to ventricular shunting proposed. Treatment of hydrocephalus in VGAMs can be achieved through obliteration of the malformation or at least diminishing the venous pressure; surgical ventricular shunting does not have to be the first treatment of hydrodynamic disorders associated with VGAMs, especially in infants.

Supratentorial cerebral arteriovenous fistulas (AVFs) in children: review of 41 cases with 63 non choroidal single-hole AVFs

SummaryPurpose. In this article we explore the various aspects of the supratentorial Single-Hole AVFs (ST AVFs) in children, focusing on their clinical features, angio-architecture, treatment indications and the role and results of endovascular management.Materials and methods. Among 1565 cases of brain AVMs seen at our neurovascular center, 620 cases were seen in the pediatric age group (≤ to 15 year-old) and 303/620 were non-Galenic brain AVMs. Among 52 brain AVFs found in children, we reviewed 41 pediatric patients with supratentorial single-hole cerebral AVFs (M/F=28/13) with a total of 63 AVFs. Mean age at presentation was 24 months. Four cases had their lesion diagnosed in utero without particular prognostic significance. 17 cases were multifocal with a total of 39 AVFs. The most common clinical presentation leading to diagnosis was cardiac sufficiency in (31.7%), epilepsy (24.4%), macrocrania (14.6%). Venous ectasia (87.8%) and pial venous stenosis (41.5%) were the most frequent angiographic features. There were 11 cases of Hemorrhagic Heriditary Telangiectasia (HHT1) (including two suspicious cases) among the 41 patients (26.8%) and among those 4 cases had single AVFs and 7 cases had multiple AVFs.Results. Thirty-five children were treated in our institution: 34/35 by embolization alone, 1/35 by a combination of embolization and radiosurgery. 32/35 patients were treated with glue alone, 3/35 with coils (2/3 with glue also). A total of 57 separate lesions were treated. On clinical follow up (from 1985–2002, mean 4.2 years), 88.6% of patients proved to be either asymptomatic, improved from previous clinical symptoms or stabilized. 40% of patients had their lesion(s) already completely excluded.Post operative mortality was 5.6% (2/35), permanent neurological morbidity 3% (1/33). Twenty-six of these 33 patients (78.6%) were neurologically normal. 5/33 patients continue to have neurological symptoms or retardation. 2/33 patients died during follow up (1 ischemic stroke from Pulmonary AVF, 1 death despite partial embolisation).Conclusion. Cerebral arteriovenous fistulae are a rare disease, but not infrequently seen in neonates and infants with AVMs. In one fourth of these patients HHT is suspected to be present. The AVFs are always superficial and fed by pial (cortical) arteries. They seldom reveal a hemorrhagic event. They are similar to those encountered in the posterior fossa or spinal cord. Endovascular treatment using NBCA was the treatment modality chosen resulting in a high rate of success and allowing children to grow up normally with no hemorrhages on follow up and no new symptom other than those already present on admission.

Magnetic Resonance Angiography in Childhood Arterial Brain Infarcts A Comparative Study With Contrast Angiography

Background and Purpose— Contrast angiography (CA) is the reference examination for the diagnosis of cerebral arterial abnormality, but this procedure is invasive. In childhood, ischemic strokes are being increasingly investigated by means of MRI, including MR angiography (MRA). Very few data are available about the accuracy of MRA compared with CA in the specific context of acute pediatric stroke. We sought to compare the results of MRA with those of CA for the study of cerebral arteries in children with arterial infarction in an arterial distribution. Methods— Twenty-four children presenting with 26 infarcts were studied. All were examined with cerebral MRI and MRA and with CA. The interval between CA and MRA was <3 days for most of the patients. Results— Arterial lesions were detected in all but 2 children. They were located in the major cerebral arteries, predominantly in the anterior circulation (85% of cases). All lesions shown by CA were present on MRA (19 cases). Patients with no lesion on MRA had normal CA (2 cases). Associated distal vascular lesions and degree of arterial stenosis were more accurately detected with CA. Conclusions— MRA is sensitive enough to provide an adequate initial evaluation of arterial brain disease in childhood.

Cerebral Proliferative Angiopathy Clinical and Angiographic Description of an Entity Different From Cerebral AVMs

Background and Purpose— The purpose of this article is to describe “cerebral proliferative angiopathy” (CPA) as a clinical entity, which may be regarded as separate from “classical” brain AVMs in angioarchitecture, natural history, clinical presentation, and, therefore, treatment and which can be discerned from other cerebral AVMs by characteristic imaging features. Methods— In a prospectively entered databank encompassing 1434 patients with brain AVMs, a subgroup of 49 patients harboring specific angiographic characteristics were identified. Their charts and imaging films were retrospectively reviewed. Results— We found a preponderance of CPA in young (mean age: 22) females (67%). Clinical symptoms were seizures, disabling headaches, and stroke-like symptoms; hemorrhagic presentations were exceptional. On cross-sectional imaging, CPA demonstrated as a diffuse network of densely enhancing vascular spaces with intermingled normal brain parenchyma. The discrepancy between the large size of the nidus and the small shunting volume, the absence of flow-related aneurysms, the presence of diffuse angiogenesis (eg, transdural supply, progressive arterial occlusion), and the small calibre of a multitude of feeding arteries and draining veins were the angiographic hallmarks of this disease. Conclusion— The diffuse angiogenetic activity is presumably related to reduced perinidal perfusion and subsequent chronic cortical ischemia. Natural history demonstrates a low risk of hemorrhage. CPA may be regarded as a separate clinical entity different to “classical” cerebral AVMs, because normal brain is interspersed with the abnormal vascular channels increasing the risk of neurological deficit in aggressive treatments, which in the light of the natural history does not seem to be indicated.