Cerebral arteriovenous malformations in children: radiology assesment

Cerebral arteriovenous malformations in children: radiology assesment Poster No.: C-1588 Congress: ECR 2015 Type: Scientific Exhibit Authors: J. S. Gaete, A. Sanchez-Montanez Garcia-Carpintero, E. Vasquez, I. Delgado Alvarez, A. Tomasello, M. A. Lemus Rosales, A. Cortes Campos, A. L. Sánchez, M. Fernández Viñas; Barcelona/ES Keywords: Paediatric, Neuroradiology brain, Head and neck, CTAngiography, MR-Angiography, Catheter arteriography, Diagnostic procedure, Arteriovenous malformations DOI: 10.1594/ecr2015/C-1588 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myesr.org Page 1 of 21

Aims and objectives Intracranial arteriovenous shunts in children can be divided into pial arteriovenous malformations (AVMs) [fig.1], vein of Galen malformations, and arteriovenous fistulae (AVF), including single-hole fistulae. Dural AVF and dural sinus malformations are rare entities within this group. [1] Pial AVMs are the most common cerebrovascular lesions in children. The lesion is thought to develop between the third and eighth week of fetal life. They are usually congenital lesions that arise from the abnormal development of the arteriolar-capillary network that exists between the arterial and venous circulations. Only 18% of AVMs that become symptomatic do so before the age of 15 years. The mortality from hemorrhage in a child is 25% [2,3]. Nevertheless brain arteriovenous malformations in pediatric population is an unfrequent pathology, with a prevalence lesser than 1% [4]. Pial AVMs are rarely seen in the first year of life. They may present in infancy, but most present in the second and third decade [1]. If the hypothesis that they are congenital is correct, this pattern implies that they undergo postnatal angioarchitecture evolution leading to presentation when the child is older. Although presentation in the newborn is infrequent, AVMs may manifest as congestive heart failure (50%), followed by hemorrhage (37.5%). In infants, hemorrhage (30%) and macrocrania (27%) are more common than heart failure (23%) [1]. The most common presentation of an AVM in childhood is hemorrhage (75%), either intraparenchymal or intraventricular, followed by seizures, which constitute approximately 15% of the presenting population. AVMs are the first cause of hemorrhagic stroke in children (30%55%) and, as such, any child with spontaneous intracranial hemorrhage should be presumed to have an AVM until proved otherwise [5,6]. Associated aneurysms are found in about 16% of children with AVMs [7]. Page 2 of 21

The radiologist should know how to assess and look for specific features to report such as: the location of the AVM (rule out the involvement of an elocuent area), size of the nidus, and type of venous drainage to give a correct Spetzler-Martin classification score, because this is important to choose the best treatment for the patient. Also the radiologist should know the different diagnostic techniques to proceed further studies when an AVM is suspected to describe its specific features. Page 3 of 21

Images for this section: Fig. 1 Radiographics Page 4 of 21

Methods and materials We did a retrospective review of 9 pediatric patients diagnosed and treated in our center from 2006 to 2014. All of the patients went through unenhanced Computed Tomography (UCT), Angio Computed Tomography(CTA), Head Resonance Magnetic angiography(mr) study and Digital Subtraction Angiography (DSA). Not necessarily the studies were done in this order because every clinical situation was different and availabity of MR or DSA were not uniform all the time. Furthermore, neurological status demanded urgent surgery in some cases. Our CT studies were made in a 32 CT slice scanner. Our MR studies were performed in a 3Tesla MR. In our protocol the Time of Flight 3D weighted image and MR-Angiography using gadolinium were included. In specific situations, a functional MR was also done. Relevant clinical data and radiologic features were collected such as: gender, age, clinical presentation, initial Modified Rankin Scale Score, AVM location, nidus size, type of venous drainage, aneurysm pressence, supplying vessels, treatment performed (surgery, previous embolization), radiological AVM remove confirmation and neurological sequeale. Page 5 of 21

Images for this section: Fig. 1 Radiographics Page 6 of 21

Fig. 2 Up to Date Page 7 of 21

Results The mean age in our study was of 10 years old. Gender: 5 males and 4 females. Clinical features at the moment of diagnosis: - Loss of consciousness was reported in 3 patients. - Seizures in 1 patient. - Focal neurological deficit was present in 4 patients. - Headache in 5 patients. - Glasgow Scale Score: 15 in 5 patients; 14 in 2 patients; and 3 in 2 patients. At the initial UCT/CTA: Hemorrhage was present in 7 of 9 patients (77%) AVM location: Fronto-parietal in 5 patients; parietal in 4 patients. CTA detected the AVM in 5 of 9 cases (55%). DSA confirmed the 5 cases and was diagnostic in those remaining without a clear visualization on CTA. MR and DSA were performed for follow-up: there were 3 persistent nidus after treatment. Spetzler-Martin Classification Score [figure 2]: -Grade 2: 6 patients -Grade 3: 3 patients The radiologic findings are shown in Table 1. Urgent surgery was performed in 3 cases to drain haemathoma which produced a mass efect, not necessarily aimed to resect vascular tangle. Elective surgery was the most frequent treatment of choice to resect the vascular tangle. The 3 patients not treated initially, during the follow-up ended in an elective surgery. Four patients went through embolization; however, this did not assure a complete nidus resection in three of these individuals. After treatment, there were four (44%) AVMs persistences demonstrated by DSA or MR. Page 8 of 21

Images for this section: Fig. 3: Female patient of 14 years old featured neurologically during the previous two weeks of multiple self limited episodes of left paresia and paresthesia along a mild dysphasia. CT was performed when holocraneal headache was added. Unenheanced CT (UCT). Axial view shows an asymmetric density in the right-fronto-parietal region. Radiology, Vall d Hebron - Barcelona/ES Page 9 of 21

Fig. 4: Same patient of Figure.3 Angio-CT (CTA) in arterial phase with maximal intensity projection (MIP) at the same level nicely shows the AVM with a diffuse nidus with venous draining to the superior sagital sinus and right superficial cortical veins. Radiology, Vall d Hebron - Barcelona/ES Page 10 of 21

Fig. 5: Same patient Figure. 3 MR shows a T2 axial weighted image (T2WI) the same findings are present however more precise venous outflow is shown. Radiology, Vall d Hebron - Barcelona/ES Page 11 of 21

Fig. 6: Same case of previous figures MR T1 sagital WI showing the right fronto-parietal AVM. Radiology, Vall d Hebron - Barcelona/ES Page 12 of 21

Fig. 7: Same patient of previous figures. MR axial view, time of flight (TOF) sequence, shows this large right fronto-parietal AVM. Radiology, Vall d Hebron - Barcelona/ES Page 13 of 21

Fig. 8: Same patient of previous figures. Digital subtraction arteriography (DSA), frontal view, contrast injected from right internal carotid artery. Radiology, Vall d Hebron - Barcelona/ES Page 14 of 21

Fig. 9: Same patient of previous figures. Digital subtraction arteriography (DSA), lateral view, contrast injected from right internal carotid artery, shows the AVM nidus as well as the superficial venous drainage during an early arterial phase. Radiology, Vall d Hebron - Barcelona/ES Page 15 of 21

Fig. 10: A 9 year old patient presented a severe headache with no focal neurologic symptoms. Axial T2 (upper right) and FLAIR weighted images show a right temporal lobe haemathoma (within an elocuent area) associated with an AVM at this level. Radiology, Vall d Hebron - Barcelona/ES Page 16 of 21

Fig. 11: A 9 year old patient presented with severe headache and no focal neurologic symptoms. Angio-TOF-3D (right upper corner) and DSA images show a vascular tangle in the right temporal and parietal lobe (AVM nidus) of 50 x 36 x 18 mm feeded by vessels from MCA, PCA, and right anterior choroid artery from the same side. This AVM affected the language area. Radiology, Vall d Hebron - Barcelona/ES Fig. 12: Table 1. Overview of radiologic findings and clinical outcomes. Location: refers to the AVM s location wether if is within a brain lobe or at a basal ganglia level. AVM size >3 cm indicates an AVM larger than 3 cm present. I.C: internal carotid artery afference; MCA: middle cerebral artery afference; ACA: anterior cerebral artery afference; PCA: posterior cerebral artery afference; Lent: lenticular-estriatal branches afference. Deep: Page 17 of 21

deep venous drainage ; Sup. : Superficial venous drainage. URG. : urgent surgery; ELECT. : elective surgery. #: indicates the finding was present. Radiology, Vall d Hebron - Barcelona/ES Page 18 of 21

Conclusion There is an obvious limitation in our small series of patients to get solid conclusions, however the AVM s treatment issue is still disccussed in medical literature and there is no clear consensus [8]. In our series the persistence of the AVM s nidus after treatment control did not have relation with any particular treatment performed. AVM are unfrequent in the general population and even more in the pediatric age. Radiologists should be aware what to look for and suspect this patholgy when brain haemathomas or unexplained increased densities are presented at this age. It is also crucial to perform specific angiographic studies to distinguish correctly their different features to get a multidisciplinary treatment approach. As a matter of fact, it was reassured that UCT was useful in the context of a ruptured AVM, to assess the location and size of the hematoma, the presence of hydrocephalus, and mass effect. However, UCT is not completely reliable to identify the precise source of bleeding. Although CTA was a more reliable approach in more than the half of the cases, the information provided was not enough to identify feeding vessels and aneurysm s presence. MR imaging was useful in all of the cases for localization of the AVMs, its size, presence of hematoma, or posthemorrhagic changes. As well it is a good strategic approach in the follow up in pediatric patients as no radiation is envolved. DSA remained as the gold standard for the diagnosis of a cerebral AVM specially to determine the supplying vessels of the AVM, the venous outflow and the presence of aneurysms. Page 19 of 21

References 1. Ozanne A, Alvarez H, Krings T, et al. Neurovascular pathology malformation of the child: aneurysmal malformations of the vein of Galen (VGAM), pial arteriovenous malformations (PAVM), dural sinus malformation (DSM). J Neuroradiol 2007;34(3): 145-66. 2. Guidetti B, Delitala A. Intracranial arteriovenous malformations. Conservative and surgical treatment. J Neurosurg 1980;53:149-52. 3. So SC. Cerebral arteriovenous malformations in children. Childs Brain 1978;4:242-50. 4. Swash M, Oxburv J. Haemorrhagic disorders. In: Swash M, Oxbury J (Eds.). Clinical Neurology, Ist Ed. New York: Churchill Livingstone, 1991; 1016. 5. Beslow LA, Jordan LC. Pediatric stroke: the importance of cerebral arteriopathy and vascular malformations. Childs Nerv Syst 2010;26(10):1263-73. 6. De Ribaupierre S, Rilliet B, Cotting J, et al. A 10-year experience in paediatric spontaneous cerebral haemorrhage: which children with headache need more than a clinical examination? Swiss Med Wkly 2008;138(5-6):59-69. 7. Anderson RC, McDowell MM, Kellner CP, et al. Arteriovenous malformation-associated aneurysms in the pediatric population. J Neurosurg Pediatr 2012;9(1):11-6. 8. Outcome after interventional or conservative management of unruptured brain arteriovenous malformations: a prospective, population-based cohort study. Wedderburn CJ, van Beijnum J, Bhattacharya JJ, Counsell CE, Papanastassiou V, Ritchie V, Roberts RC, Sellar RJ, Warlow CP, Al-Shahi Salman R, SIVMS CollaboratorsLancet Neurol. 2008;7(3):223. Page 20 of 21

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