Pediatric cerebral arteriovenous malformation

Pediatric cerebral arteriovenous malformation

Although brain arteriovenous malformations (bAVMs) account for a very small proportion of cerebral pathologies in the pediatric population, they are the cause of roughly 50% of spontaneous intracranial hemorrhages. Pediatric bAVMs tend to rupture more frequently and seem to have higher recurrence rates than bAVMs in adults 1) 2) 3) 4) 5) 6) 7).

Natural History

The natural history of untreated cerebral AVMs in children is worse than in adults, in relation to a longer life expectation, a higher annual risk of AVM bleeding (3.2% vs. 2.2%) and a higher incidence of posterior fossa and basal ganglia AVMs, most of which present with massive haemorrhages 8).


The management of pediatric bAVMs is particularly challenging. In general, the treatment options are conservative treatment, microsurgeryendovascular therapy (EVT), gamma knife radiosurgery (GKRS), proton-beam stereotactic radiosurgery (PSRS), or a combination of the above.

In 2019 Meling et al., performed a systematic review, according to the PRISMA guidelines, with the result that none of the options seem to offer a clear advantage over the others when used alone. Microsurgery provides the highest obliteration rate, but has higher incidence of neurological complications. EVT may play a role when used as adjuvant therapy, but as a stand-alone therapy, the efficacy is low and the long-term side effects of radiation from the multiple sessions required in deep-seated pediatric bAVMs are still unknown. GKRS has a low risk of complication, but the obliteration rates still leave much to be desired. Finally, PSRS offers promising results with a more accurate radiation that avoids the surrounding tissue, but data is limited due to its recent introduction. Overall, a multi-modal approach, or even an active surveillance, might be the most suitable when facing deep-seated bAVM, considering the difficulty of their management and the high risk of complications in the pediatric population 9).

In 2016 El-Ghanem et al., published a Review of the Existing Literature:

Microsurgical resection remains the gold standard for the treatment of all accessible pediatric AVMs. Embolization and radiosurgery should be considered as an adjunctive therapy. Embolization provides a useful adjunct therapy to microsurgery by preventing significant blood loss and to radiosurgery by decreasing the volume of the AVM. Radiosurgery has been described to provide an alternative treatment approach in certain circumstances either as a primary or adjuvant therapy 10).


Intracranial haemorrhage is the presenting clinical manifestation in 75-80% of paediatric patients and is associated with a high morbidity and mortality 11).

Case series

A prospectively maintained database of children between January 1997 and October 2012 for bAVMs was retrospectively queried to identify all consecutive ruptured bAVMs treated by surgery, embolization, and radiosurgery. The impact of baseline clinical and bAVM characteristics on clinical outcome, rebleeding rate, annual bleeding rate, and bAVM obliteration was studied using univariate and multivariate Cox regression analysis.

One hundred six children with ruptured bAVMs were followed up for a total of 480.5 patient-years (mean, 4.5 years). Thirteen rebleeding events occurred, corresponding to an annual bleeding rate of 2.71±1.32%, significantly higher in the first year (3.88±1.39%) than thereafter (2.22±1.38%; P<0.001) and in the case of associated aneurysms (relative risk, 2.68; P=0.004) or any deep venous drainage (relative risk, 2.97; P=0.002), in univariate and multivariate analysis. Partial embolization was associated with a higher annual bleeding rate, whereas initial surgery for intracerebral hemorrhage evacuation was associated with a lower risk of rebleeding.

Associated aneurysms and any deep venous drainage are independent risk factors for rebleeding in pediatric ruptured bAVMs. Immediate surgery or total embolization might be advantageous for children harboring such characteristics, whereas radiosurgery might be targeted at patients without such characteristics 12).


1) , 8) , 11)

Di Rocco C, Tamburrini G, Rollo M. Cerebral arteriovenous malformations in children. Acta Neurochir (Wien). 2000;142(2):145-56; discussion 156-8. PubMed PMID: 10795888.

Millar C, Bissonnette B, Humphreys RP. Cerebral arteriovenous malformations in children. Can J Anaesth. 1994;41:321–331.

Kiris T, et al. Surgical results in pediatric Spetzler-Martin grades I–III intracranial arteriovenous malformations. Childs Nerv Syst. 2005;21:69–74. discussion 75–76.

Hoh BL, et al. Multimodality treatment of nongalenic arteriovenous malformations in pediatric patients. Neurosurgery. 2000;47:346–357. discussion 357–358.

Kondziolka D, et al. Arteriovenous malformations of the brain in children: a forty year experience. Can J Neurol Sci. 1992;19:40–45.

11. Wilkins RH. Natural history of intracranial vascular malformations: a review. Neurosurgery. 1985;16:421–430.

Jankowitz BT, et al. Treatment of pediatric intracranial vascular malformations using Onyx-18. J Neurosurg Pediatr. 2008;2:171–176.

Meling TR, Patet G. What is the best therapeutic approach to a pediatric patient with a deep-seated brain AVM? Neurosurg Rev. 2019 Apr 13. doi: 10.1007/s10143-019-01101-8. [Epub ahead of print] Review. PubMed PMID: 30980204.

El-Ghanem M, Kass-Hout T, Kass-Hout O, Alderazi YJ, Amuluru K, Al-Mufti F, Prestigiacomo CJ, Gandhi CD. Arteriovenous Malformations in the Pediatric Population: Review of the Existing Literature. Interv Neurol. 2016 Sep;5(3-4):218-225. Epub 2016 Sep 1. Review. PubMed PMID: 27781052; PubMed Central PMCID: PMC5075815.

Blauwblomme T, Bourgeois M, Meyer P, Puget S, Di Rocco F, Boddaert N, Zerah M, Brunelle F, Rose CS, Naggara O. Long-term outcome of 106 consecutive pediatric ruptured brain arteriovenous malformations after combined treatment. Stroke. 2014 Jun;45(6):1664-71. doi: 10.1161/STROKEAHA.113.004292. Epub 2014 May 1. PubMed PMID: 24788975.

Inferior frontal gyrus

Inferior frontal gyrus

Broca’s area is located in the inferior frontal gyrus

Its superior border is the inferior frontal sulcus (which divides it from the middle frontal gyrus).

Its inferior border the lateral fissure (which divides it from the superior temporal gyrus), and its posterior border is the inferior precentral sulcus.

Above it is the middle frontal gyrus (the gyrus frontalis medius), behind it the precentral gyrus (the gyrus praecentralis).

The inferior frontal gyrus, like the middle frontal gyrus and the superior frontal gyrus, is more of a region than a true gyrus.

The middle frontal gyrus is usually more sinous than the inferior frontal gyrus (IFG) or superior frontal gyrus (SFG).

The uncinate fasciculus: connects the anterior temporal lobe to the inferior frontal gyrus. Damage can cause language dysfunction.


Pars opercularis

Pars triangularis

Pars orbitalis

The bone flap has been removed and the dura mater has been opened as a flap pediculated towards the greater sphenoid wing previously roungered to improve parasellar visualization. Sylvian fissureInferior frontal gyrusSuperior temporal gyrus and Middle temporal gyrus are exposed. Three pars of parasylvian inferior frontal gyrus must be distinguished: pars orbitalis (pOr) in relation to the orbital roofpars triangularis(pT) the widest area of sylvian fissure (good place for start opening of sylvian fissure); pars opercularis (pOp) where Broca’s Area is located.

Connections and Functions

Briggs et al. identified a callosal fiber bundle connecting the inferior frontal gyri bilaterally was also identified. The IFG is an important region implicated in a variety of tasks including language processing, speech production, motor control, interoceptive awareness, and semantic processing 1).

In 7 of the 14 patients, we identified nine sites where cortical stimulation interfered with syntactic encoding but did not interfere with single-word processing. All nine sites were localized to the inferior frontal gyrus, mostly to the pars triangularis and opercularis. Interference with syntactic encoding took several different forms, including misassignment of arguments to grammatical roles, misassignment of nouns to verb slots, omission of function words and inflectional morphology, and various paragrammatic constructions. The findings suggest that the left inferior frontal gyrus plays an important role in the encoding of syntactic structure during sentence production 2).


Removal of glioma from the dominant side of the inferior frontal gyrus (IFG) is associated with a risk of permanent language dysfunction. While intraoperative cortical and subcortical electrical stimulations can be used for functional language mapping in an effort to reduce the risk of postoperative neurological impairment, the extent of resection is limited by the functional boundaries. Recent reports proposed that a two-stage surgical approach for low-grade glioma in eloquent areas could avoid permanent deficits via the functional plasticity that occurs between the two operations.

In a patient with World Health Organization (WHO) grade II oligoastrocytoma in the left inferior frontal gyrus, in functional plasticity of language occurred in the interval between two consecutive surgeries. Intraoperative electrical stimulations suggested that a language area and related subcortical fiber crossed the pre-central sulcus during tumor progression owing to functional plasticity. In the present case, the authors integrated neurophysiological data into the intraoperative neuronavigation system. They also confirmed the peri-lesional shift of language area and related subcortical fiber on image findings. Consequently, the tumor was sub-totally removed with two separate resections. Permanent language disturbance did not occur, and this favorable outcome was attributed to functional plasticity. The present experience sustains the multistage approach for low-grade gliomas in the language area. A combination of intraoperative electrical stimulations and updated neuronavigation may facilitate the characterization of brain functional plasticity 3).

In an event-related fMRI study of overt speech production, Pützer et al. investigated the relationship between gestural complexity and underlying brain activity within the bilateral inferior frontal gyrus (IFG). They operationalized gestural complexity as the number of active articulatory tiers (glottal, oral, nasal) and the degree of fine-grained temporal coordination between tiers (low, high). Forty-three neurotypical participants produced three types of highly-frequent non-word CV-syllable sequences, which differ systematically in gestural complexity (simple: [‘dadada], intermediate: [‘tatata], complex: [‘nanana]). Comparing blood oxygen level-dependent (BOLD) responses across complexity conditions revealed that syllables with greater gestural complexity elicited increased activation patterns. Moreover, when durational parameters were included as covariates in the analyses, significant effects of articulatory effort were found over and above the effects of complexity. The results suggest that these differences in BOLD-response reflect the differential contribution of articulatory mechanisms that are required to produce phonologically distinct speech sounds4).



Briggs RG, Chakraborty AR, Anderson CD, Abraham CJ, Palejwala AH, Conner AK, Pelargos PE, O’Donoghue DL, Glenn CA, Sughrue ME. Anatomy and white matter connections of the inferior frontal gyrus. Clin Anat. 2019 May;32(4):546-556. doi: 10.1002/ca.23349. Epub 2019 Feb 28. PubMed PMID: 30719769.

Chang EF, Kurteff G, Wilson SM. Selective Interference with Syntactic Encoding during Sentence Production by Direct Electrocortical Stimulation of the Inferior Frontal Gyrus. J Cogn Neurosci. 2018 Mar;30(3):411-420. doi: 10.1162/jocn_a_01215. Epub 2017 Dec 6. PubMed PMID: 29211650; PubMed Central PMCID: PMC5819756.

Saito T, Muragaki Y, Miura I, Tamura M, Maruyama T, Nitta M, Kurisu K, Iseki H, Okada Y. Functional Plasticity of Language Confirmed with Intraoperative Electrical Stimulations and Updated Neuronavigation: Case Report of Low-Grade Glioma of the Left Inferior Frontal Gyrus. Neurol Med Chir (Tokyo). 2014 Feb 28. [Epub ahead of print] PubMed PMID: 24584281.

Pützer M, Moringlane JR, Sikos L, Reith W, Krick CM. fMRI and acoustic analyses reveal neural correlates of gestural complexity and articulatory effort within bilateral inferior frontal gyrus during speech production. Neuropsychologia. 2019 Jun 22;132:107129. doi: 10.1016/j.neuropsychologia.2019.107129. [Epub ahead of print] PubMed PMID: 31238044.
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