Cerebellar cavernous malformation in pediatric patients: defining clinical, neuroimaging, and therapeutic characteristics

Knerlich-Lukoschus, Friederike; Steinbok, Paul; Dunham, Christopher; Cochrane, D. Douglas

doi:10.3171/2015.1.peds14366

Cited by 22 publications

(7 citation statements)

References 37 publications

(64 reference statements)

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“…The possibility of identifying brain AVMs and other vascular malformations on -based images has been demonstrated using susceptibility-weighted imaging (SWI) (Haacke et al, 2004). In previous studies, SWI helped delineate brain AVMs (Essig et al, 1999;Gasparotti et al, 2011;George et al, 2010;Hodel et al, 2013;Huisman et al, 2010;Jagadeesan et al, 2011;Liu et al, 2015;Nabavizadeh et al, 2014;Reichenbach et al, 1998;Reichenbach and Haacke, 2001), arteriovenous fistulas (Gasparotti et al, 2011;Hodel et al, 2013;Jagadeesan et al, 2011;Nabavizadeh et al, 2014), capillary telangiectasias (Gelal et al, 2014;Lee et al, 1999;Tong et al, 2008), cavernous malformations (Dammann et al, 2013;Huisman et al, 2010;Knerlich-Lukoschus et al, 2015;Lee et al, 1999;Reichenbach et al, 2001;Thomas et al, 2008;Tong et al, 2008) and developmental venous anomalies (Fushimi et al, 2008;Horsch et al, 2014;Huisman et al, 2010;Liu et al, 2015;Reichenbach and Haacke, 2001;Thomas et al, 2008). A limitation of SWI is that it is purely a qualitative method because of the non-local nature and tissue geometry/orientation dependence of the GRE signal phase.…”

Section: Introductionmentioning

confidence: 99%

Investigating the oxygenation of brain arteriovenous malformations using quantitative susceptibility mapping

et al. 2019

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Brain arteriovenous malformations (AVMs) are congenital vascular anomalies characterized by arteriovenous shunting through a network of coiled and tortuous vessels. Because of this anatomy, the venous drainage of an AVM is hypothesized to contain more oxygenated, arterialized blood than healthy veins. By exploiting the paramagnetic properties of deoxygenated hemoglobin in venous blood using magnetic resonance imaging (MRI) quantitative susceptibility mapping (QSM), we aimed to explore venous density and oxygen saturation (SvO2) in patients with a brain AVM. We considered three groups of subjects: patients with a brain AVM before treatment using gamma knife radiosurgery (GKR); patients three or more years post-GKR treatment; and healthy volunteers. First, we investigated the appearance of AVMs on QSM images. Then, we investigated whether QSM could detect increased SvO2 in the veins draining the malformations. In patients before GKR, venous density, but not SvO2, was significantly larger in the hemisphere containing the AVM compared to the contralateral hemisphere (p = 0.03). Such asymmetry was not observed in patients after GKR or in healthy volunteers. Moreover, in all patients before GKR, the vein immediately draining the AVM nidus had a higher SvO2 than healthy veins. Therefore, QSM can be used to detect SvO2 alterations in brain AVMs. However, since factors such as flow-induced signal dephasing or the presence of hemosiderin deposits also strongly affect QSM image contrast, AVM vein segmentation must be performed based on alternative MRI acquisitions, e.g., time of flight magnetic resonance angiography or T1-weighted images. This is the first study to show, non-invasively, that AVM draining veins have a significantly larger SvO2 than healthy veins, which is a finding congruent with arteriovenous shunting.

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Section: Introductionmentioning

confidence: 99%

Investigating the oxygenation of brain arteriovenous malformations using quantitative susceptibility mapping

et al. 2019

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“…The prospective hemorrhage rate was used as the secondary outcome parameter. Consistent with prior studies, hemorrhagic events were defined as overt bleedings, which could be verified based on imaging studies and were accompanied by neurological symptoms [ 13 , 15 , 28 ].…”

Section: Methodsmentioning

confidence: 89%

How can we optimize the long-term outcome in children with intracranial cavernous malformations? A single-center experience of 61 cases

et al. 2022

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The objective is to provide a treatment algorithm for pediatric patients with intracranial cavernous malformations (CMs) based on our experience. Patients < 18 years of age who were treated either surgically or conservatively at the authors’ institution between 1982 and 2019 were retrospectively evaluated. A total of 61 pediatric patients were treated at the authors’ institution: 39 with lobar CMs; 18 with deep CMs, including 12 in the brainstem and 6 in the basal ganglia; and 4 with CMs in the cerebellar hemispheres. Forty-two patients underwent surgery, and 19 were treated conservatively. The median follow-up time was 65 months (1–356 months). In surgically treated patients, lesions were larger (2.4 cm vs 0.9 cm, p < 0.001). In patients with lobar CMs, seizures were more common (72% vs 21%, p = 0.003) in the surgery group than in conservatively managed patients. In deep CMs, modified Rankin scale (mRS) was higher (4 vs 1, p = 0.003) in the surgery group than in conservatively treated patients. At the time of last follow-up, no differences in Wieser outcome class I were seen (86% vs 67%) in lobar CMs, and mRS scores had aligned between the treatment groups in deep CMs (1 vs 0). We encountered no new permanent neurological deficit at time of last follow-up. We propose a treatment algorithm according to lesion location and size, burden of symptoms, epilepsy workup, and further clinical course during observation. A conservative management is safe in pediatric patients with asymptomatic CMs. Gross total resection should be the aim in patients with symptomatic lobar CMs. A less aggressive approach with subtotal resection, when required to prevent neurological compromise, sustainably improves neurological outcome in patients with deep CMs.

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“…Magnetic resonance imaging (MRI), specifically T2-weighted imaging, has the greatest sensitivity for CMs. [ 17 ] Gradient-echo MRI sequences show the deposition of hemosiderin in various levels of maturation, leading to the pathognomonic “popcorn” imaging appearance. [ 26 ] Unlike other vascular malformations, CMs do not appear on cerebral angiography.…”

Section: Discussionmentioning

confidence: 99%

“…However, developmental venous anomalies are frequently associated with CM, and will appear during the normal to late venous phase on conventional cerebral angiography. [ 17 ] In addition, immediately in the postoperative period, the surgical cavity could have blood in it so it may appear that there is a residual CMs. If concerned about a residual hemangioma, 3-month postoperative imaging can be performed.…”

Section: Discussionmentioning

confidence: 99%

Presentation and management of nervous system cavernous malformations in children

et al. 2022

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Cerebral cavernous malformations (CMs) are slow-flow vascular lesions that affect up to 0.5% of the pediatric population. These lesions are at risk for hemorrhage, causing seizures, and leading to neurological deficits. Here, we conduct a literature review and then present a report of a supratentorial CM in a 2-year-old patient with no significant past medical history who presented at our institution with 1 month of eye twitching. We performed a literature search of five databases of all articles published before 2020. Our inclusion criteria included cohort and case series of children with mean age under 12 years. Our search yielded 497 unique articles, of which 16 met our inclusion criteria. In our pooled literature analysis, a total of 558 children were included, 8.3% of which had a positive family history and 15.9% had multiple CMs. About 46.1% of the children had seizures, and 88.4% of those who underwent surgery had a total resection. About 85.1% of those with epilepsy were Engel Class 1 postsurgery. Over a mean follow-up of 4.1 years, 3.4% of patients had additional neurological deficits, including paresis and speech deficits. Our analysis of published literature shows surgical intervention should be considered first-line therapy for patients who are symptomatic from CM, present with seizure, and have surgically accessible lesions. Additional work is needed on outcomes and long-term effects of minimally invasive treatments, including radiosurgery and laser ablation, in pediatric populations.

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Cerebellar cavernous malformation in pediatric patients: defining clinical, neuroimaging, and therapeutic characteristics

Cited by 22 publications

References 37 publications

Investigating the oxygenation of brain arteriovenous malformations using quantitative susceptibility mapping

Investigating the oxygenation of brain arteriovenous malformations using quantitative susceptibility mapping

How can we optimize the long-term outcome in children with intracranial cavernous malformations? A single-center experience of 61 cases

Presentation and management of nervous system cavernous malformations in children

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