Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single‐institution case series

Gopalan, Vignesh; Rennie, Adam; Robertson, Fergus; Kanagarajah, Lakshmi; Toolis, Claire; Bhate, Sanjay; Ganesan, Vijeya

doi:10.1111/dmcn.13676

Cited by 27 publications

(21 citation statements)

References 14 publications

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“…Compared to a previously reported series from our service, in which 74 per cent of patients had a good outcome but where the neurodevelopmental evaluation was mainly carried out on neonates and infants (81% of the study population), the long‐term evolution described here is much poorer. In a recent review of the literature, Gopalan et al identified good outcome in 65 per cent of patients treated by embolization but did not determine average time of evaluation. A following publication from this group, however, described longer‐term evaluation (median age at testing: 5y 2mo, range: 1–11y) and noted similar results to us: that only half of the surviving patients had a good outcome …”

Section: Discussionmentioning

confidence: 99%

Long‐term outcome of vein of Galen malformation

Taffin

Maurey

Ozanne

et al. 2019

Develop Med Child Neuro

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Aim To describe the long‐term outcomes of children by the time they reached school age with vein of Galen aneurysmal malformation (VGAM). Method This was a retrospective observational study on a consecutive cohort of patients with VGAM. We included patients with at least one Francophone parent, aged between 6 and 11 years at the time of long‐term evaluation. The neurological outcome was assessed with the King’s Outcome Scale for Childhood Injury score and eight neurological and behavioural items from the Rivermead Postconcussion Symptoms questionnaire. Results All 52 patients (17 females, 32 males [data missing for n=3]) with at least one Francophone parent (5 fetuses and 47 children) were included. At the long‐term evaluation time‐point, 33 patients were alive and 19 patients had died. Risk of postnatal death was associated with severe neonatal cardiac failure (p=0.007) or isosystemic or suprasystemic pulmonary hypertension (p=0.014). Among survivors, 19 had a good outcome with normal schooling and 14 had a poor outcome. Moreover, among the good outcome patients, a large proportion had neurodevelopmental alterations. Interpretation Long‐term outcome of patients with VGAM appears to be less favourable than outcome described at the short‐ and medium‐term, even in the absence of encephalomalacia at birth. Even patients with good outcome often have neuropsychological disorders that may have repercussions on learning and requiring appropriate rehabilitation or medical management. What this paper adds Long‐term outcome appears to be less favourable than described at short‐ and medium‐term follow‐up. Even patients with good outcome at these time‐points often have minor neuropsychological disorders.

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Section: Discussionmentioning

confidence: 99%

Long‐term outcome of vein of Galen malformation

Taffin

Maurey

Ozanne

et al. 2019

Develop Med Child Neuro

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“…Previously, surgical management of VGAMs was associated with an 80%–100% mortality rate9 and has now been replaced by endovascular treatment 1. Advances in embolisation technology have led to improved patient survival1 10; lower mortality rates in embolised (12%) versus non-embolised (47%) patients have been reported 1. With increasing survival rates, the research focus is shifting to neurological and neurodevelopmental outcomes.…”

Section: Introductionmentioning

confidence: 99%

Outcome of vein of Galen malformation presenting in the neonatal period

et al. 2019

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ObjectiveVein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for this condition has improved. However, there is little reported about the medical management decisions of infants with this condition and the associated long-term neurodevelopmental outcomes. We aim to report a single centre experience of both acute treatment and long-term outcomes of VGAM for those infants admitted to our NICU soon after birth.DesignRetrospective cohort study over a 15-year period from 2001 to 2015 inclusive.SettingA quaternary NICU at The Royal Children’s Hospital, Melbourne, Australia.Participants24 newborn infants referred for management of VGAM. There were no eligibility criteria set for this study; all presenting infants were included.InterventionsNone.Main outcomes measuresClinical neuroimaging data were gathered. Surviving children were formally assessed with a battery of tests administered by a neuropsychologist and occupational therapist/physiotherapist at various ages across early to middle childhood.ResultsFifteen neonates with VGAM did not survive beyond their NICU admission. 10 of these were not offered endovascular intervention. Of the nine surviving infants, only one had a normal neurodevelopmental outcome.ConclusionsThe mortality of VGAM presenting in the neonatal period was high, and rates of normal neurodevelopmental outcome for survivors were low. These findings contribute to our understanding of which neonates should be treated and highlights the importance of providing clinical neurodevelopmental follow-up to survivors beyond their infant years.

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“…The mixed malformation carries characteristics of both types. Another dilatation of the true vein of Galen seen in cases of hyperdynamic circulation [5-8].…”

Section: Discussionmentioning

confidence: 99%

“…Type IV A is thalamic AVM, type IV B is mesencephalic AVM and type IV C is combined. Type I and II represent the mural type and type III and IV represent the choroidal type [5-8].…”

Section: Discussionmentioning

confidence: 99%

The Impact of Angiographic Geometry on the Clinical Presentation of True Vein of Galen Aneurysmal Malformations

Hassan¹,

Sultan²,

Ibrahim³

et al. 2020

Pediatr Neurosurg

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Background Purpose: Vein of Galen aneurysmal malformations (VGAM) may carry life-threatening and catastrophic sequelae in children. Their clinical presentations are differently variable between cardiac and cerebral syndromes. There may be a possible relationship between the Galenic angioarchitecture and the clinical presentation. We aimed to study the effect of the venous outflow impairment on the incidence of high-flow heart failure and the incidence of hydrocephalus. Materials and Methods: The angiographies of 21 patients with true VGAM have been studied. MR angiography, 3-dimensional CT angiogram (3D CTA), and conventional digital subtraction angiography were performed for all patients with true VGAM. Transarterial embolization was done in 1 or more sessions for all cases. Results: Among the 21 cases, 14 cases were mural type, 5 cases were choroidal type, and 2 cases were mixed. Hydrocephalus was observed more in infants (92.3%), mural types (92.9%), giant and supergiant (87.5%) aneurysms, and in cases with persistent limbic circle (90.91%). All cases of hydrocephalus were associated with significant stenosis (>70%) of the Falcine sinus draining system (100%). Conclusion: Significant stenosis (>70%) of the draining sinus is a significant factor for VGAM aneurysmal enlargement and occurrence of hydrocephalus. Probably, venous outflow impairment decreases the incidence of high-flow heart failure and increases the incidence of hydrocephalus.

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Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single‐institution case series

Abstract: Clinical and radiological features in older children with vein of Galen malformation relate to venous hypertension. Outcome is good in most cases with endovascular therapy. Mortality is low but is related to intracranial haemorrhage.

Cited by 27 publications

References 14 publications

Long‐term outcome of vein of Galen malformation

Long‐term outcome of vein of Galen malformation

Outcome of vein of Galen malformation presenting in the neonatal period

The Impact of Angiographic Geometry on the Clinical Presentation of True Vein of Galen Aneurysmal Malformations

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