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Paeds Vivasneurology-neurodisability-and-neuromuscular

Paeds Vivas · neurology-neurodisability-and-neuromuscular

Cerebral vascular malformations: Viva

Branching clinical structured oral on cerebral vascular malformations covering the classification, the neonatal high-output cardiac failure of the vein of Galen malformation, the Bicetre neonatal evaluation score, the transarterial embolisation, and the differentiation from the arteriovenous malformation and the cavernous malformation.

branching clinical structured oral4 min readVerification in progress

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RACP DWERACP DCEMRCPCH Clinical
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RACP DWERACP DCEMRCPCH Clinical
Prompt
A term neonate is transferred from the postnatal ward on day two of life with severe respiratory distress, hepatomegaly, and a gallop rhythm. The echocardiogram shows a structurally normal heart with a high-output state, and a loud cranial bruit is noted over the vertex. The examiner asks how you classify cerebral vascular malformations, how you confirm the diagnosis in this neonate, how the Bicetre neonatal evaluation score guides the management, and how you would manage an arteriovenous malformation or a cavernous malformation if the scenario shifts.

Branch 1: Classification and the bedside recognition

The candidate should classify cerebral vascular malformations into the high-flow arteriovenous malformation, the vein of Galen aneurysmal malformation, the low-flow cavernous malformation, and the developmental venous anomaly. A strong candidate states that the high-flow lesions present with haemorrhage or cardiac failure, that the low-flow lesions present with seizures, and that the developmental venous anomaly is found incidentally and left alone. The candidate should then state that this neonate, with the high-output cardiac failure and the loud cranial bruit, has the classic presentation of a vein of Galen malformation, because the malformation is the commonest extracardiac cause of high-output cardiac failure in the newborn. [4][1]

If the examiner presses on the bedside recognition, the candidate should state that the loud cranial bruit over the vertex is the clue that the heart failure is driven by an intracranial shunt and not by an intrinsic cardiac lesion. The echocardiogram shows a structurally normal heart, and the high-output state points to the extracardiac shunt. The candidate should request the urgent cranial ultrasound or the MRI brain with MRV to confirm the enlarged midline venous sac and the feeding choroidal arteries, and should arrange the early retrieval to a paediatric interventional neuroradiology centre. [5][3]

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References8ShowHide
  1. [3]Lasjaunias PL, Chng SM, Sachet M, et al The management of vein of Galen aneurysmal malformations. Neurosurgery, 2006.PMID 17053602
  2. [4]Alvarez H, Garcia Monaco R, Rodesch G, et al Vein of galen aneurysmal malformations. Neuroimaging Clin N Am, 2007.PMID 17645970
  3. [5]Cory MJ, Durand P, Sillero R, et al Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure. Pediatr Res, 2023.PMID 35422084
  4. [1]Karim S, Jain S, Martinez ML, et al Intracranial Vascular Malformations in Children. Neuroimaging Clin N Am, 2024.PMID 39461764
  5. [7]Paddock M, Lanham S, Gill K, et al Pediatric Cerebral Cavernous Malformations. Pediatr Neurol, 2021.PMID 33494000
  6. [2]Morshed RA, Winkler EA, Kim H, et al High-Flow Vascular Malformations in Children. Semin Neurol, 2020.PMID 32252098
  7. [11]Hak JF, Boulouis G, Kerleroux B, et al Pediatric brain arteriovenous malformation recurrence: a cohort study, systematic review and meta-analysis. J Neurointerv Surg, 2022.PMID 34583986
  8. [12]Smith ER, Scott RM Cavernous malformations. Neurosurg Clin N Am, 2010.PMID 20561497
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