18º Congresso Brasileiro de Neurologia Infantil

Dados do Trabalho



Apresentação do caso

This case report an eight months old patient that was prenatally diagnosed with Vein of Galen Aneurysmal Malformation (VGAM), presenting cardiomegaly and severe anemia. At birth, progressed to severe pulmonary hypertension. At 11 days old, an early endovascular procedure was performed. Despite that, the patient progressed to high-output heart failure, relying on high doses of diuretic therapy, vasodilators, and oxygen therapy. A new CT angiography showed an aneurysmal venous structure with multiple dilated and tortuous supplying arteries. A new endovascular procedure was then performed. The child underwent cardiogenic shock in the immediate postoperative period. After a period of hospitalization, the patient was discharged at four months old, on room air, with an ascending weight curve, though still using diuretics to manage cardiac failure. The child has been reaching the neuropsychomotor development milestones corresponding to their chronological age.


VGAM is a rare condition, corresponding to 1% of all cerebrovascular malformations, being the most frequent shunt in pediatrics. Untreated, they are 100% fatal. Heart failure, seen in most of these patients, is challenging to manage. Its total resolution is only possible with the correction of arteriovenous fistulas. The ideal time to perform the corrective procedure is a significant point of debate; the patient in question required two early interventions. More than 70% of the cases have a complete/near-complete resolution with only one embolization. However, subsequent approaches may be necessary. Up to 66% of patients will present complications with asymptomatic ventriculomegaly, but only 15% will present hydrocephalus with signs of intracranial hypertension. Previous studies have shown rates of adequate neuropsychomotor development in up to 60% of surviving cases. Recent studies, however, have shown that when prolonged neurological follow-up is performed up to the school stage, the prognosis may be more guarded. Even children with good neurological development will present weakness and deficits in memory and concentration.

Comentários finais

Current endovascular intervention techniques have improved quality of life and prognosis. However, mortality rates can still reach up to 30%. It is essential for neurologists and pediatricians to know this pathology, its expected evolutionary course, and current evidence so they can identify delays and deficits in neurological development and thus provide early interventions.

Referências (se houver)

1. TAFFIN, Hélène et al. Long‐term outcome of vein of Galen malformation. Developmental Medicine & Child Neurology, v. 62, n. 6, p. 729-734, 2020.
2. GANESAN, Vijeya. Vein of Galen malformation: beyond life or death. Developmental Medicine & Child Neurology, v. 62, n. 6, p. 666-666, 2020.
3. BERENSTEIN, Alejandro et al. Vein of Galen aneurysmal malformation: advances in management and endovascular treatment. Neurosurgery, v. 84, n. 2, p. 469-478, 2019.
4. HERGHELEGIU, Doru et al. Antenatal diagnosis and prognostic factors of aneurysmal malformation of the vein of Galen: A case report and literature review. Medicine, v. 96, n. 30, 2017.
5. CORY, Melinda J. et al. Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure. Pediatric Research, v. 93, n. 1, p. 39-48, 2023.
6. DE ROSA, Gabriella et al. Outcome of neonates with vein of Galen malformation presenting with severe heart failure: a case series. American Journal of Perinatology, v. 36, n. 02, p. 169-175, 2019.
7. CORNET, Marie-Coralie et al. Outcome of Neonates Presenting With Severe Cardiac Failure due to Cerebral Arteriovenous Fistula. Pediatric Neurology, v. 131, p. 25-29, 2022.
8. NUÑEZ, Francisco Brevis; DOHNA-SCHWAKE, Christian. Epidemiology, diagnostics, and management of vein of Galen malformation. Pediatric Neurology, v. 119, p. 50-55, 2021.
9. JAIN, Puneet et al. Vein of Galen Aneurysmal Dilatation in a 14-Month-Old Boy. Pediatric neurology, v. 47, n. 1, p. 71-73, 2012.
10. MCARTHUR, I.; YEO, T. H.; BHATTACHARYA, J. PP05. 4–2566: Developmental outcomes in children treated for vein of Galen malformation. European Journal of Paediatric Neurology, v. 19, p. S47, 2015.

Palavras Chave

Vein of Galen Malformation; cardiac failure; arteriovenous fistula; neonatal hydrocephalus

Fonte de Fomento (se houver)

Declaração de conflito de interesses de TODOS os autores

Eu, Sarah Reis Vilela, autora responsável pela submissão do manuscrito intitulado "VEIN OF GALEN MALFORMATION WITH HIGH OUTPUT CARDIAC FAILURE AND PREMATURE ENDOVASCULAR THERAPY: A CASE REPORT" e todos os coautores que aqui se apresentam (Pedro Lemgruber Xavier Mattoso Pavie; Laura Reis Vilela; Antonella de Albuquerque do Nascimento), declaramos que NÃO POSSUÍMOS CONFLITO DE INTERESSES de ordem: pessoal, comercial, acadêmico, político ou financeiro no manuscrito.


Malformações do sistema nervoso central


Hospital de Base do Distrito Federal - HBDF - Distrito Federal - Brasil, Hospital Materno e Infantil de Brasília - HMIB - Distrito Federal - Brasil


Sarah Reis Vilela, Laura Reis Vilela, Pedro Lemgruber Xavier Mattoso Pavie, Antonella Albuquerque Nascimento