Endoscopic Third Ventriculostomy in an Untreated Vein of Galen Malformation Presenting Lately with Acute Obstructive Hydrocephalus

Background: Mural-type vein of Galen malformations (VoGMs) mostly manifest in later stages of infancy as macrocephaly, hydrocephalus, or failure to thrive unless previously diagnosed and treated. Literature is extremely limited on the success and safety of endoscopic third ventriculostomy (ETV) in acute obstructive hydrocephalus caused by an untreated VoGM. Thus, we aimed to present a case for demonstrating the possible efficacy and safety of ETV under such conditions.

Case description: A 22-month-old boy presenting with acute-onset headache, vomiting, and lethargy was diagnosed with a mural-type VoGM and acute obstructive hydrocephalus. He was treated satisfactorily with ETV and endovascular embolization. We observed a shrinkage in malformation sac diameter of >20 mm in the magnetic resonance imaging angiography at postoperative year 1. The child showed normal motor and mental development at his outpatient clinic visit at postoperative month 15.

Conclusions: To the best of our knowledge, this is the first case in the literature with an untreated VoGM presenting with acute obstructive hydrocephalus who was treated with the combination of ETV and endovascular embolization and had an adequate follow-up period.