Spontaneous thrombosis of a vein of Galen malformation associated with acute sinusitis: a case report

• Published in: Child's nervous system Vol. 37; no. 10; pp. 3271 - 3276
• Main Authors: Pulido, Luis Fernando, Murcia Salazar, Diana, Gómez Amarillo, Diego, Useche, Juan Nicolás, Ghotme, Kemel A.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-10-2021
• Subjects: Case Report; Medicine; Medicine & Public Health; Neurosciences; Neurosurgery; Spontaneous thrombosis; Case report; Aneurysm; Acute sinusitis; Vein of Galen malformation; Arteriovenous malformation; Pulmonary hypertension
• ISSN: 0256-7040; 1433-0350
• DOI: 10.1007/s00381-021-05072-4

The vein of Galen malformation is caused by an abnormal shunting between choroidal arteries and the median prosencephalic vein during embryological development, leading to increased blood flow to the deep cerebral veins, intracranial damage, and systemic repercussions. Idiopathic spontaneous thrombosis of a vein of Galen malformation is rare, and its association with acute sinusitis has not been reported in the literature. We present the case of a girl with a postnatal diagnosis of a vein of Galen malformation at the age of 16 months, with secondary pulmonary hypertension that was adequately controlled with spironolactone. At 3 years old, while expecting elective endovascular treatment, the patient developed spontaneous thrombosis of the vein of Galen malformation, concomitant to an acute sinusitis episode, with complete resolution of the vascular malformation and secondary pulmonary hypertension. The patient continued with normal neurological development over a 5-year follow-up. We discuss the main pathophysiologic mechanisms that can explain spontaneous thrombosis of VOGMs and the patient’s outcome. Awareness of different mechanisms that can lead to spontaneous thrombosis can help in the decision-making process and prompt targeted approaches to individual patients with a vein of Galen malformation.