A lumbar arteriovenous fistula presenting with intraventricular hemorrhage and hydrocephalus

Area of Science:

• Neurology
• Pediatric Neurosurgery
• Vascular Neurology

Background:

• Pediatric spinal vascular malformations are uncommon and typically manifest with symptoms related to mass effect on neural structures.
• Arteriovenous fistulas (AVFs) are a subset of these malformations, with spinal dural/perimedullary types being particularly rare.

Observation:

• A previously healthy child presented with lethargy and headache, showing ventriculomegaly and trace intraventricular hemorrhage on initial imaging.
• Symptoms improved following cerebrospinal fluid (CSF) diversion via an external ventricular drain.

Findings:

• Further investigation identified a lumbar spinal dural/perimedullary arteriovenous fistula (AVF) as the underlying cause.
• The AVF was successfully treated with endovascular embolization.
• The patient experienced post-procedural vasospasm requiring further endovascular intervention and eventual ventriculoperitoneal shunt placement.

Implications:

• This case underscores the importance of considering the entire neuroaxis in the diagnostic workup of pediatric hydrocephalus.
• Spinal arteriovenous fistulas can present atypically, necessitating a comprehensive imaging approach.
• Early diagnosis and multimodal treatment strategies are crucial for favorable outcomes in pediatric spinal vascular malformations.