Decompressive Hemicraniectomy for a Patient With Intraventricular Hemorrhage Due to a Ruptured Arteriovenous Fistula and Refractory Intracranial Hypertension

Arteriovenous fistulas (AVFs) are a rare disease process occurring in pediatric patients with presentations ranging from asymptomatic to intracranial hemorrhage. Intracranial hypertension secondary to AVF rupture increases the risk of further neurological sequelae and hemodynamic instability, requiring immediate stabilization in the acute setting. Traditional treatments for AVF include endovascular embolization, microsurgical disconnection, or a hybrid approach; however, increased intracranial pressures secondary to intracranial hemorrhage or hydrocephalus may necessitate other forms of acute intervention such as external ventriculostomy drains (EVDs), hemorrhagic evacuation, or decompressive craniectomy. Here, we present a five-year-old, previously healthy, female who presented with a tonic-clonic seizure following two weeks of headache, nausea, and emesis after a fall. Imaging demonstrated intraventricular hemorrhage throughout the entire ventricular system secondary to a ruptured pial AVF (pAVF). Due to significantly increased intracranial pressure refractory to medical management, decompressive craniectomy was conducted, followed by endovascular embolization of the arteriovenous nidus. The patient made an excellent neurological recovery without recurrence of bleeding or residual neurological deficits. This represents the successful management of refractory intracranial pressure due to intraventricular hemorrhage and arteriovenous fistula using decompressive craniectomy despite the absence of cerebral edema or lateralizing pathology, leading to an excellent patient recovery.