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PRESENTATION #12 Case Report | Pediatric Anesthesia

PULMONARY OVERCIRCULATION FROM DURAL ARTERIOVENOUS FISTULA IN A NEONATE

Alex Younghoon Chung, MD; Jimmie Colón, MD
Ochsner Medical Center

Presenter: Jacob Boudreaux, BS
UQ – Ochsner

Pediatric intracranial arteriovenous shunts are rare presentations and comprise pial arteriovenous malformations (AVM), pial and dural arteriovenous fistulae (AVF), and vein of Galen malformations. Severity is associated with degree of shunting and ranges from benign to cardiopulmonary distress and congestive cardiac failure. We present a neonate evaluated for tachypnea and pulmonary hypertension caused by a dural AVF requiring percutaneous intervention under anesthesia. A 12-day-old presenting with tachypnea was found on echocardiogram to have severe right ventricular hypertension, an atrial septal defect with right-to-left shunting, pulmonary hypertension of 85mmHg, and dilated right ventricle and superior vena cava. Patient was admitted for suspected vein of Galen malformation and started on inhaled nitric oxide, sildenafil, and high flow oxygen for potential persistent pulmonary hypertension of the newborn (PPHN). MRI/MRA demonstrated right frontal lobe mass effect, a right middle cranial fossa dural AVF, and evidence of pulmonary overcirculation. The dural AVF acted as a high flow left-to-right shunt leading to high output cardiac failure. Nitric oxide, sildenafil, and oxygen were weaned to avoid further damage to the pulmonary vasculature from decreased pulmonary vascular resistance. Diuresis with chlorothiazide and furosemide was started, which improved RV function and pulmonary pressure. Percutaneous shunt embolization was elected after medical optimization. Anesthetic management during embolization included ketamine induction for hemodynamic stability, arterial line placement for monitoring blood pressure, and central line placement for venous access. Respiratory goals included maintaining high CO2 levels for cerebral vasodilation to facilitate surgical access and keeping pulmonary vascular resistance elevated to reduce overcirculation. Fluids were restricted to maintenance and medication administration flushes. Following successful procedure, the patient remained intubated postoperatively with dexmedetomidine infusion and fentanyl. Pediatric dural AVFs are rare and have an aggressive clinical course with mortality greater than 25%. Their effects on the neonatal cardiopulmonary system pose challenges for anesthetic intraoperative management.