Arteriovenous malformation of the trigeminal nerve root presented with venous congestive edema of the medulla oblongata and upper cervical cord: illustrative case

BACKGROUND: Arteriovenous malformation (AVM) of the trigeminal nerve root (TNR) is a rare subtype of the lateral pontine AVM. Most of them are diagnosed when they bleed or exert trigeminal neuralgia. Venous congestive edema is a rare phenomenon caused by TNR AVMs. OBSERVATIONS: An 82-year-old man was admitted with progressive limb weakness and dysphasia. Magnetic resonance imaging (MRI) revealed extensive edema of the medulla oblongata and the upper cervical cord with signal flow void at the C3 anterior spinal cord. Vertebral angiography revealed a small nidus fed mainly by the pontine perforating arteries (PPAs). The anterior pontomesencephalic vein (AMPV) was dilated, functioning as the main drainage route. This suggests that venous hypertension triggered the brainstem and upper cervical cord edema. MRI with gadolinium enhancement showed that the nidus was located around the right TNR. Because the nidus sat extrinsically on the pial surface of the right TNR’s base, microsurgical obliteration with minimum parenchymal injury was achieved. Postoperative MRI showed disappearance of the brainstem and cervical cord edema with improved clinical symptoms. LESSONS: TNR AVM is rarely associated with brainstem and upper cervical cord edema caused by venous hypertension of the congestive drainage system.