Images in Neurosurgery

Trigeminal Neuralgia Associated with Moyamoya Disease and Atypical Vascular Ectasias

A 23-year-old male presented with a six-month history of right-sided, sharp, shooting facial pain, triggered by eating or light touch. He was initially treated with wisdom tooth extraction, which failed to relieve his pain. Treatment with carbamazepine initially provided relief, but became refractory. Gabapentin and baclofen were added, but his pain persisted. MRI obtained revealed prominent flow voids along the ventral pons producing mass effect (Figure 1). Fiesta MRI detected trigeminal nerve compression (Figure 2). Angiography performed revealed multiple vascular abnormalities including steno-occlusive disease consistent with moyamoya syndrome of the right internal carotid artery (ICA) (Figure 3). Dolichoectasia of redundant enlarged serpiginous appearance in the terminal basilar artery (Figure 4). The left ICA provided cross-filling into the entire right anterior circulation (Figure 5). Based on the complexity of the vascular anomalies, microvascular decompression was not pursued and he was instead referred for stereotactic radiosurgery.

Figure 1: Axial T2-weighted MRI images, demonstrating small, right ICA flow-voids with adjacent single collateral vessels along with prominent flow voids along the ventral aspect of the pons producing mass.

Figure 2: Axial FIESTA MRI image reveals a prominent flow void displacing the right trigeminal nerve.

Figure 3: Digital subtraction angiogram of the right ICA, revealing steno-occlusive disease of the terminus of the right ICA intracranially.

Figure 4: Digital subtraction angiogram of the left vertebral artery, demonstrating dolichoectasia at the terminal basilar artery.

Figure 5: Digital subtraction angiogram of the left ICA, providing visualization of the left anterior circulation, as well as crossfilling into the right anterior circulation.

Submitted by: Rafael A. Vega, MD, PhD; rafael.vega@vcuhealth.org
Department of Neurosurgery
Virginia Commonwealth University Health
System, Medical College of Virginia