Understanding jugular venous outflow disturbance

Abstract

Extracranial venous abnormalities, especially jugular venous outflow disturbance, were originally viewed as nonpathological phenomena due to a lack of realization and exploration of their feature and clinical significance. The etiology and pathogenesis are still unclear, whereas a couple of causal factors have been conjectured. The clinical presentation of this condition is highly variable, ranging from insidious to symptomatic, such as headaches, dizziness, pulsatile tinnitus, visual impairment, sleep disturbance, and neck discomfort or pain. Standard diagnostic criteria are not available, and current diagnosis largely depends on a combinatory use of imaging modalities. Although few researches have been conducted to gain evidence-based therapeutic approach, several recent advances indicate that intravenous angioplasty in combination with stenting implantation may be a safe and efficient way to restore normal blood circulation, alleviate the discomfort symptoms, and enhance patients' quality of life. In addition, surgical removal of structures that constrain the internal jugular vein may serve as an alternative or adjunctive management when endovascular intervention is not feasible. Notably, discussion on every aspect of this newly recognized disease entity is in the infant stage and efforts with more rigorous designed, randomized controlled studies in attempt to identify the pathophysiology, diagnostic criteria, and effective approaches to its treatment will provide a profound insight into this issue.

Keywords: diagnosis; jugular venous outflow disturbance; pathophysiology; tinnitus; treatment.

Figure 1

Proposed etiologies of internal jugular vein (IJV) outflow disturbance. IJV outflow disturbance may be secondary to either extraluminal compression or intraluminal anomalies. Extraluminal compression can result from enlarged thyroid gland (A), and adjacent artery (B) or bony structures (C). Intraluminal anomalies include thrombi (D), septum (E), and elongated valve (F)

Figure 2

Neuroimaging examples of patients with bilateral internal jugular vein (IJV) stenosis. Magnetic resonance venography (MRV) images including (A,D) display the presence of bilateral IJV stenosis (short arrows) surrounded by abnormally engorged and tortuous collaterals (long arrows). Three‐dimensional reconstruction images of CT including (B,C,E,F) further reveal the IJV stenosis might due to the compression from nearby arteries

Figure 3

Cather venography of unilateral internal jugular vein (IJV) stenosis. A, shows severe stenosis of the IJV on the right side (blue arrow) and a significantly increased number of abnormal tortuous collateral veins (red arrows) before stenting. B, shows, after intervention with stenting, the former stenotic lumen is recanalized (blue arrow) and the abnormal collaterals were distinctly reduced (red arrows)

Figure 4

Fundal photographs and corresponding optical coherence tomography in a patient with bilateral internal jugular vein (IJV) stenosis. Fundal photographs and corresponding optical coherence tomography (OCT) pictures of both eyes during hospital but before stenting: (A) Right and (B) left show severe papilledema with disk elevation, periphery halo, and congested/tortuous retinal vessels; hemorrhage is noticed in the right eye; the FPG scores are 5 and 4 for right and left eyes, respectively; OCT pictures show an significant increase in retinal nerve fiber layer thickness. Fundal photographs and corresponding OCT pictures of both eyes at approximately 15 mo of follow‐up after stenting: (C) Right and (D) left show remarkable improved papilledema with disappearance of tortuous vessels and optic disc edema; the FPG scores were 0 for both eyes; the retinal nerve fiber layer thickness of each eye recovers to normal range