Introduction: Carotid Web (CW) is a rare form of focal fibromuscular dysplasia that results in an abnormal shelf-like projection of intimal fibrous tissue into the carotid bulb. It is theorized that CW leads to ischemic stroke secondary to blood flow stasis and subsequent embolization. There is uncertainty in diagnosis, prognosis, and optimal management of this uncommon entity. To address this knowledge gap, we performed a systematic literature review (SLR) of CW.
Methods: Our literature search for CW and related terms yielded 1017 results. After a preliminary assessment of all 1017 retrieved manuscripts; 72 manuscripts were reviewed in detail. A total of 31 manuscripts met entry criteria and were included in our SLR. We present the demographics, cardiovascular (CV) risk factors, neuroimaging findings, stroke recurrence or stroke free-duration, and treatment modality of CW patients.
Results: Our SLR resulted in 104 patients with CW, (median age at presentation: 45.7 [IQR 45-57], 52.3% female). The majority (68.3%) of CW patients did not have CV risk factors, however, 22.2% of patients were smokers. 47.8% of patients suffered recurrent stroke. The majority of patients were ultimately treated with antiplatelet therapy (94.4%) and 21.2% underwent carotid revascularization (4.8% carotid stenting, 16.3% carotid endarterectomy). None of the patients who underwent revascularization had a reported stroke recurrence. See Table.
Conclusions: CW is a rare disease leading to ischemic stroke in younger patients without typical CV risk factors. CW patients in our SLR were at extremely high risk for recurrent stroke (47.8%). Aggressive secondary stroke prevention measures are indicated in this patient population, however, the optimal treatment strategies remain unclear. Carotid revascularization may be the definitive treatment for certain patients with CW, but further studies are needed as incomplete reporting and potential publication bias limit our findings.