Endovascular Treatment of Congenital External Carotid-Jugular Fistula: Case Report and Review of the Literature

Abstract

Congenital vascular malformation (CVM) between the external carotid artery and the internal jugular vein is a rare disease, it originates as a consequence of arrested development during various stages of embryogenesis. The natural history of CMVs is progressive growth, it can remain clinically silent until it progresses causing local swelling accompanied by symptoms and signs of arteriovenous shunting, mass effect, ischemic insult with ulceration of skin or bleeding. In literature only few cases of CMVs have been reported, therefore there is still no consensus about the correct surgical approach to this pathology. We report an uncomplicated case of CVMs in a 15-year-old female successfully treated with coils embolization. To our knowledge, this is the eighth reported case in the literature review.

Endovascular Treatment of Congenital Internal Carotid-Jugular Fistula

“…Pulsatile mass swelling is the main clinical manifestation. However, carotid-jugular fistulas are potentially dangerous, because they may cause serious complications of cardiac failure, rupture, thromboembolism, or cerebral ischemia (5), like hemiparesis in our case.…”

“…For its rarity, neither appropriate procedural standards or definitive surgical techniques have yet been established to treat congenital internal carotid-jugular fistulas. Operative ligations, detachable balloons, coiling, and stenting were all reported as treatment options for carotid-jugular fistulas (1, 4, 5). Operative ligation was not appropriate for our case, because of its location in the cranio-cervical region.…”

Internal jugular vein agenesis: a rare vascular abnormality and incidental finding. A case of internal jugular vein agenesis in a 52-years old male

“…The incidence of developmental vascular anomalies in general population are seen in about 0.05%-0.25%, however agenesis of the IJV is rarely encountered and confirmed [2]. Different IJV abnormalities have been reported in scientific literature: partial or complete duplication [8], [9], [10], [11], [12], stenosis, complete occlusion, distortions, and intraluminal structures, such as membranes, webs, and inverted valves [13], [14], congenital external carotid artery-IJV fistula [15], congenital aneurysm [16], and fenestration [17].…”

Fetal carotid-jugular fistula

“…Studies have shown that congenital arteriovenous fistulas located in the head and neck are usually unilateral. [4] A congenital arteriovenous fistula is a benign lesion in pathological morphology, but it has a malignant tendency to grow rapidly in clinical practice and must be monitored. According to the size and location of the fistula, a congenital arteriovenous fistula can be divided into three categories by pathology.…”

“…These abnormal traffic branches are called fistulas. [3,4] In this report, we present a case of a congenital carotid-jugular (CJ) fistula in a fetus; the pregnant woman experienced termination of the pregnancy at 27 weeks, underwent induction surgery and the diagnosis was postoperatively confirmed as CJ fistula and left ear microtia. Fetal CJ fistula is an extremely rare vascular lesion.…”