Rupture of an extracranial carotid artery aneurysms (ECAA) is a very rare and life-threatening condition. In order to get a comprehensive view of past and current management of the ruptured ECAA (rECAA), we analyzed all reported cases since 1940 and two of our own cases.
We performed a comprehensive literature review in MEDLINE databases on rECAA and included two patients treated in our department.
In total 58 reports on 74 rECAAs in 74 patients were analyzed. Mean age was 50 years and male to female ratio was 2.2:1; Infection was the most common reported etiology (19/74; 26%), followed by connective tissue disorder (13/74; 18%), atherosclerosis (9/74; 12%) and previous trauma (5/74; 7%); in 28 (38%) patients information on the etiology was not available. Twenty-four (32%) patients had reconstructive surgery, 10 (14%) had endovascular treatment (EVT), 17 (23%) had ligation, 2 (3%) were treated conservatively, one (1%) died before getting definite treatment; in 20 (27%) patients information on treatment was not available. Complications after reconstruction were carotid blowout (3/24; 13%) and cranial nerve deficit (CND) (3/24; 13%). Two (8%) patients died of ECAA-unrelated cause in long-term mortality, and one (4%) died of ECAA-related cause in 30-day mortality. After endovascular (EV) approach, 1/10 of the patients developed CND. After ligation, 5 (29%) patients developed stroke, of which 3 were fatal. One conservatively treated patient had no complications and the other died of ECAA-related cause.
The most common reported etiology for rECAA was infection. Reconstructive surgery was the most common approach and safer than ligation, which carried a high risk of stroke. EVT showed promising results, especially in distally located aneurysms but the number of patients remains low.

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