Abstract
Introduction
Aneurysms of the jugular vein system are rare and high clinical suspicion is needed for diagnosis. External jugular vein aneurysms (EJVA) are considered innocent lesions that need treatment mainly for aesthetic reasons. The aim of this systematic review was to present current literature regarding diagnosis and management of EJVAs.
Methods
A literature review was conducted through the Pubmed/Medline and Scopus regarding articles referring on EJVA from 2000 to 2020. Using the PRISMA guidelines (Preferred Reporting Items for Systematic reviews and Meta-Analyses), 30 articles were identified, according to inclusion criteria. Demographics, clinical characteristics, etiology, diagnostic imaging, complications, treatment, and histopathological findings were recorded and analyzed.
Results
Twenty-seven case reports and one case series were identified, including 30 patients and 31 EJVAs. One-third of patients (30.3%) were < 18 years old (mean age 32 years, range 1–72 years) and 54% of them were females. In 51% of the cases, the lesion was characterized as a true aneurysm after histological evaluation. The presence of a soft cervical mass was the most common clinical symptom, while Valsalva maneuver pointed out the presence of an EJVA in 66.7% of patients. Diagnosis was achieved using ultrasonography, computed tomography, or magnetic resonance imaging. Forty-three percent of the patients underwent more than one radiological examination. Twenty patients underwent surgical management. The primary indication of surgical treatment was aesthetic reasons (11/20, 55%). Thrombosis was the most common EJVA complication (11/30, 36.3%).
Conclusions
Differential diagnosis of neck mass should include EJVA. High clinical suspicion and adequate imaging are important for diagnosis. Open surgical approach is the more commonly applied therapeutic strategy.
Introduction
Jugular vein system aneurysms and ectasias are uncommon pathologic lesions that may complicate differential diagnosis during neck mass investigation.1–3 Clinical presentation may be typical, although the low clinical suspicion among clinicians may hamper definite diagnosis. 3 In most patients, degeneration is the main etiopathologic mechanism, while trauma cannot be excluded. 3
While external jugular vein aneurysms (EJVAs) are considered benign clinical entities, they may be implicated by thrombotic events, as aneurysms thrombosis and pulmonary embolism. 1 , 3 Aesthetic reasons and aneurysm complications set the indication for treatment according to the current literature.3,4 Surgical excision has been applied without complications in many cases of EJVA. 1
The aim of this study was to present a systematic review of the current literature regarding the diagnosis and management of EJVAs.
Methods
Eligibility criteria
The present review was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement (PRISMA) guidelines. 5 Data extraction was performed by two independent reviewers (N.P., C.K.) using a non-blinded standardized form and any discrepancies were resolved by consulting a third reviewer (G.K.). No informed consent or institutional review board approval was required. Studies considered for inclusion and full text review fulfilled the following criteria: (1) to report on patients that were treated for EJVA (2) to provide information on clinical presentation, imaging, and management (3) to be published after 2000. The primary selection was based on title and abstract and a secondary scrutiny on the full text.
Search strategy
A data search of the current medical literature was conducted, using Pubmed/Medline (U.S. National Library of Medicine, Bethesda MD – USA) and Scopus (Elsevier, Amsterdam – Netherlands) databases. The starting date was 1 January 2000 while the search end date was set to 31 May 2020. The P.I.C.O. (patient; intervention; comparison; outcome) model was used to define the clinical questions and select relevant articles (Table 1). 6 The following search terms including Expanded Medical Subject Headings (MeSH) were used in various combinations: “aneurysm” or “external jugular vein aneurysm” or “jugular aneurysm” or “jugular ectasia”.
P.I.C.O. (patient; intervention; comparison; outcome) model was used to define the clinical questions and clinically relevant evidence in the literature.
EJVA: external jugular vein aneurysm.
Data extraction and quality assessment
A standardized data extraction Microsoft Excel file was developed. Data were retrieved from the text or tables. Extracted data included study characteristics such as author and date of publication. Furthermore, the following clinical information was collected: baseline demographics (age, sex), type of aneurysm (saccular, true aneurysm), symptoms (pain, neck swelling, soft mass), imaging modality (ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI), venography), aneurysmal complications (thrombosis, pulmonary embolism, rupture), management approach (surgical; open or endovascular and conservative treatment), technical success, and histological findings.
The quality of case reports and case series was assessed using the CARE (Case Reports Guidelines). CARE system was created to support an increased accuracy, transparency, and usefulness of case reports. 7
Definitions
The exact definition of true venous aneurysm remains controversial, while there is no size criterion in the literature to describe a venous dilatation as an aneurysm. True venous aneurysm is usually defined as a persistent isolated venous dilatation twice the normal diameter that occurs in a non-varicose vein and is not associated with pseudo-aneurysms or arteriovenous communications. 8 External jugular vein diameter is estimated at 9 mm, according to the current literature. An external jugular vein diameter of 20 mm could be used as a threshold to characterize an aneurysm. 8 Vein dilatations that did not exceed twice the diameter were characterized as ectasias. All cases were according to the reference standard.
Outcomes
Clinical presentation, aneurysm complications, imaging modalities, therapeutic management, and technical success were assessed as outcomes.
Statistical analysis
Descriptive data are presented, since this PRISMA systematic review is not comparative.
Results
Study selection
The search yielded 163 manuscripts in total, while the first case reported in the literature was recorded. No additional manuscripts were collected from other sources. After removing duplicate results, 122 manuscripts were further screened. Out of these 122 manuscripts, 63 could not be retrieved or only limited information (e.g. abstract, medical image) was available, rendering them unsuitable for this review. Out of the 59 manuscripts left, 14 were also excluded as they were irrelevant. Finally, 28 manuscripts were included (Figure 1).4,8–35 One of these manuscripts was a case series reporting on three patients, while the rest of the publications were case reports.
Out of 122 manuscripts, 63 could not be retrieved or only limited information (e.g. abstract, medical image) was available, rendering them unsuitable for this review. Finally, 28 manuscripts were included. One of these manuscripts was a case series, while the rest of publications were case reports.
Study population
In 29 patients, a unilateral aneurysm was presented, while in one case (3.3%), a bilateral presentation was recorded (in total, 30 patients and 31 EJVAs). In 10 patients, the aneurysm was located at the right supraclavicular region and in equal cases, at the left side. In nine cases, the lateralization was not reported. Ten patients were younger than 18 years. The mean age was calculated at 32 years (range 1–72 years), while 54% of patients were females. True aneurysms were reported in 51% according to histological assessment. No history of trauma was mentioned. A saccular configuration was described in six cases.
The presence of a neck mass was the most common clinical sign. A non-pulsatile, non-sensitive, soft mass was referred in more than half of patients (63.3%), while rare symptoms such as pulsatile mass (1/30 patients) was also described. Pain was detected only in cases of aneurysm thrombosis (11/30, 36.6%). In 66.7% patients, there was a significant enlargement of the lesion during the Valsalva maneuver (Table 2); 53.3% of cases underwent US evaluation, 50% CT and 13.3% MRI. Venography was performed only in one case where diagnosis was controversial. Despite the excellent sensitivity of US in the detection of these aneurysmal lesions, 43.3% of the patients underwent more than one radiological examination to confirm the diagnosis. In 69.2% of them, a US/CT combination was chosen (Table 3).
The presence of a neck mass was the most common clinical sign.
EJVA: external jugular vein aneurysm; NR: not reportedNote: Pain was detected only in cases of aneurysm thrombosis. In 66.7% patients, Valsalva maneuver was positive.
Despite US excellent sensitivity in the detection of these aneurysmal lesions, 43.3% of the patients underwent two or more radiological examinations to confirm the diagnosis.
Indications of treatment included aesthetic reasons as well as the prevention of further aneurysm complications (local thrombosis, pulmonary embolism, and rupture). In total, a surgical management is reported in 20 cases. Eleven patients (11/20, 55%) underwent surgical management due to aesthetic reasons, while the remaining cases (9/20) underwent surgery for aneurysm thrombosis. Thrombosis was presented in 11 patients (36.6%), while only one patient suffered a pulmonary embolism (3.3%). The patient that underwent venography did not suffer any thrombotic event. Aneurysm rupture was reported in one case (3.3%). The patient that suffered the aneurysm rupture was diagnosed with a hemangiothelioma of the external jugular vein during the histopathological evaluation (Table 4). In 17 out of 20 cases that underwent surgical management, an excision of the lesion was performed, while in one case (5%), an endovascular treatment with coil deployment was used. In two cases (2/20), no intra-operative details regarding the surgical management was provided No post-operative complication due to surgical management of the EJVA was recorded. In 6 out of 30 patients, a conservative approach with clinical and imaging surveillance was decided. In one case, aneurysm thrombosis – a combination of anticoagulation and surgical excision – was described. Heparin and oral anticoagulants (vitamin K antagonists) were used for EJVA thrombosis as medical treatment (one week up to three months or as a bridging before surgery).
Indications of treatment included aesthetic reasons (9/20 cases) as well as the prevention of other complications.
Note: Thrombosis was presented in 36.6% of patients, while only one patient suffered a pulmonary embolism (3.3%). Aneurysm rupture was also rare, while the same patient was diagnosed with a concomitant hemangiothelioma.
Discussion
Only case reports and case series were retrieved from databases. Regarding the etiopathological mechanisms, degenerative or congenital vein malformations are described in the current literature. 11,12,16,22 This may be a rational etiologic hypothesis explained by the young age in almost one-third of patients (<18 years). Furthermore, histopathological evaluations revealed a disorder of the elastin fibers architecture. 35 A progressive degeneration of the vein wall may explain the elder age of the remaining cases. Additionally, trauma, because of previous medical intervention, may induce jugular pseudoaneurysm. 15 Differential diagnosis includes external laryngeal diverticulae, superior mediastinum tumor, lung cupula, cystic lymphangioma, and hemangioma.11,22,27,30 While the presence of a neck vein aneurysm is often considered an innocent pathology, aneurysm thrombosis and its consequences have been reported. 16 Thrombosis was presented in 11 patients. Furthermore, a recurrent episode may conceal a neoplastic lesion. 10 Histopathological evaluation is mandatory for the detection of an underlying malignancy. 10
Clinically, patients refer a non-pulsatile, non-tender, soft mass, expanding on Valsalva maneuver. Noninvasive imaging methods, as US can be easily and safely performed and confirm diagnosis. 16 However, 43.3% of patients underwent further evaluation with CT and/or MRI.4,8–10,17–22,27,30 The low clinical suspicion may affect surgeon’s decision for the appropriate diagnostic imaging modality, while it must be acknowledged that CT and MRI permit a more detailed evaluation of aneurysm’s anatomical characteristics. As many patients with EJVA are young, radiation harm as well as higher cost and intravenous contrast enhanced should be taken under consideration before CT evaluation.
In this study, most patients (20/30) with EJVA underwent a surgical treatment.8–13,15,18–20,22–25,27,28,30–32 An excision of the lesion and ligation of the external jugular vein has been performed in most cases. In one case, an endovascular minimally invasive approach with a direct puncture of the aneurysm and coil embolization was accomplished, as the patient was treated only for aesthetic reasons and no incision was needed. 31 Considering the higher cost of an endovascular intervention and minimal character of an open surgical excision, the use of endovascular means may be not justified in such cases. Furthermore, conservative management may be a safe alternative.15,17,18,22,29 In cases of aneurysm thrombosis, anticoagulation with heparin or oral anti-coagulants, as vitamin K antagonists, has also been reported and applied successfully, despite the lack of evidence.4,29
Limitations
The quality of evidence was based only on case series and case reports, as a result of the rarity of the disease; hence, it is estimated to be very low. No specific definition of EJVA exists, as a baseline diameter is difficult to be defined. No size criterion was reported regarding surgical intervention or thrombosis in the current literature. Different diagnostic tests or combinations of them were used in each case. Furthermore, current medical management with the use of safe pharmacological factors probably would differentiate the approach of complicated EJVA in the future. Surgical management was preferred in most cases for aesthetic reasons before the evolution of further symptoms and this fact may have affected the complication rate associated to the underlying pathology.
Conclusion
Differential diagnosis of neck mass should include EJVA. High clinical suspicion and adequate imaging are important for diagnosis. Open surgical approach is the commonest more commonly applied therapeutic strategy.
Footnotes
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
