Literature DB >> 32642183

Brachiocephalic vein aneurysm: a systematic review of the literature.

Quynh Nguyen1, Jacqueline K Olive2, Dominique Vervoort3, Kevin Phan4, Jessica G Y Luc5.   

Abstract

Brachiocephalic vein aneurysms are rare lesions with only 36 cases reported in the literature. They usually present incidentally as mediastinal widening on chest X-ray, with thromboembolism or mass effect on adjacent structures, or rupture. Imaging is usually sufficient to identify and characterize the aneurysm, however, certain diagnostic pitfalls can lead to misinterpretation and misdiagnosis. Exploratory surgery is sometimes needed to confirm diagnosis. Brachiocephalic vein aneurysms have been treated both conservatively with watchful waiting, antithrombotic therapy or anticoagulation as well as surgically depending on patient presentation and aneurysm characteristics. Endovascular treatment is also becoming a therapeutic option. Prognosis following surgical treatment is excellent with no reported cases of recurrence. The present systematic review aims to describe the etiology, clinical presentation, diagnosis, management and outcomes of brachiocephalic vein aneurysms. 2020 Journal of Thoracic Disease. All rights reserved.

Entities:  

Keywords:  Brachiocephalic vein aneurysms; innominate vein aneurysms; venous aneurysms

Year:  2020        PMID: 32642183      PMCID: PMC7330298          DOI: 10.21037/jtd.2020.04.39

Source DB:  PubMed          Journal:  J Thorac Dis        ISSN: 2072-1439            Impact factor:   3.005


Introduction

Brachiocephalic vein (or innominate vein) aneurysms are extremely rare. To date, there have only been 36 cases reported in the literature. Brachiocephalic vein aneurysms are more common on the left than on the right side and are often saccular rather than fusiform (1-11). The majority of brachiocephalic vein aneurysms are asymptomatic and discovered incidentally on imaging, though some may present with mass effect on adjacent structures or rupture (2,10-17). As such, the basis of therapy is to prevent aneurysmal progression, thromboembolism or mass effect on adjacent structures, or rupture. While multiple treatment options are available, established guidelines regarding therapy for brachiocephalic vein aneurysms are lacking. The present systematic review aims to describe the etiology, clinical presentation, diagnosis, current management options and outcomes of brachiocephalic vein aneurysms.

Etiology

With the exception of trauma and iatrogenic causes, the true etiology of brachiocephalic vein aneurysms is not well understood. A number of conditions are thought to be associated with brachiocephalic vein aneurysms, including, but not limited to congenital malformation (28%, 10/36 cases) (1,2,4,5,9,12-14,18), hemangioma (8%, 3/36 cases) (5,19,20), hygroma (3%, 1/36 cases) (21), neurofibromatosis type 1 (NF1) (3%, 1/36 cases) (22), a history of vascular intervention (3%, 1/36 cases), tumor retraction (3%, 1/36 cases) (19), and degeneration of the vessel wall (3%, 1/36 cases) (23). Congenital defects in vessel structure may cause brachiocephalic vein aneurysm, as described in 28% of patients (10/36 cases) (1,2,4,5,9,12-14,18). These have been reported in association with the histological absence of smooth muscle cells in the aneurysm wall or absence of the adventitia’s longitudinal muscle layer, supporting congenital weakness of the vessel wall as a potential underlying cause (4,24). An association between brachiocephalic vein aneurysms and hemangiomas has also been described in approximately 8% of patients (3/36 reported cases) (5,19,20). Nitta et al. reported a case of congenital left and right brachiocephalic vein aneurysms in the setting of angiomatosis, a diffuse form of hemangioma (5,25,26). Akiba (19) and Nakada et al. (20) have also reported cases of thymus cavernous hemangioma in association with left brachiocephalic vein aneurysm. Pathological examination showed a transitional portion between the left brachiocephalic vein and cavernous hemangioma, and the tumor appeared to retract the lower portion of the left brachiocephalic vein (19). Brachiocephalic vein aneurysms have also been reported in association with mediastinal cystic hygromas (3%, 1/36 cases) (21). Among 15 cases of mediastinal cystic hygroma, eight patients were found to have venous aneurysms in the neck and thorax (27). The association between hygroma and venous aneurysms has been attributed to the close embryologic relationship between the lymphatic and venous systems (28). Finally, there has been one report (3%) of brachiocephalic vein aneurysm as a manifestation of NF1 (22). Pathological examination of the resected aneurysm demonstrated diffuse neurofibroma with an infiltrative pattern. While cardiac and peripheral vascular problems are known clinical complications of NF1 (29), venous aspects of the disorder are poorly understood. Only three other cases of venous aneurysms have been reported in the setting of NF1, all of which involved the internal jugular vein (29-31). Although venous aneurysms are extremely rare manifestations of NF1, they remain a possible finding.

Presentation

The majority of patients with brachiocephalic vein aneurysms are asymptomatic (36%, 13/36 cases) () (2,10-17).
Table 1

Presentation, methods of diagnosis, aneurysm size and location of previously described cases of brachiocephalic vein aneurysms

#Ref.YearAge (yrs)SexClinical presentationVenous aneurysmsDiagnostic findings
BCVTypeSize (mm)OthersCXR
1Harris (1)19285 moFSwelling on R side of neck, spasmodic cough, hoarse cry, cyanosisLSR internal jugularMediastinal shadow in thymus region
2Yokomise et al. (2)199013MAsymptomaticLS50×40SVCMediastinal shadow, shift of cardiac shadow to the L
3Pasic et al. (4)199518FAsymptomaticL, RS70×60×50SVCLarge, R paratracheal mass, partially calcified along superior aspect
4Nitta et al. (5,25,26)2005, 2006, 20081 dMRespiratory arrestL, RS (L), F (R)NoneMediastinal widening, R pneumothorax
5Hosein et al. (6)200713FNonproductive coughLS200×150NoneLarge superior mediastinal mass
6Sakai et al. (7)201148FLNoneMediastinal mass
7Sayed et al. (8)201345FCough, dyspneaLS120×120×80NoneSuperior mediastinal mass extending into L upper thoracic region, displacing upper pole of L lung
8Huang and Jiang (9)201757MLS30×35None
9Galvaing et al. (10)201872MAsymptomaticLS66×42×56None
10Shen et al. (11)201963FAsymptomaticLS47×31None
11Cai et al. (3)201943MAsymptomaticLS61×106NoneAnterior mediastinal mass
12Rappaport et al. (12)199220MAsymptomaticLSVC, azygos, hemiazygos, L inferior pulmonaryMediastinal widening
13Haniuda et al. (13)200063FAsymptomaticLS30None
14Haniuda et al. (13)200021FAsymptomaticRS40NoneR superior mediastinal mass
15Tsuji et al. (14)200416FAsymptomaticL, RS (L), F (R)110 (L), 40 (R)NoneAbnormal shadows on L & R superior mediastinum
16Mikroulis et al. (15)201060MAsymptomaticRFNoneMediastinal widening
17Dua et al. (16)201142FAsymptomaticLS70NoneSoft tissue mass in L hilar region, partially obscuring L cardiac border
18Hayashi et al. (17)201133FAsymptomaticL60×45None
19Moncada et al. (18)198523MNon-painful swelling at R sternoclavicular jointL25NoneUnremarkable
20Akiba et al. (19)201227MCoughLNoneL mediastinal widening
21Nakada et al. (20)201543MChest painLFNoneAbnormal chest shadow
22Gorenstein et al. (21)19920 dFLFSVC, R internal jugularMediastinal mass
23Bartline et al. (22)201658FPain, dyspnea, dysphagia, hoarsenessR71R internal jugular, R subclavian
24Newell et al. (23)198320MNonspecific retrosternal chest pain, sometimes associated with epigastric painL35NoneMediastinal widening
25Burkill et al. (28)199721FUnrelated findingsLSNoneMediastinal widening
26Davies and Roberts (32)199850FPain and swelling over R supraclavicular & shoulder area; dilated superficial veinsRSVCMediastinal widening
27Bansal et al. (33)201042FNonspecific pain in L infraclavicular region, dyspnea, palpitationLSNoneSuperior mediastinal widening secondary to a mass
28Buehler et al. (34)201384FBack pain radiated to neck, nausea, vomitingLS83×67NoneLarge pericardiac density extending from below aortic arch to diaphragm
29Lohrenz et al. (35)201825FNonproductive cough, dyspnea, thoracic pressure, pain in left arm, exacerbated when leaning forwardLS64–69NoneWell-defined, homogeneous mass in anterior mediastinum
30Hosaka et al. (36)201170FHoarseness, worsening coughLS40None
31Aggarwal et al. (37)201720MNonproductive coughLSNoneAnterior mediastinal mass
BCVTypeSize (mm)OthersCXR
32Pellizzari et al. (38)200887MUnrelated findingsLS13×60×22NoneHeart shadow enlargement, no mediastinal widening noted
33van der Vorst and Veger (39)201974MLS70None
34Okay et al. (40)197016FAsymptomaticLSSVCMediastinal widening
35Marmolya & Yagan (41)198947ML24NoneIsolated oval density in anterior mediastinum
36Güney et al. (42)200424MLower neck mass that enlarged with Valsalva manoeuvreL, RR internal jugularMediastinal widening, anterior mediastinal mass

F, female; M, male; mo, month; d, day; L, left; R, right; BCV, brachiocephalic vein; S, saccular; F, fusiform; SVC, superior vena cava; CXR, chest X-ray; CT, computed tomography; MRI, magnetic resonance imaging.

F, female; M, male; mo, month; d, day; L, left; R, right; BCV, brachiocephalic vein; S, saccular; F, fusiform; SVC, superior vena cava; CXR, chest X-ray; CT, computed tomography; MRI, magnetic resonance imaging. When symptomatic, brachiocephalic vein aneurysms present with swelling over the supraclavicular and shoulder area (8%, 3/36 cases) (1,18,32), pain (19%, 7/36 cases) (20,22,23,32-35), and cough (19%, 7/36 cases) (1,6,8,19,35-37). Additionally, large or thrombosed aneurysms can have a mass effect on adjacent mediastinal structures, causing other symptoms such as hoarseness (8%, 3/36 cases), dyspnea (11%, 4/36 cases), and respiratory arrest (3%, 1/36 cases).

Diagnosis

Brachiocephalic vein aneurysms are often incidental findings (56%, 20/36 cases). Widening of the mediastinum (28%, 10/36 cases) or presence of a mediastinal mass (47%, 17/36 cases) are the most common findings on chest radiographs. When incidental thoracic masses are suspected, they are often further characterized by computed tomography (CT) (conventional, contrast-enhanced CT or 3D CT) or venography [conventional, CT venography, or magnetic resonance (MR) venography]. Other imaging modalities for the diagnosis of brachiocephalic vein aneurysms include echocardiography, MR imaging (MRI) or duplex ultrasound. In some cases, more invasive approaches such as exploratory thoracoscopy are necessary to confirm diagnosis (13). On results from cross-sectional imaging methods such as CT, contrast pooling, homogeneous enhancement similar to adjacent venous structures, and continuity with the thoracic veins are often indicative of venous aneurysms (43). Although contrast-enhanced CT is often sufficient to arrive at a diagnosis of brachiocephalic vein aneurysm, common imaging pitfalls are misdiagnosis of the aneurysm as either (I) a solid mediastinal tumour (1,34) or (II) a thymoma (9). As such, should there be any suspicion for misdiagnosis; further workup should be supplemented with the use of multi-modality imaging techniques such as venography, MRI, or transthoracic Doppler study for operative planning.

Differential diagnosis

There is a wide spectrum of potential diagnoses for a mediastinal mass including thymoma, lymphoma, teratoma, neurofibroma, ectopic thyroid gland, lung neoplasm, and arterial aneurysm, among others (44). Despite its rarity, brachiocephalic vein aneurysm should be considered as a differential diagnosis upon discovery of a mediastinal mass, especially in the context of previously reported associations, including congenital malformations (1,2,4,5,9,12-14,18), hemangioma (5,19,20), hygroma (21), neurofibromatosis type 1 (NF1) (22), a history of vascular intervention and tumor retraction (19).

Management and outcomes

While multiple treatment options are available, established guidelines regarding therapy for brachiocephalic vein aneurysms are lacking. Treatment is largely determined by clinical presentation, characteristics of the aneurysm, patient decisions, and surgical candidacy. Current treatment approaches include conservative management and surgery.

Conservative management

Conservative management was reported in 12/28 cases (43%) ().
Table 2

Conservative treatment approaches and outcomes of brachiocephalic vein aneurysms

#Ref.YearAntithromboticComplicationsLength of hospital stayFollow-up
1Tsuji et al. (14)2004NoneNone2 year: dilatation of the aneurysm was not observed
2Mikroulis et al. (15)2010Antiplatelet (ASA)None15 year: asymptomatic
3Dua et al. (16)2011
4Moncada et al. (18)1985
5Newell et al. (23)19831 year: no complications
6Bansal et al. (33)2010
7Buehler et al. (34)2013
8Lohrenz et al. (35)2018NoProgression in thoracic discomfort and shoulder/arm pain; recurrent bronchopulmonary infectionSurgery needed
9Hosaka et al. (36)2011WarfarinNone1.5 months: aneurysm decreased in size, calcified along its periphery, reduced intraluminal thrombus (contrast-enhanced CT)
8 months: aneurysm and intraluminal thrombus sizes further decreased. Improved symptoms
1 year: plasma CRP within normal range, D-dimer decreased to 0.09 ug/mL
10Aggarwal et al. (37)2017Low molecular weight heparinUnexplained sudden onset syncope, cyanosis and respiratory distress3 daysSurgery needed
11Pellizzari et al. (38)2008Antiplatelet
12van der Vorst and Veger (39)2019Direct oral anticoagulant (apixaban)

CT, computed tomography; CRP, C-reactive protein.

CT, computed tomography; CRP, C-reactive protein. This approach has been suggested as a reasonable option for patients who are asymptomatic with small, non-enlarging brachiocephalic vein aneurysms (6,14,16). The majority of patients treated conservatively had saccular brachiocephalic vein aneurysms (75%, 9/12 cases). The rest had either fusiform brachiocephalic vein aneurysms (17%, 2/12 cases) or the aneurysm type was not identified. The conservative approach is also recommended for patients who are poor surgical candidates (34) or those who do not wish to receive more invasive treatment (33). Upon presentation of thrombotic material, antithrombotic therapy should be discussed. Of the 12 patients who received conservative treatment, 4 (33%) had no complications, 2 (17%) required urgent surgery, and information on follow-up for the remaining 6 (50%) was not available.

Observation only

Conservative treatment with observation only, was reported in 7 cases (58% of patients who received conservative treatment). Of these patients, 3 had no thrombi (16,33,35), 1 had thrombus but was a poor surgical candidate (34) and the presence or absence of thrombi was not mentioned in 3 cases (14,18,23). At a 2 year (14) and 1 year follow-up (23), complications or dilation of the aneurysm were not observed in two cases (14,23). During the 2 year follow-up, one case experienced worsening of symptoms and required explorative surgery (35). Follow-up information was not mentioned for the remaining four cases (16,18,33,34).

Antiplatelet therapy

Two cases (17%) of antiplatelet therapy for brachiocephalic vein aneurysm have been described (15,38). One case was treated with ASA at 160 mg/day (15), while information on the antiplatelet drug and dosing regimen for the other case is not available (38). One patient was lost to follow-up (38) and the other was on antiplatelet treatment for 15 years and remained asymptomatic (15).

Anticoagulation

Anticoagulation with warfarin, low molecular weight heparin or direct oral anticoagulant (apixaban) have been reported in 3 cases in the management of brachiocephalic vein aneurysms (36,37,39). Of the 3 cases treated with an anticoagulant, 2 had thrombosed aneurysms (36,37) whereas 1 was continued on apixaban given pre-existing atrial fibrillation (39). One patient experienced successful reduction in size of the intraluminal thrombus and the aneurysm, as evaluated by contrast-enhanced CT at 1.5 and 8 months post-treatment. One patient treated conservatively with low-molecular weight heparin (37) developed sudden onset syncope, cyanosis and respiratory distress from the aneurysm, requiring emergency surgery. One patient was lost to follow-up (39).

Surgery

Surgery with aneurysmectomy and repair was performed in 57% of patients (16/28 cases) for brachiocephalic vein aneurysms that were symptomatic (19%, 3/16 cases) (5,8,22), saccular (69%, 11/16 cases) (1-11), expanding (12%, 2/16 cases) (6,22), or containing intraluminal thrombi (44%, 7/16 cases) (4-8,11,37) ().
Table 3

Surgical treatment approaches and outcomes of brachiocephalic vein aneurysms

#Ref.YearSurgical approachCardiopulmonary bypass (CPB) requiredMethod of repairAnti-thromboticComplicationsHospital stayFollow-Up
1Harris (1)1928Transverse incision across the R sternomastoid 1in above the clavicleAneurysm was resectedPatient died of intra-operative cardiac and respiratory failure
2Yokomise et al. (2)1990Sternotomy combined with 5th intercostal thoracotomyAneurysm was resected. The proximal end of the resection line was closed with 5-0 prolene suture1 month3 weeks: no stenosis noted (angiography)
3Pasic et al. (4)1995SternotomyPartial CPB with cannulation in the ascending aorta and proximal LBCVAneurysm was opened longitudinally. Aneurysmal wall partially resected. Mediastinal venous system reconstructed using rest of aneurysmal wall and running 4-0 polypropylene suturesLow-dose heparin, warfarinNone
4Nitta et al. (5,25,26)2005, 2006, 20081st attempt: resection of LBCV aneurysm, discontinued due to complication; 2nd attempt: thymectomy, left subclavian vein ligation and jugular vein ligationSurgery discontinued due to extremely large aneurysm that bled easily58 days35 days: respiratory support with a ventilator not needed
51 days: reduction of LBCV aneurysm, development of collateral vein (angiography)
3 months: recurrent respiratory arrest. LBCV aneurysm with large collateral veins surrounding and enclosing the trachea. SVC aneurysm of the same size (which turned out to be RBCV aneurysm on autopsy) (angiography)
22 months: death due to chronic respiratory failure
5Hosein et al. (6)2007SternotomyYesAneurysm was opened longitudinally. Aneurysmal wall was resected. The underside of LBCV was reconstructed using a bovine pericardium patchNone
6Sakai et al. (7)2011Aneurysm was resectedNone
7Sayed et al. (8)2013Left thoracotomyNoAneurysm was excised including a rim of normal tissue of the BCV surrounding the neck of the aneurysmNone4 months: asymptomatic. No recurrence (angiography)
8Huang and Jiang (9)2017SternotomyAneurysm was resected. BCV was reconstructed with 5-0 prolene suturesNone2 years: no complications
9Galvaing et al. (10)2018SternotomyNoThymectomy was performed. Aneurysm was dissected. Its neck was identified and resected using an endostapler with a vascular loadNone3 days3 months: patient recovered completely from the procedure; no abnormalities noted (contrast-enhanced CT)
10Shen et al. (11)2019SternotomyNoAneurysm was opened longitudinally and resected. The defect on the underside of the BCV was closed by running 6-0 polypropylene suturesNone1.5 years: asymptomatic, no recurrence noted (contrast-enhanced CT)
11Cai et al. (3) 2019 EndovascularSelf-expanding stents were placed across the aneurysm. 2 interlock coils were inserted to embolize the L internal jugular veinWarfarin1 week1 month: complete thrombus within aneurysm sac (contrast-enhanced CT)
3-6 months: increased blood flow within aneurysm sac. Anticoagulant therapy discontinued
12 months: complete thrombus formed within aneurysm sac, intraluminal thrombus formed around the stents
18 months: aneurysm decreased in size, intraluminal thrombus increased. Patient was asymptomatic, no pulmonary embolism (pulmonary CT angiography)
12Akiba et al. (19)2012SternotomyTotal thymectomy combined with aneurysm resection. Remaining BCV was closed with 5-0 prolene sutureNone
13Nakada et al. (20)2015L-shaped sternotomy with a left-sided cervical collar incisionPartial resection of the LBCV aneurysm using a staplerPost-operative left phrenic nerve paralysis9 days5 months: no evidence of recurrence or further enlargement of the BCV
14Bartline et al. (22)2016SternotomyYesAneurysm was resected. Internal jugular vein was ligated. Femoral cryopreserved vein conduit was used for venous reconstructionIntraoperative acute right HF, requiring placement of a temporary RVAD; right HF 2 months post-operation, requiring hospital admission2 months: patency of the axillary, subclavian, and cryopreserved vein conduit (duplex ultrasound)
15Lohrenz et al. (35)2018Minimally invasive thoracoscopyNoAneurysm was drained by local compression followed by cross-clamping of the aneurysm base. Aneurysm was resected and repaired by endostaplerLow molecular weight heparinNone1 weekPost-operative: normal flow in LBCV (MRI)
16Aggarwal et al. (37)2017SternotomyYesLBCV was ligated into SVC. Aneurysm was opened, thrombus was removed. Aneurysmal wall was resectedNone3 weeks6 months: asymptomatic

L, left; R, right; BCV, brachiocephalic vein; SVC, superior vena cava; CT, computed tomography; MRI, magnetic resonance imaging; RVAD, right ventricular assist device; HF, heart failure.

L, left; R, right; BCV, brachiocephalic vein; SVC, superior vena cava; CT, computed tomography; MRI, magnetic resonance imaging; RVAD, right ventricular assist device; HF, heart failure. Surgery was also performed to confirm diagnosis (12%, 2/16 cases) (20,35), to prevent possible major complications such as thromboembolism, rupture, or venous compression with subsequent obstruction (38%, 6/16 cases) (2-4,8,10,22), or to address aneurysmal complications (12%, 2/16 cases) (35,37).

Surgical approaches

Among the reported cases, median sternotomy was the most common surgical approach (56%, 9/16 cases) given its versatility and ease for use with cardiopulmonary bypass (CPB) (2,4,6,9-11,19,22,37), followed by thoracotomy (12%, 2/16 cases) (2,8) and thoracoscopy (6%, 1/16 cases) (35). CPB was established in 4 cases (25% of patients who received surgical treatment) to provide clear anatomic details and mobilization of the aneurysm, prevent excessive blood loss, reduce the risk of embolization, and allow for decompression of the cerebral venous system in preparation for superior vena cava cross-clamping, if necessary (4,6,11,22). Endovascular treatment is also becoming a new therapeutic approach for patients with brachiocephalic vein aneurysms, as one patient (6%) was successfully treated with stent placement and coil embolization of the left brachiocephalic vein (3).

Operative outcomes

Brachiocephalic vein aneurysms were successfully resected in 81% of patients (13/16 cases). Intra-operative complications were reported in 3 cases (19%) (1,5,22,25,26). One patient died during an operation due to cardiac and respiratory failure (1). Nitta et al. reported another case where the surgery was discontinued due to friability of a large aneurysm (5). To reduce the size of the aneurysm, a thymectomy with left subclavian and jugular veins ligation was performed instead. Bartline and colleagues also described a case of intra-operative right heart failure that required implantation of a temporary right ventricular assist device (22). Post-operative complications were reported in two cases (13%), including left phrenic nerve paralysis (20) and right heart failure requiring hospitalization (22). Patients spent 3 to 58 days in the hospital after surgical resection of brachiocephalic vein aneurysms (2,3,5,10,20,35,37). Post-operative anticoagulants (19% of patients) included heparin (4), low molecular weight heparin (35), and warfarin (3,4). One patient had heparin with bridging to warfarin for 3 months following surgery (4). One patient was on low molecular weight heparin once daily for 7 days post-operatively (35). And another patient received warfarin for 3-6 months following endovascular treatment of the brachiocephalic vein aneurysm (3). The majority of patients (69%, 11/16 cases) had completion of follow-up. No complications or recurrence were noted in 8 cases (50%) (2,8-11,20,22,35). Symptoms were resolved in 3 cases (19%) (8,11,37). One patient died of chronic respiratory failure (26). Endovascular repair demonstrated aneurysmal shrinkage on chest CT 18 months after intervention, although increased intraluminal thrombus size was observed (3). The prognosis of brachiocephalic vein aneurysms is good post resection with no reported cases of recurrence.

Conclusions

Brachiocephalic vein aneurysms are rare vascular lesions that often present asymptomatically as a widening of the mediastinum on the chest radiograph. Surgical aneurysmectomy is indicated in patients with symptomatic, saccular, expanding brachiocephalic vein aneurysms; those containing intraluminal thrombi; and those presenting with complications such as recurrent thromboembolism, rupture, or mass effect on surrounding structures. Surgical outcomes are acceptable with favorable prognosis post-resection and no recurrence reported. The article’s supplementary files as
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