Skip to main content
Download PDF

Unprovoked internal jugular vein thrombosis: a case report and literature review

Thrombosis Journal volume 19, Article number: 2 (2021) Cite this article

Abstract

Background

Managing thrombosis in rare sites is challenging. Existing studies and guidelines provide detailed explanations on how to overcome lower-limb thromboses and pulmonary embolisms, but few studies have examined thrombosis in rare sites. Lack of data makes clinical practice heterogeneous. Recommendations for diagnosing, treating, and following-up internal jugular vein thrombosis are not clearly defined and mostly based on adapted guidelines for lower-limb thrombosis.

Case presentation

A 52-year-old Caucasian woman came to the Emergency Department with chest, neck, and left arm pain. Computed tomography imagery showed a left internal jugular vein thrombosis. An extensive workup revealed a heterozygous factor V Leiden gene. Therapy was initiated with intravenous unfractionated heparin, then switched to oral acenocoumarol, which resolved the symptoms. Based on this case presentation and a literature review, we summarize the causes, treatment options, and prognosis of unprovoked internal jugular vein thrombosis.

Conclusions

Managing internal jugular vein thrombosis lacks scientific data from large randomized clinical trials, partly because such thromboses are rare. Our literature review suggested that clinical treatments for internal jugular vein thrombosis often followed recommendations for treating lower-limb thrombosis. Future specific studies are required to guide clinicians on the modalities of diagnosis, screening for thrombophilia or oncologic disease, treatment duration, and follow-up.

Background

Thromboembolic disease is the third most frequent cardiovascular disease [1]. It has been thoroughly studied in recent decades, resulting in a standard diagnostic strategy, international guidelines, and new medications [2, 3].

There is scarce medical literature available on unprovoked internal jugular vein (IJV) thrombosis, essentially just case reports and short case series. IJV thrombosis is, therefore, often managed with reference to guidelines dedicated to thromboses occurring in more common sites, notably the deep veins of the lower limbs [2, 3]. Yet unusual thrombosis sites are associated with distinct risk factors and complications, and some treatments (e.g. direct oral anticoagulants) have not been tested specifically. Thus, we believe that specific recommendations are needed to guide the treatment management of patients with thromboses in uncommon sites.

This report describes a case of unprovoked IJV thrombosis and an accompanying literature review about diagnosing and treating this condition.

Case presentation

A 52-year-old Caucasian woman under treatment for hypothyroidism arrived at the Emergency Department with constrictive chest pain that had been radiating into her left arm and cervical region for 1 week, accompanied by new-onset dyspnea which had worsened 2 days before her medical visit. Her physical examination was unremarkable, except for high blood pressure (188/104 mmHg) and excess weight (body mass index = 28 kg/m2); a blood test showed a high D-dimer concentration (2170 μg/l). A computed tomography (CT) scan performed to exclude pulmonary embolism was inconclusive. However, fat infiltration around the left jugular-carotid led to a further investigation using neck ultrasound. This showed a thrombosis emerging from the base of the left subclavian vein and extending 11 centimeters into the IJV, sparing the cerebral vessels.

The patient described spontaneous hair loss and a 10 kg gain in weight over the previous year. She was taking levothyroxine but took no other medication or hormonal substitutes. There was no history of smoking, recent surgery, trauma, infectious disease, intravenous medical or recreational drug use, or past catheter insertion.

A chest CT scan and a colonoscopy revealed no cancer. A recent gynecological check-up consisting of a physical examination, a mammography, and a Pap smear found no pathology. A thyroid-stimulating hormone test and urinary cortisol were normal. Because of the patient’s young age and no obvious risk factor for thromboembolic disease, we completed the investigations with a thrombophilia workup. Antinuclear antibodies were positive at 1:320, but without other criteria suggesting overt lupus [4]. Otherwise, antinucleoprotein antibodies (SSA, SSB, RNP, Sm, Scl70, Jo1), antinucleosome antibodies, anticardiolipin antibodies, and anti-B2-glycoprotein antibodies were negative. We did not test for lupus anticoagulant due to the patient’s anticoagulation treatment and the risk of a false-positive [5]. Antithrombin, protein C, and protein S activity were within the normal ranges and were tested before the introduction of anticoagulant treatment. Only a heterozygous factor V Leiden gene (R506Q) was found positive. We concluded on the diagnosis of an unprovoked IJV thrombosis.

After 5 days of intravenous unfractionated heparin, symptoms rapidly regressed, allowing a switch to an oral anticoagulant (acenocoumarol). The patient was discharged home 1 week after admission. After 3 months, the decision to continue anticoagulation with acenocoumarol was taken according to European Society of Cardiology guidelines [2] for patients over 50 years old with unprovoked thrombosis and a low risk of major bleeding (HAS-BLED score for major bleeding risk = 1 due to hypertension, corresponding to a 3.4% risk [6]). 6 months after Emergency Department admission, the patient was symptom-free.

Discussion and conclusions

We have described a patient presenting with unprovoked IJV thrombosis, heterozygous factor V Leiden gene, and no other obvious risk factors. This rare thrombosis site reveals the limits of international guidelines based on large, randomized clinical trials mainly designed for deep-vein thrombosis in a limb or pulmonary embolism. There is, therefore, a lack of evidence which might guide clinicians in their workup and treatment of thrombosis in rare sites. Although, by their very nature, published case reports and case series provide biased information, exploring similar cases can be a first step (and sometimes the only one possible) towards collecting scientific evidence.

Medline research strategy

We searched Medline using the keywords (spontaneous OR unprovoked OR idiopathic OR primary AND internal jugular vein thrombosis AND English) and excluded reports of provoked IJV thrombosis to collect information on causes, treatment modalities, and complications. We retrieved 40 relevant articles in English (Table 1). These articles included 48 patients with unprovoked IJV from 17 countries, but mostly from Japan (9/40 articles). More than a third (17/48; 35%) were still truly idiopathic after the workup (Table 2).

Table 1 Case reports and case series of unprovoked internal jugular vein thrombosis
Full size table
Table 2 Etiologies of patients (N = 48) diagnosed with unprovoked IJV (based on a literature review)
Full size table

Internal jugular vein thrombosis epidemiology

Upper-limb thrombosis accounts for a maximum of 10% of all deep-vein thromboses [2, 47]. IJV thrombosis is even less frequent, but epidemiological studies are lacking (other unusual sites for thrombosis, such as the mesenteric vein, account for 0.002–0.006% of all inpatient admissions) [48]. Overall, the most common causes of IJV thrombosis are cancer, central venous catheter placement, and ovarian hyperstimulation syndrome (OHS) [26]. IJV thrombosis can be provoked or unprovoked. The former accounts for four out of five cases [26]. Most IJV thromboses are secondary to central catheter or pacemaker placement, extrinsic compression (by a tumor or malformation), local infections (e.g., Lemierre disease), cervical trauma, or ENT cancer. Unprovoked IJV thrombosis is uncommon and has been associated with paraneoplastic disease, thrombophilia, OHS, and idiopathic IJV [10, 26, 49,50,51]. OHS is associated with thromboembolic complications, but their mechanisms are incompletely understood [52]. In addition to the usual hemostatic changes during pregnancy [53], OHS can cause increased levels of coagulation factors [54].

Clinical presentation

The spectrum of potential clinical presentations is broad, ranging from asymptomatic disease to diffuse and non-specific pain. Neck pain and swelling are the most frequent symptoms, found in 24/48 patients (50%). IJV thrombosis is a serious event, with a potentially fatal outcome, but complications in IJV thrombosis have seldom been reported [55]. Pulmonary embolism was reported in 3 cases (6%). Surprisingly, there were no cases describing neurological complications. The risk of recurrent IJV thrombosis is unknown since the follow-up of published cases was generally interrupted after 3 to 6 months (Table 1).

Diagnostic testing

D-dimer concentrations have excellent negative predictive value, even for deep-vein thrombosis of the upper extremities. However, D-dimer concentrations have not been validated with IJV thrombosis because of the lack of prospective outcome trials. Lower D-dimer sensitivity in IJV thrombosis could be an issue, resulting in higher false-negative rates, which could lead to fatal consequences [56, 57].

Performance of imaging to diagnosis IJV is unknown. The majority of reports used ultrasonography often completed with CT (21/48; 43.8%). In cases of unprovoked IJV thrombosis, oncological disease and thrombophilia should be screened for since they are more common than in cases of lower-body thrombosis. Indeed, these conditions are frequently associated with IJV thrombosis (Table 2).

Thrombophilia is defined as a hereditary or acquired genetic abnormality predisposing the patient to thromboembolic events [58]. The F5 R506Q gene (the factor V Leiden mutation) is the most common pro-thrombotic gene mutation in Caucasians [59], affecting 3–7% of this population. It is a gain-of-function mutation in the procoagulant factors. The annual incidence of venous thromboembolism (VTE) in heterozygous carriers of the factor V Leiden mutation is approximately 0.45% [60].

Middeldorp et al. recommended not routinely screening for hereditary thrombophilia as it does not affect most patients’ clinical management, and long-term anticoagulation is generally recommended for patients with unprovoked thrombosis [58]. Thrombophilia screening is only recommended for young (age < 50) patients with a VTE, in cases of recurrent VTE, and in cases involving a family history of VTE [61]. Hereditary thrombophilia does not significantly increase the risk of recurrence (relative risk from 1.4–2.5, depending on the type of mutation [61]), and anticoagulation treatment should be stopped after 3 months in the absence of risk factors such as cancer. American guidelines also propose anticoagulation cessation after 3 months [3]. European guidelines propose individual assessments of the risks of recurrence and major bleeding in order to decide on whether anticoagulation treatment should continue [2]. In the present case, we chose to screen for thrombophilia and cancer because the thrombosis was unprovoked and located in an unusual site. The patient’s borderline age was also taken into account in accordance with an algorithm proposed by Connors [5].

In our review, only 8% (4/48) of patients with IJV thrombosis had an inherited coagulopathy, a low prevalence also seen among Caucasians if patients from Japan, Sri Lanka, India, Jordan, Israel, Turkey, and Saudi Arabia are excluded from the review. Paraneoplastic disease was far more common than inherited coagulopathy, affecting one third of patients (16/48). Thus, an active workup for oncological diseases is of the utmost importance in unprovoked IJV thrombosis.

Treatment of IJV thrombosis

There are no specific guidelines for the treatment of IJV thrombosis. Treatment is often based, by analogy, on guidelines [2, 3] for upper-limb thrombosis (Tables 1 and 3). Initial treatment (5–21 days following diagnosis) consists of parenteral therapy with low molecular weight heparin (or unfractionated heparin) with a transition using vitamin K antagonists. The alternative therapy for non-cancer patients is high-dose direct oral anticoagulants. Treatment duration is at least 3 months, with possible long-term anticoagulation depending on the cause of thrombosis [2]. To date, no randomized clinical trials have evaluated anticoagulation for upper extremity deep-vein thrombosis. The only ongoing research is a prospective clinical study assessing oral apixaban for the treatment of upper extremity deep-vein thrombosis (including IJV thrombosis). However, to the best of our knowledge, no results have been published yet [62]. The majority of cases in our literature review used unfractionated heparin (26/40; 65%), usually in association with an oral anticoagulant (19/40; 47.5%). Treatment duration varied from 2 weeks to 6 months for non-paraneoplastic cases (Table 3).

Table 3 Patient characteristics from the literature review (see text for details)
Full size table

Follow-up of IJV thrombosis

No specific guidelines exist for IJV thrombosis follow-up. Gbaguidi et al. used a venous ultrasound scan at three and 6 months. Boedeker et al. [55] proposed an ultrasound scan each month until 6 months of follow-up.

The management of internal jugular vein thrombosis is heterogenous and currently based on the management of lower extremity deep-vein thrombosis, with a low rate of complications. The lack of guidelines and large series means that the modalities of diagnosis and treatment type and duration are variable. Given the low prevalence of IJV thrombosis, large, randomized studies would be hard to carry out. Data from our literature review suggest that treatments could tend towards those used for lower limb deep-vein thrombosis, but that the modalities of diagnosis and the duration of treatment and follow-up should be clarified.

Availability of data and materials

All data generated or analyzed during this study are included in this published article.

Abbreviations

ARDS:

Acute respiratory distress syndrome

BID:

Bowel inflammatory disease

COPD:

Chronic obstructive pulmonary disease

CT:

Computed tomography

ENT:

Ear, nose, and throat

IJV:

Internal jugular vein

Jo1:

Antihistidyl transfer-RNA synthetase antibodies

MRI:

Magnetic resonance imaging

NSCLC:

Non-small cell lung cancer

OHS:

Ovarian hyperstimulation syndrome

RNP:

Antiribonucleoprotein antibodies

SSA:

Anti-Sjögren’s syndrome type A antibodies

SSB:

Anti-Sjögren’s syndrome type B antibodies

Scl70:

Anti-scleroderma antibodies

Sm:

Anti-Smith antibodies

US:

Ultrasound

VTE:

Venous thromboembolism

References

  1. Raskob GE, Angchaisuksiri P, Blanco AN, et al. Thrombosis: a major contributor to global disease burden. Arterioscler Thromb Vasc Biol. 2014;34:2363–71.

    Article  CAS  PubMed  Google Scholar 

  2. Mazzolai L, Aboyans V, Ageno W, et al. Diagnosis and management of acute deep vein thrombosis: a joint consensus document from the European Society of Cardiology working groups of aorta and peripheral vascular diseases and pulmonary circulation and right ventricular function. Eur Heart J. 2018;39:4208–18.

    Article  CAS  PubMed  Google Scholar 

  3. Kearon C, Akl EA, Comerota AJ, et al. Antithrombotic therapy for VTE disease: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012;141:e419S–96S.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Solomon DH, Kavanaugh AJ, Schur PH. Evidence-based guidelines for the use of immunologic tests: antinuclear antibody testing. Arthritis Care Res. 2002;47:434–44.

    Article  Google Scholar 

  5. Connors JM. Thrombophilia testing and venous thrombosis. N Engl J Med. 2017;377:1177–87.

    Article  PubMed  Google Scholar 

  6. Lip GYH, Frison L, Halperin JL, et al. Comparative validation of a novel risk score for predicting bleeding risk in anticoagulated patients with atrial fibrillation: the HAS-BLED (hypertension, abnormal renal/liver function, stroke, bleeding history or predisposition, labile INR, elderly, drugs/alcohol concomitantly) score. J Am Coll Cardiol. 2011;57:173–80.

    Article  CAS  PubMed  Google Scholar 

  7. Algoblan D, AlAitah L, Alotaibi AM. Internal Jugular and Subclavian Vein Thrombosis in a Post-liver Transplant Patient. Cureus. 2020;12:e6557.

    PubMed  PubMed Central  Google Scholar 

  8. Pratt DN, Patterson KC, Quin K. Venous thrombosis after nitrous oxide abuse, a case report. J Thromb Thrombolysis. 2020;49:501–3.

    Article  PubMed  Google Scholar 

  9. Agrawal A, Bajaj D, Ruben M, et al. Idiopathic Internal Jugular Vein and Subclavian Vein Thrombosis: A Rare Case Report. Cureus. 2019;11:e4005.

    PubMed  PubMed Central  Google Scholar 

  10. Hahn J, Nordmann-Kleiner M, Hoffmann TK, et al. Thrombosis of the internal jugular vein in the ENT-department - Prevalence, causes and therapy: A retrospective analysis. Auris Nasus Larynx. 2019;46:624–9.

    Article  PubMed  Google Scholar 

  11. Matsuda W, Noguchi S, Fujiyama F. Pseudotumor cerebri and lung cancer-associated jugular vein thrombosis: Role of anatomical variations of torcular herophili. eNeurologicalSci. 2018;13:18–20.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Al-Zoubi NA. Spontaneous internal jugular vein thrombosis as primary presentation of antiphospholipid syndrome: case report. Vasc Health Risk Manag. 2018;14:153–5.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Jendoubi A. Incidental detection of spontaneous floating internal jugular vein thrombosis with acute pulmonary embolism. Saudi J Anaesth. 2017;11:238–9.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Toratani M, Hayashi A, Nishiyama N, et al. Thrombosis in an Internal Jugular Vein and an Upper Limb Deep Vein Treated with Edoxaban. Intern Med Tokyo Jpn. 2017;56:1053–5.

    Article  Google Scholar 

  15. Nomura T, Kato T, Keira N, et al. Idiopathic Internal Jugular Vein Thrombosis as Embolic Origin. Intern Med Tokyo Jpn. 2016;55:847–8.

    Article  Google Scholar 

  16. Bandara AR, Wimalarathna H, Kalupahana R, et al. Internal jugular venous thrombosis due to Trousseau’s syndrome as the presenting feature of metastatic prostate carcinoma: a case report. J Med Case Rep. 2016;10:104.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Efe SC, Poci N, Unkun T, et al. Nineteen-year-old female with idiopathic thrombosis of the internal jugular vein. Perfusion. 2015;30:341–4.

    Article  CAS  PubMed  Google Scholar 

  18. Onishi A, Inoue M, Imai T, et al. Nephrotic syndrome complicated with deep venous thrombosis in the upper extremities. Case Rep Nephrol Dial. 2015;5:1–5.

    Article  PubMed  Google Scholar 

  19. van den Broek R, van Balen M, Blaauwgeers J, et al. A 28-year-old pregnant woman with a very rare cause of jugular vein thrombosis. Neth J Med. 2014;72:224–6.

    PubMed  Google Scholar 

  20. Altıntaş O, Gürsoy AE, Baran G, et al. Bilateral jugular vein and sigmoid sinus thrombosis related to an inherited coagulopathy: an unusual presentation. Case Rep Vasc Med. 2014;2014:873402.

    PubMed  PubMed Central  Google Scholar 

  21. Ghatak T, Singh RK, Baronia AK. Spontaneous central vein thrombosis in a patient with activated protein C resistance and dengue infection: An association or causation? J Anaesthesiol Clin Pharmacol. 2013;29:547–9.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Papay P, Miehsler W, Tilg H, et al. Clinical presentation of venous thromboembolism in inflammatory bowel disease. J Crohns Colitis. 2013;7:723–9.

    Article  PubMed  Google Scholar 

  23. Kunimasa K, Korogi Y, Okamoto Y, et al. Spontaneous internal jugular vein thrombosis associated with lung cancer. Intern Med Tokyo Jpn. 2013;52:1849.

    Article  Google Scholar 

  24. Honma H, Ohno T, Matsuzaki T, et al. Idiopathic internal jugular vein thrombosis in a patient with severe dizziness. J Echocardiogr. 2011;9:119–20.

    Article  PubMed  Google Scholar 

  25. Ishida A, Matsuo S, Niimura K, et al. Cervical spontaneous spinal epidural hematoma with internal jugular vein thrombosis. J Neurosurg Spine. 2011;15:187–9.

    Article  PubMed  Google Scholar 

  26. Gbaguidi X, Janvresse A, Benichou J, et al. Internal jugular vein thrombosis: outcome and risk factors. QJM Mon J Assoc Physicians. 2011;104:209–19.

    Article  CAS  Google Scholar 

  27. Serinken M, Karcioglu O, Korkmaz A. Spontaneous internal jugular vein thrombosis: a case report. Kaohsiung J Med Sci. 2010;26:679–81.

    Article  PubMed  Google Scholar 

  28. Snijders MP, Morsink M, van Spronsen DJ, et al. Internal jugular vein thrombosis as paraneoplastic syndrome of primary ovarian non-Hodgkin’s lymphoma. Eur J Gynaecol Oncol. 2010;31:675–8.

    CAS  PubMed  Google Scholar 

  29. Handley TPB, Miah MS, Majumdar S, et al. Collet-sicard syndrome from thrombosis of the sigmoid-jugular complex: a case report and review of the literature. Int J Otolaryngol. 2010. Epub ahead of print 2010. https://doi.org/10.1155/2010/203587.

  30. Leibman Y, Ayalon M, Steiner IP. Internal Jugular Venous Thrombosis After in Vitro Fertilization. J Emerg Med. 2009;37:29–31.

    Article  PubMed  Google Scholar 

  31. Chlumský J, Havlín J. Spontaneous jugular vein thrombosis. Acta Cardiol. 2009;64:689–91.

    Article  PubMed  Google Scholar 

  32. Pata YS, Ünal M, Gülhan S. Internal jugular vein thrombosis due to distant malignancies: two case reports and literature review. J Laryngol Otol. 2008;122:318–20.

    Article  CAS  PubMed  Google Scholar 

  33. Mori A, Ibata M, Mashiko S, et al. Long-term administration of a low-molecular-weight heparin contributed to successful treatment in a patient with primary mediastinal large B-cell lymphoma and venous thromboembolism. Clin Appl Thromb Off J Int Acad Clin Appl Thromb. 2008;14:468–71.

    Article  CAS  Google Scholar 

  34. Kikuchi M, Yamamoto E, Shiomi Y, et al. Case report: internal and external jugular vein thrombosis with marked accumulation of FDG. Br J Radiol. 2004;77:888–90.

    Article  CAS  PubMed  Google Scholar 

  35. Cheang PP, Fryer J, Ayoub O, et al. Spontaneous bilateral internal jugular vein thrombosis: a sign of metastasis. J Laryngol Otol. 2004;118:570–2.

    Article  CAS  PubMed  Google Scholar 

  36. Unsal EE, Karaca C, Ensarí S. Spontaneous internal jugular vein thrombosis associated with distant malignancies. Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg. 2003;260:39–41.

    Google Scholar 

  37. Khandekar AA, Kumbhalkar SD, Salkar HR, et al. Protein S deficiency presenting as deep vein thrombosis--a case report. Angiology. 2003;54:605–8.

    Article  PubMed  Google Scholar 

  38. Thomas RV, Reid W, Perry DJ. Internal jugular vein thrombosis following in-vitro fertilization in a woman with protein S deficiency and heterozygosity for the prothrombin 3’ UTR mutation, despite anticoagulation with heparin. Blood Coagul Fibrinolysis Int J Haemost Thromb. 2001;12:487–9.

    Article  CAS  Google Scholar 

  39. Van den Noortgate N, Michielsen W, Afschrift M, et al. Spontaneous internal jugular vein thrombosis: primary manifestation of a colorectal adenocarcinoma in a very old woman. Acta Clin Belg. 2000;55:94–6.

    Article  PubMed  Google Scholar 

  40. Todros T, Carmazzi CM, Bontempo S, et al. Spontaneous ovarian hyperstimulation syndrome and deep vein thrombosis in pregnancy: case report. Hum Reprod Oxf Engl. 1999;14:2245–8.

    Article  CAS  Google Scholar 

  41. Kalan A, Tariq M, Harar RP, et al. Spontaneous internal jugular vein thrombosis and recurrent laryngeal nerve palsy: a rare simultaneous presentation of an occult malignant neoplasm. J Laryngol Otol. 1996;110:1166–8.

    Article  CAS  PubMed  Google Scholar 

  42. Holland AJ, Childs PA, Beilin J, et al. Spontaneous thrombosis of the internal jugular vein as the initial presentation of the primary antiphospholipid syndrome. Aust N Z J Surg. 1996;66:854–5.

    Article  CAS  PubMed  Google Scholar 

  43. Hines JF, Tucker A, Grendys EC, et al. Spontaneous preoperative internal jugular and subclavian vein thrombosis associated with an early-stage synchronous ovarian/endometrial malignancy. Gynecol Oncol. 1995;56:298–301.

    Article  CAS  PubMed  Google Scholar 

  44. Langlieb AM, Dunton CJ, Carlson JA. Spontaneous internal jugular vein thrombosis associated with leiomyosarcoma of the omentum. Gynecol Oncol. 1992;47:125–6.

    Article  CAS  PubMed  Google Scholar 

  45. Carrington BM, Adams JE. Jugular vein thrombosis associated with distant malignancy. Postgrad Med J. 1988;64:455–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Kennedy KS, Tami TA, Friedman JC, et al. Spontaneous thrombosis of the internal jugular vein. Ann Otol Rhinol Laryngol. 1987;96:222–4.

    Article  CAS  PubMed  Google Scholar 

  47. Flinterman LE, Van Der Meer FJM, Rosendaal FR, et al. Current perspective of venous thrombosis in the upper extremity. J Thromb Haemost JTH 2008;6:1262–66.

  48. Shatzel JJ, O’Donnell M, Olson SR, et al. Venous thrombosis in unusual sites: A practical review for the hematologist. Eur J Haematol 2019;102:53–62.

  49. Binnebösel M, Grommes J, Junge K, et al. Internal jugular vein thrombosis presenting as a painful neck mass due to a spontaneous dislocated subclavian port catheter as long-term complication: a case report. Cases J. 2009;2:7991.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Weaver LA, Kanter CR, Costantino TG. Effort thrombosis provoked by saxophone performance. J Emerg Med. 2019;56:323–6.

    Article  PubMed  Google Scholar 

  51. Ilhan E, Ture M, Yilmaz C, et al. Subclavian vein thrombosis extending into the internal jugular vein: Paget-von Schroetter syndrome. J Clin Med Res. 2009;1:178–80.

    PubMed  PubMed Central  Google Scholar 

  52. Sennström M, Rova K, Hellgren M, et al. Thromboembolism and in vitro fertilization - a systematic review. Acta Obstet Gynecol Scand. 2017;96:1045–52.

    Article  PubMed  Google Scholar 

  53. Hellgren M. Hemostasis during normal pregnancy and puerperium. Semin Thromb Hemost. 2003;29:125–30.

    Article  CAS  PubMed  Google Scholar 

  54. Kodama H, Fukuda J, Karube H, et al. Characteristics of blood hemostatic markers in a patient with ovarian hyperstimulation syndrome who actually developed thromboembolism. Fertil Steril. 1995;64:1207–9.

    Article  CAS  PubMed  Google Scholar 

  55. Boedeker CC, Ridder GJ, Weerda N, et al. Etiology and therapy of the internal jugular vein thrombosis. Laryngorhinootologie. 2004;83:743–9.

    Article  CAS  PubMed  Google Scholar 

  56. Douma RA, Mos ICM, Erkens PMG, et al. Performance of 4 clinical decision rules in the diagnostic management of acute pulmonary embolism: a prospective cohort study. Ann Intern Med. 2011;154:709–18.

    Article  PubMed  Google Scholar 

  57. Righini M, Perrier A, De Moerloose P, et al. D-dimer for venous thromboembolism diagnosis: 20 years later. J Thromb Haemost JTH. 2008;6:1059–71.

    Article  CAS  PubMed  Google Scholar 

  58. Middeldorp S, van Hylckama Vlieg A. Does thrombophilia testing help in the clinical management of patients? Br J Haematol. 2008;143:321–35.

    Article  PubMed  Google Scholar 

  59. Rees DC. The population genetics of factor V Leiden (Arg506Gln). Br J Haematol. 1996;95:579–86.

    Article  CAS  PubMed  Google Scholar 

  60. Middeldorp S, Henkens CMA, Koopman MMW, et al. The incidence of venous thromboembolism in family members of patients with factor V Leiden mutation and venous thrombosis. Ann Intern Med. 1998;128:15–20.

    Article  CAS  PubMed  Google Scholar 

  61. Pruthi RK. Optimal utilization of thrombophilia testing. Int J Lab Hematol. 2017;39:104–10.

    Article  PubMed  Google Scholar 

  62. Woller SC, Stevens SM, Johnson SA, et al. Apixaban for Routine Management of Upper Extremity Deep Venous Thrombosis (ARM‐DVT): Methods of a prospective single‐arm management study. Res Pract Thromb Haemost 2019;3:340–48.

Download references

Acknowledgements

None.

Funding

No funding to declare.

Author information

Authors and Affiliations

  1. Department of Internal Medicine, Neuchâtel Hospital Network, Rue de la Maladière 45, CH-2000, Neuchâtel, Switzerland

    Loïc Payrard, Léa Iten, Jacques Donzé & Gregor John

  2. Department of Internal Medicine, University of Lausanne, Lausanne, Switzerland

    Jacques Donzé

  3. Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Jacques Donzé

  4. Department of Internal Medicine, Geneva University Hospitals (HUG), Gabrielle-Perret-Gentil 4, CH-1205, Geneva, Switzerland

    Gregor John

Authors
  1. Loïc Payrard
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Léa Iten
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jacques Donzé
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gregor John
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

LP contributed significantly to conceptualization, investigation, writing the original draft, reviewing, and editing. LI and JD contributed to data analysis, resources, visualization, and validation. GJ contributed significantly to conceptualization, validation and supervision, project administration, reviewing, and editing. All authors approved the submitted version.

Corresponding author

Correspondence to Gregor John.

Ethics declarations

Consent for publication

The patient provided her written informed consent for the case report to be published.

Competing interests

The authors declare that they have no competing interests.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Payrard, L., Iten, L., Donzé, J. et al. Unprovoked internal jugular vein thrombosis: a case report and literature review. Thrombosis J 19, 2 (2021). https://doi.org/10.1186/s12959-020-00246-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/s12959-020-00246-7

Keywords

Thrombosis Journal

ISSN: 1477-9560

Contact us