The internal jugular vein is an uncommon site of deep venous thrombosis.
Spontaneous internal jugular vein thrombosis (IJVT) is an unusual form of venous thrombosis, with no apparent predisposing causes, although many of these patients may harbour an occult malignant neoplasm.
Nonspontaneous IJVT is an uncommon condition, historically associated with deep neck infections during the pre-antibiotic era, and known as Lemierre's syndrome, necrobacillosis or postanginal septicaemia.
Today, the leading causes of nonspontaneous IJVT are trauma to the internal jugular vein from catheterisation (central venous catheter, jugular bulb or Swan-Ganz, in the internal jugular vein or subclavian vein) and repeated direct intravenous injections by drug users. Direct extension of a tumour is a rare cause.
Other causes include surgical neck dissection and hypercoagulable state (from distant malignancies or secondary to factor V Leiden, protein C, protein S, or antithrombin III deficiency, or to hypercoagulation induced by ovulation induction with gonadotrophins).
We present an unusual case of spontaneous thrombosis of the internal jugular vein.
Internal jugular vein thrombosis refers to an intraluminal thrombus occurring anywhere from the origin of the internal jugular vein in the cranium down to the point where it joins the subclavian vein to form the brachiocephalic vein.
The classic triad predisposing to intravascular thrombosis was described first by Virchow and includes endothelial damage, alteration in blood flow, and a hypercoagulable state.
The thrombosis may become secondarily infected, producing a septic thrombophlebitis.
The symptoms and signs of IJVT, either spontaneous or nonspontaneous, can often be very subtle, making it easy to overlook the diagnosis. Pain and swelling at the angle of the jaw and a palpable cord beneath the sternocleidomastoid muscle may both be absent in a significant number of patients.
If infection has developed, other objective findings may be found, such as fever and leukocytosis.
Other symptoms include sepsis, superior vena cava syndrome and/or jugular vein syndrome.
Doppler ultrasound and computed tomography or magnetic resonance imaging should confirm signs and symptoms suggesting thrombosis.
Ultrasound is a widely available test that is finding increased usage in the diagnosis of IJVT.
Findings on ultrasound include a dilated and incompressible vein, intraluminal clot (a late finding), and no response to the Valsalva manoeuvre.
Doppler blood flow imaging (duplex sonography and colour flow imaging) is very useful to confirm the diagnosis. The degree of venous obstruction can easily be determined, the efficiency of therapy can be controlled, and blood flow direction and velocity can be visualised in colour, which provides global information about haemodynamics in a short time. Furthermore, colour flow imaging can detect even small subcutaneous collaterals.
As there is a high association between subclavian and internal jugular vein thrombosis, colour Doppler investigation of both the subclavian and internal jugular veins is necessary.
Immediate anticoagulation with heparin followed by oral anticoagulation is considered the best therapeutic option to reduce associated mortality and morbidity.
Surgery is seldom needed in uncomplicated IJVT.
Internal jugular vein thrombosis can have serious, potentially life-threatening complications that include systemic sepsis following infected thrombophlebitis, pulmonary embolism, superior sagittal sinus thrombosis and pseudo-tumour cerebri.
If no risk factors for IJVT are present, a work-up for hypercoagulable states and a careful search for distant malignancy should be performed. If screening shows no obvious malignancy, further follow-up is necessary.
In this case, anticoagulation therapy resulted in complete resolution of the thrombosis and subsequent recanalisation of the internal jugular vein.