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

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.

Internal Jugular Vein Thrombosis: Unusual Diagnosis of a Cervical Mass

“…Nonetheless, the rarity of this condition makes most clinicians unaware of its presentation. The scarcity of this pathology is probably responsible for the lack of epidemiological studies about it [2][3][4]. Provoked IJVT is more common and is associated with ENT procedures, head and neck cancer, pacemaker placement, and central catheter insertion [2,4].…”

“…The scarcity of this pathology is probably responsible for the lack of epidemiological studies about it [2][3][4]. Provoked IJVT is more common and is associated with ENT procedures, head and neck cancer, pacemaker placement, and central catheter insertion [2,4]. The ovarian hyperstimulation syndrome seems to be a predisposing factor to unprovoked IJVT [3][4].…”

“…Most patients present with painful erythematous neck swelling and headache. However, the presenting symptoms could include also fever, facial swelling, papilledema, and signs of intracranial pressure elevations or neurological impairment [2,4].…”

“…In the clinical exam, the abrupt swelling of the neck and the palpation of a cord beneath the sternocleidomastoid muscle should raise suspicion for IJVT and mandate further imaging studies to confirm the diagnosis [2,4].…”

“…The former can be predisposed by ear-nose-throat (ENT) operations, head and neck neoplasms, and the placement of a central catheter. The unprovoked form is associated with paraneoplastic syndrome and ovarian hyperstimulation syndrome or it could be idiopathic IJVT [1][2][3][4]. Both forms represent a rarity, and there are no specific epidemiological studies about these entities [2][3].…”