CASE 18270 Published on 21.08.2023

Teaching Case

Section

Head & neck imaging

Case Type

Clinical Cases

Authors

Ibrahim Al-Mousllie¹, Clarissa Gevargez Lyngby², Rebeca Mirón Mombiela²

1. Hannover Medical School, Hanover, Germany

2. Herlev og Gentofte Hospital, Denmark

Patient

71 years, female

Categories

Area of Interest Anatomy, Head and neck, Oncology, Vascular, Veins / Vena cava ; Imaging Technique CT ;

Procedure Catheters ; Special Focus Aneurysms, Cancer, Drugs / Reactions, Embolism / Thrombosis ;

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Clinical History

A 71-year-old woman with a history of mamma cancer treated with mastectomy comes to the emergency department because of pain and swelling in the right neck. She received chemotherapy for the last 15 months through a Port-a-cath, which was removed 5 days ago from the right subclavian vein (Fig. 1).

Differential Diagnosis List

Bilateral fenestration with left-sided duplication of the EJV

Phlebectasia

Venous aneurysm

Imaging Findings

The contrast-enhanced CT of neck and thorax in the venous phase revealed an expanded right internal jugular vein (IJV) with a contrast defect, and to a lesser extent right external jugular vein (EJV), compatible with two thrombi (Fig. 2).

Incidentally, four venous malformations were found. The first is the bilateral termination of the facial vein (FV) into the respective EJV (Fig. 3-4). The second is the duplication of the left EJV originating from the confluence of the left FV and EJV and terminating into the left brachiocephalic vein (BV) (Fig. 4). The third is the missing left anterior jugular vein (AJV) (Fig. 4). The fourth is the bilateral fenestration of the EJV, though the left EJV is double fenestrated (Fig. 3-4). The jugular venous arch is formed by branches of the right AJV and the duplication (Fig. 5).

Discussion

Background

Venous malformations are congenital malformations of the venous system [1] with an estimated prevalence of 1% [2]. They are classified as extratruncular and truncular based on the developmental stage [3]. The first describes disorganised changes of multiple vessels. The latter is more organised and affects fewer vessels, as it occurs in more advanced developmental stages. Miscommunication of endothelial and soft muscle cells or different signalling cascades [4-5] during vasculogenesis or angiogenesis [6] are assumed to cause the malformation.

To our knowledge, this is the first reported case of a bilateral fenestrated EJV, since only unilateral fenestrated EJVs are described in the literature. Fourteen cases of unilateral fenestrated EJVs [7-20] and 12 cases of duplicated EJVs were reported [21-29]. The fenestration of a vessel is defined as the separation into two branches that rejoin [30-31], while a duplication does not rejoin [30]. Fenestrations and duplications are truncular malformations.

Another variation, prevalent in 5% of the population, is the connection of the FV to the EJV. It is an embryological remnant as the connection usually retrogresses [32].

Although thrombosis is a common complication of a Port-a-cath (PAC) [33-34], it can be hypothesised that the increased turbulence in and decreased velocity of the bloodstream caused by the fenestration and the duplication increase the risk of thrombosis. Dinh et al. (2021) published a case report of a deep venous thrombosis (DVT) in a fenestrated common iliac vein (CIV) that was previously accessed for a supraventricular tachycardia ablation. They also suspect a connection between the fenestration of the CIV and the DVT [35]. The venous malformation combined with irritation of the intima for example by a central venous catheter (CVC) is expected to play a role in thrombosis.

Clinical Perspective

The detection and radiological evaluation of venous malformation such of the EJV requires special attention due to its clinical importance in planning different procedures (measurement of the central venous pressure [36-37], cannulation in diagnostics [39], CVCs [39-41]) and surgeries (reconstructive surgery, free flap surgeries [42]), and avoiding complications.

Outcome

In our case, if chemotherapy is contemplated in the future, an alternative access point for cannulation should be considered instead of the SV or a jugular vein. Two possible alternatives are the PICC-line using peripheral veins, such as the basilic or cephalic vein [43-44], and a CVC through the femoral vein. However, the latter is associated with a higher rate of infection and thrombosis [45].

Written informed patient consent for publication has been obtained.

References

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