Endovascular Management of an Acquired Carotid-Jugular Fistula in a Child

Background

Endovascular techniques have revolutionized the management of vascular trauma, especially in cases where the nature of open surgery carries particular risk. Penetrating neck trauma can be one of these cases and is a challenging condition requiring an expedited approach. Fortunately, these injuries in children are rare, but this creates a further challenge for the vascular surgeon, as current evidence for therapeutic management is extrapolated from similar injuries in the adult population and therefore may not be applicable [1]. We report a case of an acquired carotid-jugular fistula (CJF) following penetrating neck trauma in a 12-year-old male, treated by minimally invasive endovascular covered stent.

Case Report

PROCEDURE:

The procedure was performed in the angiography suite using the Philips Allura XPer FD 20/15 x-ray system. The patient was given 100 mg aspirin and 75 mg clopidogrel before induction of anesthesia. The right common femoral artery (CFA) was punctured under ultrasound guidance and a 6 French sheath inserted followed by intra-arterial administration of 3000 IU of heparin. Selective left carotid angiogram demonstrated a high flow AVF measuring 5 mm in diameter and 12 mm in length between the left ICA and IJV with a prominent dilatation of the IJV (Figure 3). Following the diagnostic angiogram, a small micro catheter was advanced over the wire to cross the lesion. Over an exchange wire the self-expanding Fluency Plus stent graft (BARD Peripheral Vascular, Tempe, AZ, USA) measuring 6 mm in diameter and 4 cm in length was successfully deployed into the ICA with complete obliteration of the fistulous tract and satisfactory apposition of the stent (Figure 3).

Immediately post procedure, the swelling in the neck and the palpable thrill were resolved. Recovery was unremarkable, and the patient was discharged after 3 days on 100 mg aspirin and 75 mg clopidogrel which was continued for 6 weeks. Followed by lifelong aspirin 100 mg on daily basis.

POST PROCEDURE FOLLOW-UP:

CTA at 4 weeks revealed satisfactory position of the stent, a widely patent ICA and complete resolution of the CJF. At the 6-week outpatient follow-up, the patient was completely asymptomatic.

DUS at 18 months demonstrated a patent stent with normal flow velocities (peak systolic velocities (PSV)=85 cm/sec, end diastolic velocities (EDV)=25 cm/sec) and no evidence of AVF (Figure 4). The patient remains monitored by our carotid surveillance every 6 months with a duplex carotid ultrasound performed in our vascular lab.

Discussion

Carotid jugular fistulas (CJF) are an uncommon clinical entity [2–4]. Although rare, they can be congenital, however, the majority of pediatric cases are due to neck trauma, with penetrating injuries the most common of these [3,5]. Their presentation, which depends on the hemodynamic effects and degree of arterial to venous shunting, can be subtle and this often leads to delays in diagnosis of weeks or even months following the initial injury [2,4,6].

Symptoms can vary from minor to intractable tinnitus, arrhythmias, heart failure, cerebral embolization and pseudo aneurysm formation, depending on the flow through the AVF and its proximity to the heart [2–6]. Any penetrating neck trauma should warrant a high index of suspicion of a CJF and this is key for timely diagnosis. DUS is usually the initial diagnostic modality; however, it is operator dependent and cannot completely exclude CJF [3]. CTA or magnetic resonance angiography (MRA) is therefore usually performed to provide the necessary imaging required to plan treatment which aims to seal the fistula and maintain forward antegrade flow to the brain [5,7,8]. Conventional surgical ligation, division of the fistula, and vascular reconstruction was traditionally the treatment of choice, but this carries a significant morbidity and requires technical expertise owing to the small vessel diameter, relative surgical inaccessibility, and potentially scarring in the not infrequent case of delayed diagnosis [8].

There is a variety of endovascular techniques for treating CJFs in the published literature varying from coil embolization to covered stent graft placement [3,8,9]. These techniques can be advantageous as they can be done under local anesthesia allowing intra-procedural cerebral function monitoring and can treat pathology at sites which are otherwise surgically inaccessible [10]. However, they do carry unique concerns when dealing with a pediatric patient; technically the procedures can be challenging. Both access and target vessels have significantly smaller diameters than those encountered in adult patients, increasing the risk of procedural complications. The rarity of presentation means there is no current consensus regarding therapeutic management nor follow-up, including antiplatelet regimes [1]. Moreover, technical issues related to endovascular advancement of the guidewire across the distal carotid can be very challenging particularly when the shunt is substantial, and the communicating fistula is aneurysmal.

Close follow-up of these patients is clearly imperative; in-stent re-stenosis and stent mismatch are pertinent potential complications in a growing child that can be managed with re-dilatation without clinical consequence, as long as they are detected early [11]. The development of biodegradable stents offers an exciting potential solution to avoid the stent-complications unique to the pediatric patient and could make endovascular techniques universally applicable in this population [12].

The reported morbidity of carotid stent insertion for vascular trauma is encouraging, with a 3.5% risk of stroke, though long-term patency data is not available, overall mortality is less than 1% [1]. This combined with the limited vessel tortuosity in pediatric patients, makes endovascular stent placement a viable option in this age group.

Conclusions

This case demonstrated the successful management of a CJF secondary to vascular neck trauma in a pediatric patient. The safety profile and minimally invasive nature of endovascular techniques make them a viable option in pediatric patients, especially for lesions that are not easily accessible through an open approach. Yet long-term patency and follow-up plans remains unknown especially in the pediatric age group.