Endovascular Treatment for Left-side Cervical Aortic Arch with Huge Aneurysm
Article Outline
Abstract
Cervical aortic arch (CAA) is an unusual malformation of the aortic arch. CAA with aneurysm formation is very rare. A 67-year-old female patient was admitted with a huge mass on left-side neck and 2 days of dyspnea. Investigations revealed a huge aneurysm on a left-side CAA. The aneurysm was successfully repaired a using thoracic aortic stent-graft. The patient's dyspnea was resolved immediately.
Keywords: CAA, Aneurysm, Endovascular repair
Introduction
Cervical aortic arch (CAA) is a rare congenital vascular anomaly which was first described in 1914. The mechanism of development of aortic arch anomaly can be explained by the hypothetical double aortic arch systems.1 Haughton et al.2 classified CAA as five types according to the configuration of the aorta, sequence of brachiocephalic branching, and embryogenesis. CAA with aneurysm formation is much rarer. Causes of aneurysm formation with CAA may include abnormal embryologic development, pathological connective tissue, and trauma. This case report presents a D type of CAA treated endoluminally.
Case Report
A 67-year-old female patient was admitted with 2 days of serious dyspnea and a mass on left-side neck, which she had for nearly 20 years. (Fig. 1-A) The mass had been asymptomatic and grew slowly without pain. Blood pressure on the right upper extremity was 170.25/113.25
mmHg and 69.75/30mmHg in the left upper extremity. The blood pressure was 131.25/58.5mmHg in both legs. The mass was about 18.5cm×17.5cm with pulsation and a murmur. Horner's sign appeared on the left side of her face. A computed tomography angiography (CTA) showed there was a huge aneurysm with left-side CAA. A digital subtraction angiography (DSA) showed that it was distal to left common carotid artery and extended into left subclavian artery. It also showed an ipsilateral descending aorta with a normal sequence of brachiocephalic branching. (Fig. 1-B) Left vertebral artery was retrograde filled, the right one was dominant. The aneurysm protruded caudally out of thoracic outlet to left-side neck. It compressed the trachea severely.
Figure 1
(A) Preoperative appearance of the patient with left-side cervical aorta arch and aneurysm. (B) DSA showed left-side cervical aorta arch with aneurysm and brachiocephalic branchings.
Endovascular repair of the CAA aneurysm was performed with local anesthesia. Intra-operative angiography showed that length of the neck of aneurysm was 38.3mm, the diameter of proximal part was 27.5mm and its distal end was 22mm. The diameter of descending aorta was 30mm. We used one thoracic aortic stent-graft to exclude the aneurysm (Hercules-T thoracic stent-graft, Microport, China).3 Its diameter is 34mm and whole length is 175mm with a 20mm-length bare metal stent on front end. The graft is built of multiple nitinol stents attached to 155mm-length Dacron material. Right radial approach was used for placing calibrating flush catheter to the ascending aorta. Right femoral approach was used for the introduction of the stent-graft. The stent-graft was deployed just distal to the left common carotid artery. The distal landing zone was in the normal descending aorta. Intra-operative angiography showed the aneurysm was excluded with a very slight type I endoleak. Left subclavian artery was visualized in a late phase. (Fig. 2-A)
Figure 2
(A) Postoperative DSA demonstrated the exclusion of aneurysm and branchiocephalic branching filling. (B) Two weeks after operation CTA showed the exclusion of aneurysm without endoleak.
After operation her dyspnea resolved immediately. Her left upper extremity was viable without any neurologic complication. Bypass to the left subclavian artery was not performed due to the lack of ischemia. The Type I endoleak was followed up by Doppler ultrasound.
Two weeks after operation CTA showed Type I endoleak disappeared. (Fig. 2-B) At 1, 3, 6, 12 and 24 months repeat Doppler ultrasound follow-up, imaging showed that the aneurysm had completely thrombosed and no evidence of endoleak. The patient has no neurologic complications and has retained normal function of the left upper extremity.
Discussion
CAA with aneurysm formation is very rare,1 particularly with such a large sized aneurysm as is presented in this case. We found this case D type by Haughton's classification, which is ipsilateral descending aorta with a normal sequence of right brachiocephalic branching.2, 4 In this case the dyspnea and Horner's sign were caused by compression of the large aneurysm. After operation her dyspnea disappeared immediately because high-pressure blood stream was excluded by stent-graft. In the current case the left subclavian artery was filled in a retrograde fashion by the left vertebral artery pre-operatively. Her left upper extremity had no significant symptoms most likely due to a fully established collateral circulation.
Although diagnosis can be made with Doppler ultrasound, important anatomical details required for endovascular repair can only be established by CTA and DSA. Open surgical repair requires thoracotomy and cardiopulmonary bypass.4, 5 The open approach in our patient was thought to be risky, in part due to the severe tracheal displacement caused by the aneurysm thereby increasing the difficulties of general anaesthesia. Stent-graft repair CAA has previously been reported with equivalent mortality rates, but lower morbidity than traditional open surgical repair.6 Our patient had a long history with chronically compressed local tissue including trachea, sympathetic ganglion and the left subclavian artery. The well-established collateral circulation prevented ischemia to the left arm, and thus precluding any bypass surgery. The typeI endoleak was probably caused by the twisted neck of aneurysm and incompletely opened entrance of stent-graft. Repeat Doppler ultrasound follow-up showed that it stopped with thrombosis of the aneurysm and a well-attached stent-graft. If the thrombosed aneurysm continues to exert compression on the surrounding tissues it can easily be de-compressed if necessary. This way tracheal collapse, tissue defect and collateral circulation disruption can easily be avoided. Endovascular repair of CAA appears feasible and safe, despite an unusually large aneurysm.
Conflict of Interest/Funding
None.
References
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PII: S1533-3167(09)00028-4
doi:10.1016/j.ejvsextra.2009.07.004
Crown Copyright © 2009. Published by Elsevier Inc. All rights reserved.
Refers to article:
- Endovascular Treatment for Left-side Cervical Aortic Arch with Huge Aneurysm , 27 August 2009
