PÁGINA DEL RESIDENTE

What’s your diagnosis?

¿Cuál es su diagnóstico?

An 83-year-old man was referred as an emergency to the CMF Department of Hospital Universitario Marqués de Valdecilla (Santander) in June 2003 for a condition that had developed 1 month earlier consisting in a painful mass in the left preauricular and laterocervical region. In recent days it had grown rapidly and was accompanied by a bitonal voice and swallowing difficulty.

The patient’s personal history included a former smoking habit of 2 packs/day and occasional drinking. He also had diabetes mellitus type II and met criteria of chronic bronchitis type chronic obstructive pulmonary disease (COLD).

The physical examination disclosed a large cervical mass 10 x 12 cm in diameter, pulsatile, painful, and adhered to deep planes; it was situated from the submandibular region to the left supraclavicular, at the pre-sternocleidomastoid level (Fig. 1).

Computed tomography (CT) of the head and neck confirmed the presence of a heterogeneous mass with hyperdense contour and septa and hypodense center. Extensive necrosis 7 x 8 cm in diameter was located in the left cervical vascular space, which engulfed the common carotid artery and displaced neighboring structures. The internal jugular vein was amputated at its lower third (Fig.2 and 3).

Giant pseudoaneurysm of the extracranial carotid

Pseudoaneurisma gigante de carótida extracraneal

B. Rodríguez Caballero¹, M.F. García Reija², V. Vázquez Marcos¹, F. García Pire¹, G. Herrera Calvo¹, R.C. Saiz Bustillo³

1 Médico Residente.
2 Médico Adjunto.
3 Jefe de Servicio.
Servicio de Cirugía Oral y Maxilofacial
Hospital Universitario Marqués de Valdecilla Santander, España

Dirección para correspondencia

TC showed a mass directly dependent on the left common carotid artery at the level of the internal face of the carotid bulb, which suggested the presence of a large complicated pseudoaneurysm.

Therefore, with a suspected diagnosis of complicated carotid pseudoaneurysm, the patient was operated on under general anesthesia in conjunction with the cardiovascular surgery department of our hospital. Given the impossibility of orotracheal intubation, due to the displacement of cervical structures, tracheostomy was required. Later, sternotomy was performed with control of the carotid at its origin on the aorta. A large pseudoaneurysm was identified at the level of the carotid bulb. It was partially resected and repaired with a Gore-Tex patch.

The postoperative evolution was satisfactory. The patient did not present complications and was released from the hospital 20 days after surgery with a good general status and closure of the tracheostoma.

The histopathologic report on the surgical piece was a fibrotic, calcified carotid wall with thrombosis.

Discussion

Aneurysmal pathology of the extracranial carotid is uncommon. Aneurysms and pseudoaneurysms may be present; the origin of both can be either spontaneous or traumatic. Aneurysms occur when circulating blood penetrates the arterial wall through an injury in the vascular intimal layer. In contrast, pseudoaneurysms are true encapsulated paravascular hematomas that are not in direct contact with the bloodstream. Some authors maintain that carotid pseudoaneurysm includes, by definition, the bifurcation of the common carotid.^{7, 9}

Pseudoaneurysms represent 0.3% to 14% of all aneurysmal pathology and 0.5-1% of all interventions on the carotid.^1,6,9 There is a clear masculine predominance with a ratio of 2:1.⁴ The most frequent age of presentation is the fifth decade of life. Mean age decreases greatly in pseudoaneurysms of traumatic origin.^{4, 5} A high incidence of arterial hypertension (AHT), diabetes, COPD, and associated coronary disease has been reported in these patients.^7-9

With respect to etiology, many causes are known to predispose to the development carotid aneurysmal pathology (both aneurysms and pseudoaneurysms). In the early decades of the twentieth century, before the introduction of antibiotics, the most frequent etiology was infectious, mainly syphilis. At present, the most frequent causes include arteriosclerosis (42%), trauma, iatrogenic injuries (mainly related with endarterectomy and central lines), and those secondary to congenital and acquired vascular structural alterations (fibrous dysplasia, cystic necrosis of the media, Marfan’s syndrome, Ehler-Danlos’ syndrome, etc.).^1,3,4,6,9

Pseudoaneurysms have a prolonged formation period. Starting with an aggression to the carotid wall (traumatic or spontaneous), a hematoma forms and becomes encapsulated in time. The condition remains asymptomatic for weeks or months in some cases, and may increase in size until the clinical debut.⁷

The most frequent symptom is the appearance of a pulsating cervical mass that is accompanied by cervical or facial pain. The pain usually develops gradually, although it also may begin abruptly. It appears on the same side and usually is distributed throughout the anterior region of the skull, eye socket, and face. Dysfunction of adjacent nervous structures with compression of the low cranial nerves is not rare (mainly XII). In cases of large pseudoaneurysms, symptoms due to the compression of nearby cervical structures may be observed (bitonal voice, dyspnea, dysphagia, etc.).⁶ The possibility of complications due to overinfection, thromboembolism, or rupture of the pseudoaneurysm (which is very rare, but possible) must also be considered. These complications lead to ischemic cerebral pathology (ischemic cerebrovascular accident [ACV], transitory ischemic accident [AIT], or stroke), cranial nerve paralysis, oculosympathetic paralysis (occasionally manifested as total or partial Horner’s syndrome), or even shock and the death of the patient if bleeding is not controlled.^1,6

The diagnosis of pseudoaneurysm of the extracranial carotid must be based on a clinical suspicion. Due to the absolute contraindication of puncture biopsy because of the evident risk of bleeding, pseudoaneurysm must be confirmed by imaging studies, mainly Doppler ultrasonography, CT and/or magnetic resonance imaging (MRI). Arteriography will contribute information on the extension of the injury, stenosis, or irregularities of the artery.^1,4,5 The differential diagnosis must be made with other cervical tumors. The most frequent of these tumors are branchial cysts, ectodermal inclusion cysts, lymphoepithelial cysts, enlarged lymph nodes, lymphomas, and cervical primitive tumors. Among the latter, we must consider mainly the tumors that have a closer analogy with carotid pseudoaneurysm (due to location or clinical behavior), such as vascular lesions (hemangioma, benign vascular malformation, lymphangioma, hygroma, hemangiopericytoma, angiosarcoma, Kaposi sarcoma, malignant hemangiopericytoma) and paragangliomas of the vagal corpuscle or carotid corpuscle.

The primary treatment of carotid pseudoaneurysms is surgical. The indication is obligatory in patients who present a reasonable risk of morbidity and mortality. Different surgical techniques have been described. The choice of the most suitable will be made on the basis of the extension and location of the lesion. Resection and end-to-end anastomosis, partial resection and plasty with a polytetrafluorethylene patch (PTFE) or saphenous vein, or resection and reimplantation in the external carotid, can be performed.^{1, 3} Recently, techniques have been developed based on endovascular control by the introduction of metal stents and coils in the carotid lumen, which have been confirmed as a safe, minimally invasive, and effective method for the control of traumatic pseudoaneurysms ruptured in the acute phase.³ The approaches also vary, so that in pseudoaneurysms of the carotid siphon we can use a cervical approach, but in higher locations an extensive cervical exposure may be necessary, which includes parotid mobilization with identification of the facial nerve or resection of the styloid and mastoid processes with mandibular subdislocation.^1,3,5,9,10 In exceptional cases, carotid ligation is necessary, in which case there is a high rate of cerebral vascular accident (20-35%).

The prognosis has improved remarkably since the introduction of surgical treatment. The mortality is low and the morbidity and mortality consist of neurologic events and transitory or definitive cranial nerve deficit of 7.2%.¹

The underlying situation of the patient is extremely important in the prognosis of carotid pathology. For instance, in a young patient with good collateral circulation, the neurologic damage is diminished.

Conclusions

Aneurysmal pathology of the carotid artery at the extracranial level is a very uncommon pathology, but it must be considered in our specialty because proper diagnostic orientation as soon as possible can avoid complications and diagnostic or therapeutic maneuvers that may be fatal for the patient.

Dirección para correspondencia:
Dr. B. Rodríguez Caballero
Hospital Universitario "Marqués de Valdecilla"
Avda. Valdecilla nº 25
39008 Santander, España

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