Tortuous common carotid artery presenting as a pediatric submandibular neck mass

1. Case report 

A 4-year-old boy was referred with a 1-year history of a pulsatile mass in the left submandibular region. There was no previous history of trauma or surgery and the lesion had not changed in size. The patient denied any other associated symptoms and was otherwise well. On physical examination, a painless, mobile, pulsatile vessel 1cm in diameter was palpable. The remainder of the head and neck and neurologic exams were normal. Specifically there was no evidence of pharyngeal pulsations, hypoglossal or marginal mandibular nerve palsy, or neck lymphadenopathy. Magnetic resonance angiography showed multiple spiralling loops rotating 360° along a vertical axis in bilateral vertebral and internal carotid arteries. Another loop was noted in the left common carotid artery corresponding with the left submandibular mass and there were no identifiable aneurysms. Intracranial vasculature was also ectatic and tortuous suggesting dolichoectasia (Fig. 1). The patient was informed of the vascular nature of the mass and a neurosurgical consult was obtained due to the intracranial location of the vessels. Given the common carotid loop was freely mobile in the submandibular region, no limitations were suggested in his physical activities. Importantly, the family was cautioned on the possibility of bleeding from this site following trauma. He is currently followed by neurosurgery and is otherwise well.

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Fig. 1. Magnetic resonance (MR) and angiography images of pediatric submandibular mass. (a) MR scout image localizing area of interest and plane of rotation for (b) and (c). (b) Coronal MR image with large flow void (arrow) noted in left submandibular region. (c) MR angiography vessel image, tortuous common carotid loop (arrow) corresponding to left submandibular mass.

2. Discussion 

The majority of pediatric submandibular masses are inflammatory in nature and resolve either spontaneously or following a short therapeutic regime of antibiotics [1], [2]. Vascular lesions in the lateral region of the head and neck are usually proliferative vascular tumours, such as infantile hemangiomas, or vascular malformations namely lymphatic, arterial, venous, arteriovenous, capillary or mixed malformations [3]. Cervical vascular aneurysms or pseudoaneurysms may also present as lateral neck masses, and these more frequently are a consequence of blunt or penetrating trauma and spontaneous dissection [4].

Internal carotid artery (ICA) tortuosity and other anomalies are occasionally encountered during endoscopic examination of the oropharynx as pulsatile swellings [5], [6], and only one incidental finding of a tortuous common carotid artery observed during an adult neck dissection has been reported [7]. Furthermore, variations of the extracranial ICA are estimated to be present in 5% to 6% of the general population [8]. Recognizing the proximity of an aberrant ICA to the parapharyngeal wall has clinical significance in pharyngeal procedures as highlighted by Pfeiffer and Ridder [8]. These researchers developed a clinicoradiologic classification system that considers the vessel's location in relation to the pharyngeal wall in order to facilitate operative planning in these patients. Aberrations in the ICA are a recognized concern, with increased incidence in 22q11 syndrome [9], [10]. In contrast, aberrations in the common carotid are less frequently reported.

External lateral neck masses resulting from vascular aberrancy is uncommon in either adult or pediatric populations. In children, the differential diagnosis of congenital carotid artery tortuosity, includes Marfan's syndrome [11], 22q11 deletion syndrome (or velocardiofacial syndrome) [9], [10], autosomal dominant polycystic kidney disease (ADPK) [12], arterial tortuosity syndrome [13] Menkes and Moyamoya syndromes [14], [15], [16]. Significantly, however, none of these causes were found to correlate with the clinical features noted in our patient.

Marfan's syndrome causes the enlargement of distal segments of the aorta and medium sized arteries. Dilated aortic roots, aortic regurgitation, aortic dissection or carotid aneurysms are frequently encountered [11]. External neck masses, however, are not apparent in this disorder.

Chromosome 22q11 deletion syndrome, also known as velocardiofacial syndrome, is characterized by congenital cardiac defects, palate abnormalities, characteristic facies and learning disabilities [9], [10]. Ectopic ICA anomalies documented as pharyngeal wall pulsations are reported in nearly 25% of cases. External pulsatile neck masses, however, are not included in the clinical manifestations.

Autosomal dominant polycystic kidney disease is an inherited disorder affecting a variety of connective tissues with bilateral renal cysts and numerous cardiovascular anomalies. Schievink et al. [12] reported intracranial arterial dolicoectasia in ADPK resulting in cerebral ischemic symptoms. Pediatric involvement has not been reported and was not identified in this patient.

Arterial tortuosity syndrome (ATS) [13] is a rare vascular disorder with generalized and progressive tortuosity of all major arteries, joint laxity, hyperextensible skin, and severe keratoconus. The presenting patient did not have any joint or integument findings to suggest ATS was the cause of the carotid anomaly.

Menkes syndrome is an X-linked recessive neurodegenerative disorder of copper transport with infantile onset. The disease is classically associated with growth retardation, peculiar hair characteristics, and focal cerebral and cerebellar degenerations [14] and if untreated, death by 3 years of age [15]. Given the normal neurologic status and lack of other features, Menke's syndrome is an unlikely cause in our patient.

Finally, Moyamoya disease is a rare, progressive veno-occlusive cerebrovascular disorder [16] that affects children under 10 years of age. Most patients present with ischemia resulting in severe motor weakness and progressive cognitive delays. Typically there is stenosis or occlusion of the ICA with abnormalities of the anterior and middle cerebral arteries. In our case no vascular stenosis was identified and he is neurologically intact, therefore this disease was rejected as a likely diagnosis.

In adults, carotid artery tortuosity is considered a risk factor for stroke but are not treated unless symptomatic [17]. Little, however, is known about the implications and management of tortuous carotid arteries when found in children. Conservative management was elected for our patient due to the asymptomatic nature of the lesion and the potential risks associated with surgical intervention.

3. Conclusion 

Tortuosity of the ICA is of special concern to the otolaryngologist because vascular injury and the risk of hemorrhage during tonsillectomy, adenoidectomy, drainage of a peritonsillar abscess, or pharyngeal flap procedures. While common carotid artery anomalies are a far less common cause of pediatric neck masses, they too could cause devastating injury or death if biopsied or ligated unknowingly. Our case illustrates the importance of considering an aberrant common carotid artery as a rare cause of a pediatric neck mass.

Conflict of interest statement 

None.