Volume 3, Issue 4 , Pages 158-160, December 2008
Jugular phlebectasia: A cause of neck swelling—Case report
Article Outline
Summary
Jugular phlebectasia is an anomaly usually present in children presented as a soft and compressible cervical mass, which arises during straining, crying and phonation. Diagnosis is usually made through Doppler sonogram and CT scan. Treatment is either conservative or surgical, depending on the hemodynamic and aesthetic commitment. We present a jugular phlebectasia case in a 10-year-old child, associated to the atrophy of the sternocleidomastoid muscle and other vascular anomalies. We highlight the importance of a thorough investigation including magnetic resonance examination, and alert for that pathology, which is probably under diagnosed.
Keywords: Vascular disease, Neck, Abnormalities
1. Introduction
Venous aneurysms or phlebectasias are rare vascular entities in adults and children, although literature contains at least 18 reports of affected pediatric patients [1]. The differential diagnostic includes external laryngeal diverticulum, tumors or mediastinum cysts and phlebectasia [2], which could be presented in a similar manner.
Jugular vein phlebectasia is a rare venous anomaly, which has been recently diagnosed more frequently [3], [4]. It consists of an abnormal dilatation of the jugular vein that is visually presented as a soft and compressible cervical mass that increases the intra-thoracic pressure. It usually occurs in children, but it can occur in any age and affect both genders. There are reports on jugular phlebectasia in asthmatic children [5] and in Menkes disease [1]. Published cases review shows it occurs mostly on the right side. There are a few cases described that present a bilateral lesion [6].
Dilatation of the inner jugular vein with Valsalva maneuver suggests an obstructive mechanism in the neck or mediastinum, but it has an unknown cause and several hypotheses are being proposed. Most essays have found changes in the venous wall, with a decrease in its elasticity [3]. All neck veins can be affected, and the internal and external jugular, the previous jugular and the superficial communicating ones are more common.
Doppler sonogram and CT are the more useful methods in the investigation, and venography, arteriography and gamma-ray CT can also be used.
The treatment is conservative when there are no complications or deformities. Spontaneous rupture was not reported until then. The only complications described were thrombosis and Horner's syndrome. Surgical treatment, usually with aesthetic purposes, consists of the excision of the dilated portion of the vein, or a unilateral excision of the vein, usually with no further complications.
2. Case report
T.O.D. leukoderm, female gender, 10-year-old, natural from and residing in Ribeirão das Neves, Minas Gerais state, has sought for attendance with a soft and compressible cervical mass picture to the right, which arises only during straining, crying or phonation and present since birth, apparently unrelated to any underlying systemic disorder or specific genetic syndrome. During examination, she presented a cervical asymmetry with sternocleidomastoid muscle atrophy to the right, discrete hoarseness and neck swelling during continuous phonation and Valsalva maneuver (Fig. 1). Doppler sonogram, CT, and magnetic resonance evaluations evidenced the atrophy of the sternocleidomastoid muscle to the right, with normal topography of the internal jugular vein, with ecstasia and engorgement, almost triplifying its size after a Valsalva maneuver. Subclavian vein in a supraclavicular position. Common carotid artery emerging right from the aortic arch, side by side with the common left carotid. There is no brachiocephalic trunk. Right vertebral artery emerging from the subclavian. Subclavian artery in supraclavicular position emerging from the aortic arch (Fig. 2). No changes could be detected from videolaryngoscopy. Patient underwent a cardiologic evaluation and did not present an hemodynamic repercussion. We have chosen the conservative treatment because it does not present any important aesthetic complications or deformities. The patient is undergoing a follow-up and does not show changes in the clinical picture.
Fig. 1.
Macroscopic aspect of the lesion.
Fig. 2.
Cervical region CT, evidencing the lesion.
3. Discussion
Jugular phlebectasia is a rare anomaly presented similarly to Laryngocele. The lesion is predominantly present on the right side, as in the case presented. In the cases already published in literature we could not find any description of association of jugular phlebectasia with sternocleidomastoid muscle anomalies, as well as vascular anomalies such as the ones presented by the patient herein. Doppler sonogram and CT are the most useful methods in the investigation, as well as venography, arteriography and gamma-ray CT [7], [8]. Doppler sonogram and CT were complemented with magnetic resonance in order to obtain a better evaluation of the vascular anatomical variations.
When there are no complications or deformities, the treatment is conservative. Spontaneous rupture has not been reported until then. Thrombosis and Horner's syndrome were the only complications described [3]. Surgical treatment, usually with aesthetic purposes, consists of the excision of the dilated portion of the vein or the unilateral excision of the vein, usually without any complications. In the case herein we have opted for the conservative treatment, for the patient does not present any hemodynamic alterations, nor any important aesthetic complications or deformities.
4. Conclusion
A jugular phlebectasia is a rare occurrence. However, it is under diagnosed, and must be considered in neck swelling cases associated to straining. The differential diagnostic is performed without difficulties through cervical sonogram and CT, examinations suggested as being enough by several studies published. However, we suggest more detailed essays with magnetic resonance due to the possibility of presenting other anomalies, such as the ones described in the patient herein.
Acknowledgment
We are grateful to the Support Center for Scientific Publications of Santa Casa de São Paulo, Faculty of Medical Sciences for the editorial assistance.
References
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- . Jugular phlebectasia in children: is it rare or ignored?. J. Pediatr. Surg. 1999;34:1829–1832
- . Unilateral right-sided internal jugular phlebectasia in asthmatic children. J. Pediatr. Child Health. 2000;36:517–519
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PII: S1871-4048(08)00021-X
doi:10.1016/j.pedex.2008.03.002
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 3, Issue 4 , Pages 158-160, December 2008
