Case series: Internal jugular vein ectasia in pediatric patients | Ear, Nose & Throat Journal Skip to content Skip to navigation

Case series: Internal jugular vein ectasia in pediatric patients

| Reprints
January 22, 2016
by Neha A. Patel, MD; Shai Shinhar, MD

Abstract

Jugular vein ectasia is a dilation of the vein without tortuosity. This finding can easily be misdiagnosed or overlooked. Because of its rarity, many pediatricians, otolaryngologists, and pediatric surgeons are unfamiliar with this diagnosis and how to manage it. We report a series of 2 cases of internal jugular vein ectasia in pediatric patients and discuss its clinical findings, simple and noninvasive method of diagnosis, and treatment.

Introduction

Jugular vein ectasia is considered a rare cause of cervical neck swelling in children, but it actually may be underreported. In fact, one survey showed that 96% of pediatricians, 37% of otolaryngologists, and 40% of pediatric surgeons were unfamiliar with this entity.1

The most common site of jugular vein ectasia is the internal jugular system.2 Most cases of jugular vein ecstasia are unilateral, with a 5:1 predominance of the right side vs. the left side.2 Jugular vein ectasia has a slight (1.4:1) male-to-female predominance.3


Case reports

Patient 1. A 4-year-old boy with a medical history of chronic serous otitis media and a surgical history of repeat bilateral myringotomy and tube placement, presented for his follow-up with no ear complaints. His mother did report that for the preceding 2 weeks she had noticed for the first time the appearance of a neck mass in the right lateral neck that appeared when he was laughing, crying, or screaming loudly. The mass was not painful and seemed to be filled with air according to the mother. There was no associated fever, drainage, weight loss, infection, trauma, or hoarseness.

On examination, the patient had no neck tenderness, and no mass was visible initially. Flexible laryngoscopy showed a normal larynx and vocal folds with no abnormality. Computed tomography (CT) of the neck with contrast was performed to further delineate the possibility of a neck mass. The CT showed no laryngocele or abnormal soft-tissue mass.

On further examination, a soft, compressible, nonpulsatile swelling was seen on the right side of the neck when the patient coughed. It measured 3 cm transversely and 4 cm longitudinally and appeared anteromedial to the sternocleidomastoid muscle. There was no associated lymphadenopathy. The lesion had regular borders and did not elevate with deglutination.

Given the new finding on the physical exam, ultrasonography of the patient's neck was performed. The ultrasound revealed dilation in the right internal jugular vein at 14 mm, which further dilated to 19 mm during the Valsalva maneuver (figure 1). The left internal jugular vein at the same level was 9 mm in diameter at rest. There was no evidence of thrombosis. Color Doppler demonstrated turbulent flow within the right internal jugular vein. These findings were consistent with right internal jugular vein ectasia.


Figure 1. Patient 1. Ultrasonography shows the right internal jugular vein at rest (A) and during the Valsalva maneuver (B).

The patient was seen at his 6-month follow-up with no change in his clinical findings. Any change in symptoms would have warranted a follow-up ultrasound to assess for venous thrombosis and size changes. The patient had no pain or cosmetic complaints, and no surgical intervention was performed.

Patient 2. The second patient was discovered within a week of the diagnosis of the first patient. This gives us the idea that jugular vein ectasia is often overlooked. The second patient was also a 4-year-old boy, with a medical history of pharyngeal airway obstruction, asthma, chronic tonsillitis, and developmental delay and a surgical history of bilateral inguinal hernia repair.

After this patient was extubated following an adenotonsillectomy, the team discovered that when he cried, or during a Valsalva maneurver, a mass appeared in his left lateral neck (figure 2). Upon questioning, the patient's family revealed that they had noticed left greater than right neck swelling for the previous year, only when the patient was crying. The mass was not painful according to the father. There was no associated fever, drainage, weight loss, infection, trauma, or hoarseness.


Figure 2. Patient 2. Photos show the patient's neck when he is at rest (A) and during the Valsalva maneuver (B).

On examination, the patient had no neck tenderness or visible mass seen initially. Upon crying, a 2.5 x 3.5-cm soft, compressible, nonpulsatile left neck lesion appeared lateral to the sternal head of the sternocleidomastoid muscle. There was no associated lymphadenopathy. The lesion had regular borders and did not elevate with deglutination. Given the similar scenario to that of the first case, a neck ultrasound was obtained. The ultrasound findings were consistent with left jugular vein ectasia that increased in diameter with patient vocalization. Asymmetry was seen in the maximum diameter of the jugular veins at rest, with the left jugular vein measuring 1.03 cm in diameter compared with the right, which measured 0.77 cm.

Further assessment of turbulence and Valsalva-related changes were limited by patient cooperation. The patient had no pain or cosmetic complaints, so no surgical intervention was performed. This patient will also be observed for any change in clinical findings.


Discussion

Patients with jugular vein ectasia present with a mobile, round or fusiform mass that transiently enlarges with the Valsalva maneuver. While the condition is benign and often asymptomatic, it can also present with discomfort during deglutination, hoarseness upon phonation, a feeling of a cervical foreign body,2 constriction, and the sensation of choking.4

The etiology of jugular vein ectasia is unclear. The differential diagnosis includes laryngocele, superior mediastinal tumor, branchial cleft cyst, thyroglossal duct cyst, retention cyst, and cystic hygroma. The diagnosis is made by bilateral ultrasonography or color Doppler flow imaging at rest and with straining. Ultrasonography reveals fusiform dilation of the internal jugular vein and variations in size during the Valsalva maneuver, with a ratio of approximately 1.5 to 2:1.0 at rest vs. during the maneuver. It can also evaluate for thrombus formation and external compression. Color Doppler flow studies can demonstrate turbulent vascular flow within the affected segment.3 While CT scans may provide some insight into the diagnosis of this lesion, we feel that given the risks of radiographic imaging in children, ultrasound is a better first-line option.

Finally, fine-needle aspiration or drainage of a lesion with this presentation should not be done and can lead to poor outcomes. One study reported a case of a child with jugular vein ectasia that was drained because it had been misdiagnosed as a cervical abscess, necessitating patient transfer to a higher-level facility.5 A compressible lesion in the lower neck of a child should always bring to mind the diagnosis of jugular vein ectasia in order to avoid such disastrous complications.

Long-term follow-up includes serial ultrasound to evaluate for lesion growth or thrombosis. However, because of the rarity of jugular vein ectasia, no clear guidelines for the frequency and intervals of follow-up have been established. While conservative management is recommended, surgery is appropriate in cases of thrombus formation, phlebitis, cosmetic deformity, and rupture.

Case reports of surgical repair include ligation and excision, resection after proximal and distal mobilization followed by end-to end repair,5 transaxillary endoscopic ligation,6 and longitudinal constriction suture venoplasty with encapsulation.5 Surgical complications include craniofacial swelling, thrombosis, and intracranial hypertension. Bilateral lesion excision is more likely to lead to cerebral edema.

These cases bring to light the possibility that jugular vein ectasia is often overlooked by family or misdiagnosed by physicians. Although rare, this entity has a relatively characteristic clinical scenario and an easy method of diagnosis. Its management is straightforward. Physicians should be aware of the possibility of jugular vein ectasia in a child with a neck mass.

 


References

  1. Sander S, Eliçevik M, Unal M, Vural O. Jugular phlebectasia in children: Is it rare or ignored? J Pediatr Surg 1999; 34 (12): 1829-32.
  2. Aydoğan F, Taştan E, Aydin E ,et al. Bilateral internal jugular vein phlebectasia. Ear Nose Throat J 2011; 90 (3): E1-3.
  3. Chao HC, Lin SJ, Huang YC, Lin TY. Ultrasonographic diagnosis and color flow Doppler sonography of internal jugular venous ectasia in children. J Ultrasound Med 1999; 18 (6): 411-16.
  4. Gerwig WH Jr. Internal jugular phlebectasia. Ann Surg 1952; 135 (1): 130-3.
  5. Jianhong L, Xuewu J, Tingze H. Surgical treatment of jugular vein phlebectasia in children. Am J Surg 2006; 192 (3): 286-90.
  6. Chang YT, Lee JY, Wang JY, Chiou CS. Transaxillary subfascial endoscopic approach for internal jugular phlebectasia in a child. Head Neck 2010; 32 (6): 806-11.
From the Department of Otolaryngology, New York Eye and Ear Infirmary, New York, N.Y.
Corresponding author: Neha A. Patel, MD, Department of Otolaryngology, 310 East 14th St., 6th Fl., NY, NY 10003. Email: nepatel@nyee.edu
Ear Nose Throat J. 2016 January;95(1):E33