Internal Jugular Phlebectasia

A 16-year-old girl with internal jugular phlebectasia presented for followup ultrasound evaluation. At age 15 months, she was noted to have a right-sided neck mass that appeared when crying or straining. Her weight, height, and head circumference were normal. She had no cardiac anomalies and was otherwise healthy. An ultrasonogram performed at 18 months of age showed a fusiform dilation of the right internal jugular vein; it measured 0.71 to 0.79 cm along the mid-portion when the infant was at rest and 2.98 cm when she was crying. The dilation extended from the mandible down to the clavicle. An ultrasound evaluation at 22 months showed no interval change. At 16 years' follow-up, the dilation had an average diameter of 1.88 cm at rest (A and B) and a 3.95-cm diameter during a Valsalva maneuver (C and D).

Internal jugular phlebectasia is associated with few complications; those that are seen include Horner syndrome,¹ thrombosis,² heart failure,³ and slight dysphagia.⁴ Thus, most studies recommend observation.^5-8 Surgical intervention is generally reserved for cosmetic and psychological purposes.^9,10 However, the fear of rupture or thrombosis has led some to recommend surgery universally.⁹ Postoperative complications are rare but may include intracranial hypertension and thrombosis of the right internal jugular vein with infarction of the right pontine⁹ and injury to vital neck structures, especially with jugular phlebectasia resection.

Tracking the natural history of internal jugular phlebectasia can help answer the question of whether medical intervention is necessary or would benefit the patient. Reports of conservative management describe cases with 6-month follow-up⁵; 1-year follow-up with no progressive dilation⁶; 24-month follow-up with reduced dilation⁷; 3-year follow-up⁸; and 8.5-year follow-up.¹¹ In a review of 45 children with jugular vein phlebectasia, all but 5 ultimately underwent surgery; the remaining 5 children were observed for 2 to 15 years.⁹

In this patient, serial ultrasonography easily confirmed the clinical diagnosis and provided an excellent modality for follow-up. Despite the progressive dilation noted concomitantly with the patient's growth, she has continued to develop normally, has required no activity restrictions, and has remained asymptomatic.

References:

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