Jugular phlebectasia in children: is it rare or ignored?

Phlebectasia of the jugular veins is a venous anomaly that usually presents in children as a soft cystic swelling in the neck during straining. The purpose of this report is to discuss the differential diagnosis, the methods of imaging, the mode of treatment, and to demonstrate some factors that have made us believe that the condition may not be an actual rarity but rather has been ignored.

Eight cases of unilateral internal jugular phlebectasia were treated surgically (ie, excision of the dilated portion of the vein) from 1987 to 1998. The age of the patients ranged from 3 to 14 years. There were 3 girls and 5 boys. The lesions were right sided in 6, and left sided in 2 children. The patients underwent surgery after comparative ultrasonographic confirmation of the diagnosis. Furthermore, the authors prepared a simple questionnaire for evaluating the level of knowledge about this lesion among the related specialists. One hundred ten physicians were asked to describe the jugular phlebectasia and its ideal treatment.

All of the patients were discharged from the hospital 24 hours after surgery. Follow-up periods ranged from 6 months to 6 years and no complaints were noted at the time of most recent visits. Our questionnaire results showed that 96% of 73 pediatricians, 37% of 22 otorhinolaryngologists, and 40% of 15 pediatric surgeons did not know what the jugular phlebectasia was.

Color Doppler sonography alone is sufficient for the diagnosis of jugular phlebectasia. The authors recommend surgical excision in asymptomatic cases for cosmetic and psychological purposes. The rarity of the lesion may be caused by a lack of knowledge among the related physicians and the tendency of reporting only surgical results.