MyPACS.net: Radiology Teaching Files > Case 72173

never visited INTERNAL JUGULAR VEIN ECTASIA
Contributed by: Faculty and residents Children's Hospital, Radiologist, Children's Health System, Birmingham, Alabama., USA.
Patient: 3 year 2 month old male
History: presents with painless right neck mass that increases when he coughs.
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Findings: Longitudinal and transverse images through the internal jugular vein and carotid artery show perhaps a subtly enlarged vein (figures 1 and 2). Doppler imaging confirms flow in both vessels (figure 3). During the Valsalva maneuver, the internal jugular vein becomes markedly distended (figure 4). The left side is shown for comparison (figure 5).
Diagnosis: right internal jugular vein ectasia
Discussion:

CLINICAL
Internal jugular vein ectasia (phlebectasia, congenital venous cyst, venous aneurysm, essential venous dilatation) presents as a soft compressible cystic swelling in the neck during any kind of straining that increases intrathoracic pressure, such as coughing, sneezing, or crying. No bruit can be auscultated. It is considered an asymptomatic benign condition, but in a minority, there are complaints of constriction, choking, bluish dicoloration, throbmosis, tongue pain, and discomfort during physical activity (Lugo-Vicente 2001). One case of associated Horner's syndrome has been reported (Inci 1995). One small cohort of 8 children followed for 6 months showed no progression (Chao 1999).

DIFFERENTIAL DIAGNOSIS
The differential diagnosis for a cystic neck mass that increases in size with the Valsalva maneuver includes laryngocele (most common), cystic hygroma, superior mediastinal tumor, and other vascular lesions. Such venous dilatation should lead to consideration of any mechanical obstruction of the neck or mediastinum (LaMonte 1976).

IMAGING
Diagnosis can be confirmed with CT, MR, or venography, but ultrasonography will usually suffice. The vein will show a fusiform dilatation during the Valsalva maneuver. Doppler ultrasound shows an essentially flat wave indicating a venous structure (Lin 1990).

EPIDEMIOLOGY
They are considered to be rare to uncommon, although one source suggests that they are perhaps just underrecognized (Sander 1999). It has been reported not to be sex-related, although in once case boys were said to be affected more than twice as often as girls (Lugo-Vicente 2001). They usually occur on the right side (al Dousary 1997). Anatomically, venous ectasias have been described in four regions: intracranial, the neck and thorax, visceral veins, and the lower and upper extremities. In the neck they occur in the following sequence: internal jugular, external jugular, anterior jugular, and the superficial comminicans. Bilateral internal jugular vein ectasia has been reported (Gendeh 1994).

ETIOLOGY
The cause of venous ectasia is unknown. In children they have generally been thought to be congenital, although is has not been proved. Histological examinations of the wall have revealed a reduction in elastic fibers, intimal hyalinization, and no abnormality (Ekim 2002). They may be due to incompetence of the bicuspid jugular venous valve (Stocks 1997).

References:

Stocks RMS, Milburn M, Thompson J. 305 Unusual Neck Masses Secondary to Jugular Venous Abnormalities: Case Report and Discussion, Southeastern Surgical Congress ABSTRACTS Volume 63, No. 4 April 1997.

Sander S, Eliçevik M, Unal M, Vural O. Jugular phlebectasia in children: is it rare or ignored? J Pediatr Surg, 1999 Dec, 34:12, 1829-32

Lugo-Vicente HL, ed. Jugular Phlebectasia. Pediatric Surgery Update Vol 18 No 2, Aug 2001.

Inci S. Bertan V. Kansu T. Cila A. Horner's syndrome due to jugular venous ectasia. Childs Nervous System. 11(9):533-5, 1995 Sep.

Gendeh BS. Dhillon MK. Hamzah M. Bilateral internal jugular vein ectasia: a report of two cases. Journal of Laryngology & Otology. 108(3):256-60, 1994 Mar.

LaMonte SJ, Walker EA, Moran WB. Internal jugular phlebectasia. A clinicoroentgenographic diagnosis. Arch Otolaryngol, 1976 Nov, 102:11, 706-8.

Lin MJ, Chou YH, Tiu CM, Yeh TJ, Wei JF, Chang T. Duplex Doppler ultrasound of internal jugular vein ectasia. Case report. Chung Hua I Hsueh Tsa Chih (Taipei), 1990 Sep, 46:3, 190-3.

al Dousary S. Internal jugular phlebectasia. Int J Pediatr Otorhinolaryngol, 1997 Jan, 38:3, 273-80.

Yokomori K, Kubo K, Kanamori Y, Takemura T, Yamamoto T. Internal jugular phlebectasia in two siblings: manometric and histopathologic studies of the pathogenesis. J Pediatr Surg, 1990 Jul, 25:7, 762-5

Gürpinar A, Kiristioglu I, Dogruyol H. Jugular phlebectasia. Eur J Pediatr Surg, 1999 Jun, 9:3, 182-3.

Chao HC. Wong KS. Lin SJ. Kong MS. Lin TY. Ultrasonographic diagnosis and color flow Doppler sonography of internal jugular venous ectasia in children. Journal of Ultrasound in Medicine. 18(6):411-6, 1999 Jun.

Ekim H, Ozen S. Primary venous aneurysm of the external jugular vein. Eastern J of Med Vol 7 No 1, 24-25, 2002.

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Additional Details:

Case Number: 72173Last Updated: 10-06-2003
Anatomy: Face and Neck   Pathology: Vascular
Modality: USExam Date: 04-07-2003Access Level: Readable by all users
Keywords: phlebectasia, jugular vein ectasia, jugular venous aneurysm, internal jugular vein, internal jugular cyst

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