Internal jugular phlebectasia (also known as venous congenital cyst, venous aneurysm, vein ectasia or essential venous aneurysm) refers to an isolated abnormal fusiform or saccular dilatation of the internal jugular vein.1
It presents as a soft, compressible cystic swelling in the neck that increases in size during any kind of straining that increases the intrathoracic pressure, such as coughing, sneezing or crying. It is considered as an asymptomatic benign condition but, in some cases, there are complaints of constriction, choking, tongue pain and discomfort during physical activity.2
Our patient had noticed the neck swelling for over 2 years but only presented when she associated it with heaviness in her chest.
It is a rare benign vascular abnormality but may be under-reported3
possibly due to its benign nature. There is no known sex predilection, although one case reported that boys were affected twice often as girls.2
Most reported cases are in children. They usually occur on the right side as in our patient but may be bilateral.4
Anatomically, phlebectasia in the neck have been described in the following sequence in frequency: internal jugular, external jugular, anterior jugular and superficial comminicans.5
The cause of venous ectasia is unknown. It may be congenital, although this has not been proved. Histologically, examinations of the wall have revealed a reduction in elastic fibres, intimal hyalinisation and no abnormality.6
Diagnosis is usually confirmed with ultrasonography, CT, MR or venography.7
Ultrasound will often suffice;, allowing avoidance of more invasive examination, radiographic exposures and unnecessary surgical intervention. Doppler ultrasound shows an essential flat spectral wave indicating a venous structure.8
Ultrasound has been used to diagnose jugular phlebectasia since 1982.9
The differential diagnoses for a cystic neck mass that increases in size with Valsalva manoeuvre includes laryngocele, cystic hygroma, superior mediastinal tumour and other vascular lesions.10
Although cases of internal and external jugular phlebectasia have been reported mostly in children and non-African adults, as far as we are aware no case has been previously reported in the electronic literature in an African adult.3 11
Nonetheless, our case showed ultrasound and CT features described in the reported cases in children.4 12
The swelling is not known to progress rapidly and there have been no instances of spontaneous rupture of the swelling or other serious complications7 11
The complications of this condition are rare but include thrombosis, congestive heart failure and massive haemorrhage secondary to trauma.12
No treatment is indicated for this benign self-limiting condition except in a few patients who complain of symptoms and require surgical resection of the affected vein or covering with muscular segment.13
Some authors believe that ligation of the jugular vein may produce effects of venous congestion in a small subset of patients resulting in cerebral oedema. Jugular vein ligation cannot be applied in cases with bilateral affliction.14
Our patient was essentially asymptomatic from the neck mass and the pulmonary congestion observed is most likely is due to her unattended hypertensive heart disease. Surgical excision was based on cosmetic concerns.
- Internal jugular phlebectasia is a rare, benign, venous anomaly, commonly seen in children but can occur at any age.
- The cause is unknown but thought to be congenital, although this has not been proved.
- Ultrasound often suffices in making a diagnosis; thus, preventing more invasive examinations, radiographic exposures and unnecessary surgical intervention.
- Colour Doppler ultrasonography is a good modality for showing flow turbulence and real-time increase during Valsalva manoeuvre.
- It usually does not require surgical intervention although this can be offered for cosmetic concerns with satisfactory results.