Jugular vein ectasia is a localized fusiform dilation of the jugular venous system, usually the internal jugular vein. It is usually on the right side; affects males twice more than females and presents early in life. It is usually of unknown origin. The incidence of IJVP is rare; 247 cases have been reported in total in medical literature;150 cases of them are children. 1 due to this scarcity, no clear etiology or pathophysiology could be universally agreed on 2. Rossi A et al. 3 and Yu-Tang Chang et al. 4 believe that the most likely cause for this pathological entity appears to be a congenital defect of the internal jugular vein wall structure which leads to gradual decrease in the elasticity of the venous wall. Other congenital etiology like increased scalenus anticus muscle tone, anomalous reduplication of the IJV, compression of the vein between the head of the clavicle and the cupola of the right lung have been hypothesized as causative or contributing factors for the development of IJVP. 5 El Fakiri et al. hypothesized that internal jugular phlebectasia develops as a result of the superior vena cava hypertension during inspiration, while Paleri et al believes that the distribution of the valves in the superior vena cava or anomalies of the vein wall is the main etiology. Some acquired cases of adulthood phlebectasia developed as a form of post traumatic sequela, for example in the case of accidental clavicle fractures. 678. In addition, association with Neurofibromatosis Type 1 and Ehler-Danlos Syndrome have been documented in the literature. Histopathologically IJVP show a normal venous wall structure. Smooth muscle and elastic fibers disarray have been reported in a minority of cases. 9

The authors here believe that IJVP is a multifactorial abnormality; in addition to the possible congenital defect in vein wall elasticity as proposed by many authors, there is a constant rule to increased venous pressure due to anatomic reasons like clavicle fractures and upper mediastinal tumors, or due to habits or jobs associated with severe chronic straining.

diagnosis of this rare cause of childhood neck mass is a big challenge to the pediatrician and needs a high level of clinical awareness of its typical clinical manifestation as a soft, nontender, nonpulsatile neck mass with changing size (enlarges by Valsalva maneuver or straining and vanishes rapidly at rest). These clinical characteristics help to differentiate it from other causes soft neck lumps. JVP is a fusiform dilatation unlike venous aneurysm which is acquired segmental saccular fixed dilatation in adults. The term phlebectasia indicates abnormal outward dilatation of the vein without tortuosity and differs from the term varix, which implies dilatation plus tortuosity.

The differential diagnosis should include: cystic hygroma, branchial cyst, laryngocele, cavernous hemangioma, dermoid cyst and superior mediastinal mass.

Diagnosis can be confirmed by imaging study. Doppler ultrasonography (USG) of the neck is the gold standard diagnostic investigation in the developing countries. Chest and neck computerized tomography with contrast, CT- angiography, or MRI give better diagnostic accuracy and help to exclude intrathoracic masses. These imaging modalities are helpful to exclude complications and for evaluation before surgical intervention. 1011

Due to its benign nature, treatment is usually conservative. Long term clinical observation and serial USG comparative measurements are highly recommended. most case regress spontaneously. However, massive enlargement and longitudinal extension may occur which might lead to a considerable cosmetic and psychological distress.

Complications like dysphagia, dysphonia, Horner syndrome, thrombosis, and rupture of the vein wall after minor trauma are possible. Such events necessitate surgical treatment after accurate evaluation to exclude any laryngeal and mediastinal etiology. 121314