PHLEBECTASIA OF THE INTERNAL JUGULAR VEIN : A Novel Approach to Management
GB DAVER*, SANTOSH BALAKRISHNAN**, TARUN SINGHAL***
*Professor and Head, **Lecturer, ***Chief Resident, Department of General Surgery, Grant Medical College, Sir JJ Group of Hospitals, Mumbai - 400 008. India.
A fifteen-year-old boy presented to us with a neck swelling that appeared on straining or coughing. Investigations revealed an internal jugular phlebectasia on the right side. In spite of reassurance that there were no chances of the swelling bursting or causing any complications, the parents wanted surgical correction of the unsightly swelling. Ligation of the affected Jugular could not be carried out for fear of causing cerebral oedema since the contralateral vein could not compensate for the flow. Hence the whole affected vein was sheathed in an 8 mm Poly tetra fluro ethylene (PTFE) tube graft from the site of its emergence to the point where it disappeared behind the right sternoclavicular joint. This cured the patient of the swelling and at the same time did not hamper the venous drainage of the brain.
Jugular vein ectasia is a rare benign dilatation of the jugular veins. It is commonly seen in children or young adults.[1-3] While the patients may present at an older age, the history often dates back to the childhood. The patient presents with an expansile cystic swelling in the neck, which becomes more prominent on straining or on performing a Valsalva manoeuvre. Except for the unsightly swelling there are no other complaints. The swelling is not known to progress rapidly and there have been no instances of spontaneous rupture of the swelling or other serious complications.[1,2,4] The possible differential diagnosis for the swelling could include a branchial cyst, cystic hygroma, laryngocoele, cavernous haemangioma and superior mediastinal cysts.[2-4]
Treatment may be sought on account of the swelling for cosmetic reasons. Though most often reassurance is all that is needed, a few patients may want the swelling to be removed. Ligation of the jugular vein, which has been the only method of treatment applied so far, may pose a danger in a small subset of cases due to the resultant cerebral oedema. Any such disastrous outcome of the treatment of a benign condition would be unacceptable. At our institution, we succeeded in treating such a patient, by wrapping the dilated segment in an 8-m.m. diameter PTFE tube graft.
After investigating the patient to rule out any other cause of jugular ectasia, such as superior mediastinal mass causing obstruction, or an Arteriovenous (AV) malformation, the patient is taken for the surgery. The exact extent of the ectatic segment is accessed by either a spiral Computed Tomography (CT) scan with dynamic contrast enhancement and 3-D reconstruction or by an angiography [Magnetic Resonance (MR) or Digital subtraction angiography (DSA)]. An 8 mm PTFE tube graft of the appropriate length is kept ready. Under appropriate anaesthesia the whole of the ectatic jugular vein is dissected out of the carotid sheath from its origin at the base of the skull to the point where it goes retrosternal. The PTFE graft is cut open longitudinally and wrapped around the vein in its entire extent. Few tributaries of the vein may have to be ligated for the same. Head high position is given causing the vein to collapse and the cut edges of the graft are sutured to each other using 4-0 polypropylene sutures on atraumatic needle. Care is taken to prevent inadvertent damage to the jugular vein or the contents of the carotid sheath. This reinforcement prevents the vein from dilating, and at the same time preserves its function.
A fifteen-year-old boy was brought to us by the mother with history of a swelling appearing on the right side of the neck on straining or on performing the Valsalva manoeuvre. This worried the mother, and at the same time the boy was subject to a lot of ridicule among peers. Clinical examination revealed a cystic fluctuant swelling of 15 cm X 5cm in the region of the right anterior triangle of the neck, which increased in size on performing a Valsalva manoeuvre. It completely regressed on sitting up or standing and was totally compressible. There was no bruit or hum over the swelling, and it was not transilluminant or pulsatile. With a differential diagnosis of an AV malformation, or a laryngocoele in mind, a CT scan was ordered which revealed an ectasia of the right internal jugular vein (Fig. 1) with no demonstrable cause. To rule out any AV malformation, a colour Doppler study and an MR angiography were performed and these too did not demonstrate any cause for the pathology. The patient and his mother were reassured, but they desired that the swelling be removed. The child was the son of a widowed mother and any complications, which may ensue on ligation of an internal jugular vein, were unacceptable. Hence with due informed consent of the patient and the mother about ligation of the vein as well as the new procedure, the child was taken up for surgery.
On table after dissecting out the whole vein (Fig. 2), proximal and distal clamping of the vein was done using vascular clamps. However, the proximal segment of the vein was seen to bulge and become tense which did not regress even after a few minutes had elapsed after clamping. We were doubtful if the contralateral jugular vein would be able to compensate for the high flow rates of the dilated vein that was to be ligated. Hence the plan to ligate and excise the vein was abandoned and the wrap procedure as described was performed (Fig. 3).
Postoperatively the swelling did not appear and the recovery was uneventful. The child went home on the seventh postoperative day. A follow up visit after two months revealed no swelling and a follow up Doppler showed normal blood flow pattern in the treated vein.
Fig. 1: Contrast enhanced CT scan of the neck showing dilated internal jugular vein. Fig. 2: Dilated right internal jugular vein (operative picture). Fig. 3: Right internal jugular vein wrapped in 8 mm PTFE tube graft.
Jugular vein phlebectasia is a benign condition seen in children and young adults, which is not known to cause any complications. Considering the age group affected, cosmetic defects caused by the swelling may be the only reason for seeking treatment.[1-5] Many authors advise a conservative approach based on reassurance,[2,5] but due to cosmetic reasons these have been excised with ligation of the jugular vein with no gross side effects.[1,4,5] Ligation of the jugular vein may produce effects of venous congestion in a small subset of patient resulting in cerebral oedema. Jugular vein ligation is too radical a procedure for such a benign condition, and this can definitely not be applied in cases with bilateral affliction. Pathological studies by different groups have shown that veins are histopathologically normal.[1,2,4] Hence in such cases where cosmesis alone is the reason for surgery, the procedure described by us should suit the requirement well in being effective in curing the patient of the swelling without causing any physiological alteration. REFERENCES
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