Bilateral Internal Jugular Vein Ectasia: a Report of Two Cases

The Journal of Laryngology and Otology March 1994, Vol. 108, pp. 256-260

Bilateral internal jugular vein ectasia: a report of two cases

B. S. GENDEH, M.S.*, M. K. DHILLON, M.S.f, M. HAMZAH, M.S.*

Abstract Internal jugular vein ectasia is a venous anomaly commonly presenting as a unilateral neck swelling in children and adults. Literature reports of bilateral presentation are rare. Bilateral Doppler ultrasonography is the diagnostic investigation of choice. The possible pathology, aetiology and management are discussed. Conservative management of bilateral cases is recommended in uncomplicated cases. Key words: Jugular veins, internal, Ultrasonography

Introduction clavicle with a mean enlargement measuring 5.4 mm which Internal jugular vein ectasia was first described by Harris (1928). It is a rare condition in which there is a fusiform or saccular dilatation of the internal jugular vein. Only two cases of bilateral neck swelling has been reported (Leung et al., 1983; Walsh etal., 1992) whereas 32 cases of unilateral swellings have been reported under a variety of names including venous cyst, venous aneurysm, venectasia, venous ectasia, aneurysmal varix and venoma.

Case reports Case 1 A healthy four-year-old Chinese boy presented with a two- month history of painless recurrent bilateral neck swelling which was noticeable on exertion (Figure 1). There was no history of dysphagia or previous neck trauma. Examination during Val- salva manoeuvre revealed a soft, nontender bilateral mass in the lower anterior triangle of the neck which disappeared at rest. A venous hum was not detectable on auscultation. The transillum- ination test was negative. Ultrasonography of the neck revealed a nontortuous dilatation of the distal segment of the right and left jugular vein with mean enlargement measurements of 5.2 and 4.8 mm respectively on Valsalva manoeuvre (Figure 2).

Case 2 A thirty-six-year-old Chinese teacher presented with a one and a half-year history of nonprogressive, painless, intermittent swelling of the left neck which was noticeable on coughing or swallowing (Figure 3). The patient had a history of unproductive intermittent chronic cough for the past five years, each episode lasting for several days. There was no history of hoarseness, dysphagia or previous neck trauma. Examination on swallowing or on a Valsalva manoeuvre revealed a soft compressible, nontender, bilateral neck swelling at the anterior border of the sternocleidomastoid muscle just above the clavicle which was compressible on digital pressure. No venous hum was detectable on auscultation. An ultrasound of the neck revealed a markedly FIG. 1 dilated segment of the left internal jugular vein just above the Bilateral neck swelling noticeable on exertion in a male child.

From the Department of Otorhinolaryngology*, National University of Malaysia (NUM), Kuala Lumpur and the Department of Radiologyt, General Hospital, Kuala Lumpur, Malaysia. This paper was presented at the XVth World Congress of Otorhinolaryngology, Head and Neck Surgery, Istanbul, Turkey, June 1993. Accepted for publication: 15 September 1993.

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Fia. 2 Ultrasonography of neck of child revealing dilatation of distal segment of right (above) and left (below) jugular vein on Valsalva manoeuvre.

peritoneal and cisterna chyli. All the other sacs separate from their parent veins to establish independent systems except the jugular sacs which retain their connection with the jugular venous system. Well formed valves are present at the site of entrance of the lymphatic channels into the veins to prevent reflux of blood into the lymphatic channels. Steinberg and Watson (1966) believe that the absence of incompetence of these valves will lead to a persistent embryonic lymph sac which will become evident on Valsalva manoeuvre. Ectasia of the internal jugular vein (IJV) presents a soft cystic swelling mainly in the right lower third of the neck at the anterior border of sternocleidomastoid muscle (Bowdler and Singh, 1986) predominantly affecting young children. The swelling is precipitated by Valsalva manoeuvre and disappears at rest. A venous hum may be present and the swelling can classically be obliterated by pressure from above. Most histopathological specimens revealed a localized dilatation in the venous wall with histologically normal venous structure (Gordon et al., 1976; Passariello et al., 1979; Bowdler and Singh, 1986). Several authors found no significant abnormality (Gerwig, 1952; Gordon etal., 1976), but Danis (1982) reported elastic tissue dysplasia, focal intimal thickening with an increased amount of connective tissue and predominant smooth muscle cells. The differential diagnosis of a cystic swelling in the lower neck of a child or a young adult must include a branchial cyst, thyroglossal cyst, dermoid cyst, cavernous haemangioma, cystic hygroma, laryngocoele and a persistent jugular sac (Steinberg and Watson, 1966). The association with the Valsalva manoeuvre occurs in laryngocoeles, superior mediastinal cysts and ectasia of internal jugular vein. A chest radiograph which includes the lower neck region, performed during Valsalva manoeuvre and at rest may exclude the former two, but further radiological evaluation is required to confirm the diagnosis of FIG. 3 ectasia. Prominant left neck swelling noticeable on exertion in an adult male. Percutaneous venography either by direct puncture or by the transfemoral route was the previous investigation of choice became evident on exertion. A smaller, but similar finding with a (Okay et al., 1970; Gordon et al., 1976; Passariello et al., 1979). mean enlargement measuring 4.6 mm involving the right inter- The contrast media will outline the jugular venous system and nal jugular vein was also detected on exertion (Figure 4). This will show a localized dilatation during Valsalva manoeuvre. was further confirmed by a left internal jugular venogram Aneurysms of superficial communicating veins are best visual- (Figure 5). ized by direct injection of contrast media into the proximal Bilateral internal jugular vein ectasia was diagnosed in both dilated vein. This invasive technique is, however, potentially the cases. In the absence of complications, conservative manage- dangerous and the non-invasive techniques of ultrasonography ment was recommended in both. The patients remained well (Stevens et al., 1982) and CT scanning (Som et al., 1985) have with the swelling unchanged at more than two and three years been advocated. Ultrasonography is the investigation of choice; follow-up in Cases 1 and 2 respectively. it is widely available, comparatively inexpensive, of enormous value in children and accurately defines the extent of the lesion Discussion and it relationship with the surrounding structures in the lower neck. Jugular venous ectasia is a rare clinical entity (Gordon et ah, 1976; Passariello et al., 1979; Leung et al., 1983). In the majority Treatment largely depends on the presenting symptoms and of cases, it is idiopathic and probably congenital in nature the cosmetic effect. Excision of the mass employing a supra- especially in children (LaMonte et al., 1976; Danis, 1982), but clavicular incision can be offered especially to females mainly several predisposing factors have been suggested: superior for cosmetic reasons. An unsightly mass in the lower neck on mediastinal irradiation (Harris, 1928), anomalous reduplication Valsalva manoeuvre should be weighed against a surgical scar of internal jugular vein (Som et al., 1985), increased scalenus and the possible complications of the operation, which may anterior muscle tone (Rowe, 1946), compression of the jugular require splitting of the sternum (Steinberg and Watson, 1966). In bulb between the head of the clavicle and the cupula of the right this case report of bilateral internal jugular vein ectasia, surgical lung(Gerwig, 1952; LaMonte et al., 1976), trauma and congeni- excision would be hazardous with a high risk of cerebral oedema tal muscular defect within the wall of the vein itself (Yokomori et and its consequences. As there are no reported cases of rupture of al., 1990). The dilatation could be secondary to mechanical com- the untreated internal jugular vein ectasia (Gordon et al., 1976; pression of the left innominate vein by a high, tortuous, aorta in Passariello et al., 1979; Leung et al., 1983) we advocate a con- hypertension or the compression of the venous structure in pec- servative approach. tus excavatum (Gordon etal., 1976; Passariello etal., 1979). The ectatic segment has been attributed to inflammation, trauma and Conclusion degenerative changes by Schatz and Fine (1962). The other poss- Internal jugular vein ectasia can occur bilaterally although it ible aetiology is that it is a persistent jugular lymph sac. It has may appear clinically unilateral. Ultrasonography is the inves- been established that lymphatics are derived from veins. They tigation of choice and should be performed on both sides of the begin as a series of six lymphatic sacs: two are paired, the jugular neck. In bilateral cases of jugular ectasia, a conservative policy and the posterior lymph sac, while two are unpaired, the retro- should be adopted.

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4 . OCM FIG. 4 Ultrasonography of neck of adult male revealing a markedly dilated segment of right (above) and left (below) jugular vein.

Harris, R. I. (1928) Congenital venous cyst of mediastinum. Annals of Surgery 88: 953-954. LaMonte, S. A., Walker, E. A., Moran, W. B. (1976) Internal jugular phlebectasia. Archives of Otolaryngology 102: 706-708. Leung, A., Hampson, H. B., Singh, M. P., Carr, D. (1983) Ultrasonic diagnosis of bilateral congenital venous aneurysms. British Journal of Radiology 56: 588-591. Okay, N. H., Bryk, D., Kroop, I. G., Budow, J. (1970) Phlebectasia of the internal and great mediastinal veins. Radiology 95: 629-630. Passariello, R., Lozzi, F., Cusalena, G., Colarossi, G., Rossi, P., Sim- onettic, G. (1979) Angiographic diagnosis of jugular venous system dilatation in children: a report of five cases. Pediatric Radiology 8: 247-250. Rowe, M. I. (1946) Contracture of scalenus anterior causing aneurysmal varix of the right internal jugular vein. Journal of Bone and Joint Surgery 28: 147-150. Schatz, I. J., Fine, G. (1962) Venous aneurysms. New England Journal of Medicine 266: 1310-1312. Som, P. M., Shugar, J. M. A., Sacher, M, Lanzieri, C. F. (1985) Internal jugular vein phlebectasia and duplication: CT features. Journal of Computer Assisted Tomography 9: 390-392. Steinberg, L., Watson, R. C. (1966) Lymphangiographic and angio- FIG. 5 graphic diagnosis of persistent jugular lymph sac. Report of a A left jugular venogram of adult male showing a markedly dilated case. New England Journal of Medicine 275: 1471-1474. segment of left internal jugular vein on Valsalva manoeuvre. Stevens, R. K., Fried, A. H., Hood, T. R. (1982) Ultrasonic diagnosis of jugular vein aneurysm. Journal of Clinical Ultrasound 10: Acknowledgements 85-87. Walsh, R. M., Murty, G. E., Bradley, P. I. (1992) Bilateral internal The authors wish to thank Dr Lokman Saim, F.R.C.S., Ed., jugular phlebectasia. Journal of Laryngology and Otology 106: Head of the Ear, Nose and Throat Department, National Univer- 753-754. sity of Malaysia for giving us access to the case notes. We are Yokomori, K., Kubo, K., Kanamari, Y., Takemura, T., Yamamoto, T. also indebted to Ms Fuziah for clerical assistance and Mr Kama- (1990) Internal jugular phlebectasia in two siblings: manometric rulzaman for photography. and histopathologic studies of the pathogenesis. Journal of Pae- diatric Surgery 25: 762-765. References Bowdler, D. A., Singh, S. D. (1986) Internal phlebectasis. Inter- national Journal of Paediatric Otorhinolaryngology 12: Address for correspondence: 165-171. Dr B. S. Gendeh, Danis, R. K. (1982) Isolated aneurysm of the internal jugular vein. Lecturer, ENT Department, Journal of Paediatric Surgery 17: 130-131. Medical Faculty, Gerwig, W. H. (1952) Internal jugular phlebectasia. Annals of Sur- U.K.M., gery 135: 130-133. Jalan Raja Muda Aziz-50300, Gordon, D. H., Rose, J. S., Kottheier, P., Levin, D. C. (1976) Jugular Kuala Lumpur, venous ectasia in children. Radiology 118: 147-149. Malaysia.