|Year : 2019 | Volume
| Issue : 1 | Page : 81-83
Internal jugular phlebectasia: A rare case report in an Indian female
Vela D Desai
Department of Oral Medicine and Radiology, Jaipur Dental College, Jaipur, Rajasthan, India
|Date of Web Publication||12-Jun-2019|
Dr. Vela D Desai
B: 406, Trimurthy Apartments, Opposite BSNL Telecom Colony, Malviya Nagar, Jaipur - 302 017, Rajasthan
Source of Support: None, Conflict of Interest: None
Phlebectasia is an abnormal dilatation of an isolated vein, which may affect any vein, and is rarely symptomatic. Internal jugular vein phlebectasia (JVP) presents as fusiform neck mass during straining or triggered by Valsalva maneuver. Due to its rarity, it is often misdiagnosed and ignored. Most cases are reported in children and sometimes later in age. The authors here report a case of right internal JVP, with an unusual coexisting presentation of tongue deformity in an Indian female patient. The aim of documenting this case is to sensitize the oral physician of this rare neck mass which is seldom reported in the dental literature. Furthermore, phlebectasia should be one of the differential diagnosis of neck mass. Although there are rarely any complications recorded in the literature, thorough investigations (ultrasound with Doppler) are needed for correct diagnosis and referral if needed. Diagnosis would prevent unnecessary surgical interventions. The patients are generally followed up by conservative treatment.
Keywords: Internal jugular vein, phlebectasia, swelling, tongue, trauma
|How to cite this article:|
Desai VD. Internal jugular phlebectasia: A rare case report in an Indian female. Arch Med Health Sci 2019;7:81-3
| Introduction|| |
Jugular vein phlebectasia (JVP) also known as venous congenital cyst, venous aneurysm, venectasia, venom, venous ectasia, or essential venous dilatation refers to a congenital fusiform or saccular dilatation of the jugular vein that appears as a soft, compressible mass in the neck on straining such as coughing, crying, sneezing, and speaking or may be triggered by the Valsalva maneuver. The term “phlebectasia” indicates abnormal outward dilatation of the vein without tortuosity and should not be misdiagnosed with varix that presents as dilatation along with tortuosity. All neck veins can be affected, but the internal and external jugular, the previous jugular, and the superficial communicating ones are most commonly affected.,,, Most of the literature of this rare venous anomaly is reported in the medical literature only; there are no records of this congenital defect in the dental literature and rarely has been mentioned in the differential diagnosis of neck swellings. Phlebectasia has been reported with hoarseness of voice. As an oral physician, one needs to have a thorough knowledge of this often misdiagnosed venous neck anomaly and advice appropriate referral if needed. The author here reports a case of one such anomaly with a brief review of the literature.
| Case Report|| |
A middle-aged female reported to the Department of Oral Medicine and Radiology with a complaint of pain in the lower right and left upper back teeth for few days. Pain was insidious in onset, mild, dull, and intermittent. The medical, family, and dental history were noncontributory. General physical examination showed an uncommon presentation of a swelling in the neck on speaking and laughing. At rest, examination of the neck showed no abnormality [Figure 1]; however, on performing Valsalva maneuver, the swelling appeared on the right side of the neck (anterior triangle) and anterior to the sternocleidomastoid muscle [Figure 2] measuring approximately 5 cm × 2 cm, with normal skin color. The swelling was elongated, fusiform in shape anterior to the sternocleidomastoid muscle, with a well-demarcated and smooth surface. On palpation, the mass was nontender, nonpulsatile, soft, and compressible which appeared only on coughing, laughing, and speaking for a long time. History revealed that the asymptomatic intermittent swelling was present since childhood and the patient did not undergo any investigation or treatment for the same. There was no history of pain, facial puffiness, and difficulty in breathing, speech, swallowing change in voice, cough, or chest pain. There was no history of trauma to the neck region or any previous neck infection or surgery.
|Figure 2: Appearance of a fusiform swelling in the neck as the patient speaks|
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Intraoral examination revealed pale-yellow oral mucosa. Tongue presented with an abnormal folded appearance at the tip [Figure 3]. Detailed history revealed that when the patient was an infant (2 months old), she accidentally fell off her mother's laps and injured the tongue. No treatment was instituted then. The patient did not report any pain or difficulty in speech, swallowing, or taste abnormality. Generalized attrition was evident. Phlebectasia of the right internal jugular vein was considered as an incidental finding and posttraumatic tongue defect.
The patient was referred for a thorough evaluation of the cardiovascular and renal system. Extraction of the root stumps and their after replacement, diet counseling, and nutritional supplements were suggested. The patient is being followed up for her dental treatment and was informed about the treatment options and also complications of phlebectasia. As treatment is indicated if any complications arise; otherwise, conservative management is recommended.
| Discussion|| |
Venous dilatation was first reported in 1928, and phlebectasia was the term which was used by Gerwig to describe an abnormal fusiform dilatation of a vein. Its etiology remains unknown. Many hypotheses have been proposed as a cause for phlebectasia including 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, trauma, and congenital origin.,,
Soft, round, or fusiform neck swelling in a child below 13 years of age is the typical presentation, though it has been reported in a wide age interval (ranging from 5 months to 68 years).
Phlebectasia is seen more commonly on the right IJV because the right innominate vein lies in contact with the right apical pleura. Therefore, any increase in the intrathoracic pressure could be directly communicated to the right IJV. The left vein, being placed more medially, is not subjected to such stress. Furthermore, the right IJV valves are placed at a higher level than the left-sided valves. The valves play an important role in preventing retrograde blood flow. Valves are almost never seen in the right brachiocephalic vein, but the incidence of the valves in the left brachiocephalic vein is 4%–8%, most being competent.
The clinical presentation of the reported case was classical for the clinical diagnosis of JVP, so the authors did not advice any further investigations, and referral was considered for thorough evaluation. The differential diagnosis for the neck swelling could be branchial cyst, cystic hygroma, laryngocoele, cavernous hemangioma, varix, and superior mediastinal cysts. Thyroglossal duct cyst, dermoid cyst, bronchogenic cyst, cervical adenitis, pharyngocele, neumocele and metastatic adenopathy, and Menkes disease also need to be ruled out.,,
Ultrasonograph with color Doppler before, during, and after the Valsalva maneuver is the preferred choice of investigation for the diagnosis. Ultrasound is a noninvasive, accurate, and readily reproducible imaging technique in this condition, which defines its extent and its relationship with the other structures in the neck. Contrast-enhanced computed tomography (CT) is the other modality of choice for the investigation. CT, angiography, and magnetic resonance venography are also indicated.
Management of IJV phlebectasia is usually conservative, as there are no complications or deformity with regular follow-up. Treatment is recommended only in patients who present with phlebitis, thrombus formation, Horner's syndrome, (intractable) cough, or for cosmetic purpose. The various surgical treatment options available are: ligation of the affected vein, resection of the dilated segment, and sheathing of the affected segment in a polytetrafluoroethylene tube graft. Some authors believe that ligation of the jugular vein may produce effects of venous congestion in few patients resulting in cerebral edema.,,,,,
Investigations for the tongue defect were not indicated in the present case as the patient denied any kind of procedure. The patient had no difficulty in tongue movements, speech, and taste. She is being followed up for other oral findings. The patient was counseled regarding the nature of the condition, and regular follow-up was advised for phlebectasia. Furthermore, asked to seek immediate medical advice if the symptoms increased or new symptoms such as hoarseness, difficulty in breathing, or swallowing occurred.
| Conclusion|| |
The aim of documenting this case is to sensitize the oral physician of this rare neck mass which is seldom reported in the dental literature. Furthermore, phlebectasia should be one of the differential diagnoses of the neck mass.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Yilmaz MD, Kahveci OK. Jugular phelebectacia. Kocatepe Tip Derg 2003;1:71-4.
Bowdler DA, Singh SD. Internal jugular phlebectasia. Int J Pediatr Otorhinolaryngol 1986;12:165-71.
Paleri V, Gopalakrishnan S. Jugular phlebectasia: Theory of pathogenesis and review of literature. Int J Pediatr Otorhinolaryngol 2001;57:155-9.
Kwok KL, Lam HS, Nq DK. Unilateral right-sided internal jugular phlebectasia in asthmatic children. J Paediatr Child Health 2000;36:17-519.
Chakraborty S, Dey PK, Roy A, Bagchi NR, Sarkar D, Pal S, et al.
Internal jugular vein phlebectasia presenting with hoarseness of voice. Case Rep Vasc Med 2013;2013:386961.
Gerwig WH Jr. Internal jugular phlebectasia. Ann Surg 1952;135:130-3.
Rowe MJ Jr. Contracture of the scalenus anterior, causing aneurysmal varix of right internal juglar vein, a case report. J Bone Joint Surg Am 1946;28:147.
Zukschwerdt L. Rare localization of a venectasia. Dtsch Z Chir 1929;216:283-5.
Rossi A, Tortori-Donati P. Internal jugular vein phlebectasia and duplication: Case report with magnetic resonance angiography features. Pediatr Radiol 2001;31:134.
Chang YT, Lee JY, Wang JY, Chiou CS. Transaxillary subfascial endoscopic approach for internal juglar phlebectasia in a child. Head Neck 2010;32:806-11.
Momooa T, Johkura K, Kuroiwab Y. Jugular phlebectasia: A manometric study in an unanesthetized patient. J Clin Neurosci 2008;15:914-6.
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 Surg1990;25:762-5.
Hu X, Li J, Hu T, Jiang X. Aneurism of the internal jugular vein. Am. J Otolaringol Head Neck Med Surg 2005;26:172-4.
La Monte SJ, Walker EA, Moran WB. Internal jugular phlebectasia. A clinicoroentgeographic diagnosis. Arch Otolaryngol 1976;102:706-8.
Harris RI. Congenital venous cyst of mediastinum. Ann Surg 1928;88:953-6.
Malik V, Kumari A, Murthy T. Unusual case of focal neck swelling: Phlebectasia of internal jugular vein with intracranial extension. Int J Appl Basic Med Res 2015;5:58-60.
Abdulla AS, Aldabagh MH. Congenital phlebectasia of internal jugular vein. Dohuk Med J 2008;2:155-60.
Jianhong L, Xuewu J, Tingze H. Surgical treatment of jugular vein phlebectasia in children. Am J Surg 2006;192:286-90.
Nedumaran B, Krishnasamy A. Internal jugular venous ectasia in an adult female. J Clin Diagn Res 2018;12:7-8.
Alenezi M, Alaglan A, Almutairi A, Alanazy S, Al Wutayd O. Unilateral internal jugular vein phlebectasia in an adult: Management and one year follow-up. SAGE Open Med Case Rep 2019;7:2050313X19836351.
Cheah SC, Wong HT, Lau CY. Rare left-sided presentation of internal jugular vein ectasia in an adult. Ann Saudi Med 2018;38:381-2.
Agrawal R. Left external jugular phlebectasia: Rare presentation in adults; sign of a deep, dangerous lesion? Exp Rhinol Otolaryngol 2018;2:1-3.
[Figure 1], [Figure 2], [Figure 3]