Internal jugular vein thrombosis – A rare presentation of mediastinal lymphoma

Internal jugular vein thrombosis – A rare presentation of mediastinal lymphoma

Respiratory Medicine CME 3 (2010) 273–275 Contents lists available at ScienceDirect Respiratory Medicine CME journal homepage: www.elsevier.com/loca...

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Respiratory Medicine CME 3 (2010) 273–275

Contents lists available at ScienceDirect

Respiratory Medicine CME journal homepage: www.elsevier.com/locate/rmedc

Case report

Internal jugular vein thrombosis – A rare presentation of mediastinal lymphoma Mohammad Shameem*, Jamal Akhtar, Rakesh Bhargava, Zuber Ahmed, Ummul Baneen, Nafees Ahmad Khan Department of Tuberculosis and Chest Disease, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh 202002, UP, India

a r t i c l e i n f o

a b s t r a c t

Article history: Received 21 September 2009 Accepted 25 September 2009

Internal jugular vein (IJV) thrombosis is an extremely rare vascular disease. It is usually secondary to intravenous drug abuse, prolonged central venous catheterization or deep head-neck infections or trauma. Associated malignancies are uncommon and not well documented in the etiology of IJV thrombosis. A 25-year-old male presented with complains of neck swelling on left side for last 1 month and shortness of breath for last 10 days with no ear or nose complain. Examination of his neck showed swelling along the anterior border of the left sternocleidomastoid muscle. An ultrasound scan of the neck and CECT neck revealed left IJV thrombosis. CECT thorax showed anterior mediastinal mass which on biopsy comes out to be non Hodgkin lymphoma. IV heparin was given for IJV thrombosis and patient was referred to radiotherapy department for further treatment. Ó 2009 Elsevier Ltd. All rights reserved.

Keywords: Internal jugular vein thrombosis Distant malignancy Mediastinal lymphoma

1. Introduction Internal jugular vein (IJV) thrombosis is an extremely rare disease. It is usually due to intravenous drug abuse, prolonged central venous catheterization or deep head-neck infections or trauma.1,2 Related malignancies, either known or occult, are also uncommon and not well documented in the etiology of IJV thrombosis.3,4 IJ thrombosis itself can have serious potentially life-threatening complications that include systemic sepsis, chylothorax, papilledema, airway edema, and pulmonary embolism. The diagnosis often is very challenging and requires, first and foremost, a high degree of clinical suspicion. Here, we are presenting a case of left internal jugular vein thrombosis occurring as the first manifestation of lymphoma. 2. Case report A 25-year-old patient presented in emergency of our hospital with complains of neck swelling on left side for last 1 month and shortness of breath for last 10 days. There was no history suggestive of any ear or nose disease. Also there was no history suggestive of intravenous drug abuse or trauma. Physical examination showed low grade fever (99F), pulse rate 100/min, blood pressure 130/90, respiratory rate 32/min, with prominent accessory muscles of respiration. The patient had no pallor, clubbing or peripheral edema. There was no cervical, axillary * Corresponding author. Tel.: þ91 9412731835. E-mail address: [email protected] (M. Shameem). 1755-0017/$36.00 Ó 2009 Elsevier Ltd. All rights reserved. doi:10.1016/j.rmedc.2009.09.020

or inguinal lymphadenopathy. There was no discharge from ear or nose. Examination of his neck showed left sided neck swelling along the anterior border of sternocleidomastoid muscle. There was no abnormality on otoscopic examination of ear. Findings on oral cavity examination were normal. Indirect laryngoscopic examination was also normal. In respiratory system examination, on inspection movements were decreased on left side of chest wall, trachea was central. Palpation revealed decreased chest expansion on left side with decreased vocal fremitus. Percussion note was dull on left side. On auscultation intensity of breath sound was decreased on left side with no added sound. There was no abnormality on CVS examination, CNS examination and abdominal examination. On laboratory examination CBC showed Hb 10.6%, WBC count 13,600/mm, ESR was 20/h. Renal function test, liver function test and ECG were normal. Fibrin degradation products, levels of fibrinogen, prothrombin and activated partial thromboplastin times were within normal range. Chest X-ray (Fig. 2) at presentation revealed homogenous opacity of left hemithorax with CPA blunting. A previous chest X-ray(PA View) showed mediastinal widening (Fig. 1) .USG neck was done in which there was thrombosis involving the complete extent of left internal jugular vein and thrombus in left sub-clavian vein was also found (Figs. 4 and 5). CECT neck showed thrombosis of left internal jugular vein (Fig. 3). CECT thorax showed lobulated mass lesion in anterior mediastinum with left side pleural effusion (Fig. 6). FNAC of mass lesion was performed which comes out to be malignant lymphoma. On biopsy it comes out to be non Hodgkin lymphoma. Heparin was given by intravenous route for thrombosis.

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Fig. 1. CXR PA view shows mediastinal widening in previous X-ray. Fig. 3. CECT neck shows thrombosis of left internal jugular vein.

The patient was further referred to radiotherapy department where patient was taken on CHOP (cyclophosphamide, adriamycin, vincristine and prednisolone) regimen. 3. Discussion Internal jugular vein thrombosis was first described by Long as a complication of peritonsillar abscess in 1912.5 It refers to an intraluminal thrombus occurring anywhere from the intracranial internal jugular vein to the junction of the internal jugular and subclavian vein. It is a rare vascular disease. Its most common cause is prolonged central vein catheterization. About 66% of patients with IJV catheters have proof of thrombus formation on ultrasound or at autopsy.6 Other common causes are head and neck infection, surgery, intravenous drug abuse7 and neck massage. Up to a third of patients following a neck dissection will have a thrombus in the IJV.8 Harada et al. showed that the most significant narrowing of the IJV following neck dissection occurs in the first week after surgery and that patency is gradually restored within three months.9 A case of IJV thrombosis following neck dissection was reported by Arslan et al.10 Similar case was also reported by Quraishi et al.11 A case of internal

Fig. 2. CXR at presentation shows homogenous opacity in lower zone of left hemithorax with blunting of left CPA.

jugular vein thrombosis following neck massage was reported by Wada et al.12 Related malignancies are also exceptional and not well documented in the etiology of IJV thrombosis.3 The association of cancer and thrombophlebitis was first observed by Trousseeau13 and this association still bears his name. Trousseau in 1861 stated that if the diagnosis of a suspected carcinoma of an internal organ could not be verified, the sudden and spontaneous appearance of thrombophlebitis in a large vein afforded necessary proof for diagnosis. Sack et al.14 in their review of 541 Trousseau’s syndrome cases, reported that underlying malignancy was detected in the lung {25.6%}, pancreas {17.4%}, stomach {16.8%}, colon {15.2%}, prostate {6.5%} and head and neck {2%}. Chen et al. also reported a case of left internal jugular vein thrombosis secondary to lung tumour.15 Pata et al., reported two cases of internal jugular vein thrombosis associated with breast and lung cancer.16 Carrington et al. reported two cases of IJVT in patients with advanced malignancy, one from an ovarian cancer and the second a mesothelioma.4 We are reporting a case of left IJV thrombosis associated with mediastinal lymphoma in which first complain to appear was left sided neck swelling.

Fig. 4. USG of neck shows thrombosis of left internal jugular vein involving complete extent

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Fig. 5. USG of neck shows thrombus in left sub-clavian vein.

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imaging (MRI), nuclear medicine scan, ultrasound and contrast venogram. Ultrasound scan is safe, non-invasive, and cost-effective and a doppler can detect flow rate. CT scan with intravenous contrast is considered by many to be the investigation of choice. MRI provides better soft tissue definition and sensitivity to blood flow rates compared to CT scanning and does not require exposure to contrast material or radiation.22 In patients with infected IJV thrombosis culture from the infection source (oropharynx, ears, catheter tip, etc.) and blood cultures should be done. Uncomplicated IJV thrombosis requires more indepth investigations, for example protein C, S, antithrombin-3deficiency tests and DIC screen which includes prothrombin time (PT), activated partial thromboplastin time (APTT), fibrin split products and fibrinogen. Treatment of patients with IJV thrombosis is done with heparin; but it is usually unsuccessful. Patients with JVT may need to be on long-term oral anticoagulation therapy. Treatment of the underlying cause or malignancy is the most definitive therapy, but usually in these particular diseases is also unsuccessful. Conflict of interest

The thrombosis may become secondarily infected, producing a septic thrombophlebitis. An infected IJ thrombus caused by extension of an oropharyngeal infection is referred to as Lemierre syndrome. This has also been termed necrobacillosis or postanginal septicaemia.18,19 A case of Lemierre’s syndrome was reported by Varkey Maramattom, Wijdicks .17 The classic triad predisposing to intravascular thrombosis was described first by Virchow and includes blood vessel trauma, stasis of blood flow, and a hypercoagulable state.5 IJV thrombosis generally present with pain and swelling in the neck. Tovi et al. described the following clinical manifestation in a large series of patients with septic IJV thrombosis: fever [83%], leukocytosis [78%], cervical pain [66%], neck swelling [72%], cord sign [39%], sepsis syndrome [39%], pleuro-pulmonary complications [28%], superior vena cava syndrome [11%], chylothorax [5%] and jugular foramen syndrome [6%].20 IJV thrombosis can lead to complications like septic emboli, pulmonary embolism, elevated intracranial pressure, facial edema, intracranial venous thrombosis and loss of vision.21 Imaging studies used in IJV thrombosis diagnosis include contrasted computed tomography (CT) scan, magnetic resonance

Fig. 6. CECT thorax shows lobulated mass lesion in anterior mediastinum suggestive of lymphoma with left side pleural effusion.

The authors have no conflict of interest to disclose. References 1. Sengupta S, Kalkonde Y, Khot R, Paithankar M, Salkar R, Salkar H. Idiopathic bilateral external jugular vein thrombosis. A case report. Angiology 2001;52(1):69–71. 2. Chowdhury K, Bloom J, Black MJ, Al-Noury K. Spontaneous and nonspontaneous internal jugular vein thrombosis. Head Neck 1990;12:168–73. 3. Unsal EE, Karaca C, Ensari S. Spontaneous internal jugular vein thrombosis associated with distant malignancies. Eur Arch Otorhinolaryngol 2003;260:39–41. 4. Carrington BM, Adams JE. Jugular vein thrombosis associated with distant malignancy. Postgrad Med J 1988;64:455–8. 5. Cohen JP, Persky MS, Reede DL. Internal jugular vein thrombosis. Laryngoscope 1985;95:1478–82. 6. Hubsch PJ, Stiglbauer RL, Schwaighofer BW. Internal jugular and subclavian vein thrombosis caused by central venous catheters. Evaluation using Doppler blood flow imaging. J Ultrasound Med 1988 Nov;7(11):629–36. 7. Myers EM, Kirkland Jr LS, Mickey R. The head and neck sequelae of cervical intravenous drug abuse. Laryngoscope 1988 Feb;98(2):213–8. 8. Leontsinis TG, Currie AR, Mannell A. Internal jugular vein thrombosis following functional neck dissection. Laryngoscope 1995 Feb;105(2):169–74. 9. Harada H, Omura K, Takeuchi Y. Patency and calibre of the internal jugular vein after neck dissection. Auris Nasus Larynx 2003 Aug;30(3):269–72. 10. Arslan N, Dursun E, Oquz B, Oquz H, Safak MA, Demirci M, et al. Internal jugular vein thrombosis following functional and selective neck dissections. Kulak Burun Bogaz Ihtis Durg 2008 Nov–Dec;18(6):355–61. 11. Quraishi HA, Wax MK, Granke K, Rodman SM. Internal jugular vein thrombosis after functional and selective neck dissection. Arch Otolaryngol Head Neck Surg 1997 Sept;123(9):969–73. 12. Wada Y, Yanagihara C, Nishimura Y. Internal jugular vein thrombosis associated with shiatsu massage of the neck. J Neurol, Neurosurg Psychiatr 2005;76:142–3. 13. Trousseau A. Plegmasia alba dolens. Clinique medicale de l’Hotel-Dieu de Paris, London. N Sydenham Soc 1865;3:94. 14. Sack Jr GH, Levin J, Bell W. Trousseau’s syndrome and other manifestations of clinical disseminated coagulopathy in patients with neoplasm: clinical, pathologic and therapeutic features. Medicine 1977;56:1–37. 15. Chen KH, Chan YJ, Lian CC, Chang HJ, Yeow KM. Left internal jugular vein thrombosis due to lung tumour. Chang Gung Med J 2003 Jun;26(6):458–62. 16. Pata YS, Unal M, Gulhan S. Internal jugular vein thrombosis due to distant malignancies: two case reports and literature review. J Laryngol Otol 2008 Mar;122(3):318–20 [Epub 2007 May14]. 17. Varkey Maramattom B, Wijdicks EFM. Bilateral internal jugular vein thrombosis: a benign presentation of Lemierre’s Syndrome? Cerebrovasc Dis 2005;19:139–40. 18. Lemierre A. On certain septicaemias due to anaerobic organisms. Lancet 1936;1:701–3. 19. Kristensen LH, Prag J. Human necrobacillosis, with emphasis on Lemierre’s syndrome. Clin Infect Dis 2000;31:524–32. 20. Tovi F, Fliss DM, Gatot A. Septic jugular thrombosis with abscess formation. Ann Otol Rhinol Laryngol 1991 Aug;100(8):682–4. 21. Gutteridge IF, Royle JP, Cockburn DM. Spontaneous internal jugular vein thrombosis and venous-stasis retinopathy. Stroke 1987;18:808–11. 22. Carpenter JP, Holland GA, Baum RA. Magnetic resonance venography for the detection of deep venous thrombosis: comparison with contrast venography and duplex Doppler ultrasonography. J Vasc Surg 1993 Nov;18(5):734–41.