Jugular Venous Ectasia

 

TITLE OF CASE Do not include “a case report”

  Deep vein thrombosis with bilateral jugular vein ectasia

SUMMARY Up to 150 words summarising the case presentation and outcome (this will be freely available online)

A 55-Year-old male patient presented with pain, left thigh swelling and swollen veins in the cervical area. Further investigation revealed deep vein thrombosis in the patient’s left thigh with internal jugular vein ectasia on left and external jugular vein ectasia on right. The ectasia may have remained unnoticed due to the availability of only one government doctor per 11528 people in India where it is reported patients may not see a doctor during their lifetimes. This patient remained undiagnosed for 55 years as the imaging modalities such as ultrasonography and CECT were unavailable in the primary care hospital settings and the ectasia diagnosis was missed. The deep vein thrombosis was treated with anticoagulants. For the ectasias biannual follow up was recommended as the patient was asymptomatic and surgery complication risks were high The patient was counselled about future care and potential complications.

BACKGROUND Why you think this case is important – why did you write it up?

A case report contributes to the medical literature and familiarizes practitioners with the condition, differential diagnosis and best practice interventional care. The patient lived and laboured in an area without physician availability or diagnostic facilities. The importance of this case is the presentation is rare enough that medical students and many physicians may only see this in a textbook. We provide video footage and radiographic evidence of the case as a learning tool. Without treatment a patient may not survive complications should they present in an understaffed facility with complications. This case report alerts physicians and medical students to observe such swellings and to appropriately educate the patient with regards to complications.

CASE PRESENTATION Presenting features, medical/social/family history

A 55-year-old male patient came to the outpatient department with a complaint of generalized weakness and pain in the upper left thigh region of three years duration. He has a previous history of ulcers in this region but there were no unhealed ulcers present. There was no history of smoking, diabetes mellitus and hypertension. The patient had no history of hoarseness of voice, trauma or puncture, dysphagia and shortness of breath.

While narrating his history we found something moving on his left side of the neck, like a rat crawling under a bed sheet. Looking at the neck in a closer angle, the swelling did not appear. When he resumed sharing his history as a daily wage labourer, we noted a prominent 3×3 cm swelling on the lower left side of the neck during conversation only. (video 1). When the patient turned his head, we noticed a similar swelling on his lower right side of the neck (but a smaller one) which was 2×3 cm, but the anatomical position was not bilateral.(video 2).

On physical examination, his left thigh was swollen as compared to the right thigh. whereas the swellings on the neck were soft, painless, non-pulsatile, and compressible in nature. There was no family history of similar findings.

INVESTIGATIONS If relevant

In view of unilateral swelling and pain on the left thigh, the recurrent slow healing ulcer and the history of prolonged standing at his workplace, we investigated all swellings with compression ultrasonography with doppler and noticed a increased venous outflow on the superficial veins of the thigh. A diagnosis of deep vein thrombosis (DVT) was made. At this juncture we needed to rule out lower neck swelling as a site for DVT or associated malignancy as this can be a contributory risk factor.

We further investigated the neck swellings based on the anatomical location, it was suspected that the left swelling and right swelling might be due to the external jugular vein and the internal jugular vein, respectively. We used another imaging modality to resolve the diagnostic uncertainty.We evaluated the case first with ultrasonography with doppler followed by contrast-enhanced computed tomography (CECT) of the neck, where it became evident swellings were due to the external jugular vein ectasia on left side (figure 1, 2, 3) and internal jugular vein ectasia on right side (figure 4, 5, 6). Although this was an incidental finding as a result of the investigation, it served to rule out other concerns, reassure the patient, and the attending team who had not seen this before in spite of many years in practice. The patient shared his relief as he was concerned that it could be a source of the current problem or perhaps a heart illness or brain cyst that may burst in the future.

DIFFERENTIAL DIAGNOSIS If relevant

The differential diagnosis of deep vein thrombosis are baker's cyst, cellulitis, lymphedema, venous or arterial aneurysm, enlarged lymph nodes compressing the veins, heterotopic ossification, hematoma, and muscle tears [1] and the swellings of the lower neck include tumors in the mediastinum, branchial cyst, cavernous haemangioma, dermoid cyst [2].

TREATMENT If relevant

The patient was prescribed paracetamol for pain reduction and started on warfarin for anticoagulation. A rapport was established by meeting the patients concerns and the patient shared this developed trust and confidence to continue with warfarin therapy in the form of anticoagulants. It is challenging for patients to spend money and follow up with an intervention that may cause bruising and other side effects unless they are convinced of its worth. The patient was asymptomatic to swellings on the neck. Further treatment other than watchful waiting was not advised. Among 46 patients treated with surgery, the involved vein was ligated or excised in 32 patients. The cosmetic effects were satisfactory however three children faced postoperative complications of postoperative intracranial hypertension and 2 other children had neck and craniofacial swelling[3] The patient was reassured about the incidental finding of jugular vein ectasia and the possible complications of surgical management at this stage and risk management discussed. The complications for leaving the condition untreated although rare do include pulmonary embolism, massive bleeding secondary to trauma, thrombus formation, and congestive cardiac failure (CCF). However, should symptoms develop, surgical treatment by wrapping the dilated segment of the affected vein in a polytetrafluoroethylene (PTFE) tube graft is recommended. [4]

OUTCOME AND FOLLOW-UP

The patient was asymptomatic to swellings on the neck. Further treatment other than watchful waiting was not advised. He was advised to return for follow-up every six months to one year. The patient continued to take warfarin as per the instructions.

DISCUSSION Include a very brief review of similar published cases

These ectasias are generally reported between 5 months to 68 years but are most commonly investigated before the age of 13.[5]. The unilateral internal jugular vein phlebectasia reportings are occasionally reported in India, China, Sub Saharan Africa and other lower resource income countries. However bilateral internal and external jugular vein ectasia is extremely rare. Cases may go undiagnosed due to the doctor patient ratio. In India, for example, only one government doctor is present for 11528 people [6].The duration of the patient visit to doctor is another factor for concern. Only 48 seconds per appointment is the norm in Bangladesh with less than 5 minutes per appointment in other Asian and African countries. In contrast, more than 10 minutes is allotted per appointment in European and American countries with the highest being Sweden where a patient and doctor appointment lasts an average of 22.5 minutes [7]. Yaadhavakrishnan RD et al reports in lower middle income countries only one in 150 freshly graduated doctors is familiar with knowledge of Jugular vein Ectasia whereas only 23 pediatricians from 312 respondents, 21 from 328 Otorhinolaryngologists, 4 from 369 general practitioners were familiar with Jugular vein Ectasia [8].This makes a strong case for reporting this finding with video and radiographic films to increase awareness amongst medical students, allied health professionals, general physicians, consultants and surgeons. This can potentially reduce the complications and mortality due to this condition. [9]

LEARNING POINTS/TAKE HOME MESSAGES 3 to 5 bullet points – this is a required field

• Presenting the finding in a case study with evidence can help others learn about this condition as it is not commonly observed.

• Jugular vein ectasia can be diagnosed by a noninvasive ultrasonography and CECT.

• These ectasias can be observed incidentally, only during talking, coughing, straining and valsalva maneuver

• Increasing doctor-patient interaction time, improving physicians knowledge in such cases and reducing doctor workload may diagnose more cases but this is not in the doctor’s control.

• Jugular venous ectasias are not treated until they become symptomatic to avoid complications associated with its surgical management.

REFERENCES Vancouver style (Was the patient involved in a clinical trial? Please reference related articles)

1.DiVittorio R, Bluth EI, Sullivan MA. Deep Vein Thrombosis: Diagnosis of a Common Clinical Problem. The Ochsner Journal. 2002;4(1):14-17.

Source:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3399231/

2.Walsh RM, Murthy GE, Bradley PJ. Bilateral internal jugular phlebectasia. The Journal of Laryngology & Otology. Cambridge University Press; 1992; 106(8):753-4

Source:https://www.cambridge.org/core/journals/journal-of-laryngology-and-otology/article/bilateral-internal-jugular-phlebectasia/740F431E37BE8798179A0FC544B4F922

3. Jianhong L, Xuewu J, Tingze H. Surgical treatment of jugular vein phlebectasia in children.. Am J Surg. 2006 Sep; 192(3): 286-90.

Source: https://www.ncbi.nlm.nih.gov/pubmed/16920419

4.Sqn LH, Nambiar S. Focal ectasia of internal jugular vein. Medical J of armed forces India 2009;65: 282-283.

5.Bora MK. Internal Jugular Phlebectasia: Diagnosis by Ultrasonography, Doppler and Contrast CT. Journal of Clinical and Diagnostic Research : JCDR. 2013;7(6):1194-1196.

Source:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3708235/

6. Bagchii S. India has low doctor to patient ratio, study finds. British Medical Journal BMJ 2015;351:h5195.

Source: http://www.bmj.com/content/351/bmj.h5195.long

7. Irving G, Neves AL, Dambha-Miller H, et al.International variations in primary care physician consultation time: a systematic review of 67 countries BMJ Open 2017;7:e017902.

Source:http://bmjopen.bmj.com/content/7/10/e017902

8.Yaadhavakrishnan RD, Navaneethan N. Jugular Phlebectasia: Clinical Scenario in India. Indian Journal of Otolaryngology and Head & Neck Surgery. 2015;67(1):13-17.

Source :https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4298588/

9.Sippel S, Muruganandan K, Levine A, Shah S. Review article: Use of ultrasound in the developing world. International Journal of Emergency Medicine. 2011;4:72.

Source:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3285529/

FIGURE/VIDEO CAPTIONS figures should NOT be embedded in this document

Video 1 showing the focal dilation of left external jugular vein while talking.

Figure 1 showing the 3D constructed contrast-enhanced computed tomography image of dilation of left external jugular vein.

Figure 2 and Figure 3 showing the focal dilation of left external jugular vein in contrast-enhanced computed tomography as well as doppler.

Video 2 showing the dilation of right internal jugular vein while talking.

Figure 4 showing the 3D constructed contrast-enhanced computed tomography image of dilation of right internal jugular vein.

Figure 5 and Figure 6 showing the Focal dilation of right internal jugular vein in contrast-enhanced computed tomography as well as doppler.

PATIENT’S PERSPECTIVE Optional but strongly encouraged – this has to be written by the patient or next of kin

It is the first time, I noticed such a swelling on either side of my neck after looking at the video shown by the Mr.Madhava sai Sivapuram and horrified to know that what is going to happen to me? After a little talk with Dr. Biswas, who advised me to do some high cost non invasive imaging (Thanks to him which was done for free) , I happen to know to that there are some masses which are present on either side of neck. I was horrified to know whether is it going give me any heart attack as these are vessels close to my heart; if it also occurs to all my vessels am I going to swell like a gas balloon the entire time I talk. But Dr.Biswas has assured me that it is going to cause any heart attack and not going to swell like a gas balloon, To some extent I was relieved by knowing that but there is a sense of dilemma still stuck in my head why is that it is happening to me?; what is causing it?, But I believed in my doctor as he stated that as of now there would be no problem but asked for continuous follow up for every 6 months to 1 year is required as such and has given his contact number through which I could be in touch. I really be grateful to Dr.Biswas for helping me out in such unknown dilemma and reassuring me.