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Table of Contents
Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 33-43

A 41-year-old man with end-stage renal disease on maintenance haemodialysis with pericardial effusion

1 Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
2 Department of Medicine, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
3 Department of Radiology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

Date of Web Publication8-Jan-2019

Correspondence Address:
R Ram
Professor and Head, Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JCSR.JCSR_12_18

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How to cite this article:
Lakshmi B S, Neeharika D, Anil Kumar C V, Reddy M H, Chandra V S, Nagaraj R D, Sarala S, Ram R, Kumar V S. A 41-year-old man with end-stage renal disease on maintenance haemodialysis with pericardial effusion. J Clin Sci Res 2018;7:33-43

How to cite this URL:
Lakshmi B S, Neeharika D, Anil Kumar C V, Reddy M H, Chandra V S, Nagaraj R D, Sarala S, Ram R, Kumar V S. A 41-year-old man with end-stage renal disease on maintenance haemodialysis with pericardial effusion. J Clin Sci Res [serial online] 2018 [cited 2019 May 21];7:33-43. Available from: http://www.jcsr.co.in/text.asp?2018/7/1/33/249623

A 41-year-old male had been diagnosed to have chronic glomerulonephritis on renal biopsy 11 years ago. He has been on thrice a week maintenance hemodialysis for the past 7 years. He was initiated on hemodialysis through a right internal jugular vein (IJV) catheter. A left radiocephalic arteriovenous fistula (AVF) was secured. During these 7 years, there were secondary failures of left radiocephalic, left brachiocephalic, and right radiocephalic AVFs. Right and left IJVs were cannulated twice each as a temporary vascular access whenever an AVF had failed. The IJV catheters were retained at least for 8 weeks on each of those four occasions. He received antihepatitis B and antipneumococcal vaccine. He had been on regular erythropoietin. His residual renal function was 200 mL/day. He complained progressive breathlessness during the last 45 days, and at presentation, it was associated with orthopnea. There was a history of progressive facial swelling of similar duration. There was only a minimal respite in breathlessness and facial swelling even after a dialysis session. There was a history of head fullness, nasal stuffiness, and light-headedness. Bending forward aggravated the symptoms. There was a history of left shoulder discomfort, abdominal discomfort, and nausea. There was a history of right upper quadrant abdominal pain. There was no history of syncope, palpitation, fever, night sweats, cough, and expectoration. There was also no history of hematuria, pyuria, and graveluria. There was no history of joint pains, skin rash, photosensitivity, hair loss, oral ulcers, red eyes, epistaxis, haemoptysis, haematemesis, hematochezia, and tingling numbness. He had normal appetite and no history of loss of weight.

He did not have diabetes mellitus. There was no past history of bronchial asthma, tuberculosis, epilepsy, jaundice, accidents, and fractures.

There was no history of the use of over-the-counter medications such as painkillers, herbal medicine, or any other treatment. He was neither a smoker nor consumes alcohol. His medications included telmisartan, calcitriol, phosphate binder, vitamins, iron sucrose, and erythropoietin.

On examination, there was edema of feet and face, neck vein distension on both the sides, no pallor, no cyanosis, no clubbing, no jaundice, no lymphadenopathy, and no koilonychia. Pulse: 90 bpm, pulsus paradoxus present. regular, no radiofemoral delay; Blood pressure was 140/90 mmHg, right upper limb supine and standing. Respiratory rate was 28/minute. Temperature was 98.6° F. Breast and thyroid examination was normal.

Cardiovascular system examination revealed engorged neck veins including IJVs with no wave pattern noticed. Multiple engorged veins were noticed on anterior chest wall till fifth ribs and also on the back till the inferior angles of scapulae [Figure 1]. Apex beat was not localized on inspection, and there was a difficulty in palpating it. There was dullness in the left second intercostal space and widening of the area of cardiac flatness to percussion. On auscultation, the heart sounds were muffled. There were no murmurs, adventitious sounds, or pericardial friction rub. Respiratory system examination revealed that the trachea shifted to the right, no flattening or bulges on the chest wall, reduced chest movement on left infrascapular area, stony dull note on percussion on bilateral infrascapular areas, and absent breath sounds in same areas. No crackles, no rhonchi, and no pleural rub were heard. Gastrointestinal examination revealed that soft, smooth, nontender, nonpulsatile hepatomegaly, 1 cm below right costal margin, no splenomegaly, no shifting dullness, and bowel sounds were normal. Central nervous system was unremarkable. Laboratory investigations are listed in [Table 1].
Figure 1: Clinical photograph showing multiple engorged veins on the back

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Table 1: Laboratory investigations

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The patient underwent daily hemodialysis without heparin. The haemodialysis clearance of the dialyser × time/volume of distribution of urea (Kt/V) was found to be 1.4 and urea reduction ratio (URR) was 65%. The patient underwent diagnostic pericardiocentesis followed by therapeutic pericardiocentesis. Tube drainage of pericardial fluid was established. More than six liters of the pericardial fluid were drained.

  Differential Diagnosis Top

Differential diagnosis presented by Dr. Neeharika, 1st year Postgraduate Junior Resident in MD (Medicine), Sri Venkateswara Institute of Medical Sciences, Tirupati.

The patient is a 41-year-old gentleman, came with complaints of breathlessness and facial puffiness of 45-day duration. Considering breathlessness, there are several causes. Here in this patient, as breathlessness was associated with facial swelling the cardiac conditions such as massive pericardial effusion, cardiac tamponade, constrictive pericarditis, high output cardiac failure, respiratory conditions like tension pneumothorax, apical lobe malignancy or occult malignancy compressing superior vena cava (SVC), uremia, and SVC thrombosis are the possibilities to be considered. In the above list, tension pneumothorax or cardiac tamponade were unlikely as these conditions were usually acute presentations.

Further clinical history described that he also had a history of head fullness, nasal stuffiness, and lightheadedness which were aggravated on bending forward which might be a clue for increased intracranial pressure. He had a history of abdominal pain in the right upper quadrant, nausea, and vomiting. In the past, he was found to have hypertension for the first time 11 years back. About 4 years later, he was diagnosed to have chronic kidney disease (CKD). For sometime, he was on conservative management. CKD progressed over a period of 2 years to end-stage renal disease (ESRD). He was initiated on hemodialysis when serum creatinine was 9.8 mg/dL and the patient had uremic symptoms such as oliguria, anasarca, and shortness of breath. The patient was initiated on hemodialysis through right IJV catheter with AVF secured. He was on maintenance hemodialysis from then with a residual renal function of 200 mL/day. During this period, there were late AVF failures of left brachiocephalic AVF, and then right radiocephalic AVF during which period temporary vascular access through the right and left IJV catheters two times each were secured. He was vaccinated against hepatitis B and pneumococcus. He was using telmisartan, calcitriol, vitamins, phosphate binders, iron sucrose, and erythropoietin.

The AVF failures for the purpose of discussion might be divided as early and late failures. Early failure represented an AVF that was never usable for dialysis or that fails within 3 months of use. This might also be referred as primary failure. The late AVF failure represented as failure of AVF that occurred after a period of normal usage. The distinction of early and late AVF was necessitated because certain unique lesions cause early failure. These unique lesions were also major causes of late AVF failure if they were not corrected in the early phase. Brief information on AVF failures was given in the [Table 2].[1]
Table 2: Arteriovenous fistula failure*

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Modified from the hypercoagulable states also had to be considered, in addition to the causes given in the [Table 2].[1]

At presentation, temperature was 98.4°F, pulse rate is 90 beats/minute, blood pressure was 140/90 mmHg with no postural drop, pulsus paradoxus was appreciated, respiratory rate was 28/minute, with oxygen saturation 98% while breathing on ambient air. There was no conjunctival pallor, scleral icterus, peripheral edema, and peripheral lymphadenopathy. Oropharynx was clear with no exudates or erythema. There were engorged veins including IJV with no wave pattern noticed and multiple distended veins over the anterior chest wall till fifth rib and back till inferior angle of the scapula. There was no visible apex beat and hardly felt on palpation and heart sounds were muffled. There was dullness in the second intercostal space with widening of cardiac dullness to percussion. There were no murmurs or adventitious sounds or pericardial rub.

The cardiovascular examination was notable for distended veins in the neck including the IJV, with no pulsatile waveform pointing impaired conduction of right atrial pressure to the IJV. It suggested the possibility of obstruction anywhere between the right atrium and the IJV. This was buttressed by the presence of engorged veins over the anterior chest wall and inferior angle of scapula. This is an opportunity to review the consequences of the obstruction of the SVC.

The clinical signs of the obstruction of the SVC are mainly cyanosis, due to venous stasis with normal arterial oxygenation and edema of the upper chest, arms, neck, and face. Vein varicosities of the proximal tongue and dark purple ears are also possible. Other signs or symptoms are coughing, epistaxis, hemoptysis, dysphagia, dysphonia, and hoarseness caused by vocal cord congestion, esophageal, retinal, and conjunctival bleeding. In the case of significant cephalic venous stasis, headache, dizziness, buzzing, drowsiness, stupor, lethargy, seizures, and even coma may be encountered. Psychosis, probably due to carbon dioxide accumulation, may also occur.[2],[3],[4],[5],[6],[7],[8],[9],[10],[11]

Dyspnea can be directly related to pulmonary congestion caused by lymphatic stasis, combination with pulmonary atelectasis, pleural effusion and pericardial effusion, direct compression of the mass on the airways, on the heart, or on the pulmonary artery, and laryngeal edema.

The anatomic classification the SVC obstruction is based on level of obstruction:[12]

  1. obstruction of the SVC, proximal to the azygos vein entry point
  2. obstruction of the SVC along with the azygos vein and
  3. obstruction of the SVC, distal to the azygos vein entry point.

When there is obstruction of the SVC, proximal to the azygos vein entry point, there is no impediment to normal blood flow through the azygos vein. Venous drainage coming from the head, neck, shoulders, and arms cannot directly reach the right atrium. Several veins, the most important being the right superior intercostal vein, provide a longer but effective collateral path for the blood flow from the superior tract of the SVC. The blood flow from the superior tract of the SVC is reversed and directed to the azygos vein, mainly through the right superior intercostal vein.

The azygos collateral venous system is deep; the presence of superficial vessels is usually lacking. However, they are still possible in the area of the internal thoracic vein's superficial tributaries [Figure 2].
Figure 2: Obstruction of the superior vena cava, proximal to the azygos vein entry point

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When there is obstruction of the SVC along with the azygos vein, the only blood return is possible by minor vessels to IVC (cava-cava or anazygotic circulation). From the internal thoracic veins, blood is forced to the intercostal veins, then to the azygos and the hemiazygos veins. The flow is thus reversed into the ascending lumbar veins to the iliac veins. Direct anastomosis between the azygos' origin and the IVC and between the hemiazygos and the left renal vein are also active. In addition, the internal thoracic veins can flow into the superior epigastric veins. From the superior epigastric veins, blood is carried to the inferior epigastric veins across the superficial system of the cutaneous abdominal veins and finally to the iliac veins. Another course is between the thoracoepigastric vein (collateral of the axillary vein) and the external iliac vein.

In these conditions, the collateral circulation is partly deep and partly superficial. The important symptoms are dyspnea and pleural effusion and the superficial-dilated vascular routes, the main sign of collateral circulation appear swollen and nonpulsating. The ensuing slow blood flow may be responsible for superimposed thrombosis. The renal impairment is possible as the SVC obstruction affects the lumbar plexus (mostly the ascending lumbar veins, left side) which congests the renal vein [Figure 3].
Figure 3: Obstruction with the azygos involvement

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When there is an obstruction of the SVC, distal to the azygos vein entry point, the blood flow is distributed from the upper body into the azygos and the hemiazygos veins, in which the flow is reversed, to the IVC tributaries. In this type, the superficial collateral system is not always evident. The congestion and dilatation of the azygos and the hemiazygos is significant. The hemodynamic changes lead to edema and cyanosis of the upper chest and pleural effusion. Pleural effusion accumulates slow and right sided, probably due to anatomical reasons: there is a wider anastomosis between the hemiazygos and the IVC than between the azygos and the IVC [Figure 4].
Figure 4: Obstruction of the lower superior vena cava, distal to the azygos vein

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The invisible apex beat and muffled heart sounds point to pericardial effusion. Widened area of cardiac dullness reinforces pericardial effusion. The finding of a flat percussion note over the lower half of the sternum, extending upward as far as the level of the second costal cartilage, proved to be the leading sign in the diagnosis of pericardial effusion.[13] Widened area of cardiac dullness also differentiates between cardiac tamponade and constrictive pericarditis as the latter would not have cardiac dullness.

On examination of respiratory system, there was deviation of trachea to the right side. Lungs were clear to auscultation except for decreased breath sounds in bilateral infrascapular areas which was evident by dullness on percussion in same areas. There were also decreased movements on the left side, tracheal shift to right, and decreased movements on the left side which suggested bilateral pleural effusion with the left more than right.

The abdomen was soft, nontender, with normal bowel sounds. Smooth, nontender, nonpulsatile liver is palpable 1 cm below the costal margin which can be congestive hepatomegaly. Examination of the central nervous system is unremarkable.

With these findings, the diagnosis could be the SVC obstruction (preazygous) and pericardial effusion and pleural effusion. The aetiology of the obstruction and the link between the three had to be discerned out.

Patient underwent daily haemodialysis without heparin. The dialysis was optimized with Kt/V ratio of 1.4 and urea reduction ratio of 65%. The management strategy of pericardial effusion in a hemodialysis patient includes, ascertaining whether the pericardial involvement was fibrinous, effusive, or constrictive. Acute fibrinous pericarditis with or without a small effusion usually responds to conservative measures. Patients in preterminal renal failure who have fibrinous pericarditis should be dialyzed. The use of nonsteroidal inflammatory agents or steroids is controversial. If a patient on maintenance dialysis develops pericarditis, the treatment regimen should be reassessed since ineffective dialysis was a common cause of pericarditis. Specific attention should be paid to the adequacy of fistula flow. Although the recirculation of AVF was not tested in this patient, a Kt/V of 1.4 indicated that the patient was not underdialyzed. In the presence of pericarditis, the dialysis regimen must be intensified; daily dialysis in such patients was recommended. Patients with pericarditis are at high risk of cardiac tamponade when routine heparin anticoagulation is administered during hemodialysis, and strict supervision of heparin anticoagulation (Lee White coagulation time, 9–16 min) or heparin-free dialysis was advisable. Regional anticoagulation has been advocated but is delicate without tight coagulation controls. Pericarditis usually resolves within 2 weeks of the onset of daily dialysis treatment. However, large effusions (>250 mL) were resistant to conservative treatment. Pericardial effusions which do not respond to dialysis should be drained. A pericardiocentesis using a needle inserted blindly is dangerous because it can precipitate cardiac arrhythmia. It also carries the risk of coronary artery puncture. Therefore, this maneuver should be reserved for emergency situations of life-threatening cardiac tamponade. Tamponade can set in early, particularly when effusions develop rapidly and pericardial membranes are still poorly compliant. The preferred approach to persistent pericardial effusions is primary surgical fenestration,[14] which may be performed by subxiphoid pericardiotomy under local anesthesia or, preferably, pericardiectomy under general anesthesia. The success of pericardiostomy may be limited in the presence of loculated effusion, particularly surrounding the posterior wall. Pericardiectomy involves extended resection of the pericardial sac and in the case of constrictive pericarditis, decortication of the fibrotic tissue. The treating team has performed the majority of these steps to manage the pericardial effusion.

The list of investigations in the table had to be reviewed to move forward.

Among the investigations, serum creatinine and urea were high which decreased after daily dialysis. Serum parathyroid hormone levels were 243 pg/dL which was desired value for an ESRD patient. The value of thyroid-stimulating hormone was elevated. The initial value was 18.1 ml u/L which decreased over a period of 8 weeks to 3.5 ml u/L.

Fahr[15] first evolved the clinical concept of “myxedema heart” in which functional cardiac inadequacy was associated with an enlarged dilated heart, slow indolent heart action, and low ECG complexes, with flattened or inverted T-waves. All these abnormalities were reversible by specific therapy. However, it was left to Gordon[16] to describe the first case of myxedema complicated by a pericardial effusion.

The incidence of pericardial effusion in untreated hypothyroidism is reported to be 30% to 80%[17],[18],[19],[20] as detected by echocardiography. However, in these studies, the patients manifested overt clinical features of myxedema. But in the present population of hypothyroid patients, in whom the diagnosis is being established in its early stage, the reported incidence is only 3%–8%.[21],[22] Considered that pericardial effusion is a “constant, early, and major factor” in the syndrome of myxedema heart. In myxedematous pericardial effusion also, the underlying endocrine disorder may be overlooked and the patient subjected to repeated pericardial aspiration. Kern et al.[21] had cited an example where the patient underwent 75 pericardial taps over a period of 16 years before thyroid therapy was finally instituted. The pathogenesis of pericardial effusion is obscure. It seems likely that the extravasation of hygroscopic mucopolysaccharides into body cavities is largely responsible for such effusions,[23] but increased capillary permeability with the resultant albumin leak into interstitial and extracellular space, reduced lymph clearance – probably due to poor lymphatic tone[24],[25],[26] may also play a part.

The pericardial effusion in hypothyroidism is slow-forming and is rarely large to cause tamponade. However, there were reports of cardiac tamponade in hypothyroidism.[27],[28] The term “gold paint effusion” was applied to describe the golden brown appearance of the pericardial fluid in hypothyroidism. It was due to the shimmering satin cholesterol crystals. The high cholesterol content of the fluid had been attributed to disturbances in lipid metabolism; possibly, a churning action of the heart plays a role in the precipitation of cholesterol from pericardial fluid or the poor absorptive capacity of the pericardium may be a major factor.[27]

In this patient, the improvement in the thyroid function tests had not reduced the pericardial effusion. This should stimulate to explore the possibility of another cause for the pericardial effusion. Connective tissue profile was negative, coagulation profile was normal, and there were no abnormalities of protein C, protein S, antithrombin III, and normal levels of serum homocysteine levels renders the hypercoagulable state as a cause of fistula failures as unlikely.

Positron emission tomography revealed metabolically quiescent pleural and pericardial effusion with metabolically quiescent mediastinal lymphadenopathy. Pleural fluid is transudate as per Lights criteria,[29] cultures were sterile and ADA levels were 26 IU/L. Pericardial fluid examination also suggested transudate, cultures were sterile and ADA levels were 17.3 U/L.

The investigations lead to a juncture where the causes of pericardial effusion as mentioned in conventional medical tomes were all excluded. This list includes infections, neoplastic disease, myxedema, severe pulmonary hypertension, radiation therapy, acute myocardial infarction, aortic dissection, trauma, and autoimmune disease related such as systemic lupus erythematosus, rheumatoid arthritis, and granulomatosis with polyangiitis and drug induced.

The patient was distinguished by two major clinical features, SVC obstruction and pericardial effusion with the majority of causes excluded. The link between these two was possible. I am aware of the reports of the SVC obstruction that could cause increased hydrostatic venous pressures which in turn restricted the capacity of the lymphatics in the parietal pleura resulting in accumulation of pleural fluid. In addition, it could lead to obstruction of orifice of thoracic duct that caused increased intraluminal pressure, causing back pressure in communicating vessels and leakage of pleural lymphatics into the pleural and pericardial space.[30]

  Discussant's Diagnosis Top

The possibility of SVC obstruction (preazygous) or bilateral brachiocephalic obstruction was considered. It could be due to repeated catheterization of the IJVs which lead to pericardial effusion due to reduced venous and lymphatic drainage from the pericardium.

  Radiologist's Diagnosis Top

(Dr. Sarala, Assistant Professor, Department of Radiology, Sri Venkateswara Institute of Medical Sciences, Tirupati).

This patient of ESRD had a radiocephalic fistula in the right forearm. There was mild narrowing of feeding artery just proximal to anastomosis, the fistula site was patent and the draining cephalic vein was patent in the forearm and in distal two-thirds of the arm. There was narrowing of cephalic vein in the proximal one-third of the arm. No flow noted in the distal part of the cephalic vein in the deltopectoral groove where it pierces the clavipectoral fascia to open into the axillary vein to form the subclavian vein. It suggested of thrombosis. Thrombosis with total occlusion noted in entire left brachiocephalic vein, distal part of the right brachiocephalic vein, and in the proximal SVC. Distal part of the SVC and the opening of the azygous vein into the SVC were patent. The thrombus was seen extending into the distal-most part of bilateral internal thoracic veins which were its tributaries. The perforating branches (tributaries) of the internal thoracic veins were dilated.

The pericardium is supplied by the musculophrenic and the pericardiophrenic branches of the internal thoracic arteries. The internal thoracic veins accompany the branches of the internal thoracic artery. These veins drain the pericardium through the musculophrenic and the pericardiophrenic veins. In the proximal segment, the internal thoracic vein runs as two venae comitantes which ascend upward along the lateral border of the sternum and unite into a single vein at the level of the third costal cartilage to form the internal thoracic vein. It ascends upward behind the sternal end of the clavicle to open into the corresponding the brachiocephalic vein. The lymphatics that drain the pericardium also accompany the corresponding veins.

Multiple tortuous collaterals are seen in the proximal half of the right arm, in anterior and posterior chest wall, in the neck, and in the anterior abdominal wall. The azygous vein is dilated which was receiving venous return from the upper limb and head-and-neck region through the paravertebral venous plexus, the lumbar veins, the intercostal veins, and the left superior intercostal vein which were all dilated apart from the hemiazygous and the accessory hemiazygous venous systems which normally drain into the azygos vein. The azygous vein forms an important channel of communication between the SVC and the inferior vena cava when either of these two was occluded. Collaterals were noted in anterior abdominal wall between the superior epigastric and the inferior epigastric veins which drain into the external iliac veins and also between the lateral thoracic veins and the superficial epigastric veins which drain into the femoral veins.

In this patient, moderate pericardial effusion is seen. Moderate left pleural effusion with passive collapse of the left lower lobe was also seen. Encysted pleural collections were noted on the right side. The thrombosis of the bilateral internal thoracic veins led to venous hypertension, which resulted in pericardial effusion was a credible postulation.

Venous thrombosis near the confluence of the left jugular and left subclavian veins can obstruct the drainage of the thoracic duct which was the largest lymphatic vessel of the lymphatic system and can also result in the chylopericardium.

Diagnosis based on computed tomography angiogram: Status postright radiocephalic AVF showing mild stenosis of radial artery just proximal to fistula, thrombosis with total occlusion of distal cephalic vein near the termination, thrombosis with total occlusion of entire left brachiocephalic vein, and distal right brachiocephalic vein with extension of thrombosis into distal part of bilateral internal thoracic veins resulting in multiple collaterals in right proximal arm, anterior and posterior chest wall, anterior abdominal wall, and dilated azygos vein [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]. Pericardial and bilateral pleural effusions with passive collapse of underlying lungs.
Figure 5: Three-dimensional computed tomography angiogram of the right upper limb and neck vessels with bone showing right fistula, feeding artery, patent draining vein in the forearm, thrombosed draining vein in the distal part at the level of proximal arm and in the deltopectoralgroove (a). Multiple collaterals in the abdominal and chest wall, in proximal right arm, and in neck noted. Computed tomography angiogram SSD images without bones showing feeding artery, patent draining vein in the forearm, thrombosed draining vein in the distal part at the level of proximal arm, and in the deltopectoralgroove. Multiple collaterals in the abdominal and chest wall, in proximal right arm, and in neck noted (b)

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Figure 6: Thick maximum intensity projection computed tomography angiogram image in the right forearm showing short-segment stenosis of radial artery just proximal to fistula, patent fistula and patent draining vein in the forearm

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Figure 7: Maximum intensity projection computed tomography angiogram showing occlusion of cephalic V in its distal part up to its termination. Multiple collaterals noted in the proximal arm and in the chest wall

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Figure 8: Thick maximum intensity projection coronal reformatted computed tomography angiogram (a) and the diagrammatic representation of the same findings (b). Thrombosis with total occlusion of distal part of the right brachiocephalic vein and entire left brachiocephalic vein. The thrombus is seen extending into distal part of bilateral internal thoracic veins near their termination into the respective brachiocephalic veins. Collaterals seen in the neck. Pericardial effusion is also noted

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Figure 9: Right internal thoracic vein with its dilated perforating tributaries seen with thrombosis in its distal part and also in the right brachiocephalic vein into which it is draining (a and b). Patent superior vena cava and patent dilated azygos vein is also seen. Collaterals noted in the anterior chest wall

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Figure 10: Computed tomography angiogram showing multiple collaterals in the anterior and posterior chest wall, anterior abdominal wall, neck and in the right arm (a-d)

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Figure 11: Computed tomography showing bilateral pleural effusion and pericardial effusion, collaterals in anterior and posterior chest wall (a and b). Dilated azygos vein is seen (a)

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  Nephrologist's Diagnosis Top

Nephrologist's [the primary physician treating this patient] diagnosis by Dr. Hari Krishna Reddy, Second year postgraduate Senior Resident in DM (Nephrology), Sri Venkateswara Institute of Medical Sciences).

The pericardial space normally contains 15–50 mL of fluid, which serves as lubrication for the visceral and parietal layers of the pericardium. This fluid is thought to originate from the visceral pericardium and is essentially an ultrafiltrate of the plasma. The mechanisms of noninflammatory pericardial effusion include increased capillary permeability, increased capillary pressure, decreased plasma oncotic pressure, and lymphatic obstruction.

In 8%–10% of haemodialysis patients, pericardial effusion may occur. It is a reflection of under dialysis.[31] To counter ineffective dialysis, our patients were received heparin-free daily dialysis and Kt/V and URR were ensured to be optimal.

In our patient, we have excluded the main causes of pericardial effusion. These were mentioned by the discussant.

Pericardium is drained by the pericardiophrenic veins which open into the internal thoracic (mammary) vein which are venae comitantes to the inferior half of the internal thoracic artery. The internal thoracic vein ends in the brachiocephalic vein. Sometimes, left pericardiophrenic vein drains into the left superior intercostal vein which ends in the left brachiocephalic vein. A few pericardial veins also drain into azygos vein on the right side which ends in superior vena cava.[32] The lymphatics of pericardium drain into thoracic duct which opens into the internal jugular and the subclavian vein junction.[32]

The thrombotic occlusion of the right and left brachiocephalic veins and stenosis of the left subclavain vein notwithstanding the presence of dilated multiple intercostal veins and the azygos vein lead to insufficient pericardial fluid drainage. The proportion of the insufficient fluid versus insufficient lymph drainage determined the nature of pericardial fluid accumulated.

Numerous patient reports of pericardial tamponade after a central venous catheter insertion exist in the pediatric literature, although almost all are limited to the neonatal population.[33],[34],[35],[36] Tamponade caused by SVC obstruction has been documented in adult oncology patients with tumor compression/infiltration of the SVC.[37],[38] In nononcology patients, it was reported only in three patients[39],[40] of which one is in a 5-year-old maintenance hemodialysis patient.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Ahmann FR. A reassessment of the clinical implications of the superior vena caval syndrome. J Clin Oncol 1984;2:961-9.  Back to cited text no. 4
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Armstrong BA, Perez CA, Simpson JR, Hederman MA. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys 1987;13:531-9.  Back to cited text no. 6
Yellin A, Rosen A, Reichert N, Lieberman Y. Superior vena cava syndrome. The myth – The facts. Am Rev Respir Dis 1990;141:1114-8.  Back to cited text no. 7
Schraufnagel DE, Hill R, Leech JA, Pare JA. Superior vena caval obstruction. Is it a medical emergency? Am J Med 1981;70:1169-74.  Back to cited text no. 8
Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg 1990;160:207-11.  Back to cited text no. 9
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]

  [Table 1], [Table 2]


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