Renal vein thrombosis is a condition characterized by renal dysfunction secondary to a clot blocking the renal vein, which drains blood from the kidney. It is typically seen in patients suffering from nephrotic syndrome, but may also be diagnosed in individuals with renal malignancies, and hypercoagulable states. Clinical symptoms are variable and may be often masked by underlying conditions. Therefore diagnosis is based on clinical suspicion and confirmed with the radiological identification of the blood clot.
Presentation
Renal vein thrombosis (RVT) is a relatively rare condition in which a clot occludes the renal vein draining blood from the kidney and leads to renal vein infarction [1]. The exact reason why there is a predilection of the renal vein for thrombosis in as yet unknown [2]. It occurs most often in patients with nephrotic syndrome but can also affect patients with renal malignancies, anti-glomerular basement membrane disease [3], Behçet syndrome, hypercoagulable states like pregnancy [4], antiphospholipid antibody syndrome and following renal transplantation.
The clinical presentation of RVT in adults is usually insidious and asymptomatic. The first indication of the condition may be when the clot in the renal vein travels to the lungs causing pulmonary embolism with dyspnea and chest pain. Some cases may present with unilateral lower limb swelling. The onset is often sudden in children and some adults with fever, nausea, vomiting, flank pain, oliguria, gross hematuria, and proteinuria which progresses swiftly to renal failure especially in bilateral renal vein thrombosis or in patients with a single kidney [5]. The first symptom is usually flank pain occurring posterior to the lower ribs and in the region of the hips. In patients with renal malignancies, anorexia and weight loss may be predominant while patients with nephrotic syndrome may have anasarca.
Hematological
- Hemophilia A
While clotting factor deficiencies are well known causes of hemophilia, their levels should also be measured in the workup of transplant candidates with a history of multiple vascular access thrombosis. [ncbi.nlm.nih.gov]
Entire Body System
- Amyloidosis
Excluding renal cancer other possible causes of thrombosis are medical conditions such as amyloidosis, multiple myeloma, nephrotic syndrome, thrombophlebitis. [ncbi.nlm.nih.gov]
[…] for renal vein thrombosis sickle cell disease 2 polycythemia maternal diabetes indwelling umbilical venous catheters in adults, renal vein thrombosis can result from a variety of disorders, including: nephrotic syndrome systemic lupus erythematosus amyloidosis [radiopaedia.org]
Sickle cell nephropathy Amyloidosis Diabetic nephropathy Renal vasculitis Lupus nephritis Allograft rejection 5. 2/3rd Cases are bilateral Endothelial Damage Homocystinuria Endovascular intervention Surgery Venous stasis Dehydration Compression [slideshare.net]
Also systemic lupus erythematosus (SLE) and amyloidosis. Renal cell carcinoma - by extrinsic pressure on the renal vein or invasion of the renal vein or inferior vena cava. [patient.info]
- Anemia
Routine laboratory tests like complete blood count, urinalysis, serum blood urea nitrogen (BUN) and creatinine levels may reveal anemia, oliguria/proteinuria, and elevated BUN/ creatinine levels due to renal dysfunction. [symptoma.com]
Megaloblastic anemia occurs when a person has a low number of red blood cells (anemia), and the remaining red blood cells are larger than normal (megaloblastic). [ghr.nlm.nih.gov]
Laboratory tests may reveal hematuria, proteinuria, polycythemia, hemolytic anemia, thrombocytopenia and possibly acute kidney injury. The etiology for a hypercoagulable state should be investigated. [ncbi.nlm.nih.gov]
Respiratoric
- Cough
Further investigations for the cough showed thromboembolism of the posterior and lateral basal segments of the right lower lobe. Moreover he was found to have thrombosis of the right upper pole renal vein. [ncbi.nlm.nih.gov]
Excessive bleeding Signs of excessive bleeding can include: passing blood in your pee passing blood when you poo or having black poo severe bruising prolonged nosebleeds (lasting longer than 10 minutes) bleeding gums vomiting blood or coughing up blood [nhs.uk]
In infants: fever and chills; blood in the urine Chest pain, shortness of breath, cough, possibly with bloody sputum, profuse perspiration, anxiety, and palpitations, due to pulmonary emboli Renal Vein Thrombosis Prevention Preventive use of anticoagulant [healthcommunities.com]
Symptoms include : Difficulty breathing Coughing Chest pain Renal Vein Thrombosis Diagnosis Renal vein thrombosis is diagnosed by imaging studies. This allows the doctor to visualize the clot or the disruption in blood flow. [healthhype.com]
Gastrointestinal
- Vomiting
A 31-year-old male with known atresia of the inferior vena cava who received a deceased donor renal transplant 7 years previously presented to hospital with severe graft site pain and a week of nausea, vomiting, and chills. [ncbi.nlm.nih.gov]
Warfarin can cause rashes, diarrhea, nausea (feeling sick) or vomiting, and hair loss. [en.wikipedia.org]
A 61-year-old woman whose medical history was notable for hypertension and type 2 diabetes reported a two-day history of increasing dull pain in the right flank and nonbloody, bilious vomiting. [nejm.org]
However, onset may be acute, causing renal infarction with nausea, vomiting, flank pain, gross hematuria, and decreased urine output. [msdmanuals.com]
The onset is often sudden in children and some adults with fever, nausea, vomiting, flank pain, oliguria, gross hematuria, and proteinuria which progresses swiftly to renal failure especially in bilateral renal vein thrombosis or in patients with a single [symptoma.com]
- Nausea
A 31-year-old male with known atresia of the inferior vena cava who received a deceased donor renal transplant 7 years previously presented to hospital with severe graft site pain and a week of nausea, vomiting, and chills. [ncbi.nlm.nih.gov]
However, onset may be acute, causing renal infarction with nausea, vomiting, flank pain, gross hematuria, and decreased urine output. [msdmanuals.com]
The onset is often sudden in children and some adults with fever, nausea, vomiting, flank pain, oliguria, gross hematuria, and proteinuria which progresses swiftly to renal failure especially in bilateral renal vein thrombosis or in patients with a single [symptoma.com]
Cardiovascular
- Hypertension
Risk factors for renal vascular disease include: age female gender atherosclerosis hypertension, particularly new onset of hypertension in an older person smoking high cholesterol diabetes A risk factor is anything that may increase a person’s chance [surgery.med.miami.edu]
A 61-year-old woman whose medical history was notable for hypertension and type 2 diabetes reported a two-day history of increasing dull pain in the right flank and nonbloody, bilious vomiting. [nejm.org]
[…] artery disease - legs, Peripheral intravenous line - infants, Portacaval shunting, Pulmonary angiography, Pulmonary arteriovenous fistula, Pulmonary embolus, Renal arteriography, Renal cell carcinoma, Renal vein thrombosis, Renal venogram, Renovascular hypertension [ufhealth.org]
Most patients (10 of 14) had generalized vague illness and nephrotic syndrome but 4 were initially seen with acute symptoms of flank pain, hematuria or hypertension. [ncbi.nlm.nih.gov]
Urogenital
- Flank Pain
RVT should always be included in the differential diagnosis of flank pain and hematuria, and because RVT can induce acute renal injury. A 19-year-old man was admitted to our hospital because he complained of right flank pain and oliguria for 3 days. [ncbi.nlm.nih.gov]
The first symptom is usually flank pain occurring posterior to the lower ribs and in the region of the hips. In patients with renal malignancies, anorexia and weight loss may be predominant while patients with nephrotic syndrome may have anasarca. [symptoma.com]
- Hematuria
A 38-year-old woman presented with flank pain and hematuria suggestive of renal calculus on ultrasound. [ncbi.nlm.nih.gov]
When the cause is a renal cancer, its signs (eg, hematuria, weight loss) predominate. [msdmanuals.com]
Workup
Diagnosis in patients with RVT is difficult as the clinical presentation is often overshadowed by anasarca or weight loss and anemia due to the predisposing conditions like nephrotic syndrome and renal malignancies respectively. Routine laboratory tests like complete blood count, urinalysis, serum blood urea nitrogen (BUN) and creatinine levels may reveal anemia, oliguria/proteinuria, and elevated BUN/ creatinine levels due to renal dysfunction.
The diagnosis of RVT is based on radiological tests. Ultrasonography in the acute stage may reveal renal enlargement with hyperechogenicity [6] and is the first radiological investigation to be performed although it cannot confirm the diagnosis. Computed tomogram with angiography (CTA) is the gold standard investigation with almost 100% sensitivity and specificity [7] as it can detect the occluding thrombus with abnormal renal perfusion [8]. The radiologist can also deduce the presence of a thrombus on CTA indirectly due to signs of renal enlargement, renal vein dilatation, poor renal venous drainage on a nephrogram, delayed corticomedullary differentiation, and renal fascial thickening [7]. Instead of a CTA, magnetic resonance imaging (MRI) is preferred in patients with renal impairment as it can be performed without using nephrotoxic contrast [9]. However, it has a lower sensitivity and specificity as compared to CTA [10].
Treatment
Treatment of renal vein thromboses has previously been limited to conventional anticoagulation. [ncbi.nlm.nih.gov]
Prognosis
Prognosis Prognosis is determined by the effects on nephrotic syndrome, renal dysfunction or the complications resulting from thromboembolism. Prognosis of any underlying cause is worsened by the onset of acute renal vein thrombosis. [patient.info]
In this report, we describe a fetus with unilateral RVT and summarize the literature regarding prenatal diagnostic criteria and postnatal prognosis. [ncbi.nlm.nih.gov]
Clinical Investigation and Epidemiology Summary Background: Renal vein thrombosis (RVT) complicating the nephrotic syndrome is associated with a poor prognosis. [link.springer.com]
Etiology
Nephrotic syndrome preceded the hospital admission of the patient and was the etiological cause of renal vein thrombosis. [ncbi.nlm.nih.gov]
Harrison, Milne, and Steiner (8) recognized 4 etiologic varieties of renal-vein thrombosis: [1] thrombosis of the inferior vena cava and renal veins secondary to extension of thrombi from pelvic or leg veins; [2] thrombotic occlusion of the inferior vena [annals.org]
There are a host of etiologies for renal vein thrombosis, including trauma, compression by neoplasms, renal vein invasion by renal cell carcinoma, and nephrotic syndrome with membranous nephropathy and hypercoagulable state. [library.med.utah.edu]
Epidemiology
Department of Clinical Epidemiology, Leiden University Medical Centre, Leiden, the Netherlands Department of Hematology, Academic Medical Centre, Amsterdam, the Netherlands Search for more papers by this author First published: 15 January 2014 Cited by [doi.org]
Epidemiology The overall prevalence is unknown, as renal vein thrombosis is probably underdiagnosed. it is rare in children and usually due to severe illness, eg asphyxia, severe infection, dehydration. [patient.info]
Clinical Investigation and Epidemiology Summary Background: Renal vein thrombosis (RVT) complicating the nephrotic syndrome is associated with a poor prognosis. [link.springer.com]
[…] common non–catheter-related thromboembolism in the neonatal period May also be associated with nephrotic syndrome, hypercoagulable states, and oral contraceptive use May present with a clinical triad of flank mass, gross hematuria, and thrombocytopenia Epidemiology [unboundmedicine.com]
Pathophysiology
Moreover, diffusion-weighted MRI appears to provide important insights into the pathophysiology of delayed graft function and deserves further investigation. [ncbi.nlm.nih.gov]
Effective strategies to prevent and treat AD remain elusive despite major efforts to understand its basic biology and clinical pathophysiology. Significant investments in therapeutic ... [doi.org]
The pathophysiology of venous thrombosis has been reported to involve a combination of three interrelated factors: endothelial damage, stasis, and hypercoagulability. [bmcurol.biomedcentral.com]
Prevention
Fibrinolytic and heparin therapy may play a role in preventing chronic renal failure in neonates with bilateral RVT. However, the use of fibrinolytics early after any major surgery requires tremendous caution. [ncbi.nlm.nih.gov]
If you have experienced renal vein thrombosis, call your provider if you have: Decrease in urine output Breathing problems Other new symptoms Prevention In most cases, there is no specific way to prevent renal vein thrombosis. [mountsinai.org]
The treatment helps to prevent the formation of new clots and reduces the risk of clot traveling to other locations in the body (embolization). You may get medicines that prevent blood clotting (anticoagulants). [nlm.nih.gov]
References
- Mansi MK. Postpartum renal infarction secondary to spontaneous renal vein thrombosis. J Urol. 2001;165:893–894.
- Harris SL, Smith MP, Laurie A, Darlow BA. Neonatal renal vein thrombosis and prothrombotic risk. Acta Paediatr. 2010 Jul;99(7):1104-7.
- Bailey P, Sarfraz F, Ravanan R. Anti-GBM disease and renal vein thrombosis. BMJ Case Rep. 2011 Nov 15;
- Mouhsine A, Belkouch A, El Mehdi A et al. Renal vein thrombosis in the postpartum period: a case report. J.Med Case Rep. 2014; 8:462
- Harrison CV, Milne MD, Steiner RE. Clinical aspects of renal vein thrombosis. QJ Med. 1956;25:285–301.
- Ricci MA, Lloyd DA. Renal venous thrombosis in infants and children. Arch Surg. 1990;125:1195–1199. doi: 10.1001/archsurg.1990.01410210121019.
- Alvarez-Castells A, Sebastia CC, Quiroga GS. Computerized tomography angiography of renal vessels. Arch Esp Urol. 2001;54:603–615.
- Kawashima A, Sandler CM, Ernst RD, et al. CT evaluation of renovascular disease. Radiographics. 2000;20 (5):1321-40.
- Tempany CM, Morton RA, Marshall FF. MRI of the renal veins: assessment of nonneoplastic venous thrombosis. J Comput Assist Tomogr. 1992;16 (6): 929-34.
- Hodgson DJ, Jan W, Rankin S, et al. Magnetic resonance renal angiography and venography: an analysis of 111 consecutive scans before donor nephrectomy. BJU Int. 2006;97:584–586.