Renal vein thrombosis I82.3

Renal vein thrombosis (NVT) was first described by Rayer in 1840 (Jorch 2010). Schauwecker was the first to describe a connection between NVT and amyloidosis in 1930, and Siegmund a connection between NVT and myeloma in 1935 (Zollinger 1966).

Renal vein thrombosis is defined as a thrombosis of the renal vein with the risk of loss of renal function (Manski 2019). It is usually a complication of an accompanying chronic underlying disease (Segerer 2014).

NVT is divided into a:

• acute course (occurs predominantly with severe bilateral parenchymal damage in young children [Remmele 2013])
• chronic form of progression (found in older children and adults) (Kuhlmann 2015).

In 0.09% of all autopsies, NVT is found as a terminal event. A chronic course can be detected in 0.04 % of autopsies (Remmele 2013).

NVT is a rare disease in newborns with an incidence of 2.2 per 100,000 live births. The proportion of male patients is approximately 67% (Jorch 2010).

In patients with nephrotic syndrome, however, hypercoagulability leads to thrombosis and/or thromboembolic complications in up to 25 % of cases in the course of the disease (Kuhlmann 2015).

The causes of renal vein thrombosis can be divided into 3 broad groups:

• 1. endothelial damage:
  • Homocystinuria
  • endovascular interventions
  • surgical interventions with vascular endothelial damage (Kasper 2015).
• 2. venous stasis:
  • dehydration (most frequent cause in paediatrics)
  • Risk factors in neonates and infants are:
    • difficult births
    • Asphyxia
    • cyanotic cardiac vitals
    • hypotension (Manski 2019)
  • Compression (e.g., due to abdominal neoplasia, Ormond disease [Kasper 2015])
  • Kinking of the renal veins (change in position of vessels due to e.g. malposition, arteriosclerosis etc. [Schild 2003])
  • in cases of kidney transplantation:
    • too narrow venous anastomosis
    • extraluminal compression due to e.g. a hematoma or a lymphocele (Hegele 2015)
• 3. thrombophilia:
  • antiphospholipid syndrome
  • Thrombophilia e. g. in:
    • nephrotic syndrome
    • membranous glomerulonephritis
    • deficiency of antithrombin, protein S, protein C
    • Factor V Leiden mutation
    • use of oral contraceptives (Kasper 2015)

In infants, thrombosis develops centripetally from the periphery (so-called peripheral thrombosis); in older children or adults, it develops centrifugally from the renal vein to the periphery (central thrombosis).

(Hofmann 2005)

The tendency to thrombosis in nephrotic syndrome is due to hypercoagulability, which is caused by:

• renal loss of antithrombin III (this leads to a low concentration of inhibitors in the renal veins).
• thrombocytosis
• increased platelet aggregation
• Increased hepatic protein synthesis with renal protein loss and thus increased synthesis of clotting factors (Kuhlmann 2015).

The left kidney is more frequently affected by thrombosis than the right. In 2/3 of the cases there is a bilateral thrombosis (Kasper 2015).

In the chronic form, there are usually no clinical symptoms (Kuhlmann 2015).

In the acute form, the following may occur:

• flank pain
• sometimes a tumor can be felt in the flank due to enlargement of the kidney (Manski 2019)
• macrohaematuria (Kuhlmann 2015)
• Knocking pain (Kasper 2015)
• Swelling of the left-sided testis (Herold 2021) or left ovary with left-sided thrombosis (Keller 2010)
• Functional deterioration of renal performance in transplanted kidney (Hegele 2015)
• Anuria in bilateral thrombosis (Hofmann 2005)

Sonography

Typical but not specific findings of NVT are:

• enlargement of the renal volume to 2 - 3 times the original size
• high echogenicity
• reduction of the corticomedullary differentiation
• overall unstable texture

(Hofmann 2005)

• pendulum flow detectable (Risler 2008)
• Extension of the thrombus into the v. cava possible (Manski 2019)

In Z. n. kidney transplantation:

• unilateral enlargement of the kidneys
• increased echogenic kidneys
• increased visualization of capsular vessels

(Risler 2008)

• increase of the size of the graft
• emphasized medullary papillae (Keller 2010)

Color Doppler sonography

• Renal vein flow is reduced (in infants) or absent (in older children or in adults)
• The resistance index of the arterial pathway increases to ≥ 1 and thus diastolic flow becomes negative (Hofmann 2005)

In patients after kidney transplantation:

• Decrease of the resistance index
• Blood stasis propagating backwards via the renal vessels to the renal artery with:
  • Flow acceleration by more than 2.5 times the poststenotic value or > 180 cm / sec (Keller 2010)

Computed tomography

CT is the safest diagnostic tool with a sensitivity of 100% (Kasper 2015).

• Proteinuria (Herold 2021)
• Hematuria
• Decrease in GFR (Kuhlmann 2015)

• hemolytic-uremic syndrome (in infants with bilateral NVT [Hofmann 2005])

• acute renal failure (Keller 2010)
• Pulmonary embolism (Kasper 2015)

Therapeutically, prompt anticoagulation with full heparinization should be given (Manski 2019).

Endovascular thrombolysis can also be considered. However, this is usually reserved for severe cases (Kasper 2015).

Following acute symptomatology, marcoumarization is required, the duration of which depends on the creatinine level and the risk profile (Manski 2019).

Dosage recommendation:

For Quick or INR- values that are initially in the normal range, the following dosage can be started in adults:

• Marcumar 3 mg 2 x 1 tbl. /d in the first 3 days, then dose adjustment according to the result of the INR- value (Herold 2018).

Parallel treatment of the triggering disease is mandatory (Kasper 2015).

If life-threatening complications occur in the course of NVT, nephrectomy is required (Kasper 2015).

In cases of renal transplantation:

If there is significant venous outflow obstruction in Z. n. transplanted kidney, surgical revision is indicated (Hegele 2015).

In newborns, irreversible kidney damage is seen in 70.6 % of those affected and in 3.0 % there is a need for permanent renal replacement therapy (Jorch 2010).

The lethality in infants ranges from 65% to 95%. The chronic form, which occurs predominantly in adults, can lead to fatal pulmonary embolism (Remmele 2013).

If left untreated or if there is no response to therapy, the kidney shrinks within a few weeks, and sonographically it can sometimes hardly be distinguished from the surrounding area after a few months (Hofmann 2005).

After kidney transplantation:

After surgical treatment of the transplanted kidney, there is often a loss of function of the graft (Hegele 2015).

Patients with the above-mentioned diseases or risk factors or with recurrent thromboses can be given a vena cava filter as a preventive measure (Kasper 2015).

1. Hegele A et al (2015) Urology: intensive course for continuing education. Thieme Verlag 525
2. Herold G et al (2021) Internal medicine. Herold Publishers 658
3. Herold G et al (2018) Internal medicine. Herold Publishers 634
4. Hofmann V et al (2005) Ultrasound diagnostics in pediatrics and pediatric surgery: textbook and atlas. Thieme Verlag 476 - 477
5. Jorch G et al (2010) Neonatology: the medicine of the premature and mature infant. Georg Thieme Publishers 382 - 383
6. Kasper D L et al (2015) Harrison's Principles of Internal Medicine. Mc Graw Hill Education 1847 - 1848, 1863.
7. Kasper D L et al (2015) Harrison's internal medicine. Georg Thieme Verlag 2275 - 2276, 2294
8. Keller C K et al (2010) Practice of nephrology. Springer Verlag 3, 34, 108, 186
9. Kuhlmann U et al. (2015) Nephrology: pathophysiology - clinic - renal replacement procedures. Thieme Verlag 104 - 105
10. Manski D (2019) The urology textbook. Dirk Manski Publishers 243
11. Remmele W et al. (2013) Pathology 5: Male genitalia - Kidney - Urinary tract and urethra - Skeletal system - Joints, tendons and tendon gliding tissue, bursae, fascia - Skin. Springer Verlag 150
12. Risler T et al (2008) Specialist nephrology. Elsevier Urban and Fischer Publishers 124
13. Schild H (2003) Angiography. Thieme Verlag 335
14. Schwiegk H et al (2013) Renal diseases Springer Verlag 631.
15. Segerer K, Wanner C. (2014) Renal infarction and renal vein thrombosis. In: Steffel J et al. Kidney and draining urinary tract. Springer textbook. Springer, Berlin, Heidelberg. 171 - 174 https://doi.org/10.1007/978-3-642-28236-2_21
16. Zollinger H U et al. (1966) Kidney and draining urinary tract Springer Verlag 131.