Idiopathic spontaneous extensive iliocaval thrombosis presenting with renal infarct
- 1 Urology and Renal Transplant, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
- 2 Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
- Correspondence to Dr Hira Lal; hiralal2007@yahoo.co.in
Abstract
We describe an extremely rare case of idiopathic spontaneous extensive venous thrombosis in a young man involving the inferior vena cava, the iliac veins and both renal veins associated with right haemorrhagic renal infarction with non-functioning right kidney.
Background
Renal infarction is a rare cause of acute flank and abdominal pain.1 Renal vein thrombosis should be considered in patients with renal infarction or any unexplained deterioration in renal function, particularly in patients with the nephrotic syndrome or renal tumours, hypercoagulable states and after surgery or trauma to the renal vessels. Haemorrhagic infarction of the kidneys due to thrombosis of the inferior vena cava (IVC) and renal veins is an extremely rare entity as opposed to haemorrhagic renal infarction resulting from localised renal vein thrombosis.2 This is probably the first case being reported about an adult with extensive iliocaval thrombosis with renal infarction. Appropriate line of management and long-term renal outcomes are still not well known.3 4
Case presentation
A 27-year-old man, a tobacco chewer, with no comorbidities presented with right flank pain and purple red discolouration of right flank with gross total painless haematuria for 2 weeks. He also reported mild breathlessness and bilateral lower limb swelling of 10 days duration. The patient, a resident of India, had gone to work in arid climate in Saudi Arabia for 2 years prior to presentation. There was no previous similar history or any significant medical or surgical history.
Physical examination revealed the patient to be conscious and alert but distressed. He was lethargic and dehydrated and febrile with temperature of 37.9°C; his blood pressure was 100/70 mm Hg and pulse rate was 90/min. He was breathing rapidly with respiratory rate of 30/min. He had bilateral pitting pedal oedema extending into both legs and feet. On abdominal examination, Grey-Turner sign was present on right side with right flank tenderness. The abdomen was distended but there was no lump. Catheterisation obtained blood-tinged urine. Chest was clear with no adventitious sounds. The patient was admitted initially as a case of retroperitoneal haemorrhage.
Investigations
The haemoglobin was 90 g/L and total leucocyte count was 18.5×109/L with a differential count of 26% lymphocytes, 1% basophils, 5% eosinophils and 68% polymorphonuclear leucocytes.
Markers of blood coagulation (Prothrombin Time, International Normalised Ratio and activated Partial Thromboplastin Time) and biochemical tests, including creatinine and liver enzymes, were normal except for raised alkaline phosphatase and lactate dehydrogenase. Urine analysis showed haematuria with 10–15 red blood cells/hpf and mild proteinuria while urine pH was 5. Chest X-ray showed both lung fields to be normal. Supportive management was started in the form of analgesics, parenteral antibiotics and intravenous fluids. Patient’s urine output remained normal since day of admission and during hospital stay. The patient was immediately referred to radiology suite after stabilisation for contrast-enhanced CT (CECT) scan with CT angiography of the abdomen and chest. The right kidney was bulky with mild thickening of the perirenal fascia. There was no enhancement in right kidney. CECT demonstrated hypodense filling defect in IVC and both renal veins (figure 1), more in right side; the filling defect extending superiorly upto infrahepatic segment of IVC and inferiorly into bilateral iliac veins. There was no abnormal morphology associated with IVC. CECT chest was unremarkable except for subpleural consolidation in both lungs in lower lobes.
Contrast-enhanced CT scan of abdomen in axial plane at the level of renal veins showing bulky non-enhancing right kidney with thrombus in inferior vena cava and both renal veins.
MR angiography was done to evaluate the extent of thrombus which showed bulky right kidney of 12.6×8 cm with cortical rim sign and no parenchymal enhancement (figure 2). It also showed patchy areas of diffusion restriction with corresponding low apparent diffusion coefficient (ADC) values suggestive of renal infarct with perirenal fat stranding. Thrombus was seen in bilateral common iliac veins, bilateral renal veins and in IVC expanding its lumen and extending superiorly into infrahepatic segment and in left gonadal vein. Right renal artery was markedly narrow. Left kidney was normal in size and enhancement. On Doppler ultrasonography of both lower limbs, distal extent of thrombus was confirmed upto superficial femoral veins bilaterally.
MRI abdomen—(A) T2-weighted sequence in axial plane at the level of kidneys showing enlarged right kidney with altered signal with loss of normal signal in inferior vena cava (IVC) and both renal veins; (B) diffusion-weighted sequence showing diffusion restriction in right kidney and IVC and (C) postcontrast liver acceleration volume acquisition sequence in coronal plane showing non-filling of IVC superiorly upto infrahepatic location with non-opacification of both renal veins and non-enhancing right kidney with cortical rim sign.
Evaluation for thrombophilia was done by haematologist and tests for anticardiolipin antibodies and antibeta2 gp1 antibodies were negative. Antithrombin III activity level was 80% (normal 80%–120%). Blood sample for factor V Leiden mutation was positive (heterozygous). Flow cytometry of peripheral blood sample for Paroxysmal Nocturnal Hemoglobinuria (PNH) panel comprising of CD45/CD64/CD15/CD157/FLAER (Fluorescent Aerolysin) was also negative. methylenetetrahydrofolate reductase (MTHR) genotype was heterozygous for the sample.
Differential diagnosis
Initially on the basis of presentation, patient was suspected to have spontaneous renal haemorrhage likely due to rupture of any vascular neoplasm with extension to the subcapsular and perirenal spaces. A remote possibility of severe acute pancreatitis was also kept. However, CT scan and later MRI ruled out the other diagnoses.
Treatment
Risk of pulmonary embolism was explained to the patient and his attendants and he was started on intravenous heparin after consulting cardiovascular surgeon and then was overlapped with oral warfarin which was continued thereafter and heparin was stopped. In view of extensive iliocaval venous thrombosis and risk of haemorrhage from the infarcted kidney, endovascular or catheter-directed interventions were not performed.
Patient’s pain subsided within 2 days but his fever continued for 5 days of starting antibiotics. Total leucocyte count also decreased to 9300/mm3 after 5 days and further to 7900/mm3 after 10 days of admission. His general condition improved and he was able to walk independently. Renal Doppler done after 7 days of starting anticoagulant therapy did not show any vascularity in right kidney with thickened renal cortex. Diethylenetriaminepentacetate (DTPA) scan was done after 10 days of starting anticoagulants which showed non-functioning right kidney and normally functioning left kidney with glomerular filtration rate (GFR) 53 mL/min.
Outcome and follow-up
Patient was continued on oral anticoagulant therapy and MRI abdomen 2 months after starting therapy showed right kidney returning to normal size of 9.2×6.2 cm with cortical rim sign and no parenchymal enhancement. It also showed patchy areas of diffusion restriction with corresponding low ADC values suggestive of renal infarct and perirenal fat stranding. There was no perirenal collection. Thrombus was seen in bilateral common iliac veins, bilateral renal veins and in IVC extending superiorly into infrahepatic segment and in left gonadal vein. Length of IVC thrombus and IVC expansion had decreased.
Patient was continued on oral anticoagulant with target INR of 1.5–2 and MRI abdomen at 1 year showed shrunken right kidney of 6×4 cm with no postcontrast enhancement and loss of corticomedullary differentiation (figure 3). There was marked narrowing of IVC, mainly in infrarenal part. Multiple anterior abdominal wall, lumbar and paravertebral venous collaterals were seen. No definite thrombus was seen in this scan. GFR of right kidney was 0 and left kidney was 74 mL/min. Presently patient is on oral anticoagulants.
Follow-up MRI abdomen—(A) T2-weighted sequence in axial plane at the level of kidneys shows small contracted right kidney; (B) T2-weighted sequence in coronal plane showing small right kidney with narrowing of inferior vena cava (IVC) and (C) postcontrast liver acceleration volume acquisition (LAVA) sequence in coronal plane showing non-opacification of IVC with formation of multiple paravertebral venous collaterals.
Discussion
Haemorrhagic infarction of the kidney due to thrombosis of the renal vein and IVC is a very rare disease, usually encountered in the neonatal period. However, it may rarely occur in adults, as in this case.
In our case, patient had thrombosis involving bilateral renal veins; however, renal infarction occurred in right side only since multiple tributaries of left renal vein help in development of collateral flow leading to unobstructed blood flow. An extensive literature search failed to identify any other similar case reported in adults.
The main aetiologic factors for renal vein thrombosis have been listed as dehydration, infection, polycythemia, sickle cell disease etc in neonates and nephrotic syndrome, systemic lupus erythematosus, amyloidosis, glomerulonephritis, collagen vascular disease, diabetes and tumour thrombus in adult patients. However, a definite cause for iliocaval thrombosis has yet to be confirmed as in the case which we have presented and it may be inferred that the thrombosis would have started when the patient was working in arid climate 15 days prior to presenting to the hospital.
In our case, the thrombus was found in the femoral veins, the iliac veins and the IVC, and so it was not a localised process limited to the kidneys.2 Thrombosis of the IVC in the absence of congenital abnormalities is rare and is usually a result of a predisposing hypercoagulable state along with an acquired pathology in the IVC or one of its adjacent structures.5 However, the haematological workup to identify any coagulational abnormality, whether genetic or otherwise, was negative. No obvious risk factors for initiation of thrombosis like any anatomic congenital abnormality in IVC were found and the aetiology was considered as idiopathic. Anticoagulation, the current first-choice treatment for venous thromboembolism, was promptly started in this case. Catheter-directed thrombolysis was not attempted in this case as patient had haemorrhagic renal infarction with haematuria and the golden period of 14 days had already passed when patient presented to us.5 The thrombus did not propagate in this case, as evident on follow-up MRI scans.
Patient’s perspective
I was admitted to the hospital in a very distressing condition and was told that a clot has formed in the biggest vessel in my body with bleeding in my right kidney. I was very scared, however the doctors gave me hope by persisting and managing all aspects. Gradually I improved and presently I am asymptomatic on continuous medications and I have been told that my right kidney has shrunken and not likely to cause any problem.
Learning points
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Renal infarction is an extremely rare complication of another rare condition, idiopathic iliocaval thrombosis which may present as retroperitoneal haemorrhage.
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There are no specific guidelines regarding its diagnosis and management due to paucity of the literature.
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Awareness of this entity and multidisciplinary approach are necessary for the diagnosis and optimal management of this condition.
Footnotes
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Twitter @babu_drjmt
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Contributors NK, AS and HL conducted the study including patient recruitment, data collection and data analysis. NK and NM did the literature review and prepared the manuscript draft. All the authors had complete access to the study data. The manuscript was finally reviewed by AS and approved by HL.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Competing interests None declared.
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Patient consent for publication Obtained.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.
References
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