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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2020  |  Volume : 31  |  Issue : 5  |  Page : 1110-1116
Spontaneous Renal Artery Dissection with Bilateral Renal Infarction Successfully Treated with Rivaroxaban


Department of Medicine, Section of Nephrology, The Aga Khan University Hospital, Karachi, Sindh, Pakistan

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Date of Web Publication21-Nov-2020
 

   Abstract 


Spontaneous renal artery dissection (SRAD) causing bilateral renal infarction is a rare condition. It may present with nonspecific symptoms, resulting in delayed diagnosis. We report a case of SRAD in an adult male who presented with flank pain and fever. The patient was initially worked up for possible pyelonephritis, which came back negative. Later, a diagnosis of SRAD with bilateral renal infarction was made on contrast-enhanced computed tomography (CT) abdomen followed by CT angiogram. The patient was treated with rivaroxaban and antihypertensive therapy. He was followed up for 12 months after the initial presentation and repeat imaging showed no new infarcts and a stable renal function.

How to cite this article:
Haider A, Abdul Razzaque MR, Yaqub S. Spontaneous Renal Artery Dissection with Bilateral Renal Infarction Successfully Treated with Rivaroxaban. Saudi J Kidney Dis Transpl 2020;31:1110-6

How to cite this URL:
Haider A, Abdul Razzaque MR, Yaqub S. Spontaneous Renal Artery Dissection with Bilateral Renal Infarction Successfully Treated with Rivaroxaban. Saudi J Kidney Dis Transpl [serial online] 2020 [cited 2020 Dec 3];31:1110-6. Available from: https://www.sjkdt.org/text.asp?2020/31/5/1110/301179



   Introduction Top


Spontaneous renal artery dissection (SRAD) with renal infarction is a rare disease which often poses a diagnostic challenge to the treating physicians. Flank pain is the most common clinical presentation and most of the times, it is mistaken as nephrolithiasis or pyelonephritis. Other common underlying causes, especially thromboembolic phenomena, need to be considered and investigated first before making this uncommon diagnosis with vascular imaging studies. Various imaging modalities such as computed tomography (CT), intravenous urogram (IVU), or magnetic resonance imaging (MRI) may be helpful in diagnosing the condition, however renal angiogram remains the gold standard. Management options include conservative treatment of hypertension (HTN), use of anticoagulants, endovascular procedures, open vascular surgery, and total or partial nephrectomy. We report a case of SRAD in an adult male who presented with flank pain and fever. The patient was initially worked up for possible pyelonephritis, and eventually a diagnosis of SRAD with bilateral renal infarction was made on contrast-enhanced CT.


   Case Report Top


Written informed consent was obtained from the patient for the publication of this case.

A 56-year-old male presented to the emergency room with abdominal pain for seven days, and vomiting and fever for three days. The pain was severe in intensity and had started in the left lower quadrant initially and later migrated to the right lower quadrant and only temporarily relieved with oral analgesics. He had two episodes of nonbilious, nonpro-jectile vomiting and had low-grade fever. There were no associated urinary symptoms.

He had past history of benign prostatic hyperplasia and Helicobacter pylori gastritis. On examination, his heart rate was 91 beats/min (regular sinus rhythm) and blood pressure (BP) was 140/90 mm Hg, and he was afebrile. His abdomen was soft but tender in the right lumbar region and right iliac fossa with normal bowel sounds. [Table 1] shows the laboratory investigations on presentation. Electrocardiogram revealed normal sinus rhythm. Blood and urine cultures did not grow any organisms. Ultrasound kidneys were unremarkable [Figure 1].
Figure 1: Ultrasound images of the bilateral kidneys.

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Table 1: Laboratory parameters on presentation.

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Considering acute pyelonephritis as the provisional diagnosis, a non-contrast CT scan of the kidneys and bladder was performed which showed no evidence of urolithiasis, obstructive uropathy, or pyelonephritis. He was managed with intravenous fluids and intravenous antibiotics initially. He continued to be febrile over the next 48 h with persistent abdominal pain, rising total leukocyte count (TLC), and worsening kidney functions (creatinine 2.1 mg/dL). He then underwent CT abdomen with intravenous contrast.

The CT scan showed multiple wedge-shaped low-attenuated areas in the cortices of both kidneys, suggestive of renal infarcts, more marked in the upper and lower poles of the right kidney and upper pole of the left kidney, predominantly involving the anterior branches. There was mild perinephric fat stranding, more marked on the right side; there was no evidence of renal vein thrombosis and no significant artherosclerotic disease or renal artery abnormality [Figure 2].
Figure 2: Contrast-enhanced abdominal computed tomography scan showing bilateral infarction more on right side of the kidney.

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Additional testing was conducted to determine a possible underlying cause. These tests included a hypercoagulability workup including prothrombin time, partial thromboplastin time, antiphospholipid antibodies, protein C and S activity, antithrombin III activity, homocysteine level, and autoimmune profile including antinuclear antibody and antinuclear cytoplasmic antibodies. All the test results were within normal limits. Transthoracic echocardiography revealed no evidence of right-to-left intra-cardiac shunts, thrombus, or cardiac tumor. CT angiogram of mesenteric and renal arteries was undertaken which showed features suggestive of SRAD with renal infarctions bilaterally [Figure 3]. After discussion with vascular surgeons, he was started on anticoagulation with rivaroxaban. In the meantime, the patient's condition stabilized and the fever subsided. Amlodipine 5 mg was added for better BP control. His serum creatinine remained static on 2.1 mg/dL and TLC improved. He was discharged from the hospital and was advised clinic follow-up.
Figure 3: Computed tomography angiogram showing the linear filling defect in the bilateral renal arteries, consistent with dissection and bilateral infarctions.

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He was followed up three months later with repeat ultrasound abdomen. There were no new infarctions and anticoagulation was continued for six months and then stopped. At one-year follow-up, he remained asymptomatic with good BP control on a single agent and serum creatinine improved to 1.4 mg/dL with no new infarcts on repeat imaging despite being off anticoagulation for six months [Figure 4].
Figure 4: Ultrasound images of the bilateral kidneys on follow-up after 1 year.

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   Discussion Top


We have described a case of bilateral renal artery dissection as an isolated, nontraumatic event, and the initial presentation was that of ureteric colic or pyelonephritis. He was successfully treated with a newer oral anticoagulant without any endovascular intervention. SRAD is a rare entity with approximately 200 cases reported in the literature so far.[1] However, it is speculated that the actual incidence of this condition may be much higher than reported because it may be underdiagnosed or in many cases, it may be silent and may undergo spontaneous resolution. Due to its rarity and nonspecific clinical presentation, SRAD may often be a diagnostic and therapeutic challenge for clinicians. It occurs predominantly in the 4th decade of life, with males being more commonly affected than females (male-to-female ratio of 4:1).[2] The left renal artery is more frequently involved in this condition possibly because of its shorter length, predisposing it to dissection in case of trauma.[3] However, in 10%–15% of cases, SRAD has been found to affect bilateral renal arteries.[3]

SRAD may be associated with certain conditions including fibromuscular dysplasia, malignant HTN, severe atherosclerosis, Marfan syndrome, Ehlers–Danlos syndrome, sub-adventitial angioma, cystic medial necrosis, and extreme physical exertion.[4] It is also reported to occur as an extension of aortic dissection, post percutaneous angioplasty or any blunt abdominal trauma.[5],[6] However, in most of the cases, the etiology may remain uncertain.

SRAD can present clinically with severe HTN, hematuria, proteinuria, flank pain, or acute kidney injury. However, in most cases, the initial presentation can be nonspecific with symptoms suggestive of renal colic, as in our scenario. A study performed at the University of Michigan over 12 years evaluated the characteristics of 17 patients having a SRAD and found flank/lumbar pain to be the most common clinical presentation.[7]

Uncontrolled HTN may be both the cause and effect of SRAD. HTN is found commonly in SRAD patients in many studies. Although there are case reports where patients are normotensive, many series have reported HTN associated with SRAD.[3] Alamir et al found malignant HTN at presentation in their series of SRAD cases,[8] while Ramamoorthy et al reported fibromuscular dysplasia and atherosclerotic renal artery disease associated with their series of SRAD cases.[5]

The differential diagnosis of SRAD includes thromboembolism with or without renal infarction, renal vein thrombosis, renal abscess, ureteric calculi, and pyelonephritis.[9] The diagnosis must be made as early as possible to increase the chances of renal revascularization and recovery. Because of this vague clinical presentation, it may simulate urolithiasis or pyelonephritis. The rarity and nonspecific presentation of SRAD often lead to a delayed or a missed diagnosis. Nonspecific laboratory findings may be noted in these patients. Urinalysis could reveal hematuria and proteinuria in about half of the patients, and kidney function is usually only slightly impaired. Serum lactate dehydrogenase, a marker for tissue damage, is classically elevated, as was evident in our case as well.

A definitive diagnosis can only be made on imaging. Multiple imaging modalities including CT scan, MRI with gadolinium contrast, CT angiogram, IVU, and intravascular ultrasound can be used to visualize the dissection or an area of renal hypo-perfusion. CT angiogram has been the gold standard, and it enables the extent and nature of the dissection as well as provides potential therapeutic options.[6] Conventional noninvasive imaging such as ultrasound, Doppler, or plain CT imaging can be misleading, such as in our case, where all these imaging results came out negative. In order to ensure timely diagnosis of this condition, clinicians must have a high index of suspicion for this rare phenomenon. Once common etiologies are ruled out on noncontrast/noninvasive studies, one must keep a low threshold to do contrast studies earlier in the course.

Management of patients with SRAD usually focuses on restoring patency to the affected artery and treating the underlying cause if possible. Given the rarity of this condition, with literature discussing only case reports and case series, the best treatment strategy is not known and so should be individualized. Management options include conservative medical management, surgical therapy, and endovascular procedures.[3] Conservative treatment includes BP control, pain management, and anticoagulation. Anticoagulation therapy provides satisfactory short-term results, but its long-term impact is unknown and is not reported in literature. Furthermore, there are no recommendations regarding the optimal duration and choice of anticoagulation due to lack of available data. Parental heparin (fractionated or unfractionated) followed by warfarin has been used in most of the cases as part of conservative management.[10]

There is limited data available on the role of new anticoagulants, such as rivaroxaban in such patients, and we could find only two case reports and both had unilateral SRAD, unlike our case. Dauchy et al reported the case of a 54-year-old female with right-sided flank pain, and renal artery angiogram demonstrated a spon-taneous dissection of the superior branch of the right renal artery.[11] Percutaneous intervention was not performed due to the risk of jeopardizing the other vessels, and she was conservatively treated with rivaroxaban along with hydrochlorothiazide and metoprolol, and repeat angiogram performed after eight -week follow-up showed healing of the dissection. Ababneh Md et al reported the case of a 56- year-old male with acute abdominal pain. His workup showed right-sided renal infarction, secondary to renal artery occlusion.[12] After multiple unsuccessful attempts of revascularization, he was kept on medical treatment initiated on anticoagulation with unfractionated heparin infusion which was later switched to rivaroxaban and was continued for six months, mimicking the recommendations for the treatment of pulmonary emboli without hemodynamic compromise. Our patient had bilateral SRAD with initially worsening kidney functions. We used rivaroxaban for a total of six months with good results. Not only that his renal functions improved but also repeat imaging at one-year follow-up showed no new infarcts. Moreover, his BP was well controlled on small dose of just one anti-hypertensive agent.

Surgical intervention may be considered in selected patients. It may include vascular reconstruction via arterial bypass, endovascular intervention (stenting or coiling), or nephrectomy. Surgical intervention in the form of arterial bypass is reserved for cases with correctable dissection causing hemodynamically significant occlusion of the main or major segmental renal arteries, uncontrolled renovascular HTN resistant to medical treatment, significantly deteriorating renal function, or for those with failed endovascular intervention.[9] Nephrectomy is preferred if the infarct size is large, severely compromised renal function, or if the revascularization is not possible due to involvement of a branch artery.[13] Endovascular approach has been described in case reports and series with good outcomes at follow-up in terms of better BP control and no signs of occlusion or restenosis. It may include stenting or coiling and is considered in cases where an early diagnosis is made and hence there is enough residual renal function.[14]

In cases where conservative management is opted for, a serial angiography may be obtained and if it shows stability of the dissection and no worsening in renal function, conservative approach should be continued, but if there is acute deterioration in renal function, urgent surgical intervention may be necessary.[6]

Spontaneous resolution has been seen in a few cases and is attributed to the re-entry phenomenon of the false lumen back into the true lumen or due to a complete obliteration of the dissected segment by thrombosis and organization.[15] The major long-term effect is malignant HTN, and mortality generally results from renal failure, which is more common with bilateral lesions.[5]


   Conclusion Top


SRAD is a rare but important cause of flank pain, and clinicians should be mindful of it as a differential diagnosis. It can easily be missed due to its rarity and nonspecific clinical presentation, hence causing delay in diagnosis and appropriate management. Conventional imaging modalities can be helpful, but CT angiogram is the gold standard for diagnosis. Nonsurgical management with adequate BP control and anti-coagulation can provide significant short-term results, but long-term follow-up is required and in selected cases, surgical interventions may be beneficial.


   Declaration of Patient Consent Top


The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Conflict of interest: None declared.



 
   References Top

1.
Kanofsky JA, Lepor H. Spontaneous renal artery dissection. Rev Urol 2007;9:156-60.  Back to cited text no. 1
    
2.
Béroniade V, Roy P, Froment D, Pison C. Primary renal artery dissection. Presentation of two cases and brief review of the literature. Am J Nephrol 1987;7:382-9.  Back to cited text no. 2
    
3.
Renaud S, Leray-Moraguès H, Chenine L, Canaud L, Vernhet-Kovacsik H, Canaud B. Spontaneous renal artery dissection with renal infarction. Clin Kidney J 2012;5:261-4.  Back to cited text no. 3
    
4.
Lacombe M. Isolated spontaneous dissection of the renal artery. J Vasc Surg 2001;33:385-91.  Back to cited text no. 4
    
5.
Ramamoorthy SL, Vasquez JC, Taft PM, McGinn RF, Hye RJ. Nonoperative management of acute spontaneous renal artery dissection. Ann Vasc Surg 2002;16:157-62.  Back to cited text no. 5
    
6.
Mudrick D, Arepally A, Geschwind JF, Ronsivalle JA, Lund GB, Scheel P. Spontaneous renal artery dissection: Treatment with coil embolization. J Vasc Interv Radiol 2003;14:497-500.  Back to cited text no. 6
    
7.
Afshinnia F, Sundaram B, Rao P, Stanley J, Bitzer M. Evaluation of characteristics, associations and clinical course of isolated spontaneous renal artery dissection. Nephrol Dial Transplant 2013;28:2089-98.  Back to cited text no. 7
    
8.
Alamir A, Middendorf DF, Baker P, Nahman NS Jr., Fontaine AB, Hebert LA. Renal artery dissection causing renal infarction in otherwise healthy men. Am J Kidney Dis 1997;30:851-5.  Back to cited text no. 8
    
9.
Stawicki SP, Rosenfeld JC, Weger N, Fields EL, Balshi JD. Spontaneous renal artery dissection: Three cases and clinical algorithms. J Hum Hypertens 2006;20:710-8.  Back to cited text no. 9
    
10.
Guérin E, Vandueren E, Dubois E, Delcour C, Wautrecht JC, Verhelst G. Spontaneous renal artery dissection in an otherwise healthy male. Acta Chir Belg 2006;106:703-6.  Back to cited text no. 10
    
11.
Dauchy EM, Modica M, Masri N. A rare cause of abdominal pain. J La State Med Soc 2017; 169:54-5.  Back to cited text no. 11
    
12.
Ababneh Md B, Ali MJ, Lopez FA. Clinical case of the month: A 56-year-old man with sudden onset abdominal pain. J La State Med Soc 2016;168:63-5.  Back to cited text no. 12
    
13.
Peynircioglu B, Pişkinkaya S, Özer Ç, Çil B, Yorgancioglu C, Arici M. Isolated spontaneous renal artery dissection: Diagnosis and endovascular management. Diagn Interv Radiol 2011;17:101-4.  Back to cited text no. 13
    
14.
Pellerin O, Garçon P, Beyssen B, et al. Spontaneous renal artery dissection: Long-term outcomes after endovascular stent placement. J Vasc Interv Radiol 2009;20:1024- 30.  Back to cited text no. 14
    
15.
Mori H, Hayashi K, Tasaki T, Hori T, Yamasaki T, Amamoto Y. Spontaneous resolution of bilateral renal artery dissection: A case report. J Urol 1986;135:114-6.  Back to cited text no. 15
    

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Correspondence Address:
Sonia Yaqub
Department of Medicine, Section of Nephrology, The Aga Khan University Hospital, Karachi, Sindh, 74800
Pakistan
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DOI: 10.4103/1319-2442.301179

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