DOI: 10.1148/rg.283075106
RadioGraphics 2008;28:855-867
© RSNA, 2008
Rare Causes of Hematuria Associated with Various Vascular Diseases Involving the Upper Urinary Tract1
Noriaki Muraoka, MD,
Toyohiko Sakai, MD, PhD,
Hirohiko Kimura, MD, PhD,
Hidemasa Uematsu, MD, PhD,
Kazuya Tanase, MD,
Osamu Yokoyama, MD, PhD, and
Harumi Itoh, MD, PhD
1 From the Departments of Radiology (N.M., T.S., H.K., H.U., H.I.) and Urology (K.T., O.Y.), University of Fukui, 23 Matsuoka-Shimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui 910-1193, Japan. Presented as an education exhibit at the 2006 RSNA Annual Meeting. Received May 9, 2007; revision requested July 10 and received September 7; accepted September 18. All authors have no financial relationships to disclose.
Address correspondence to N.M. (e-mail: nmuraoka{at}u-fukui.ac.jp).
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Abstract
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Hematuria is a commonly encountered symptom of a wide spectrum of diseases, including calculi, tumors, and vascular abnormalities. In rare cases, hematuria is caused by life-threatening vascular diseases. When hematuria is encountered, physicians sometimes fail to include vascular diseases in the differential diagnosis because of their rare association with hematuria. Likewise, radiologists often fail to do so because of the low frequency of occurrence of these diseases. Multidetector computed tomography performed with the bolus injection technique should be the first-line diagnostic test when vascular disease is suspected. Radiologists should be familiar with the various imaging findings of hematuria caused by vascular disease. They should also be familiar with the management options (including endovascular techniques) for hematuria caused by vascular disease, since in some cases affected patients can be treated with interventional procedures.
© RSNA, 2008
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LEARNING OBJECTIVES
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After reading this article and taking the test, the reader will be able to:
- Identify various vascular diseases that can cause hematuria.
- Discuss the utility of multidetector CT and angiography in detecting these vascular diseases.
- Describe the interventional procedures used to treat these diseases.
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Introduction
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Hematuria is one of the most commonly encountered indications of disease, ranging from an incidental finding to a significant symptom that requires immediate treatment. Hematuria can also be associated with various diseases of the urinary system, including renal or ureteral calculus, urothelial neoplasms (eg, renal cell carcinoma, transitional cell carcinoma, metastases), trauma, infection, and hemorrhagic cystitis.
The cause of hematuria is usually appropriately diagnosed by physicians on the basis of clinical presentation, cytologic findings, and serologic findings; however, imaging studies (intravenous urography, ultrasonography [US], or computed tomography [CT]) are sometimes necessary to confirm the diagnosis or exclude the possibility of other diseases of the urinary system such as calculus or malignant tumor. Thus, familiarity with the imaging characteristics of various diseases in patients with hematuria is crucial for radiologists.
Among the causes of hematuria, vascular disease is extremely rare; therefore, physicians sometimes fail to include this possibility in the differential diagnosis. For the same reason, radiologists seldom include vascular diseases in the differential diagnosis, even though angiography or interventional techniques are useful in diagnosing and managing these diseases. Therefore, radiologists should be familiar with the various imaging findings caused by vascular disease in patients with hematuria and the usefulness of endovascular management in such cases.
In this article, we discuss and illustrate the CT and angiographic findings of vascular disease associated with hematuria, including iliac artery–ureteral fistula (IAUF), renal arteriovenous malformation (AVM), ruptured renal artery aneurysm, renal artery pseudoaneurysm, spontaneous isolated renal artery dissection, renal ischemia associated with aortic dissection, "nutcracker" syndrome, and retroaortic left renal vein. The prevalence of these diseases is shown in the Table. In addition, we briefly discuss the role of interventional procedures for these diseases.
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Iliac Artery–Ureteral Fistula
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IAUF is an uncommon but potentially life-threatening cause of hematuria. Approximately 90 cases of IAUF have been reported (1–3). Predisposing factors include pelvic or vascular surgery, extensive ureteral mobilization, an indwelling ureteral stent, and pelvic irradiation. Diagnosing IAUF is difficult, since the clinical manifestation is variable, ranging from microscopic or intermittent hematuria to life-threatening hematuria. Even when IAUF is suspected, radiologic confirmation with various imaging modalities may still be difficult.
CT scans are usually negative or nonspecific for IAUF because they rarely show bleeding, and the fistulous communications are almost never seen. Unenhanced CT sometimes demonstrates a high-attenuation hematoma that flows retrograde in the collecting system, but the origin of the hemorrhage is rarely detectable. Conventional contrast material–enhanced thick-section CT often fails to demonstrate a pseudoaneurysm; however, thin-section multidetector CT may be useful in demonstrating a small pseudoaneurysm adjacent to the iliac artery, thereby helping confirm the diagnosis (Fig 1a–1c) (4). Even angiography may fail to demonstrate IAUF. Use of (a) angiography after removal of the ureteric stent and (b) reformatted images in various planes could be considered if conventional angiography has failed to demonstrate the cause of hematuria (Fig 1d).
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Figure 1a. IAUF in a 78-year-old man who underwent radical cystectomy and ureterocutaneostomy for bladder cancer. Pulsatile bleeding occurred from the skin-ureteral fistula. (a–c) Axial (a) and oblique sagittal (b) contrast-enhanced multidetector CT scans and coronal reformatted CT image (c) obtained before treatment show a left common iliac artery pseudoaneurysm (arrow) near the ureteral stent (arrowheads). (d) Aortogram obtained before treatment also shows the pseudoaneurysm (arrow). (e) Aortogram obtained after placement of a stent-graft (arrowheads) helps confirm complete exclusion of the pseudoaneurysm. (f) Contrast-enhanced CT scan shows no evidence of endoleak and helps confirm the preservation of blood flow inside the stent.
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Figure 1b. IAUF in a 78-year-old man who underwent radical cystectomy and ureterocutaneostomy for bladder cancer. Pulsatile bleeding occurred from the skin-ureteral fistula. (a–c) Axial (a) and oblique sagittal (b) contrast-enhanced multidetector CT scans and coronal reformatted CT image (c) obtained before treatment show a left common iliac artery pseudoaneurysm (arrow) near the ureteral stent (arrowheads). (d) Aortogram obtained before treatment also shows the pseudoaneurysm (arrow). (e) Aortogram obtained after placement of a stent-graft (arrowheads) helps confirm complete exclusion of the pseudoaneurysm. (f) Contrast-enhanced CT scan shows no evidence of endoleak and helps confirm the preservation of blood flow inside the stent.
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Figure 1c. IAUF in a 78-year-old man who underwent radical cystectomy and ureterocutaneostomy for bladder cancer. Pulsatile bleeding occurred from the skin-ureteral fistula. (a–c) Axial (a) and oblique sagittal (b) contrast-enhanced multidetector CT scans and coronal reformatted CT image (c) obtained before treatment show a left common iliac artery pseudoaneurysm (arrow) near the ureteral stent (arrowheads). (d) Aortogram obtained before treatment also shows the pseudoaneurysm (arrow). (e) Aortogram obtained after placement of a stent-graft (arrowheads) helps confirm complete exclusion of the pseudoaneurysm. (f) Contrast-enhanced CT scan shows no evidence of endoleak and helps confirm the preservation of blood flow inside the stent.
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Figure 1d. IAUF in a 78-year-old man who underwent radical cystectomy and ureterocutaneostomy for bladder cancer. Pulsatile bleeding occurred from the skin-ureteral fistula. (a–c) Axial (a) and oblique sagittal (b) contrast-enhanced multidetector CT scans and coronal reformatted CT image (c) obtained before treatment show a left common iliac artery pseudoaneurysm (arrow) near the ureteral stent (arrowheads). (d) Aortogram obtained before treatment also shows the pseudoaneurysm (arrow). (e) Aortogram obtained after placement of a stent-graft (arrowheads) helps confirm complete exclusion of the pseudoaneurysm. (f) Contrast-enhanced CT scan shows no evidence of endoleak and helps confirm the preservation of blood flow inside the stent.
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Figure 1e. IAUF in a 78-year-old man who underwent radical cystectomy and ureterocutaneostomy for bladder cancer. Pulsatile bleeding occurred from the skin-ureteral fistula. (a–c) Axial (a) and oblique sagittal (b) contrast-enhanced multidetector CT scans and coronal reformatted CT image (c) obtained before treatment show a left common iliac artery pseudoaneurysm (arrow) near the ureteral stent (arrowheads). (d) Aortogram obtained before treatment also shows the pseudoaneurysm (arrow). (e) Aortogram obtained after placement of a stent-graft (arrowheads) helps confirm complete exclusion of the pseudoaneurysm. (f) Contrast-enhanced CT scan shows no evidence of endoleak and helps confirm the preservation of blood flow inside the stent.
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Figure 1f. IAUF in a 78-year-old man who underwent radical cystectomy and ureterocutaneostomy for bladder cancer. Pulsatile bleeding occurred from the skin-ureteral fistula. (a–c) Axial (a) and oblique sagittal (b) contrast-enhanced multidetector CT scans and coronal reformatted CT image (c) obtained before treatment show a left common iliac artery pseudoaneurysm (arrow) near the ureteral stent (arrowheads). (d) Aortogram obtained before treatment also shows the pseudoaneurysm (arrow). (e) Aortogram obtained after placement of a stent-graft (arrowheads) helps confirm complete exclusion of the pseudoaneurysm. (f) Contrast-enhanced CT scan shows no evidence of endoleak and helps confirm the preservation of blood flow inside the stent.
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Conventional treatment for IAUF involves exclusion or ligation (1,5); however, surgical repair of the involved vessel is associated with high mortality rates, particularly in high-risk groups. Regarding nonsurgical treatment for IAUF, Keller et al (6) reported the successful use of angiographic occlusion techniques incorporating coil embolization, although the risk of potential leg ischemia remains. The use of autologous vein–covered stents and "homemade" polytetrafluoroethylene- or dacron-covered stents (Fig 1e, 1f) has also been reported (2,3). Stent-graft treatment of infected sites is controversial because of the risk of delayed infection (7,8). Before stent-graft placement, laboratory findings such as C-reactive protein level and leukocyte count should be examined and several treatment options discussed in evaluating the risk of infection at the fistula.
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Renal AVM
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The prevalence of congenital renal AVM is less than 0.04% (9). Congenital renal AVM consists of multiple irregular vessels without an associated elastic component. Congenital renal AVMs of the kidney are sometimes called cirsoid AVMs. These tortuous, varixlike vessels are immediately beneath the urothelium and lead to hematuria as the presenting finding in up to 72% of cases (10). They account for slightly less than one-quarter of all renal AVMs (11). In contrast, acquired lesions are aneurysmal, typically with a solitary communication between the artery and vein. They account for almost three-quarters of all renal AVMs (12) and have been associated with renal biopsy (the most common cause), other renal surgical procedures (eg, nephrectomy, heminephrectomy, nephrolithotomy), trauma (usually penetrating), and malignant tumors. Idiopathic lesions are also aneurysmal, with a single cavernous channel and well-defined arterial and venous elements. They account for 3%–5% of all renal AVMs (12). Acquired and idiopathic l esions cause increased venous return and high cardiac output, and are more likely to result in cardiovascular signs and symptoms. Clinical symptoms include hematuria, systolic hypertension, and abdominal pain (13), which usually manifest after the 4th decade of life.
Multidetector CT is useful in evaluating renal AVM and fistula. Unenhanced scans show only renal caliceal or pelvic hemorrhage and cortical atrophy. On early phase scans obtained with the bolus injection technique, renal AVM is clearly seen as a vascular-attenuation mass located in the renal sinus and surrounding the pelvicaliceal system (Fig 2a). Delayed phase CT scans commonly show renal AVM to have the same attenuation as the inferior vena cava. In addition, the renal and left gonadal veins are often dilated; however, definition of the communicating renal artery and draining vein is often poor (14).
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Figure 2a. Renal AVM in a 72-year-old woman who presented with gross hematuria and left flank pain. (a) Sagittal reformatted CT image demonstrates abnormal vessels in the left kidney (arrows). (b) Left renal arteriogram reveals a cirsoid AVM approximately 3 cm in diameter (arrows). (c) Left renal arteriogram shows the selection of the main feeding artery with use of a microcatheter (arrow). (d) Left renal arteriogram obtained after alcohol ablation shows total occlusion of the AVM.
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Figure 2b. Renal AVM in a 72-year-old woman who presented with gross hematuria and left flank pain. (a) Sagittal reformatted CT image demonstrates abnormal vessels in the left kidney (arrows). (b) Left renal arteriogram reveals a cirsoid AVM approximately 3 cm in diameter (arrows). (c) Left renal arteriogram shows the selection of the main feeding artery with use of a microcatheter (arrow). (d) Left renal arteriogram obtained after alcohol ablation shows total occlusion of the AVM.
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Figure 2c. Renal AVM in a 72-year-old woman who presented with gross hematuria and left flank pain. (a) Sagittal reformatted CT image demonstrates abnormal vessels in the left kidney (arrows). (b) Left renal arteriogram reveals a cirsoid AVM approximately 3 cm in diameter (arrows). (c) Left renal arteriogram shows the selection of the main feeding artery with use of a microcatheter (arrow). (d) Left renal arteriogram obtained after alcohol ablation shows total occlusion of the AVM.
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Figure 2d. Renal AVM in a 72-year-old woman who presented with gross hematuria and left flank pain. (a) Sagittal reformatted CT image demonstrates abnormal vessels in the left kidney (arrows). (b) Left renal arteriogram reveals a cirsoid AVM approximately 3 cm in diameter (arrows). (c) Left renal arteriogram shows the selection of the main feeding artery with use of a microcatheter (arrow). (d) Left renal arteriogram obtained after alcohol ablation shows total occlusion of the AVM.
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Color Doppler US is the first-line imaging procedure for renal AVM because of its low cost, less invasive nature, and wide availability. Color Doppler US is reported to have successfully demonstrated a small, mosaiclike vascular area with posterior color spots (representing tissue vibration) in a patient with renal AVM (15). Yokoyama and Tsuji (16) concluded that color Doppler US is excellent for demonstrating turbulent blood flow within the kidney. Takebayashi et al (17) reported that color Doppler US revealed a small peripheral malformation that was indistinct at selective angiography; however, they concluded that it was difficult to distinguish arteriovenous fistula from aneurysm at color Doppler US, and that flow in normal vessels grouped in the renal hilum obscures the lighter-colored flow in a small central renal AVM.
Magnetic resonance (MR) angiography has demonstrated major feeding vessels and multiple intralesional vessels in high-flow lesions, features that are absent in low-flow lesions (18). Although there have been few reports of the use of MR angiography for renal AVM, the modality is considered to be less invasive than angiography in this setting; however, the small feeding vessels of a renal AVM are not clearly identified at MR angiography. Furthermore, priority should be given to color Doppler US or CT because of the relatively high cost and poor temporal resolution of MR angiography.
Although they might be helpful, diagnostic tools such as multidetector CT, color Doppler US, and MR angiography are not considered sufficient to confirm a diagnosis of renal AVM. Angiography remains the standard of reference for confirming or excluding the existence of renal AVM and is also useful prior to embolization (Figs 2b, 2c, 3a, 3b).
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Figure 3a. Renal AVM in a 39-year-old woman who presented with gross hematuria and right flank pain. (a) Right renal arteriogram reveals a cirsoid AVM approximately 2 cm in diameter (arrow). (b) Right renal arteriogram shows embolization of the two main feeding arteries (arrows) with absolute ethanol. (c) Right renal arteriogram obtained after alcohol ablation shows total occlusion of the AVM.
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Figure 3b. Renal AVM in a 39-year-old woman who presented with gross hematuria and right flank pain. (a) Right renal arteriogram reveals a cirsoid AVM approximately 2 cm in diameter (arrow). (b) Right renal arteriogram shows embolization of the two main feeding arteries (arrows) with absolute ethanol. (c) Right renal arteriogram obtained after alcohol ablation shows total occlusion of the AVM.
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Figure 3c. Renal AVM in a 39-year-old woman who presented with gross hematuria and right flank pain. (a) Right renal arteriogram reveals a cirsoid AVM approximately 2 cm in diameter (arrow). (b) Right renal arteriogram shows embolization of the two main feeding arteries (arrows) with absolute ethanol. (c) Right renal arteriogram obtained after alcohol ablation shows total occlusion of the AVM.
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Embolization is considered a primary treatment option in cases of renal AVM because it preserves maximal unaffected normal renal parenchyma while eliminating the risk of recurrent hemorrhage. The goal of AVM embolization is eradication of the nidus, where the artery and vein communicate (19). Recent reports describe the ablation of feeding vessels with various embolic agents, including absolute ethanol (Figs 2d, 3c) (20), polyvinyl alcohol, metallic coils, and n- butyl-2-cyanoacrylate (21). AVM radiofrequency ablation has also been attempted (22).
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Ruptured Renal Artery Aneurysm
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The prevalence of renal artery aneurysm at angiography varies from 0.3% (23) to 0.7% (24). The natural history of renal artery aneurysm is poorly documented. Although rupture is considered to be extremely rare (25), several risk factors have been reported, such as pregnancy, size greater than 1.5 cm, and noncalcified saccular morphologic features (26,27). Rupture of a renal artery aneurysm usually causes the flank pain or abdominal pain associated with hematuria (28,29).
A contrast-enhanced arterial phase CT scan demonstrates a massive perinephric hematoma (Fig 4a) that extends into the anterior or posterior pararenal space and continues caudally into the pelvis. A ruptured aneurysm and extravasation manifest as a highly enhanced lesion in the hematoma. A greater degree of extravasation extends into the surrounding tissue on delayed phase scans than on arterial phase scans.
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Figure 4a. Ruptured renal artery aneurysm in a 68-year-old man who presented with microhematuria and sudden left flank pain. CT demonstrated massive hemorrhage around the left kidney, and the patient lapsed into hemorrhagic shock. (a) Contrast-enhanced arterial phase CT scan reveals a massive hematoma with extravasation (arrow). (b) Left renal arteriogram obtained during treatment shows a ruptured renal artery aneurysm (arrow). (c) Selective angiogram of the renal artery shows the aneurysm and extravasation. (d) Angiogram shows successful coil embolization of the feeding artery with four Tornado microcoils (Cook, Bloomington, Ind). Two coils were 4 mm in diameter and two were 3 mm in diameter. (Case courtesy of Toru Yamamoto, MD, PhD, and Jun Yoshikawa, MD, PhD, Fukui Prefectural Hospital, Fukui, Japan.)
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Figure 4b. Ruptured renal artery aneurysm in a 68-year-old man who presented with microhematuria and sudden left flank pain. CT demonstrated massive hemorrhage around the left kidney, and the patient lapsed into hemorrhagic shock. (a) Contrast-enhanced arterial phase CT scan reveals a massive hematoma with extravasation (arrow). (b) Left renal arteriogram obtained during treatment shows a ruptured renal artery aneurysm (arrow). (c) Selective angiogram of the renal artery shows the aneurysm and extravasation. (d) Angiogram shows successful coil embolization of the feeding artery with four Tornado microcoils (Cook, Bloomington, Ind). Two coils were 4 mm in diameter and two were 3 mm in diameter. (Case courtesy of Toru Yamamoto, MD, PhD, and Jun Yoshikawa, MD, PhD, Fukui Prefectural Hospital, Fukui, Japan.)
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Figure 4c. Ruptured renal artery aneurysm in a 68-year-old man who presented with microhematuria and sudden left flank pain. CT demonstrated massive hemorrhage around the left kidney, and the patient lapsed into hemorrhagic shock. (a) Contrast-enhanced arterial phase CT scan reveals a massive hematoma with extravasation (arrow). (b) Left renal arteriogram obtained during treatment shows a ruptured renal artery aneurysm (arrow). (c) Selective angiogram of the renal artery shows the aneurysm and extravasation. (d) Angiogram shows successful coil embolization of the feeding artery with four Tornado microcoils (Cook, Bloomington, Ind). Two coils were 4 mm in diameter and two were 3 mm in diameter. (Case courtesy of Toru Yamamoto, MD, PhD, and Jun Yoshikawa, MD, PhD, Fukui Prefectural Hospital, Fukui, Japan.)
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Figure 4d. Ruptured renal artery aneurysm in a 68-year-old man who presented with microhematuria and sudden left flank pain. CT demonstrated massive hemorrhage around the left kidney, and the patient lapsed into hemorrhagic shock. (a) Contrast-enhanced arterial phase CT scan reveals a massive hematoma with extravasation (arrow). (b) Left renal arteriogram obtained during treatment shows a ruptured renal artery aneurysm (arrow). (c) Selective angiogram of the renal artery shows the aneurysm and extravasation. (d) Angiogram shows successful coil embolization of the feeding artery with four Tornado microcoils (Cook, Bloomington, Ind). Two coils were 4 mm in diameter and two were 3 mm in diameter. (Case courtesy of Toru Yamamoto, MD, PhD, and Jun Yoshikawa, MD, PhD, Fukui Prefectural Hospital, Fukui, Japan.)
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Renal artery angiography is the diagnostic modality of choice for clearly depicting an aneurysm and extravasation (Fig 4b, 4c); however, angiography is usually not necessary to confirm the diagnosis. Surgical repair is usually selected for the treatment of ruptured renal artery aneurysm, but endovascular treatment with embolic material (Fig 4d) has also recently been reported (28).
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Renal Artery Pseudoaneurysm
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Renal artery pseudoaneurysm is a recognized complication of percutaneous renal procedures (biopsy and nephrostomy) (Fig 5a), open or laparoscopic partial nephrostomy, and renal trauma (30). The prevalence of renal artery pseudoaneurysm following open partial nephrectomy is less than 0.43% (31). Renal artery pseudoaneurysm commonly causes hematuria within 4 weeks after intervention (29). Farrell et al (32) reported that hematuria results from erosion of the pseudoaneurysm into the adjacent renal collecting system. The possibility of renal artery pseudoaneurysm should be considered in any patient who develops flank pain and hematuria after renal intervention.
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Figure 5a. Renal artery pseudoaneurysm in a 63-year-old man with staghorn calculi in the left renal pelvis. Pulsatile bleeding occurred following nephrostomy for treatment access. (a) Contrast-enhanced CT scan shows a nephrostomy tube in the renal pelvis (arrow), along with extravasation (arrowheads). (b) Left renal angiogram obtained during intervention shows a renal artery pseudoaneurysm (arrow). (c) Selective angiogram helps identify the feeding vessel (arrowheads) and pseudoaneurysm (arrow). (d) Angiogram obtained after coil embolization with two Tornado microcoils (Cook) 3 mm in diameter and a straight 10-cm liquid coil (Berenstein; Boston Scientific/Target Therapeutics, Fremont, Calif) 0.016 inch in diameter helps confirm exclusion of the pseudoaneurysm. (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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Figure 5b. Renal artery pseudoaneurysm in a 63-year-old man with staghorn calculi in the left renal pelvis. Pulsatile bleeding occurred following nephrostomy for treatment access. (a) Contrast-enhanced CT scan shows a nephrostomy tube in the renal pelvis (arrow), along with extravasation (arrowheads). (b) Left renal angiogram obtained during intervention shows a renal artery pseudoaneurysm (arrow). (c) Selective angiogram helps identify the feeding vessel (arrowheads) and pseudoaneurysm (arrow). (d) Angiogram obtained after coil embolization with two Tornado microcoils (Cook) 3 mm in diameter and a straight 10-cm liquid coil (Berenstein; Boston Scientific/Target Therapeutics, Fremont, Calif) 0.016 inch in diameter helps confirm exclusion of the pseudoaneurysm. (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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Figure 5c. Renal artery pseudoaneurysm in a 63-year-old man with staghorn calculi in the left renal pelvis. Pulsatile bleeding occurred following nephrostomy for treatment access. (a) Contrast-enhanced CT scan shows a nephrostomy tube in the renal pelvis (arrow), along with extravasation (arrowheads). (b) Left renal angiogram obtained during intervention shows a renal artery pseudoaneurysm (arrow). (c) Selective angiogram helps identify the feeding vessel (arrowheads) and pseudoaneurysm (arrow). (d) Angiogram obtained after coil embolization with two Tornado microcoils (Cook) 3 mm in diameter and a straight 10-cm liquid coil (Berenstein; Boston Scientific/Target Therapeutics, Fremont, Calif) 0.016 inch in diameter helps confirm exclusion of the pseudoaneurysm. (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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Figure 5d. Renal artery pseudoaneurysm in a 63-year-old man with staghorn calculi in the left renal pelvis. Pulsatile bleeding occurred following nephrostomy for treatment access. (a) Contrast-enhanced CT scan shows a nephrostomy tube in the renal pelvis (arrow), along with extravasation (arrowheads). (b) Left renal angiogram obtained during intervention shows a renal artery pseudoaneurysm (arrow). (c) Selective angiogram helps identify the feeding vessel (arrowheads) and pseudoaneurysm (arrow). (d) Angiogram obtained after coil embolization with two Tornado microcoils (Cook) 3 mm in diameter and a straight 10-cm liquid coil (Berenstein; Boston Scientific/Target Therapeutics, Fremont, Calif) 0.016 inch in diameter helps confirm exclusion of the pseudoaneurysm. (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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Imaging findings are similar to those in ruptured renal artery aneurysm and include a dense collection of contrast material within the renal hilum, perinephric hemorrhage, and a slightly delayed nephrogram relative to the contralateral kidney. Although differentiating between renal artery pseudoaneurysm and ruptured renal artery aneurysm may be difficult at diagnostic imaging (28), it is less difficult clinically because renal artery pseudoaneurysm usually occurs after renal intervention (Fig 5b, 5c).
Selective embolization with an endovascular technique is the preferred therapy for hemodynamically stable patients with renal artery pseudoaneurysm (Fig 5d), whereas surgical intervention is usually necessary for hemodynamically unstable patients (31,33).
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Spontaneous Isolated Renal Artery Dissection
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Renal artery dissection is not usually isolated, manifesting instead as an extension of aortic dissection. However, isolated renal artery dissection may occur after percutaneous angioplasty or blunt trauma. By definition, spontaneous isolated renal artery dissection is not associated with any intervention or trauma. Although almost 200 cases of spontaneous isolated renal artery dissection have been reported in the literature, the frequency of occurrence of this entity is considered to be low. In a large arteriographic series, spontaneous isolated renal artery dissection was encountered in less than 0.05% of all patients (34). However, its true prevalence may be higher, since spontaneous regressions of this entity are known to have occurred (35,36).
The cause of spontaneous isolated renal artery dissection is not well understood. Such dissection is known to be associated with several vascular diseases, including fibromuscular dysplasia (37,38), malignant hypertension, severe atherosclerosis (39), Marfan syndrome, and Ehlers-Danlos syndrome. Isolated dissection in the segmental arteries in men can be due to a variant of fibromuscular dysplasia (40). In contrast, spontaneous isolated renal artery dissection (male-to-female ratio, 4:1) is reported to have occurred in healthy men without any vascular disease in the 4th to 6th decades of life (34).
The clinical manifestations of spontaneous isolated renal artery dissection include lumbar or flank pain (77% of cases), hypertension (~100%), gross hematuria (18%), and impaired renal function (9%) (41). Clinical diagnosis is usually difficult because of the vague nature of the symptoms and the rarity of the condition; therefore, it is important for radiologists to be familiar with the imaging findings of spontaneous isolated renal artery dissection.
Spontaneous isolated renal artery dissection is diagnosed on the basis of the detection of an intimal flap in the renal artery with various imaging modalities. The recent development of multidetector CT has made this detection much easier on arterial phase thin-section multidetector CT scans (Fig 6a). The presence of renal infarction may help radiologists diagnose spontaneous isolated renal artery dissection, since renal infarction is often associated with renal artery dissection (42,43).
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Figure 6a. Spontaneous isolated renal artery dissection in a 35-year-old man who presented with acute onset of left flank pain, microhematuria, and hypertension (180/100 mm Hg). (a) Contrast-enhanced arterial phase thin-section multidetector CT scan shows an intimal flap in the left renal artery (arrow). (b) Posteroanterior left renal arteriogram shows the original proximal stenosis and the intimal flap that subsequently developed (arrow). (c) Anterior oblique left renal arteriogram shows false lumen enhancement (arrow) and subsequent true lumen enhancement. (d) Anterior oblique left renal arteriogram shows dissection into the segmental branch with occlusion of the middle and lower segmental branches (arrows). (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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Figure 6b. Spontaneous isolated renal artery dissection in a 35-year-old man who presented with acute onset of left flank pain, microhematuria, and hypertension (180/100 mm Hg). (a) Contrast-enhanced arterial phase thin-section multidetector CT scan shows an intimal flap in the left renal artery (arrow). (b) Posteroanterior left renal arteriogram shows the original proximal stenosis and the intimal flap that subsequently developed (arrow). (c) Anterior oblique left renal arteriogram shows false lumen enhancement (arrow) and subsequent true lumen enhancement. (d) Anterior oblique left renal arteriogram shows dissection into the segmental branch with occlusion of the middle and lower segmental branches (arrows). (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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Figure 6c. Spontaneous isolated renal artery dissection in a 35-year-old man who presented with acute onset of left flank pain, microhematuria, and hypertension (180/100 mm Hg). (a) Contrast-enhanced arterial phase thin-section multidetector CT scan shows an intimal flap in the left renal artery (arrow). (b) Posteroanterior left renal arteriogram shows the original proximal stenosis and the intimal flap that subsequently developed (arrow). (c) Anterior oblique left renal arteriogram shows false lumen enhancement (arrow) and subsequent true lumen enhancement. (d) Anterior oblique left renal arteriogram shows dissection into the segmental branch with occlusion of the middle and lower segmental branches (arrows). (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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Figure 6d. Spontaneous isolated renal artery dissection in a 35-year-old man who presented with acute onset of left flank pain, microhematuria, and hypertension (180/100 mm Hg). (a) Contrast-enhanced arterial phase thin-section multidetector CT scan shows an intimal flap in the left renal artery (arrow). (b) Posteroanterior left renal arteriogram shows the original proximal stenosis and the intimal flap that subsequently developed (arrow). (c) Anterior oblique left renal arteriogram shows false lumen enhancement (arrow) and subsequent true lumen enhancement. (d) Anterior oblique left renal arteriogram shows dissection into the segmental branch with occlusion of the middle and lower segmental branches (arrows). (Case courtesy of Kazuyuki Kinoshita, MD, and Tadashi Sagoh, MD, PhD, Fukui Red Cross Hospital, Fukui, Japan.)
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The role of angiography in the diagnosis of spontaneous isolated renal artery dissection is controversial. We believe that angiography is not always necessary for detecting the intimal flap (Fig 6b–6d); however, angiography can demonstrate multifocal stenoses or microaneurysms in small arteries and can help evaluate potential vascular diseases (eg, fibromuscular dysplasia) associated with spontaneous isolated renal artery dissection.
The treatment policy for spontaneous isolated renal artery dissection also remains controversial. Some investigators have found medical therapy to be as effective as surgery in controlling hypertension (43), whereas others have argued for more aggressive surgical revascularization to preserve renal parenchyma and in patients who are unresponsive to medical therapy (36,41). Other studies have reported that nephrectomy was still required in 30%–40% of cases and that medical treatment for hypertension was required after surgical intervention in approximately one-half of patients (35,41). A percutaneous interventional procedure for spontaneous isolated renal artery dissection has not been reported, except for one case of endovascular embolization for accessory renal artery dissection (44).
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Renal Ischemia Associated with Aortic Dissection
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Aortic dissection is a potentially life-threatening condition and is known to possibly involve the renal artery. Jenq et al (45) reported that renal artery involvement should be considered when hematuria, pyuria, and deterioration of renal function occur following aortic dissection. More than one-third of patients with aortic dissection have various symptoms that indicate involvement of the visceral or iliac arterial branches (46). Because morbidity and mortality rates increase with involvement of branch vessels such as the renal artery in aortic dissection (47), it is important to evaluate the entire aorta, determine the distal extent of the dissection, and detect any systemic involvement. Renal ischemia with aortic dissection is usually caused by extension of dissection from the aorta to the renal artery. It has also been reported that renal ischemia can result from true lumen obliteration with aortic dissection, without involvement of the renal artery itself (48). Therefore, radiologists should focus not only on the renal artery but also on the morphologic features of the aorta itself when the patient presents with signs or symptoms (eg, hematuria) that indicate renal ischemia associated with aortic dissection.
Cross-sectional imaging with multidetector CT is currently the imaging technique of choice for the diagnosis and evaluation of aortic dissection, with a sensitivity and specificity of nearly 100% (46,47). CT is more sensitive than catheter aortography in diagnosing aortic dissection (49) and is comparable to MR imaging and transesophageal echocardiography in this setting (50). The main finding at contrast-enhanced CT is an intimal flap that separates the true lumen from the false lumen. The CT appearance of renal artery involvement in aortic dissection is the same as that of isolated renal artery dissection (Fig 7a), but it is important to determine whether the renal artery has received blood flow from a true lumen or a false lumen.
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Figure 7a. Renal ischemia associated with aortic dissection in a 63-year-old man who presented with right-sided back pain and hematuria following a traffic accident. Thoracic and abdominal CT demonstrated type B aortic dissections with left renal artery dissection. One month after the accident, urine output was reduced and renal function deteriorated. Endovascular exclusion of the primary tear in the thoracic aorta with a stent-graft was planned. (a) Contrast-enhanced CT scan shows renal artery dissection and segmental renal infarction (arrow). (b) Aortogram obtained in the true lumen before treatment shows only the stump of the left renal artery (arrow), with no renal parenchymal enhancement. (c) Thoracic aortic angiogram obtained after treatment shows contrast material filling the false lumen (black arrows) via an entry site at the aortic arch (white arrow). (d) Aortogram obtained after placement of a stent-graft at the aortic arch shows revascularization of the left renal artery (arrows), which improved the perfusion of the true lumen.
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Figure 7b. Renal ischemia associated with aortic dissection in a 63-year-old man who presented with right-sided back pain and hematuria following a traffic accident. Thoracic and abdominal CT demonstrated type B aortic dissections with left renal artery dissection. One month after the accident, urine output was reduced and renal function deteriorated. Endovascular exclusion of the primary tear in the thoracic aorta with a stent-graft was planned. (a) Contrast-enhanced CT scan shows renal artery dissection and segmental renal infarction (arrow). (b) Aortogram obtained in the true lumen before treatment shows only the stump of the left renal artery (arrow), with no renal parenchymal enhancement. (c) Thoracic aortic angiogram obtained after treatment shows contrast material filling the false lumen (black arrows) via an entry site at the aortic arch (white arrow). (d) Aortogram obtained after placement of a stent-graft at the aortic arch shows revascularization of the left renal artery (arrows), which improved the perfusion of the true lumen.
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Figure 7c. Renal ischemia associated with aortic dissection in a 63-year-old man who presented with right-sided back pain and hematuria following a traffic accident. Thoracic and abdominal CT demonstrated type B aortic dissections with left renal artery dissection. One month after the accident, urine output was reduced and renal function deteriorated. Endovascular exclusion of the primary tear in the thoracic aorta with a stent-graft was planned. (a) Contrast-enhanced CT scan shows renal artery dissection and segmental renal infarction (arrow). (b) Aortogram obtained in the true lumen before treatment shows only the stump of the left renal artery (arrow), with no renal parenchymal enhancement. (c) Thoracic aortic angiogram obtained after treatment shows contrast material filling the false lumen (black arrows) via an entry site at the aortic arch (white arrow). (d) Aortogram obtained after placement of a stent-graft at the aortic arch shows revascularization of the left renal artery (arrows), which improved the perfusion of the true lumen.
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Figure 7d. Renal ischemia associated with aortic dissection in a 63-year-old man who presented with right-sided back pain and hematuria following a traffic accident. Thoracic and abdominal CT demonstrated type B aortic dissections with left renal artery dissection. One month after the accident, urine output was reduced and renal function deteriorated. Endovascular exclusion of the primary tear in the thoracic aorta with a stent-graft was planned. (a) Contrast-enhanced CT scan shows renal artery dissection and segmental renal infarction (arrow). (b) Aortogram obtained in the true lumen before treatment shows only the stump of the left renal artery (arrow), with no renal parenchymal enhancement. (c) Thoracic aortic angiogram obtained after treatment shows contrast material filling the false lumen (black arrows) via an entry site at the aortic arch (white arrow). (d) Aortogram obtained after placement of a stent-graft at the aortic arch shows revascularization of the left renal artery (arrows), which improved the perfusion of the true lumen.
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The efficacy of stent-graft repair in both acute and chronic aortic dissection is now widely accepted (51,52). In most cases, entry closure with endovascular stent-graft placement improves the collapsed true lumen, bowel ischemia, lower extremity ischemia, and renal ischemia (Fig 7b–7d); in some patients, however, the aortic branches are supplied solely by the false lumen. In chronic dissection, the intimal flap may fuse to the opposite wall of the vessel if reentry develops in a branch vessel. As a result, the branch becomes totally dependent on the blood flow from the false lumen. Special considerations, including creation of a double-barreled aorta below the site of stent-graft placement, are required in such cases to avoid ischemic complications when surgical intervention is performed. Similar techniques should be considered mandatory when endovascular procedures are used. Percutaneous fenestration is mainly performed in cases of dynamic involvement of the aortic branches; its safety and efficacy have been demonstrated in several studies (48).
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Nutcracker Syndrome
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Nutcracker syndrome is defined as compression of the left renal vein between the superior mesenteric artery and the aorta and is associated with urinary abnormalities (Fig 8a). It has been postulated that the mechanism that produces the hematuria is an increase in left renal vein pressure, which may cause minute rupture of thin-walled veins into the collecting system (53). Hematuria and flank pain can also cause renal vein thrombosis (54).
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Figure 8a. Nutcracker syndrome in an 84-year-old woman with hematuria. The patient’s condition improved with intravenous infusion of hemostat and better nutrition. (a) Contrast-enhanced CT scan shows stenosis of the left renal vein (arrow) between the superior mesenteric artery and the aorta. (b) Venous phase left renal angiogram shows massive reflux in the left gonadal vein.
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Figure 8b. Nutcracker syndrome in an 84-year-old woman with hematuria. The patient’s condition improved with intravenous infusion of hemostat and better nutrition. (a) Contrast-enhanced CT scan shows stenosis of the left renal vein (arrow) between the superior mesenteric artery and the aorta. (b) Venous phase left renal angiogram shows massive reflux in the left gonadal vein.
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Doppler US, multidetector CT, MR imaging, and angiography all demonstrate left renal vein stenosis with proximal distention and the presence of collateral pathways. The main collateral pathway is the left gonadal vein, which displays early enhancement during the portal phase at angiography, MR imaging, or contrast-enhanced CT (Fig 8b). The diagnostic criterion is a pressure gradient greater than 3 mm Hg as measured with the pullback method at renal venography (55).
In nutcracker syndrome, the elevated pressure gradient is accompanied by repeated bouts of gross hematuria and left flank pain or repeated documentation of gross hematuria from the left ureteral orifice only. This is a very important feature because many asymptomatic patients have a narrowed vein without an elevated pressure gradient but lack any gross hematuria or left flank pain.
The treatment is chosen when certain clinical signs and symptoms (left flank pain, hematuria) are manifested. Left renal vein transposition, superior mesenteric artery transposition, and nephrectomy have all been reported as surgical repair methods (56). The surgery for nutcracker syndrome is not inconsequential, and it is important to link the objective radiologic findings with the critical clinical findings before performing surgery.
Endovascular treatment for this syndrome with stent-graft placement has recently been reported (57,58).
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Retroaortic Left Renal Vein
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Retroaortic left renal vein (Fig 9a) is one of the most common congenital anomalies and may cause left renal vein hypertension according to the criteria for nutcracker syndrome (59). The anatomy and embryogenesis of this anomaly are well described in the literature (60,61). In an autopsy series, retroaortic left renal vein was seen in 1.5%–3.4% of cases, with circumaortic left renal vein being seen in 1.8%–16.8% (61). Knowledge of the anomalies of the left renal vein is important for radiologists who perform renal or adrenal venography and venous sampling. These vascular anomalies can be mistaken for lymphadenopathy at CT (62). Few patients with these anomalies have clinical symptoms.
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Figure 9a. Retroaortic left renal vein in a 57-year-old woman with hematuria. The patient’s condition improved with intravenous infusion of hemostat and better nutrition. (a) Contrast-enhanced CT scan shows stenosis of the left renal vein (arrow) between the abdominal aorta and a vertebral body. (b) Contrast-enhanced portal phase CT scan shows early enhancement of the left gonadal vein (arrow).
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Figure 9b. Retroaortic left renal vein in a 57-year-old woman with hematuria. The patient’s condition improved with intravenous infusion of hemostat and better nutrition. (a) Contrast-enhanced CT scan shows stenosis of the left renal vein (arrow) between the abdominal aorta and a vertebral body. (b) Contrast-enhanced portal phase CT scan shows early enhancement of the left gonadal vein (arrow).
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As in nutcracker syndrome, the main collateral pathway is the left gonadal vein (Fig 9b), and the diagnostic criterion is a pressure gradient greater than 3 mm Hg as measured with the pullback method at renal venography (55).
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Conclusions
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In rare cases, hematuria is caused by life-threatening vascular diseases. Therefore, radiologists should be familiar with the various imaging findings of hematuria caused by vascular disease. Multidetector CT performed with the bolus injection technique should be the first-line diagnostic test when vascular disease is suspected. Radiologists should also be familiar with the management policy (including endovascular techniques) for hematuria caused by vascular disease, since some of these patients can be treated with interventional procedures.
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Footnotes
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Abbreviations: AVM = arteriovenous malformation, IAUF = iliac artery-ureteral fistula
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Abstract
LEARNING OBJECTIVES
