Case Report
Urologia Internationalis
Received: November 3, 2014 Accepted after revision: February 24, 2015 Published online: April 18, 2015
Urol Int DOI: 10.1159/000381270
Anterior Nutcracker Syndrome with Left Gonadal Vein Varicosities on Multiphasic Computed Tomography: An Unexpected Cause of Pyeloureteral Junction Obstruction Maddalena Di Carlo a Caterina Gaudiano a Fiorenza Busato a Simone Pucci a Riccardo Schiavina b Valerio Vagnoni b Rita Golfieri a Departments of a Radiology and b Urology, University of Bologna, Sant’Orsola-Malpighi Hospital, Bologna, Italy
Key Words Nutcracker syndrome · Left renal vein · Pyeloureteral junction obstruction · Left flank pain
Abstract The anterior nutcracker syndrome is defined by the compression of the left renal vein between the aorta and superior mesenteric artery, usually related to the occurrence of hematuria. We report the case of an uncommon complication of the nutcracker syndrome. A 75-year-old woman was referred to our institution for left flank pain without hematuria. Multiphasic computer tomography urography showed a condition of left renal vein entrapment between the aorta and superior mesenteric artery with the development of left gonadal vein varicosities at the level of the renal hilum; a pyeloureteral junction compression with dilation of the pyelocalyceal system coexisted. To our knowledge, this is the first report of the association between nutcracker syndrome and pyeloureteral junction obstruction. © 2015 S. Karger AG, Basel
Introduction
Nutcracker syndrome (NCS) refers to the compression of the left renal vein (LRV) between the aorta and superior mesenteric artery (anterior NCS) or spine (posterior NCS), with increased pressure within the LRV and development of varices of the renal pelvis and ureter [1, 2]. Clinically, this condition can be silent or involve episodes of hematuria (macro-/microscopic) [3–5]; orthostatic proteinuria has also been reported [6]. Other symptoms include flank pain and discomfort in the pelvic or scrotal area caused by a varicocele or ovarian vein congestion [6]. Some reports have suggested, in fact, that LRV congestion can cause the development of venous varicosities in the left gonadal vein bed, possibly responsible for hematuria and/or varicocele. Nevertheless, to the best of our knowledge, this is the first report in the literature of the association between anterior NCS and pyeloureteral junction obstruction.
Case Report
© 2015 S. Karger AG, Basel 0042–1138/15/0000–0000$39.50/0 E-Mail [email protected] www.karger.com/uin
Maddalena Di Carlo, MD Department of Radiology, Sant’Orsola-Malpighi Hospital Via Albertoni 15 IT–40138 Bologna (Italy) E-Mail magda.dicarlo @ hotmail.it
Downloaded by: NYU Medical Center Library 198.143.38.1 - 7/26/2015 10:46:20 PM
A 75-year-old woman with slight left flank pain without hematuria was referred to our institution. Her blood test revealed a normal level of serum creatinine and no leukocytosis. Urinalysis showed
Color version available online
a
b
Fig. 2. Computed tomography: the multiplanar reconstruction of
Fig. 3. Computed tomography: the multiplanar reconstruction of
the nephrographic phase in coronal oblique plane shows large collateral varices along the left gonadal vein directed to the internal iliac vein (arrows). The LRV is not dilated (arrowhead).
the nephrographic phase in axial oblique plane shows the thin LRV compressed between the aorta and the superior mesenteric artery (arrow).
no microhematuria or bacterial infection. An abdominal ultrasonography, performed as a screening test, demonstrated moderate dilation of the left pyelocalyceal system without dilation of the ureter and a dysplasia of the pyeloureteral junction was suspected. Multiphasic computed tomography (CT) with angiographic study and excretory phase was carried out in order to better evaluate this condition and define the preoperative planning. CT confirmed the moderate dilatation of the left pyelocalyceal system with shrinkage and kinking of the pyeloureteral junction and proximal ureter (fig. 1), which were entrapped by some tortuous varicosities developing
along the course of the left gonadal vein (fig. 2). A compression of the LRV between the superior mesenteric artery and the aorta was also noted (fig. 3) and a diagnosis of the anterior NCS was made. Renal scintigraphy showed a slight decrease in the excretion rate of the left kidney (48% for the left kidney and 52% for the right one) with retention of radioactive urine in the dilated pelvis, which was responsive to the diuretic stimulus. The patient refused surgery and was subjected to a conservative approach with ultrasonographic follow-up in order to monitor the pyelocalyceal dilatation.
2
Urol Int DOI: 10.1159/000381270
Di Carlo/Gaudiano/Busato/Pucci/ Schiavina/Vagnoni/Golfieri
Downloaded by: NYU Medical Center Library 198.143.38.1 - 7/26/2015 10:46:20 PM
Fig. 1. Computed tomography. a The volume-rendering technique of the urinary excretory phase shows the kinking of the pyeloureteral junction (arrow) with dilatation of the pyelocalyceal system. b The multiplanar reconstruction of the nephrographic phase shows the close relationship between the varices and the pyeloureteral junction (arrow).
Discussion
Some authors have suggested the differentiation between the ‘nutcracker phenomenon’, related to the reduction of the angle between the aorta and the superior mesenteric artery, and the ‘nutcracker syndrome’, related to the onset of the clinical symptoms. It has been postulated, in fact, that the increased pressure within the LRV can cause the development of varices in the venous peripyelic and the periureteral bed; the rupture of the thin septa between the small varices and collecting system, especially in the calyceal fornix, can result in hematuria [3–5]. For the same reason, it can produce congestion of the gonadal vein with dilatation, venous reflux, and development of varicocele or ovarian vein congestion. The diagnosis can be difficult, often delayed, because no robust clinical diagnostic criteria exist, and most patients are diagnosed only after the exclusion of other causes of flank pain or hematuria. Ishidoya et al. [7] suggested the following conditions are required for diagnosis: (1) there is no urological disease associated with hematuria and flank pain, (2) the distance between the superior mesenteric artery and the aorta is
Urologia Internationalis
Received: November 3, 2014 Accepted after revision: February 24, 2015 Published online: April 18, 2015
Urol Int DOI: 10.1159/000381270
Anterior Nutcracker Syndrome with Left Gonadal Vein Varicosities on Multiphasic Computed Tomography: An Unexpected Cause of Pyeloureteral Junction Obstruction Maddalena Di Carlo a Caterina Gaudiano a Fiorenza Busato a Simone Pucci a Riccardo Schiavina b Valerio Vagnoni b Rita Golfieri a Departments of a Radiology and b Urology, University of Bologna, Sant’Orsola-Malpighi Hospital, Bologna, Italy
Key Words Nutcracker syndrome · Left renal vein · Pyeloureteral junction obstruction · Left flank pain
Abstract The anterior nutcracker syndrome is defined by the compression of the left renal vein between the aorta and superior mesenteric artery, usually related to the occurrence of hematuria. We report the case of an uncommon complication of the nutcracker syndrome. A 75-year-old woman was referred to our institution for left flank pain without hematuria. Multiphasic computer tomography urography showed a condition of left renal vein entrapment between the aorta and superior mesenteric artery with the development of left gonadal vein varicosities at the level of the renal hilum; a pyeloureteral junction compression with dilation of the pyelocalyceal system coexisted. To our knowledge, this is the first report of the association between nutcracker syndrome and pyeloureteral junction obstruction. © 2015 S. Karger AG, Basel
Introduction
Nutcracker syndrome (NCS) refers to the compression of the left renal vein (LRV) between the aorta and superior mesenteric artery (anterior NCS) or spine (posterior NCS), with increased pressure within the LRV and development of varices of the renal pelvis and ureter [1, 2]. Clinically, this condition can be silent or involve episodes of hematuria (macro-/microscopic) [3–5]; orthostatic proteinuria has also been reported [6]. Other symptoms include flank pain and discomfort in the pelvic or scrotal area caused by a varicocele or ovarian vein congestion [6]. Some reports have suggested, in fact, that LRV congestion can cause the development of venous varicosities in the left gonadal vein bed, possibly responsible for hematuria and/or varicocele. Nevertheless, to the best of our knowledge, this is the first report in the literature of the association between anterior NCS and pyeloureteral junction obstruction.
Case Report
© 2015 S. Karger AG, Basel 0042–1138/15/0000–0000$39.50/0 E-Mail [email protected] www.karger.com/uin
Maddalena Di Carlo, MD Department of Radiology, Sant’Orsola-Malpighi Hospital Via Albertoni 15 IT–40138 Bologna (Italy) E-Mail magda.dicarlo @ hotmail.it
Downloaded by: NYU Medical Center Library 198.143.38.1 - 7/26/2015 10:46:20 PM
A 75-year-old woman with slight left flank pain without hematuria was referred to our institution. Her blood test revealed a normal level of serum creatinine and no leukocytosis. Urinalysis showed
Color version available online
a
b
Fig. 2. Computed tomography: the multiplanar reconstruction of
Fig. 3. Computed tomography: the multiplanar reconstruction of
the nephrographic phase in coronal oblique plane shows large collateral varices along the left gonadal vein directed to the internal iliac vein (arrows). The LRV is not dilated (arrowhead).
the nephrographic phase in axial oblique plane shows the thin LRV compressed between the aorta and the superior mesenteric artery (arrow).
no microhematuria or bacterial infection. An abdominal ultrasonography, performed as a screening test, demonstrated moderate dilation of the left pyelocalyceal system without dilation of the ureter and a dysplasia of the pyeloureteral junction was suspected. Multiphasic computed tomography (CT) with angiographic study and excretory phase was carried out in order to better evaluate this condition and define the preoperative planning. CT confirmed the moderate dilatation of the left pyelocalyceal system with shrinkage and kinking of the pyeloureteral junction and proximal ureter (fig. 1), which were entrapped by some tortuous varicosities developing
along the course of the left gonadal vein (fig. 2). A compression of the LRV between the superior mesenteric artery and the aorta was also noted (fig. 3) and a diagnosis of the anterior NCS was made. Renal scintigraphy showed a slight decrease in the excretion rate of the left kidney (48% for the left kidney and 52% for the right one) with retention of radioactive urine in the dilated pelvis, which was responsive to the diuretic stimulus. The patient refused surgery and was subjected to a conservative approach with ultrasonographic follow-up in order to monitor the pyelocalyceal dilatation.
2
Urol Int DOI: 10.1159/000381270
Di Carlo/Gaudiano/Busato/Pucci/ Schiavina/Vagnoni/Golfieri
Downloaded by: NYU Medical Center Library 198.143.38.1 - 7/26/2015 10:46:20 PM
Fig. 1. Computed tomography. a The volume-rendering technique of the urinary excretory phase shows the kinking of the pyeloureteral junction (arrow) with dilatation of the pyelocalyceal system. b The multiplanar reconstruction of the nephrographic phase shows the close relationship between the varices and the pyeloureteral junction (arrow).
Discussion
Some authors have suggested the differentiation between the ‘nutcracker phenomenon’, related to the reduction of the angle between the aorta and the superior mesenteric artery, and the ‘nutcracker syndrome’, related to the onset of the clinical symptoms. It has been postulated, in fact, that the increased pressure within the LRV can cause the development of varices in the venous peripyelic and the periureteral bed; the rupture of the thin septa between the small varices and collecting system, especially in the calyceal fornix, can result in hematuria [3–5]. For the same reason, it can produce congestion of the gonadal vein with dilatation, venous reflux, and development of varicocele or ovarian vein congestion. The diagnosis can be difficult, often delayed, because no robust clinical diagnostic criteria exist, and most patients are diagnosed only after the exclusion of other causes of flank pain or hematuria. Ishidoya et al. [7] suggested the following conditions are required for diagnosis: (1) there is no urological disease associated with hematuria and flank pain, (2) the distance between the superior mesenteric artery and the aorta is
