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Diagnosis of pelviureteral duplication by ultrasonically-guided antegrade pyelography
EUROPEAN JOURNAL OF
U~VBA$@~N© ELSEVIER
European Journal of Ultrasound 2 (1995) 215-218
Clinical report
Diagnosis of pelviureteral duplication by ultrasonically-guided antegrade pyelography P e r R e j n e r t J e n s e n , a , Sture H e g e l u n d a, T o r b e n K j a e r b aDepartment of Radiology, Roskilde Hospital, 4000 Roskilde, Denmark bDepartment of Surgery, Roskilde Hospital, 4000 Roskilde, Denmark Received 3 September 1994; revision received 14 February 1995; accepted 16 February 1995
Abstract
The case of a 14-year-old boy with pelviureteral duplex system and megaureter detected by ultrasound and corroborated by ultrasonically-guided antegrade pyeiography is presented
Keywords: Pelviureteral duplication; Antegrade pyelography; lnterventional ultrasound
1. Introduction
Congenital anomalies at the ureterovesical junction are not uncommon. Ureteroceles (UC) are four to six times more common in girls than in boys, and occur bilaterally in 10% of the cases (Vaughan et al. 1979). UCs are subdivided into either simple or ectopic. Simple UCs arise at the normal ureteric orifice in the trigone. The ectopic variety is any UC which does not open in the lateral angle of the trigone. The majority of UCs are ectopic and they usually are associated with a duplicated renal collecting system. Duplication has an incidence ranging from 1.7% to 4.2% (Caione et al. 1989). UC and ureteral ectopy * Corresponding author, Teglparken 8, DK-4200 Slagelse, Denmark.
without duplex system anomalies are rare (Share et al. 1989). The aim of this report is to demonstrate a case of pelviureteral duplication diagnosed by ultrasound (US) and verified by ultrasonically-guided antegrade pyelography. Moreover, we emphasize the special imaging problems regarding the detection of this anomaly. 2. Case report
A 14-year-old boy without previous urological problems was admitted to hospital after 2 days of right flank pain, accompanied by fever up to approximately 39°. Because of a suspected abscess, US examination was performed. This examination revealed a cystiform angular mass with a thickened wall at the right renal upper pole. The renal paren-
0929-8266/95/$09.50 © 1995 Elsevier Science Ireland Ltd. All rights reserved SSD! 0929-8266(95)00103-X
216
P.R. Jensen et al./ European Journal of Ultrasound 2 (1995) 215-218
graphy (IVU) was performed showing apparently normal conditions, however, the outline of the right upper pole was poorly defined. The pelvicalyceal systems were slightly asymmetrical, but otherwise they appeared normal and only one ureter was disclosed on each side (Fig. 3). Filling defects or other signs of UC were not found and a persistent urachus was not suspected. Cystoscopy demonstrated a normal ureteric orifice on the left side, On the right side of the bladder base an abnormal bulge was found. Medi-
Fig. I. Cystiform angular mass at right renal upper pole (arrows).
chyma was normal and there was no sign of hydronephrosis (Fig. 1). An immobile tube-like structure with a thick wall was detected in the paravesical retroperitoneum (Fig. 2). An intravenous uro-
Fig. 2. A tube-like structure in the paravesical retroperitoneum (arrows).
Fig. 3. Urography with apparently normal conditions. The outline of the right kidney pole is poorly defined, indicating increased renal substance between the contrast-filled calyces and the top of the kidney.
P.R. Jensen et al./ European Journal of Ultrasound 2 (1995) 215-218
217
al to the bulge a supposed lower pole ureteric orifice was seen and a catheter could be inserted, but no orifice was found in relation to the bulge itself. Retrograde contrast injection into the orifice revealed a cavity associated with the bladder. The assumption was then that the previously mentioned bulge could not be a true ureterocele. To exclude an abscess at the upper renal pole, an ultrasonically-guided puncture was performed, whereby clear yellow fluid was aspirated. Contrast medium was injected into the cavity demonstrating an upper renal moiety, consisting of a single rounded calyx and a megaureter, which could be
followed through the retroperitoneum and causing the abnormal configuration of the bladder (Figs. 4 and 5). Surgery was performed. The operation confirmed the pre-operative findings of a flaccid cystic cavity at the right renal upper pole. The cavity was an upper moiety directly continuing in a broad, immobile and thickened ureter. The lower moiety ureter was normal. Both right ureters were dissected distally until 2 cm from the bladder wall, where they were non-separable. A catheter was inserted into the megaureter and could be pushed straight into the bladder. Intra-operatively, only one ureteric orifice was demonstrated on the right side. The upper moiety and the megaureter were
Fig. 4. Contrast-filling of the upper moiety calyx and megaureter through the inserted puncture needle,
Fig. 5. Contrast-filling of the megaureter contributing to the abnormal bulging in the bladder (arrows).
218
P.R. Jensen et al./ European Journal of Ultrasound 2 (1995) 215-218
removed surgically. However, the distal part of the megaureter was preserved together with the common ureteric orifice of the upper and lower moiety. 3. Discussion
The clinical importance of UC primarily lies in the obstructive uropathy and subsequent renal damage with recurrent urinary infection. The diagnosis of an ectopic UC is usually made radiographically. However, US is generally the first choice as part of a screening program, when investigating recurrent urinary tract infections in infants. Hereby, kidney size can be measured and, if present, pelvicalyceal dilatation detected. Voiding cystourethrography may show bladder anomalies or vesicoureteral reflux. Usually, the diagnosis of UC is obtained by IVU showing the dilatation of the distal ureter which is surrounded by a 'cobrahead' halo, representing the bladder wall. Indications for performing IVU are typically suspicion of vesicoureteral reflux and urostasis, but for detecting a UC there must be sufficient excretory function of the kidney. When ureteral obstruction is present, various degrees of ureteral and pelvicalyceal dilatation will be found. With pelviureteral duplication, the UC almost invariably arises from the upper moiety ureter. When the upper pole system is poorly functioning and fails to be visualized on IVU, duplication can be suspected by the indirect urographic signs: downward and lateral displacement of the lower pole calyces, lateral displacement and tortuosity of the lower pole ureter, renal axis deviation, increased renal substance thickness between the lower calyceal group and the top of the renal outline, and deficiency in the number of calyces on the affected side. US picks up the vast majority of duplex systems if the renal sinus is split. It is possible to visualize pelvicalyceal dilatation and megaureter, and with a slightly filled bladder, a UC is normally clearly visualized (Griffin et al. 1983). However, as pointed out by Share et al. (1990), some cases of pelviureteral duplication cannot be visualized on primary investigation by both IVU,
US and cystoscopy. Our case report emphasizes this difficulty. A small dysplastic upper pole segment with a single calyx and no excretory function, collapsed ureter and ureteral ectopy are conditions particularly difficult to demonstrate. IVU will fail to demonstrate contrast filling, US will not show dilatation and no sign of an ureteric orifice will be found on endoscopic examination (absence of incontinence). An endoscopic puncture of the UC and subsequent retrograde ureterogram will delineate the pathology. In our case report it was necessary to exclude abscess and to document the connection between what was interpreted as a possible upper pole system and a distal ureterectasis. This was performed by ultrasonically-guided puncture of the upper pole cystic cavity followed by antegrade pyelography (Hancke 1991). As identification of the relevant ureteric orifices failed and the intravesical bulge was conceived as not being a true UC, retrograde pyelography was not possible. However, the antegrade pyelography revealed a pelviureteral duplication with an upper pole moiety, consisting of scarred renal parenchyma with a single rounded calyx, and a tortuous megaureter which distally contributed to the intravesical balooning. The poorly defined right upper kidney pole on IVU was in accordance with this realization. However the anomaly was not clearly demonstrated on IVU, US and cystoscopy. References Caione P, Zaccara A, Capozza N, De Gennaro M. How prenatal ultrasound can affect the treatment of ureterocele in neonates and children. Eur Urol 1989; 16: 195-199. Griffin J, Jennings C, MacErlean D. Ultrasonic evaluation of simple and ectopic ureteroceles. Clin Radiol 1983; 34" 55-57. Hancke S. Ultrasonically-guided fine needle pyelography. Scand J Urol Nephrol 1991; Suppl. 137: 49-51. Share JC, Lebowitz RL. Ectopic ureterocele without ureteral calyceal dilatation (Ureterocele disproportion): Findings on urography and sonography. Am J Roentgenol 1989; 152: 267-271. Share JC, Lebowitz RL. The unsuspected double collecting system on imaging studies and at cystoscopy. Am J Roentgenol 1990; 155: 561-564. Vaughan, McKay, Behrman. Nelson: Textbook of Paediatrics. Saunders, Philadelphia, London, Toronto 1979: 1565.
U~VBA$@~N© ELSEVIER
European Journal of Ultrasound 2 (1995) 215-218
Clinical report
Diagnosis of pelviureteral duplication by ultrasonically-guided antegrade pyelography P e r R e j n e r t J e n s e n , a , Sture H e g e l u n d a, T o r b e n K j a e r b aDepartment of Radiology, Roskilde Hospital, 4000 Roskilde, Denmark bDepartment of Surgery, Roskilde Hospital, 4000 Roskilde, Denmark Received 3 September 1994; revision received 14 February 1995; accepted 16 February 1995
Abstract
The case of a 14-year-old boy with pelviureteral duplex system and megaureter detected by ultrasound and corroborated by ultrasonically-guided antegrade pyeiography is presented
Keywords: Pelviureteral duplication; Antegrade pyelography; lnterventional ultrasound
1. Introduction
Congenital anomalies at the ureterovesical junction are not uncommon. Ureteroceles (UC) are four to six times more common in girls than in boys, and occur bilaterally in 10% of the cases (Vaughan et al. 1979). UCs are subdivided into either simple or ectopic. Simple UCs arise at the normal ureteric orifice in the trigone. The ectopic variety is any UC which does not open in the lateral angle of the trigone. The majority of UCs are ectopic and they usually are associated with a duplicated renal collecting system. Duplication has an incidence ranging from 1.7% to 4.2% (Caione et al. 1989). UC and ureteral ectopy * Corresponding author, Teglparken 8, DK-4200 Slagelse, Denmark.
without duplex system anomalies are rare (Share et al. 1989). The aim of this report is to demonstrate a case of pelviureteral duplication diagnosed by ultrasound (US) and verified by ultrasonically-guided antegrade pyelography. Moreover, we emphasize the special imaging problems regarding the detection of this anomaly. 2. Case report
A 14-year-old boy without previous urological problems was admitted to hospital after 2 days of right flank pain, accompanied by fever up to approximately 39°. Because of a suspected abscess, US examination was performed. This examination revealed a cystiform angular mass with a thickened wall at the right renal upper pole. The renal paren-
0929-8266/95/$09.50 © 1995 Elsevier Science Ireland Ltd. All rights reserved SSD! 0929-8266(95)00103-X
216
P.R. Jensen et al./ European Journal of Ultrasound 2 (1995) 215-218
graphy (IVU) was performed showing apparently normal conditions, however, the outline of the right upper pole was poorly defined. The pelvicalyceal systems were slightly asymmetrical, but otherwise they appeared normal and only one ureter was disclosed on each side (Fig. 3). Filling defects or other signs of UC were not found and a persistent urachus was not suspected. Cystoscopy demonstrated a normal ureteric orifice on the left side, On the right side of the bladder base an abnormal bulge was found. Medi-
Fig. I. Cystiform angular mass at right renal upper pole (arrows).
chyma was normal and there was no sign of hydronephrosis (Fig. 1). An immobile tube-like structure with a thick wall was detected in the paravesical retroperitoneum (Fig. 2). An intravenous uro-
Fig. 2. A tube-like structure in the paravesical retroperitoneum (arrows).
Fig. 3. Urography with apparently normal conditions. The outline of the right kidney pole is poorly defined, indicating increased renal substance between the contrast-filled calyces and the top of the kidney.
P.R. Jensen et al./ European Journal of Ultrasound 2 (1995) 215-218
217
al to the bulge a supposed lower pole ureteric orifice was seen and a catheter could be inserted, but no orifice was found in relation to the bulge itself. Retrograde contrast injection into the orifice revealed a cavity associated with the bladder. The assumption was then that the previously mentioned bulge could not be a true ureterocele. To exclude an abscess at the upper renal pole, an ultrasonically-guided puncture was performed, whereby clear yellow fluid was aspirated. Contrast medium was injected into the cavity demonstrating an upper renal moiety, consisting of a single rounded calyx and a megaureter, which could be
followed through the retroperitoneum and causing the abnormal configuration of the bladder (Figs. 4 and 5). Surgery was performed. The operation confirmed the pre-operative findings of a flaccid cystic cavity at the right renal upper pole. The cavity was an upper moiety directly continuing in a broad, immobile and thickened ureter. The lower moiety ureter was normal. Both right ureters were dissected distally until 2 cm from the bladder wall, where they were non-separable. A catheter was inserted into the megaureter and could be pushed straight into the bladder. Intra-operatively, only one ureteric orifice was demonstrated on the right side. The upper moiety and the megaureter were
Fig. 4. Contrast-filling of the upper moiety calyx and megaureter through the inserted puncture needle,
Fig. 5. Contrast-filling of the megaureter contributing to the abnormal bulging in the bladder (arrows).
218
P.R. Jensen et al./ European Journal of Ultrasound 2 (1995) 215-218
removed surgically. However, the distal part of the megaureter was preserved together with the common ureteric orifice of the upper and lower moiety. 3. Discussion
The clinical importance of UC primarily lies in the obstructive uropathy and subsequent renal damage with recurrent urinary infection. The diagnosis of an ectopic UC is usually made radiographically. However, US is generally the first choice as part of a screening program, when investigating recurrent urinary tract infections in infants. Hereby, kidney size can be measured and, if present, pelvicalyceal dilatation detected. Voiding cystourethrography may show bladder anomalies or vesicoureteral reflux. Usually, the diagnosis of UC is obtained by IVU showing the dilatation of the distal ureter which is surrounded by a 'cobrahead' halo, representing the bladder wall. Indications for performing IVU are typically suspicion of vesicoureteral reflux and urostasis, but for detecting a UC there must be sufficient excretory function of the kidney. When ureteral obstruction is present, various degrees of ureteral and pelvicalyceal dilatation will be found. With pelviureteral duplication, the UC almost invariably arises from the upper moiety ureter. When the upper pole system is poorly functioning and fails to be visualized on IVU, duplication can be suspected by the indirect urographic signs: downward and lateral displacement of the lower pole calyces, lateral displacement and tortuosity of the lower pole ureter, renal axis deviation, increased renal substance thickness between the lower calyceal group and the top of the renal outline, and deficiency in the number of calyces on the affected side. US picks up the vast majority of duplex systems if the renal sinus is split. It is possible to visualize pelvicalyceal dilatation and megaureter, and with a slightly filled bladder, a UC is normally clearly visualized (Griffin et al. 1983). However, as pointed out by Share et al. (1990), some cases of pelviureteral duplication cannot be visualized on primary investigation by both IVU,
US and cystoscopy. Our case report emphasizes this difficulty. A small dysplastic upper pole segment with a single calyx and no excretory function, collapsed ureter and ureteral ectopy are conditions particularly difficult to demonstrate. IVU will fail to demonstrate contrast filling, US will not show dilatation and no sign of an ureteric orifice will be found on endoscopic examination (absence of incontinence). An endoscopic puncture of the UC and subsequent retrograde ureterogram will delineate the pathology. In our case report it was necessary to exclude abscess and to document the connection between what was interpreted as a possible upper pole system and a distal ureterectasis. This was performed by ultrasonically-guided puncture of the upper pole cystic cavity followed by antegrade pyelography (Hancke 1991). As identification of the relevant ureteric orifices failed and the intravesical bulge was conceived as not being a true UC, retrograde pyelography was not possible. However, the antegrade pyelography revealed a pelviureteral duplication with an upper pole moiety, consisting of scarred renal parenchyma with a single rounded calyx, and a tortuous megaureter which distally contributed to the intravesical balooning. The poorly defined right upper kidney pole on IVU was in accordance with this realization. However the anomaly was not clearly demonstrated on IVU, US and cystoscopy. References Caione P, Zaccara A, Capozza N, De Gennaro M. How prenatal ultrasound can affect the treatment of ureterocele in neonates and children. Eur Urol 1989; 16: 195-199. Griffin J, Jennings C, MacErlean D. Ultrasonic evaluation of simple and ectopic ureteroceles. Clin Radiol 1983; 34" 55-57. Hancke S. Ultrasonically-guided fine needle pyelography. Scand J Urol Nephrol 1991; Suppl. 137: 49-51. Share JC, Lebowitz RL. Ectopic ureterocele without ureteral calyceal dilatation (Ureterocele disproportion): Findings on urography and sonography. Am J Roentgenol 1989; 152: 267-271. Share JC, Lebowitz RL. The unsuspected double collecting system on imaging studies and at cystoscopy. Am J Roentgenol 1990; 155: 561-564. Vaughan, McKay, Behrman. Nelson: Textbook of Paediatrics. Saunders, Philadelphia, London, Toronto 1979: 1565.