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Ureteropelvic junction obstruction in both upper and lower moieties of a duplex system: A rare association
Journal of Pediatric Urology (2008) 4, 167e169
CASE REPORT
Ureteropelvic junction obstruction in both upper and lower moieties of a duplex system: A rare association Prashant Jain*, Beejal Sanghvi, Hemanshi Shah, Sandesh Parelkar Department of Pediatric Surgery, KEM Hospital, Parel, Mumbai, India Received 11 April 2007; accepted 13 April 2007 Available online 23 July 2007
KEYWORDS Ureteropelvic junction; Duplex moiety; Ureterocele
Abstract Ureteropelvic junction (UPJ) obstruction is a rare association with duplex system. It has been reported infrequently in lower and upper pole moieties. This is the first case of both upper and lower moieties UPJ obstruction to be reported in the literature. This case adds one more variant to the known wide spectrum of duplex systems. ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Ureteropelvic junction (UPJ) obstruction is a rare but well-known association with duplex moieties, involving mainly the lower moiety [1]. Upper moiety UPJ obstruction is a very rare occurrence. The case reported here is an unusual variant of duplex moiety, having UPJ obstruction in both the moieties. The management of UPJ in a single moiety is challenging, and this rare association further increased the challenge.
Case report An 8-year-old male child presented with complaints of absent testis in the right hemiscrotum. He had no urinary * Corresponding author. Tel.: þ91 022 09324522179. E-mail address: [email protected] (P. Jain).
complaints. Serum creatinine and urine examination were normal. Ultrasonography revealed bilateral duplex moiety. The right upper moiety had paper thin parenchyma with mild dilatation of the ureter with ureterocele. The lower moiety was normal. There was hydronephrosis of both the left moieties suggesting an element of UPJ obstruction in both moieties. IVU was suggestive of right upper nonfunctioning moiety with mild hydronephrosis and hydroureter of lower moiety with ureterocele. There was UPJ obstruction of both the moieties on the left side. VCUG was normal. The results of magnetic resonance urography were consistent with the above findings (Fig. 1). Renal scan showed non-functioning right upper moiety and obstructed pattern of both moieties in left duplex system with moderately reduced function. Cystourethroscopy showed rightsided ureterocele with two ureteric orifices on the left side. Diagnostic laparoscopy followed by orchiopexy was done for non-palpable undescended testis.
1477-5131/$30 ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2007.04.008
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P. Jain et al.
Figure 1
Magnetic resonance urography.
The management of UPJ obstruction of both moieties of the left duplex system was technically challenging. At surgery, the left duplex system with dilatation of the extrarenal pelvis of both moieties and normal ureters with preserved renal cortex was seen. Dismembered pyeloplasty over a double J stent was done for both the moieties preserving the vessels crossing the UPJ (Figs. 2 and 3). The patient was also operated for right upper pole heminephrectomy. Follow-up DTPA scan after 3 months showed maintained renal functions with non-obstructed pattern of curve on the left side.
Discussion No case has been reported in the literature to date with UPJ obstruction involving both moieties. UPJ obstruction in the lower pelvis is considered to be either congenital or extrinsic compression with aberrant or accessory renal vessels. Also, high-grade VUR can cause angulation and kinking of a dilated and tortuous ureter with secondary fixation from periureteral inflammation [2]. The upper moiety usually has no true pelvis and reflux is not common, which makes UPJ obstruction a rare phenomenon. The causes of upper moiety UPJ obstruction reported are either mainly due to primary UPJ obstruction [3] or kinking secondary to VUR [4]. Probably in our patient the cause of UPJ obstruction was aberrant renal vessels. The case described here had the unusual variation of duplex system with UPJ obstruction in both the duplex moieties of the same side. Upper pole UPJ obstruction has been reported infrequently. David et al. [3] reported 190 children operated for UPJ obstruction between 1977 and 1993. In seven of these, obstruction was associated with duplex collecting system representing 3.7% of children
Figure 2 Intraoperative photograph with dialated upper and lower moiety pelvis with crossing vessels.
treated for UPJ obstruction, and of these three had upper pole obstruction. In a critical review, in response to a case of upper pole UPJ obstruction by Dewan et al. [4], Ng [5] reviewed a total of 10 cases of upper pole UPJ obstruction. The presence of two ipsilateral renal units requires greater precision in evaluation and management [4]. Ultrasound and IVU may not delineate precisely the complexity of this anomaly. In our experience, magnetic resonance urography gives better delineation of anatomy as compared to other investigations. The management of upper and lower pole UPJ obstruction will depend on the renal function, available length of ureter and associated reflux. Ng [5] formulated a surgical
Figure 3
Intraoperative scan.
Ureteropelvic junction obstruction algorithm for the management of upper pole UPJ obstruction which depends on the renal function, length of available ureter, extrarenal or intrarenal pelvis and obstructing band, with available options of pyeloplasty, pyelopyelostomy, pyeloureterostomy, ureterolysis with or without ureterouretostomy, and heminephrectomy. These guidelines are similar to those used in the management of lower pole UPJ obstruction, except for greater emphasis on intrarenal pelvis. This was a rare case of bilateral complete duplication with UPJ obstruction of both functioning moieties of the same side with extrarenal pelvis. There was no VUR. The options available were separate pyeloplasty of both the moieties, or pyeloplasty of the lower moiety with pyelopyelostomy for the upper moiety. We performed a classical Anderson-Hynes dismembered pyeloplasty of both moieties with two separate double J stents. This case adds one more variant to the known wide spectrum of duplex systems. Diagnosis, treatment and the
169 ultimate goal of preserving maximum renal function does not differ in a duplicated system, whether the upper or lower pole is involved.
References [1] Pieretti RV. Congenital hydronephrosis in the lower segment of a duplex system. Pediatr Surg Int 1995;10:565e6. [2] Shelfo SW, Keller MS, Weiss RM. Ipsilateral pyeloureterostomy for managing lower pole reflux with associated ureteropelvic junction obstruction in duplex systems. J Urol 1997;157: 1420e2. [3] Joseph David B, Bauer Stuart B, Colodny Arnold H, et al. Lower pole ureteropelvic junction obstruction and incomplete renal duplication. J Urol 1989;141:896e9. [4] Dewan PA, Penington E, Jeyaseelan D. Upper pole pelviureteric junction obstruction. Pediatr Surg Int 1989;13:290e2. [5] Ng JWT. Upper pole pelviureteric junction obstruction: a critical review. Pediatr Surg Int 1999;15:298e9.
CASE REPORT
Ureteropelvic junction obstruction in both upper and lower moieties of a duplex system: A rare association Prashant Jain*, Beejal Sanghvi, Hemanshi Shah, Sandesh Parelkar Department of Pediatric Surgery, KEM Hospital, Parel, Mumbai, India Received 11 April 2007; accepted 13 April 2007 Available online 23 July 2007
KEYWORDS Ureteropelvic junction; Duplex moiety; Ureterocele
Abstract Ureteropelvic junction (UPJ) obstruction is a rare association with duplex system. It has been reported infrequently in lower and upper pole moieties. This is the first case of both upper and lower moieties UPJ obstruction to be reported in the literature. This case adds one more variant to the known wide spectrum of duplex systems. ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Ureteropelvic junction (UPJ) obstruction is a rare but well-known association with duplex moieties, involving mainly the lower moiety [1]. Upper moiety UPJ obstruction is a very rare occurrence. The case reported here is an unusual variant of duplex moiety, having UPJ obstruction in both the moieties. The management of UPJ in a single moiety is challenging, and this rare association further increased the challenge.
Case report An 8-year-old male child presented with complaints of absent testis in the right hemiscrotum. He had no urinary * Corresponding author. Tel.: þ91 022 09324522179. E-mail address: [email protected] (P. Jain).
complaints. Serum creatinine and urine examination were normal. Ultrasonography revealed bilateral duplex moiety. The right upper moiety had paper thin parenchyma with mild dilatation of the ureter with ureterocele. The lower moiety was normal. There was hydronephrosis of both the left moieties suggesting an element of UPJ obstruction in both moieties. IVU was suggestive of right upper nonfunctioning moiety with mild hydronephrosis and hydroureter of lower moiety with ureterocele. There was UPJ obstruction of both the moieties on the left side. VCUG was normal. The results of magnetic resonance urography were consistent with the above findings (Fig. 1). Renal scan showed non-functioning right upper moiety and obstructed pattern of both moieties in left duplex system with moderately reduced function. Cystourethroscopy showed rightsided ureterocele with two ureteric orifices on the left side. Diagnostic laparoscopy followed by orchiopexy was done for non-palpable undescended testis.
1477-5131/$30 ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2007.04.008
168
P. Jain et al.
Figure 1
Magnetic resonance urography.
The management of UPJ obstruction of both moieties of the left duplex system was technically challenging. At surgery, the left duplex system with dilatation of the extrarenal pelvis of both moieties and normal ureters with preserved renal cortex was seen. Dismembered pyeloplasty over a double J stent was done for both the moieties preserving the vessels crossing the UPJ (Figs. 2 and 3). The patient was also operated for right upper pole heminephrectomy. Follow-up DTPA scan after 3 months showed maintained renal functions with non-obstructed pattern of curve on the left side.
Discussion No case has been reported in the literature to date with UPJ obstruction involving both moieties. UPJ obstruction in the lower pelvis is considered to be either congenital or extrinsic compression with aberrant or accessory renal vessels. Also, high-grade VUR can cause angulation and kinking of a dilated and tortuous ureter with secondary fixation from periureteral inflammation [2]. The upper moiety usually has no true pelvis and reflux is not common, which makes UPJ obstruction a rare phenomenon. The causes of upper moiety UPJ obstruction reported are either mainly due to primary UPJ obstruction [3] or kinking secondary to VUR [4]. Probably in our patient the cause of UPJ obstruction was aberrant renal vessels. The case described here had the unusual variation of duplex system with UPJ obstruction in both the duplex moieties of the same side. Upper pole UPJ obstruction has been reported infrequently. David et al. [3] reported 190 children operated for UPJ obstruction between 1977 and 1993. In seven of these, obstruction was associated with duplex collecting system representing 3.7% of children
Figure 2 Intraoperative photograph with dialated upper and lower moiety pelvis with crossing vessels.
treated for UPJ obstruction, and of these three had upper pole obstruction. In a critical review, in response to a case of upper pole UPJ obstruction by Dewan et al. [4], Ng [5] reviewed a total of 10 cases of upper pole UPJ obstruction. The presence of two ipsilateral renal units requires greater precision in evaluation and management [4]. Ultrasound and IVU may not delineate precisely the complexity of this anomaly. In our experience, magnetic resonance urography gives better delineation of anatomy as compared to other investigations. The management of upper and lower pole UPJ obstruction will depend on the renal function, available length of ureter and associated reflux. Ng [5] formulated a surgical
Figure 3
Intraoperative scan.
Ureteropelvic junction obstruction algorithm for the management of upper pole UPJ obstruction which depends on the renal function, length of available ureter, extrarenal or intrarenal pelvis and obstructing band, with available options of pyeloplasty, pyelopyelostomy, pyeloureterostomy, ureterolysis with or without ureterouretostomy, and heminephrectomy. These guidelines are similar to those used in the management of lower pole UPJ obstruction, except for greater emphasis on intrarenal pelvis. This was a rare case of bilateral complete duplication with UPJ obstruction of both functioning moieties of the same side with extrarenal pelvis. There was no VUR. The options available were separate pyeloplasty of both the moieties, or pyeloplasty of the lower moiety with pyelopyelostomy for the upper moiety. We performed a classical Anderson-Hynes dismembered pyeloplasty of both moieties with two separate double J stents. This case adds one more variant to the known wide spectrum of duplex systems. Diagnosis, treatment and the
169 ultimate goal of preserving maximum renal function does not differ in a duplicated system, whether the upper or lower pole is involved.
References [1] Pieretti RV. Congenital hydronephrosis in the lower segment of a duplex system. Pediatr Surg Int 1995;10:565e6. [2] Shelfo SW, Keller MS, Weiss RM. Ipsilateral pyeloureterostomy for managing lower pole reflux with associated ureteropelvic junction obstruction in duplex systems. J Urol 1997;157: 1420e2. [3] Joseph David B, Bauer Stuart B, Colodny Arnold H, et al. Lower pole ureteropelvic junction obstruction and incomplete renal duplication. J Urol 1989;141:896e9. [4] Dewan PA, Penington E, Jeyaseelan D. Upper pole pelviureteric junction obstruction. Pediatr Surg Int 1989;13:290e2. [5] Ng JWT. Upper pole pelviureteric junction obstruction: a critical review. Pediatr Surg Int 1999;15:298e9.