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Management of ectopic ureterocele with horseshoe kidney
Journal of Pediatric Urology (2009) 5, 400e401
CASE REPORT
Management of ectopic ureterocele with horseshoe kidney George Chiang*, George Kaplan Division of Urology, Rady Children’s Hospital San Diego, 7930 Frost Street #300, San Diego, CA 92123, USA Received 5 January 2009; accepted 26 February 2009 Available online 28 March 2009
KEYWORDS Horseshoe kidney; Ureterocele; Partial nephrectomy
Abstract The combination of a horseshoe kidney and ureterocele is a rare complex with no definite genetic or developmental association. Only two cases have been reported historically. Diagnosis and treatment of such a complex can be varied amongst providers. We performed a partial nephrectomy with lower pole reimplant after marsupialization of the ureterocele. The patient currently has no evidence of reflux and has been infection free. ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Ectopic ureterocele can be a challenge in both diagnosis and therapy. Multimodal imaging and surgical interventions may be required in complex cases. We present the youngest case to our knowledge of an ectopic ureterocele and horseshoe kidney and the third to be reported in the English language.
Case report A 2-month-old full-term female was admitted with a febrile UTI and known history of a left ectopic ureterocele on antibiotic prophylaxis. A prior VCUG showed no evidence of reflux. Attempts at endoscopic incision were unsuccessful and a nephrostomy tube was placed (Fig. 1). A DMSA scan was performed to determine surgical approach which
* Corresponding author. Tel.: þ1 858 279 8527; fax: þ1 858 279 8876. E-mail address: [email protected] (G. Chiang).
demonstrated the presence of an isthmus and minimal left upper pole function. Further anatomy was delineated using a CT scan (Fig. 2), with visualization of a horseshoe kidney and the ureterocele. A left upper pole partial nephrectomy with open ureterocele marsupialization and lower pole reimplantation was performed with a postoperative IVP (Fig. 3), demonstrating normal emptying of the horseshoe kidney and a slight bladder wall defect. Follow-up VCUG demonstrated no reflux and the patient was symptom free at 6 months.
Discussion The incidence of ureterocele has been reported in up to 1 in 500 based upon autopsy studies [1]. Ureterocele formation may be related to the timing of the absorption of the mesonephric duct into the urogenital sinus or failure of Chwalla’s membrane to rupture [2]. Presenting symptoms include infection, hematuria, and a palpable abdominal mass. Treatment is still controversial and can include
1477-5131/$36 ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2009.02.208
Management of ectopic ureterocele with horseshoe kidney
Figure 1
401
Placement of nephrostomy tube.
endoscopic puncture, upper pole nephrectomy, reconstruction at the bladder level, or non-operative management. Factors influencing treatment decision include type of presentation, age of patient, type of ureterocele, renal function, and presence of VUR and UTI [2]. Horseshoe kidney occurs in 0.2e0.3% of the general population. It results from fusion of the two nephrogenic blastemas during week 4 of gestation while these structures are in close apposition in the fetal pelvis, thus preventing normal migration and rotation, and resulting in a ventral position of the collecting system. Horseshoe kidney can be associated with a variety of congenital anomalies with reports ranging between 33% and 79% of cases [3].
Figure 3
Postoperative IVP.
There have been three documented reports of an ectopic ureterocele with a horseshoe kidney. The treatment in a 3-year-old boy involved an upper pole nephrectomy whereas surgery in a 55-year-old man involved an upper pole ureteropyelostomy with ligation of the distal ureter and unroofing of the ureterocele [4,5]. Given the minimal function of the upper pole as well as the significant infectious reservoir, we performed an upper pole nephroureterectomy via a flank incision. The accompanying extensive resection of the ureterocele and mobilization of the lower pole ureter necessitated a reimplant procedure via a low Pfannenstiel incision. The presence of the horseshoe kidney actually enabled easier access to the upper pole and ureter via a flank incision, since these were rotated ventrally. Appropriate functional studies and imaging are integral to surgical planning for these complex anomalies.
Conflict of interest/funding None.
References
Figure 2 CT ureterocele.
scan
visualizing
horseshoe
kidney
and
[1] Berdon WE, Baker DH, Becker JA, Uson AC. Ectopic ureterocele. Radiol Clin North Am 1968;6:205e14. [2] Merlini E, Lelli Chiesa P. Obstructive ureterocele-an ongoing challenge. World J Urol 2004;22:107e14. [3] Knutson T, Hawas B. Horseshoe kidney with a circumcaval ureter. Scand J Urol Nephrol 2004;38:348e50. [4] Sumner TE, Volberg FM, Munitz A, Harrison LH, Mashburn AM. Unilateral duplex horseshoe kidney with ectopic ureterocele. South Med J 1985;78:199e201. [5] Mikuma N, Adachi H, Takatsuka K, Yokoo A, Wada H, Takahashi A. Ectopic ureterocele with a horseshoe kidney in an adult. Int J Urol 1996;3:243e4.
CASE REPORT
Management of ectopic ureterocele with horseshoe kidney George Chiang*, George Kaplan Division of Urology, Rady Children’s Hospital San Diego, 7930 Frost Street #300, San Diego, CA 92123, USA Received 5 January 2009; accepted 26 February 2009 Available online 28 March 2009
KEYWORDS Horseshoe kidney; Ureterocele; Partial nephrectomy
Abstract The combination of a horseshoe kidney and ureterocele is a rare complex with no definite genetic or developmental association. Only two cases have been reported historically. Diagnosis and treatment of such a complex can be varied amongst providers. We performed a partial nephrectomy with lower pole reimplant after marsupialization of the ureterocele. The patient currently has no evidence of reflux and has been infection free. ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Ectopic ureterocele can be a challenge in both diagnosis and therapy. Multimodal imaging and surgical interventions may be required in complex cases. We present the youngest case to our knowledge of an ectopic ureterocele and horseshoe kidney and the third to be reported in the English language.
Case report A 2-month-old full-term female was admitted with a febrile UTI and known history of a left ectopic ureterocele on antibiotic prophylaxis. A prior VCUG showed no evidence of reflux. Attempts at endoscopic incision were unsuccessful and a nephrostomy tube was placed (Fig. 1). A DMSA scan was performed to determine surgical approach which
* Corresponding author. Tel.: þ1 858 279 8527; fax: þ1 858 279 8876. E-mail address: [email protected] (G. Chiang).
demonstrated the presence of an isthmus and minimal left upper pole function. Further anatomy was delineated using a CT scan (Fig. 2), with visualization of a horseshoe kidney and the ureterocele. A left upper pole partial nephrectomy with open ureterocele marsupialization and lower pole reimplantation was performed with a postoperative IVP (Fig. 3), demonstrating normal emptying of the horseshoe kidney and a slight bladder wall defect. Follow-up VCUG demonstrated no reflux and the patient was symptom free at 6 months.
Discussion The incidence of ureterocele has been reported in up to 1 in 500 based upon autopsy studies [1]. Ureterocele formation may be related to the timing of the absorption of the mesonephric duct into the urogenital sinus or failure of Chwalla’s membrane to rupture [2]. Presenting symptoms include infection, hematuria, and a palpable abdominal mass. Treatment is still controversial and can include
1477-5131/$36 ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2009.02.208
Management of ectopic ureterocele with horseshoe kidney
Figure 1
401
Placement of nephrostomy tube.
endoscopic puncture, upper pole nephrectomy, reconstruction at the bladder level, or non-operative management. Factors influencing treatment decision include type of presentation, age of patient, type of ureterocele, renal function, and presence of VUR and UTI [2]. Horseshoe kidney occurs in 0.2e0.3% of the general population. It results from fusion of the two nephrogenic blastemas during week 4 of gestation while these structures are in close apposition in the fetal pelvis, thus preventing normal migration and rotation, and resulting in a ventral position of the collecting system. Horseshoe kidney can be associated with a variety of congenital anomalies with reports ranging between 33% and 79% of cases [3].
Figure 3
Postoperative IVP.
There have been three documented reports of an ectopic ureterocele with a horseshoe kidney. The treatment in a 3-year-old boy involved an upper pole nephrectomy whereas surgery in a 55-year-old man involved an upper pole ureteropyelostomy with ligation of the distal ureter and unroofing of the ureterocele [4,5]. Given the minimal function of the upper pole as well as the significant infectious reservoir, we performed an upper pole nephroureterectomy via a flank incision. The accompanying extensive resection of the ureterocele and mobilization of the lower pole ureter necessitated a reimplant procedure via a low Pfannenstiel incision. The presence of the horseshoe kidney actually enabled easier access to the upper pole and ureter via a flank incision, since these were rotated ventrally. Appropriate functional studies and imaging are integral to surgical planning for these complex anomalies.
Conflict of interest/funding None.
References
Figure 2 CT ureterocele.
scan
visualizing
horseshoe
kidney
and
[1] Berdon WE, Baker DH, Becker JA, Uson AC. Ectopic ureterocele. Radiol Clin North Am 1968;6:205e14. [2] Merlini E, Lelli Chiesa P. Obstructive ureterocele-an ongoing challenge. World J Urol 2004;22:107e14. [3] Knutson T, Hawas B. Horseshoe kidney with a circumcaval ureter. Scand J Urol Nephrol 2004;38:348e50. [4] Sumner TE, Volberg FM, Munitz A, Harrison LH, Mashburn AM. Unilateral duplex horseshoe kidney with ectopic ureterocele. South Med J 1985;78:199e201. [5] Mikuma N, Adachi H, Takatsuka K, Yokoo A, Wada H, Takahashi A. Ectopic ureterocele with a horseshoe kidney in an adult. Int J Urol 1996;3:243e4.