Case Report Solitary crossed ectopia with vesicoureteric junction obstruction: A rare case report Raashid Hamid, Sajad Wani, Aejaz Baba, Gowhar Mufti Department of Paediatric and Neonatal Surgery, SKIMS, Srinagar, Jammu and Kashmir, India Address for correspondence: Dr. Raashid Hamid, Married Doctors Hostel, A-Block, Room No. S2, SKIMS, Soura, Srinager - 190 011, Jammu and Kashmir, India. E-mail: [email protected]
ABSTRACT Solitary crossed renal ectopia (SCRE) is a very rare anomaly of urinary tract. Most cases are diagnosed incidentally. We report a case of SCRE associated with vesicoureteric junction obstruction in a 7-year-old child which has not been reported in the literature till date.
Access this article online Website: www.jiaps.com DOI: 10.4103/0971-9261.151560 Quick Response Code:
KEY WORDS: Ectopia, renal ectopia, solitary crossed renal ectopia, vesicoureteric junction obstruction
INTRODUCTION Crossed renal ectopia (RE) is a very rare anomaly with an incidence of 1 in 7500 autopsies or 1 in 14,000 pediatric admissions.[1,2] Solitary crossed RE (SCRE) occurs due to a combination of unilateral agenesis and RE.[3] It is diagnosed incidentally on routine ultrasound or autopsies. The most common associated urological anomaly is vesicoureteric reflux (VUR).[2,4] We report a case of SCRE associated with vesicoureteric junction (VUJ) obstruction in a 7-yearold male child.
CASE REPORT A 7-year-old male child was being evaluated for urinary tract infection. Ultrasonography abdomen revealed hydroureteronephrosis with a solitary left kidney with a nonvisualization of the right kidney. General and systemic examination was normal. There was a history of cleft palate surgery at 2 years of age. Routine urine examination revealed pyuria. Renal functions were normal (serum creatinine: 0.7; urea: 30). There was no associated cardiac or vertebral anomaly.
Voiding cystourethrography (VCU) was performed which did not reveal any reflux. Intravenous urogram (IVU) findings demonstrated that the ureter from the left kidney was inserted into the bladder on the right side after crossing the midline [Figure 1]. Furthermore, ureter and pelvis were grossly dilated. Renal radionuclide 99m-technetium (99mTc) -scan was again suggestive of absent right kidney and ectopic left kidney with VUJ obstruction (delayed drainage of whole ureter and kidney) [Figure 2]. Cystoscopy showed one ureteric orifice on the right side which was normal in caliber and could not be cannulated. Upon exploration, diagnosis of VUJ obstruction was confirmed, and intravesical ureteric reimplantation along with tapering of ureter was done. Postoperative period was uneventful, and patient was discharged on 9th postoperative day.
DISCUSSION Renal ectopia describes a kidney that lies outside the renal fossa. Simple RE refers to the kidney that remains in ipsilateral retroperitoneal space. Crossed RE exists when a kidney is located on the opposite side of the midline from where ureters enter the
Cite this article as: Hamid R, Wani S, Baba A, Mufti G. Solitary crossed ectopia with vesicoureteric junction obstruction: A rare case report. J Indian Assoc Pediatr Surg 2015;20:95-7. Source of Support: Nil, Conflict of Interest: None declared.
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2
95
Hamid, et al.: Solitary crossed ectopia with vesicoureteric junction obstruction
Figure 1: Intravenous urogram showing solitary right to left ectopia with grossly dilated ureter crossing the midline
bladder. It can be with fusion, without fusion, solitary or bilateral. SCRE results due to renal agenesis of one side and contralateral displacement of the ureter to the opposite side. The crossed RE is a rare disorder, furthermore the incidence of SCRE is exceedingly low, and till now only 35 cases have been reported in the literature.[3,5] Like simple RE, SCRE is also asymptomatic and is diagnosed incidentally. SCRE can be diagnosed by ultrasonography and radionuclide scintigraphy using 99mTc-dimercaptosuccinic acid (DMSA) in most of the asymptomatic cases. Excretory urography and multidetector three-dimensional computed tomography urography are excellent for delineating RE.[5] In our case, IVU and renal scan were used to arrive at the final diagnosis. 99mTc-DMSA scintigraphy is useful in the diagnosis of simple RE, renal cortical lesions, and function of cortical mass. [6] A high incidence of associated urological abnormalities has been reported in patients with RE. Hydronephrosis is the most significant finding in RE, and the associated VUR is present in 25-70% of cases.[2,4] Dilatation of the ectopic renal pelvis due to the ureteropelvic junction obstruction is present in 37% of patients.[7] Other abnormalities associated with RE include abnormalities of spinal growth and genital abnormalities such as anorectal malformation, hypospadias, and sacral agenesis. In our case, SCRE (right to left) was associated with VUJ obstruction which was the cause of hydroureteronephrosis. To our best knowledge, this is the first case report in the world literature although VUJ obstruction with crossed RE has been reported in one case which was bilateral.[8] In this case, the ectopic kidney was poorly functioning, while the contra lateral orthotopic kidney was malrotated, with fetal appearance. The global glomerular filtration 96
Figure 2: Renal radionuclide scan showing crossed ectopia with obstruction to drainage at vesicoureteric junction and proximal dilated ureter and pelvis
rate in our patient was 84 ml/min. IVU clinched the diagnosis in our case. Ectopic kidneys are more disease prone than normal kidney, and they are often hypoplastic or dysplastic.[2] Some authors encourage a VCUG in boys and cystoultrasonography in girls affected by crossed RE for the high risk of associated VUR as postnatal US is a poor predictor of VUR.[9] Crossed renal ectopic kidneys are smaller than simple renal ectopic kidneys and have inherently reduced function.[4] A solitary crossed ectopic renal unit will have further worsening of function if associated with an obstructive or refluxing lesion. Furthermore, it is important to distinguish the VUR from VUJ obstruction as association of VUJ obstruction causes a more rapid deterioration of renal function particularly in a solitary renal unit. Most patients with SCRE do not require treatment for this condition, but because of associated VUJ obstruction reimplantation was unavoidable in our case. We recommend that the urological evaluation should be sought due to the high proportion of these cases have associated urological anomalies.
CONCLUSION This case of SCRE is probably the 36th case reported. The association of SCRE with VUJ obstruction is probably the first case we reported. Intravenous urography, magnetic resonance urography, and 99mTc-DMSA are the investigations used for the diagnosis of such unusual cases. SCRE usually requires treatment for associated abnormalities rather than for renal problems.
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2
Hamid, et al.: Solitary crossed ectopia with vesicoureteric junction obstruction
An associated VUJ obstruction once documented should be treated urgently to prevent deterioration of renal function.
REFERENCES 1. Malek RS, Kelalis PP, Burke EC. Ectopic kidney in children and frequency of association with other malformations. Mayo Clin Proc 1971;46:461-7. 2. Kheradpir MH, Bodaghi E. Crossed renal ectopia in children. Pediatr Surg Int 1992;7:69-72. 3. Livermore LJ, Thiruchelvam N. Whose side are you on? The diagnostic conundrum of solitary crossed renal ectopia. J Pediatr Urol 2010;6:83-6. 4. Guarino N, Tadini B, Camardi P, Silvestro L, Lace R, Bianchi M. The incidence of associated urological abnormalities in children with renal ectopia. J Urol 2004;172:1757-9.
5. Kesan K, Gupta R, Gupta A, Kothari P, Ranjan R, Mudkhedkar K, et al. Solitary crossed renal ectopia with vesicoureteric reflux presenting with impaired renal function in a neonate. J Clin Neonatol 2013;2:140-2. 6. Gordon I. Pediatric aspects of radionuclides in nephrourology. In: Muray IP, EII PJ, editors. Nuclear Medicine in Clinical Diagnosis and Treatment. 2nd ed. London: Churchill Living Stone; 1998. p. 277-90. 7. Kramer SA, Kelalis PP. Ureteropelvic junction obstruction in children with renal ectopy. J Urol (Paris) 1984;90:331-6. 8. Arena F, Arena S, Paolata A, Campenni A, Zuccarello B, Romeo G. Is a complete urological evaluation necessary in all newborns with asymptomatic renal ectopia? Int J Urol 2007;14:491-5. 9. McIlroy PJ, Abbott GD, Anderson NG, Turner JG, Mogridge N, Wells JE. Outcome of primary vesicoureteric reflux detected following fetal renal pelvic dilatation. J Paediatr Child Health 2000;36:569-73.
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2
97
Copyright of Journal of Indian Association of Pediatric Surgeons is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
ABSTRACT Solitary crossed renal ectopia (SCRE) is a very rare anomaly of urinary tract. Most cases are diagnosed incidentally. We report a case of SCRE associated with vesicoureteric junction obstruction in a 7-year-old child which has not been reported in the literature till date.
Access this article online Website: www.jiaps.com DOI: 10.4103/0971-9261.151560 Quick Response Code:
KEY WORDS: Ectopia, renal ectopia, solitary crossed renal ectopia, vesicoureteric junction obstruction
INTRODUCTION Crossed renal ectopia (RE) is a very rare anomaly with an incidence of 1 in 7500 autopsies or 1 in 14,000 pediatric admissions.[1,2] Solitary crossed RE (SCRE) occurs due to a combination of unilateral agenesis and RE.[3] It is diagnosed incidentally on routine ultrasound or autopsies. The most common associated urological anomaly is vesicoureteric reflux (VUR).[2,4] We report a case of SCRE associated with vesicoureteric junction (VUJ) obstruction in a 7-yearold male child.
CASE REPORT A 7-year-old male child was being evaluated for urinary tract infection. Ultrasonography abdomen revealed hydroureteronephrosis with a solitary left kidney with a nonvisualization of the right kidney. General and systemic examination was normal. There was a history of cleft palate surgery at 2 years of age. Routine urine examination revealed pyuria. Renal functions were normal (serum creatinine: 0.7; urea: 30). There was no associated cardiac or vertebral anomaly.
Voiding cystourethrography (VCU) was performed which did not reveal any reflux. Intravenous urogram (IVU) findings demonstrated that the ureter from the left kidney was inserted into the bladder on the right side after crossing the midline [Figure 1]. Furthermore, ureter and pelvis were grossly dilated. Renal radionuclide 99m-technetium (99mTc) -scan was again suggestive of absent right kidney and ectopic left kidney with VUJ obstruction (delayed drainage of whole ureter and kidney) [Figure 2]. Cystoscopy showed one ureteric orifice on the right side which was normal in caliber and could not be cannulated. Upon exploration, diagnosis of VUJ obstruction was confirmed, and intravesical ureteric reimplantation along with tapering of ureter was done. Postoperative period was uneventful, and patient was discharged on 9th postoperative day.
DISCUSSION Renal ectopia describes a kidney that lies outside the renal fossa. Simple RE refers to the kidney that remains in ipsilateral retroperitoneal space. Crossed RE exists when a kidney is located on the opposite side of the midline from where ureters enter the
Cite this article as: Hamid R, Wani S, Baba A, Mufti G. Solitary crossed ectopia with vesicoureteric junction obstruction: A rare case report. J Indian Assoc Pediatr Surg 2015;20:95-7. Source of Support: Nil, Conflict of Interest: None declared.
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2
95
Hamid, et al.: Solitary crossed ectopia with vesicoureteric junction obstruction
Figure 1: Intravenous urogram showing solitary right to left ectopia with grossly dilated ureter crossing the midline
bladder. It can be with fusion, without fusion, solitary or bilateral. SCRE results due to renal agenesis of one side and contralateral displacement of the ureter to the opposite side. The crossed RE is a rare disorder, furthermore the incidence of SCRE is exceedingly low, and till now only 35 cases have been reported in the literature.[3,5] Like simple RE, SCRE is also asymptomatic and is diagnosed incidentally. SCRE can be diagnosed by ultrasonography and radionuclide scintigraphy using 99mTc-dimercaptosuccinic acid (DMSA) in most of the asymptomatic cases. Excretory urography and multidetector three-dimensional computed tomography urography are excellent for delineating RE.[5] In our case, IVU and renal scan were used to arrive at the final diagnosis. 99mTc-DMSA scintigraphy is useful in the diagnosis of simple RE, renal cortical lesions, and function of cortical mass. [6] A high incidence of associated urological abnormalities has been reported in patients with RE. Hydronephrosis is the most significant finding in RE, and the associated VUR is present in 25-70% of cases.[2,4] Dilatation of the ectopic renal pelvis due to the ureteropelvic junction obstruction is present in 37% of patients.[7] Other abnormalities associated with RE include abnormalities of spinal growth and genital abnormalities such as anorectal malformation, hypospadias, and sacral agenesis. In our case, SCRE (right to left) was associated with VUJ obstruction which was the cause of hydroureteronephrosis. To our best knowledge, this is the first case report in the world literature although VUJ obstruction with crossed RE has been reported in one case which was bilateral.[8] In this case, the ectopic kidney was poorly functioning, while the contra lateral orthotopic kidney was malrotated, with fetal appearance. The global glomerular filtration 96
Figure 2: Renal radionuclide scan showing crossed ectopia with obstruction to drainage at vesicoureteric junction and proximal dilated ureter and pelvis
rate in our patient was 84 ml/min. IVU clinched the diagnosis in our case. Ectopic kidneys are more disease prone than normal kidney, and they are often hypoplastic or dysplastic.[2] Some authors encourage a VCUG in boys and cystoultrasonography in girls affected by crossed RE for the high risk of associated VUR as postnatal US is a poor predictor of VUR.[9] Crossed renal ectopic kidneys are smaller than simple renal ectopic kidneys and have inherently reduced function.[4] A solitary crossed ectopic renal unit will have further worsening of function if associated with an obstructive or refluxing lesion. Furthermore, it is important to distinguish the VUR from VUJ obstruction as association of VUJ obstruction causes a more rapid deterioration of renal function particularly in a solitary renal unit. Most patients with SCRE do not require treatment for this condition, but because of associated VUJ obstruction reimplantation was unavoidable in our case. We recommend that the urological evaluation should be sought due to the high proportion of these cases have associated urological anomalies.
CONCLUSION This case of SCRE is probably the 36th case reported. The association of SCRE with VUJ obstruction is probably the first case we reported. Intravenous urography, magnetic resonance urography, and 99mTc-DMSA are the investigations used for the diagnosis of such unusual cases. SCRE usually requires treatment for associated abnormalities rather than for renal problems.
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2
Hamid, et al.: Solitary crossed ectopia with vesicoureteric junction obstruction
An associated VUJ obstruction once documented should be treated urgently to prevent deterioration of renal function.
REFERENCES 1. Malek RS, Kelalis PP, Burke EC. Ectopic kidney in children and frequency of association with other malformations. Mayo Clin Proc 1971;46:461-7. 2. Kheradpir MH, Bodaghi E. Crossed renal ectopia in children. Pediatr Surg Int 1992;7:69-72. 3. Livermore LJ, Thiruchelvam N. Whose side are you on? The diagnostic conundrum of solitary crossed renal ectopia. J Pediatr Urol 2010;6:83-6. 4. Guarino N, Tadini B, Camardi P, Silvestro L, Lace R, Bianchi M. The incidence of associated urological abnormalities in children with renal ectopia. J Urol 2004;172:1757-9.
5. Kesan K, Gupta R, Gupta A, Kothari P, Ranjan R, Mudkhedkar K, et al. Solitary crossed renal ectopia with vesicoureteric reflux presenting with impaired renal function in a neonate. J Clin Neonatol 2013;2:140-2. 6. Gordon I. Pediatric aspects of radionuclides in nephrourology. In: Muray IP, EII PJ, editors. Nuclear Medicine in Clinical Diagnosis and Treatment. 2nd ed. London: Churchill Living Stone; 1998. p. 277-90. 7. Kramer SA, Kelalis PP. Ureteropelvic junction obstruction in children with renal ectopy. J Urol (Paris) 1984;90:331-6. 8. Arena F, Arena S, Paolata A, Campenni A, Zuccarello B, Romeo G. Is a complete urological evaluation necessary in all newborns with asymptomatic renal ectopia? Int J Urol 2007;14:491-5. 9. McIlroy PJ, Abbott GD, Anderson NG, Turner JG, Mogridge N, Wells JE. Outcome of primary vesicoureteric reflux detected following fetal renal pelvic dilatation. J Paediatr Child Health 2000;36:569-73.
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2
97
Copyright of Journal of Indian Association of Pediatric Surgeons is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
