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Volume 5, Issue 2 (2024)
J Clinic Care Skill 2024, 5(2): 77-80 |
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Ethics code: IR.YUMS.REC.1399.184
History
Received: 2023/08/27 | Accepted: 2024/05/28 | Published: 2024/06/21
Received: 2023/08/27 | Accepted: 2024/05/28 | Published: 2024/06/21
How to cite this article
Rabani S. Unilateral Multiple Renal Congenital Anomalies; A Case Report. J Clinic Care Skill 2024; 5 (2) :77-80
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Authors
S.M.R. Rabani * 
Departments of Urology, Faculty of Medicine, Yasuj University of Medical Sciences, Yasuj, Iran
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Introduction
Congenital anomalies of the urinary tract have a wide spectrum including upper and lower urinary tract anomalies such as renal hypoplasia, dysplasia, aplasia, ectopia, supernumerary, duplication, ureteropelvic junction obstruction (Ureter-pelvic Junction Obstruction: UPJO), ureterovesical junction obstruction (Uretero-vesical Junction Obstruction: UVJO), vesicoureteral reflux (Vesico-ureteral Reflux: VUR), ureterocele, posterior urethral valve, bladder exstrophy, and many other anomalies. The causes of these wide ranges of urinary tract anomalies are related to abnormal embryologic development of the urinary tract [1, 2]. The urinary tract system is substantially derived from the intermediate mesoderm [3, 4], and based on the underlying defect and the disturbances in the developmental process, different types of congenital anomalies may develop in the urinary system.
Patient and Methods
A 19-year-old army male presented with left lower quadrant (LLQ) abdominal pain after blunt abdominal trauma, accompanied by gross hematuria. He was healthy and asymptomatic until he had blunt abdominal trauma during sports activity and developed pain and gross hematuria.
Written informed consent for publication of the clinical details and clinical images were obtained from the patient. The datasets used during the current study are available from the corresponding author on reasonable request.
Findings
On arrival at the emergency room, physical examination revealed LLQ tenderness with stable vital signs. Primary laboratory data showed normal renal function tests and gross hematuria. Ultrasonography suggested ectopic pelvic left kidney with hydronephrosis and UPJO with distal ureteral dilatation. Intravenous pyelography confirmed the same findings (Figure 1). Renal DTPA (Diethylene Triamine Penta Acetic Acid) scanning was done that suggested ectopia, a double collecting system, lower moiety UPJO, and a common distal ureter with UVJO (Figure 2). Cystoscopy and trying to perform ureteroscopy failed to pass a catheter into the left ureter that was a plan for retrograde uretero-pyelography because the UVJ area was very narrow. Exploration was done through Gibson’s retroperitoneal incision. The upper moiety ureter was significantly dilated, but the lower moiety was thin and narrow; Both of them ended in a tapered segment typical for an adynamic segment and UVJO. Pyeloplasty and ureteroneocystostomy were carried out simultaneously. The patient has an uneventful post-operative 18-month follow-up.
Figure 1. IVP (Intravenous Pyelography) suggestive for functional, ectopic, irregular, left kidney with hydroureteronephrosis
Figure 2. Renal DTPA scanning confirms the IVP findings
Discussion
The development of the urinary tract is a very complex process and depends on regular and precise events. Normal development of the urinary system also needs the integration of a variety of cell populations that have different embryologic origins [1, 3]. The final products of these events are the functional and conduit parts of the urinary system. The evolution of our knowledge in genetics and molecular biology has greatly advanced our understanding of the pathophysiology of urinary tract congenital anomalies [5, 6]. Although anomalies of the urinary tract are among the most common anomalies of the body, there are also a great number of varieties of anomalies in the upper and lower urinary tract systems. The upper urinary tract is affected by congenital anomalies and structural anomalies (such as continuous fetal lobulation, hypertrophied Bertin column and dromedary hump), fusion anomalies (such as horseshoe kidney and pancake kidney), renal position anomalies (such as malrotation of the kidney, simple renal ectopy and crossed renal ectopy), renal number (e.g. renal agenesis and extra kidney), and abnormalities in the development of the urinary collecting system (e.g. pyelocalyceal diverticulum, megacalycosis, ureteropelvic junction obstruction, collecting system duplex, megaverter, ectopic ureter, and ureter) are classified [7]. Therefore, understanding the embryonic development and the anatomical structure of the upper urinary tract is very important for a better understanding of these cases.
In this patient, we accidentally encountered unilateral multiple renal congenital anomalies: Ectopia; Double collecting system; UPJO, UVJO, and as an operation finding there was a suggestion for the lower moiety ureter reflux to the upper moiety ureter.
Each of these anomalies has its own pathophysiology and clinical presentation, which will be discussed briefly.
Renal ectopia: The cause of renal ectopia may be a defect in the ascent process of the kidney or an abnormal ureteric bud. An ectopic kidney may be asymptomatic for life. The incidence of renal ectopia is between one in 2200 to one in 3000 autopsies, and it occurs between the 6th and 9th weeks of gestation. The most common position is opposite the sacrum and below the aortic bifurcation [7, 8]. Van Den Bosch et al.'s study diagnosed kidney ectopy in 41 cases, in which 32% of the patients were diagnosed during prenatal screening. Urological abnormalities were found in 66% of patients and vesicoureteral reflux was found in 13 cases. that there was no adverse effect on blood pressure or kidney function in children with renal ectopy. However, periodic follow-up seems necessary, at least until adulthood [9].
Duplicated system: This may be complete or partial. The incidence and prevalence of urinary tract duplication have been reported to be 0.7-4% of the population [10]. It is the result of the unusual development of the ureteral bud. In a complete duplicated system, the Wiegert-Meyer rule can be seen in 95% of the cases with consequences of vesicoureteral reflux and ureterocele or ectopic ureter. The duplication system is more common in women than in men with a ratio of 2:1 [11]. It can be asymptomatic or associated with bladder reflux (VUR), urinary incontinence, obstructive uropathy as well as renal parenchymal scarring or dysplasia and reduction [10]. Awareness of duplex abnormalities and awareness according to the symptoms observed in all imaging methods, especially ultrasound. Then, understanding the basic rules governing duplex abnormalities is important to guide a thoughtful evaluation of the child [10]. Dino et al. report a 35-year-old woman who presented with left flank pain. A CT scan of the abdomen has shown a complete duplex system on the left side with hydroureteronephrosis of the lower part and an obstructing middle ureteral stone. It states that the kidney was a complete duplex. It is rare to have lower half hydronephrosis caused by medial ureteral stone [12].
UPJO: Ureter-pelvic junction obstruction (UPJO) is the most common cause of hydronephrosis in sick children. They are usually diagnosed in the perinatal period as hydronephrosis in routine ultrasound, and are characterized by symptoms of abdominal discomfort, flank pain, or vomiting in childhood [13]. It accounts for about 50% of all intrauterine detectable hydronephrosis and it is really the most common cause of congenital obstructive nephropathy [14, 15]. Minimally invasive surgery (MIS), initially with laparoscopic pyeloplasty (LP) and then with robot-assisted laparoscopic pyeloplasty (RALP), has played an important role in the treatment of UPJO in the past 10 years [16, 17]. UPJO accounts for 11% of all hydronephrosis affecting pregnancy. About 1-2% of all pregnancies, the incidence is estimated at 1:750 1:1500 live births. Currently, they are diagnosed with prenatal ultrasound in the second trimester [18].
UVJO: It is an obstruction at the level of the end of the ureter where it enters the bladder. It is the result of an anatomical or a functional abnormality in the distal segment of the ureter [19]. It is a well-known fact that congenital anomalies of the upper and lower urinary tracts are among the most common anomalies in the body. All of these anomalies have been reported frequently and separately, but the novelty of our presentation is the collection of several anomalies in an asymptomatic male, collagen accumulation with muscle hypertrophy around the distal ureter has been identified as the cause of UVJO [20]. UVJO is the second most common cause of hydronephrosis in newborns, accounting for approximately 20% of cases, with an estimated incidence of 36 per 100000 live births [19].
Conclusion
Anomalies of the urinary tract may compromise the functional or conduit parts of the urinary tract. Multiple anomalies can be seen accidentally in an asymptomatic patient. In this reported case, renal ectopia, double collecting system, UPJO, UVJO were seen and also data suggested reflux of the lower moiety ureter into the upper moiety ureter, because the lower moiety ureter was narrow while the other was dilated.
Acknowledgments: I thank Dr Zafar Masoumi (Department of Radiology, Beheshti Teaching Hospital) for his constructive suggestions and for performing sonography and IVP.
Ethical Permission: Approval for the study was obtained from the Ethical Committee in Biomedical Research (Yasuj University of Medical Sciences: IR.YUMS.REC.1399.184) and written informed consent was obtained from the study participant. The patient has undergone all stages of diagnosis and therapy in accordance with the standard protocol
Conflicts of Interests: The author declares that they have no competing interests.
Authors’ Contribution: Rabani SMR (First Author), Introduction Writer/Methodologist/Main Researcher/Discussion Writer/Statistical Analyst (100%)
Funding/Support: Funding information is not applicable.
Congenital anomalies of the urinary tract have a wide spectrum including upper and lower urinary tract anomalies such as renal hypoplasia, dysplasia, aplasia, ectopia, supernumerary, duplication, ureteropelvic junction obstruction (Ureter-pelvic Junction Obstruction: UPJO), ureterovesical junction obstruction (Uretero-vesical Junction Obstruction: UVJO), vesicoureteral reflux (Vesico-ureteral Reflux: VUR), ureterocele, posterior urethral valve, bladder exstrophy, and many other anomalies. The causes of these wide ranges of urinary tract anomalies are related to abnormal embryologic development of the urinary tract [1, 2]. The urinary tract system is substantially derived from the intermediate mesoderm [3, 4], and based on the underlying defect and the disturbances in the developmental process, different types of congenital anomalies may develop in the urinary system.
Patient and Methods
A 19-year-old army male presented with left lower quadrant (LLQ) abdominal pain after blunt abdominal trauma, accompanied by gross hematuria. He was healthy and asymptomatic until he had blunt abdominal trauma during sports activity and developed pain and gross hematuria.
Written informed consent for publication of the clinical details and clinical images were obtained from the patient. The datasets used during the current study are available from the corresponding author on reasonable request.
Findings
On arrival at the emergency room, physical examination revealed LLQ tenderness with stable vital signs. Primary laboratory data showed normal renal function tests and gross hematuria. Ultrasonography suggested ectopic pelvic left kidney with hydronephrosis and UPJO with distal ureteral dilatation. Intravenous pyelography confirmed the same findings (Figure 1). Renal DTPA (Diethylene Triamine Penta Acetic Acid) scanning was done that suggested ectopia, a double collecting system, lower moiety UPJO, and a common distal ureter with UVJO (Figure 2). Cystoscopy and trying to perform ureteroscopy failed to pass a catheter into the left ureter that was a plan for retrograde uretero-pyelography because the UVJ area was very narrow. Exploration was done through Gibson’s retroperitoneal incision. The upper moiety ureter was significantly dilated, but the lower moiety was thin and narrow; Both of them ended in a tapered segment typical for an adynamic segment and UVJO. Pyeloplasty and ureteroneocystostomy were carried out simultaneously. The patient has an uneventful post-operative 18-month follow-up.
Figure 1. IVP (Intravenous Pyelography) suggestive for functional, ectopic, irregular, left kidney with hydroureteronephrosis
Figure 2. Renal DTPA scanning confirms the IVP findings
Discussion
The development of the urinary tract is a very complex process and depends on regular and precise events. Normal development of the urinary system also needs the integration of a variety of cell populations that have different embryologic origins [1, 3]. The final products of these events are the functional and conduit parts of the urinary system. The evolution of our knowledge in genetics and molecular biology has greatly advanced our understanding of the pathophysiology of urinary tract congenital anomalies [5, 6]. Although anomalies of the urinary tract are among the most common anomalies of the body, there are also a great number of varieties of anomalies in the upper and lower urinary tract systems. The upper urinary tract is affected by congenital anomalies and structural anomalies (such as continuous fetal lobulation, hypertrophied Bertin column and dromedary hump), fusion anomalies (such as horseshoe kidney and pancake kidney), renal position anomalies (such as malrotation of the kidney, simple renal ectopy and crossed renal ectopy), renal number (e.g. renal agenesis and extra kidney), and abnormalities in the development of the urinary collecting system (e.g. pyelocalyceal diverticulum, megacalycosis, ureteropelvic junction obstruction, collecting system duplex, megaverter, ectopic ureter, and ureter) are classified [7]. Therefore, understanding the embryonic development and the anatomical structure of the upper urinary tract is very important for a better understanding of these cases.
In this patient, we accidentally encountered unilateral multiple renal congenital anomalies: Ectopia; Double collecting system; UPJO, UVJO, and as an operation finding there was a suggestion for the lower moiety ureter reflux to the upper moiety ureter.
Each of these anomalies has its own pathophysiology and clinical presentation, which will be discussed briefly.
Renal ectopia: The cause of renal ectopia may be a defect in the ascent process of the kidney or an abnormal ureteric bud. An ectopic kidney may be asymptomatic for life. The incidence of renal ectopia is between one in 2200 to one in 3000 autopsies, and it occurs between the 6th and 9th weeks of gestation. The most common position is opposite the sacrum and below the aortic bifurcation [7, 8]. Van Den Bosch et al.'s study diagnosed kidney ectopy in 41 cases, in which 32% of the patients were diagnosed during prenatal screening. Urological abnormalities were found in 66% of patients and vesicoureteral reflux was found in 13 cases. that there was no adverse effect on blood pressure or kidney function in children with renal ectopy. However, periodic follow-up seems necessary, at least until adulthood [9].
Duplicated system: This may be complete or partial. The incidence and prevalence of urinary tract duplication have been reported to be 0.7-4% of the population [10]. It is the result of the unusual development of the ureteral bud. In a complete duplicated system, the Wiegert-Meyer rule can be seen in 95% of the cases with consequences of vesicoureteral reflux and ureterocele or ectopic ureter. The duplication system is more common in women than in men with a ratio of 2:1 [11]. It can be asymptomatic or associated with bladder reflux (VUR), urinary incontinence, obstructive uropathy as well as renal parenchymal scarring or dysplasia and reduction [10]. Awareness of duplex abnormalities and awareness according to the symptoms observed in all imaging methods, especially ultrasound. Then, understanding the basic rules governing duplex abnormalities is important to guide a thoughtful evaluation of the child [10]. Dino et al. report a 35-year-old woman who presented with left flank pain. A CT scan of the abdomen has shown a complete duplex system on the left side with hydroureteronephrosis of the lower part and an obstructing middle ureteral stone. It states that the kidney was a complete duplex. It is rare to have lower half hydronephrosis caused by medial ureteral stone [12].
UPJO: Ureter-pelvic junction obstruction (UPJO) is the most common cause of hydronephrosis in sick children. They are usually diagnosed in the perinatal period as hydronephrosis in routine ultrasound, and are characterized by symptoms of abdominal discomfort, flank pain, or vomiting in childhood [13]. It accounts for about 50% of all intrauterine detectable hydronephrosis and it is really the most common cause of congenital obstructive nephropathy [14, 15]. Minimally invasive surgery (MIS), initially with laparoscopic pyeloplasty (LP) and then with robot-assisted laparoscopic pyeloplasty (RALP), has played an important role in the treatment of UPJO in the past 10 years [16, 17]. UPJO accounts for 11% of all hydronephrosis affecting pregnancy. About 1-2% of all pregnancies, the incidence is estimated at 1:750 1:1500 live births. Currently, they are diagnosed with prenatal ultrasound in the second trimester [18].
UVJO: It is an obstruction at the level of the end of the ureter where it enters the bladder. It is the result of an anatomical or a functional abnormality in the distal segment of the ureter [19]. It is a well-known fact that congenital anomalies of the upper and lower urinary tracts are among the most common anomalies in the body. All of these anomalies have been reported frequently and separately, but the novelty of our presentation is the collection of several anomalies in an asymptomatic male, collagen accumulation with muscle hypertrophy around the distal ureter has been identified as the cause of UVJO [20]. UVJO is the second most common cause of hydronephrosis in newborns, accounting for approximately 20% of cases, with an estimated incidence of 36 per 100000 live births [19].
Conclusion
Anomalies of the urinary tract may compromise the functional or conduit parts of the urinary tract. Multiple anomalies can be seen accidentally in an asymptomatic patient. In this reported case, renal ectopia, double collecting system, UPJO, UVJO were seen and also data suggested reflux of the lower moiety ureter into the upper moiety ureter, because the lower moiety ureter was narrow while the other was dilated.
Acknowledgments: I thank Dr Zafar Masoumi (Department of Radiology, Beheshti Teaching Hospital) for his constructive suggestions and for performing sonography and IVP.
Ethical Permission: Approval for the study was obtained from the Ethical Committee in Biomedical Research (Yasuj University of Medical Sciences: IR.YUMS.REC.1399.184) and written informed consent was obtained from the study participant. The patient has undergone all stages of diagnosis and therapy in accordance with the standard protocol
Conflicts of Interests: The author declares that they have no competing interests.
Authors’ Contribution: Rabani SMR (First Author), Introduction Writer/Methodologist/Main Researcher/Discussion Writer/Statistical Analyst (100%)
Funding/Support: Funding information is not applicable.
Keywords:
References
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