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Published on: October 12, 2017
Congenital urethral anomalies in boys. Part I: posterior urethral valves
1Department of Pediatric Urology, Children's Hospital Medical Center, Tehran University of Medical Sciences, Tehran, Iran. kajbafzd@sina.tums.ac.ir.
Insights
Congenital urethral anomalies are complex, with subtypes and comorbidities potentially causing chronic kidney disease. Understanding fetal obstruction effects on bladder and kidney function is crucial for effective treatment.
Area of Science:
- Pediatric Urology
- Nephrology
- Developmental Biology
Background:
- Congenital urethral anomalies represent a spectrum of conditions, not a single disease entity.
- Posterior urethral valve (PUV) subtypes and associated comorbidities can lead to chronic renal failure in children.
- Fetal lower urinary tract obstruction significantly impacts postnatal bladder and renal function.
Purpose of the Study:
- To review evidence on PUV subtypes and comorbidities contributing to chronic kidney disease.
- To describe the effects of fetal urinary obstruction on postnatal bladder and renal function.
- To compare current findings with conventional approaches to congenital urethral anomalies.
Main Methods:
- Extensive literature review of congenital urethral outlet obstruction, including presentations, diagnosis, and treatments.
- Evaluation of 739 published papers on posterior urethral valves, with a focus on a quarter of those.
- Analysis of radiologic presentations from Iranian patients treated over 25 years.
Main Results:
- Significant overlap in presentations observed prior to widespread antenatal diagnosis.
- The natural history of these anomalies is becoming clearer.
- The posterior urethral diaphragm hypothesis is gaining traction compared to older classifications.
Conclusions:
- Further molecular research is essential to elucidate the pathophysiology of renal and bladder dysfunction in congenital urethral obstruction.
- Management requires urologists with specialized expertise in valves and rare congenital urethral anomalies.
- Antenatal diagnosis and understanding of fetal obstruction impacts are critical.
Introduction:
The aims of this review are one, to consider that congenital urethral anomalies are not a simple disease entity in all patients. This is accomplished by reviewing the evidence for presence of posterior urethral valve subtypes and comorbidity of various unexplained clinical conditions in some children leading to chronic renal failure. The review's second aim is to describe the effects of fetal lower urinary tract obstruction on postnatal bladder function and the consequence of bladder dysfunction on the remaining postnatal renal function.
Materials And Methods:
The literature was extensively reviewed concerning the different types of congenital urethral outlet obstruction presentations, diagnosis, different types of treatment modalities, morbidity, mortality, and new concepts for this old problem. These findings were compared with conventional approaches to these anomalies. The 739 published papers on posterior urethral valves were evaluated, and a quarter of those are addressed. All radiologic presentations and figures in this review were selected from among the records of Iranian patients treated by the author during the last 25 years.
Results:
A significant overlap of presentation before antenatally diagnosed era was observed. The natural history of these anomalies is becoming clear and the hypothesis of posterior urethral diaphragm is popular among several investigators in comparison to the original valves classification by Young in 1903.
Conclusion:
Further molecular investigation of the urinary tract is needed to better understand the pathophysiology of renal and bladder function in children who are born with antenatally diagnosed congenital urethral obstruction. These anomalies must be treated by urologists with a vast experience with valves and other rare congenital urethral anomalies.
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