Congenital urethral anomalies in boys. Part I: Posterior Urethral valves

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. 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. 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. 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

Congenital urethral anomalies in boys. Part II

In the second part of this article, congenital urethral anomalies other than posterior urethral valve were reviewed. The anomalies considered in the current review were anterior urethral valve, lacuna magna, syringocele, Cobb's collar, duplication of urethra, megalourethra, and prostatic urethral polyps. The literature was extensively reviewed concerning the presentations, diagnosis, different types of treatment modalities, morbidity, mortality, and new concepts for the above disorders. Anterior urethral valves or diverticula are the most prevalent congenital anomalies of anterior urethra. The lacuna magna is the largest depression in the dorsal aspect of the fossa navicularis. It is demonstrable on a well-performed voiding cystourethrography of the distal urethra. The dilated Cowper's gland duct is the other missed diagnosed anomaly of the urethra in boys. The congenital narrowing of the bulbar urethra with a variable clinical presentation and obstruction grade and different types of anterior urethral obstruction are the most common presentation of these anomalies. However, other symptoms or signs including, hematuria, bloody spotting on underwear, discomfort or sever pain in the vicinity of the glance, interrupted voiding, infection, bulging of anterior urethra, enuresis, and postvoiding dribbling are the only nonspecific manifestations of these disorders. All of these disorders are demonstrable on a well-performed voiding cystourethrography of the distal urethra. The urologist must be aware about these uncommon congenital anomalies and the anterior urethra should be carefully evaluated for such anomalies. Diagnosis of these entities is elusive unless the physician is looking for them. Nonspecific symptoms mentioned here besides radiographic findings can be a valuable clue for diagnosis