Clinical presentation and diagnosis of posterior urethral valves
- Nicholas Holmes, MD
Nicholas Holmes, MD
- Clinical Professor of Surgery
- University of California, San Diego
Posterior urethral valves (PUV) are obstructing membranous folds within the lumen of the posterior urethra that are the most common etiology of urinary tract obstruction in the newborn male, occurring in 1 in 5000 to 8000 pregnancies (figure 1) [1,2]. PUV are also the most common cause of chronic renal disease due to urinary tract obstruction in children .
The diagnosis and presentation of patients with posterior urethral valves will be reviewed here. The management of these patients is discussed separately. (See "Management of posterior urethral valves".)
The pathogenesis of posterior urethral valves (PUV) appears to be due to an obstructing persistent urogenital membrane (figure 1). Although the exact mechanism resulting in this obstruction is not completely understood , it appears that the normal embryologic development of the male urethra between weeks 9 and 14 of gestation is disrupted.
Normal male urethral embryology — Between weeks four and six of gestation, the cloaca is divided into the anorectal canal and the urogenital sinus. Over the following weeks of gestation, the urogenital sinus is divided by the entrance of the mesonephric duct into the cephalad vesicourethral canal, giving rise to the bladder and pelvic urethra. The caudal portion of the urogenital sinus (genital tubercle) forms the phallic urethra. As the genital tubercle elongates in the male fetus, the urethral groove and folds are created. As the penile urethra grows, it moves towards the urethral plate of the glans penis. Complete fusion of the entire urethra occurs at about 14 weeks gestation.
PUV embryology — Several disruptions of the male embryologic urethral development have been proposed as the mechanism resulting in PUV . These are based upon published anatomical descriptions in the literature and include the following :
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- Chitrit Y, Bourdon M, Korb D, et al. Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? Prenat Diagn 2016; 36:831.
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- Ghanem MA, Wolffenbuttel KP, De Vylder A, Nijman RJ. Long-term bladder dysfunction and renal function in boys with posterior urethral valves based on urodynamic findings. J Urol 2004; 171:2409.
- Bomalaski MD, Anema JG, Coplen DE, et al. Delayed presentation of posterior urethral valves: a not so benign condition. J Urol 1999; 162:2130.
- Ziylan O, Oktar T, Ander H, et al. The impact of late presentation of posterior urethral valves on bladder and renal function. J Urol 2006; 175:1894.
- Engel DL, Pope JC 4th, Adams MC, et al. Risk factors associated with chronic kidney disease in patients with posterior urethral valves without prenatal hydronephrosis. J Urol 2011; 185:2502.
- Sarhan O, Zaccaria I, Macher MA, et al. Long-term outcome of prenatally detected posterior urethral valves: single center study of 65 cases managed by primary valve ablation. J Urol 2008; 179:307.
- DeFoor W, Clark C, Jackson E, et al. Risk factors for end stage renal disease in children with posterior urethral valves. J Urol 2008; 180:1705.
- Taskinen S, Heikkilä J, Rintala R. Posterior urethral valves: primary voiding pressures and kidney function in infants. J Urol 2009; 182:699.
- Wells JM, Mukerji S, Chandran H, et al. Urinomas protect renal function in posterior urethral valves--a population based study. J Pediatr Surg 2010; 45:407.
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- Heikkilä J, Rintala R, Taskinen S. Vesicoureteral reflux in conjunction with posterior urethral valves. J Urol 2009; 182:1555.
- Heikkilä J, Taskinen S, Toppari J, Rintala R. Posterior urethral valves are often associated with cryptorchidism and inguinal hernias. J Urol 2008; 180:715.
- Normal male urethral embryology
- PUV embryology
- CLINICAL MANIFESTATIONS
- - Prenatal
- - Postnatal
- Renal and urologic manifestations
- - Chronic kidney disease
- - Vesicoureteral reflux
- - Bladder dysfunction
- - Other urologic findings
- Associated anomalies
- Other studies
- - Radionuclide scans
- - Laboratory studies
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS