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Posterior Urethral Valves
Priyanka Jha, MBBS
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Posterior urethral valves (PUV)
                • Bladder outlet obstruction (BOO)
              • Definitions

                • Urethral membrane acts as valve, resulting in lower urinary tract obstruction (LUTO)
                  • Obstruction is usually partial
                  • Posterior urethra obstructed by valves
                  • Occurs exclusively in males

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Johal N et al: Contractile function of detrusor smooth muscle from children with posterior urethral valves - the role of fibrosis. J Pediatr Urol. 17(1):100.e1-e10, 2021
                                          2. Buffin-Meyer B et al: A single-center study to evaluate the efficacy of a fetal urine peptide signature predicting postnatal renal outcome in fetuses with posterior urethral valves. Pediatr Nephrol. 35(3):469-75, 2020
                                          3. Dębska M et al: Urethroplasty with balloon catheterization in fetal lower urinary tract obstruction: observational study of 10 fetuses. Ultrasound Obstet Gynecol. 56(6):916-20, 2020
                                          4. Expert Panel on Pediatric Imaging et al: ACR Appropriateness Criteria® antenatal hydronephrosis-infant. J Am Coll Radiol. 17(11S):S367-79, 2020
                                          5. Garel J et al: The perineal midsagittal view in male fetuses - pivotal for assessing genitourinary disorders. Pediatr Radiol. 50(4):575-82, 2020
                                          6. Strizek B et al: Vesicoamniotic shunting for fetal megacystis in the first trimester with a Somatex® intrauterine shunt. Arch Gynecol Obstet. 302(1):133-40, 2020
                                          7. Vinit N et al: Biometric and morphological features on magnetic resonance imaging of fetal bladder in lower urinary tract obstruction: new perspectives for fetal cystoscopy. Ultrasound Obstet Gynecol. 56(1):86-95, 2020
                                          8. Vinit N et al: Fetal cystoscopy and vesicoamniotic shunting in lower urinary tract obstruction: long-term outcome and current technical limitations. Fetal Diagn Ther. 47(1):74-83, 2020
                                          9. Brownlee E et al: Current epidemiology and antenatal presentation of posterior urethral valves: Outcome of BAPS CASS National Audit. J Pediatr Surg. 54(2):318-21, 2019
                                          10. Cohen J et al: Fetal cardiac findings and hemodynamic changes associated with severe lower urinary tract obstruction in utero. Ultrasound Obstet Gynecol. 54(6):780-85, 2019
                                          11. Fontanella F et al: Antenatal workup of early megacystis and selection of candidates for fetal therapy. Fetal Diagn Ther. 45(3):155-61, 2019
                                          12. Fontanella F et al: Antenatal staging of congenital lower urinary tract obstruction. Ultrasound Obstet Gynecol. 53(4):520-4, 2019
                                          13. Graupner O et al: Myocardial function in fetuses with lower urinary tract obstruction: is there a cardiac remodeling effect due to renal damage? Prenat Diagn. 39(7):495-504, 2019
                                          14. Jain S et al: Developmental pathology of congenital kidney and urinary tract anomalies. Clin Kidney J. 12(3):382-99, 2019
                                          15. Kajbafzadeh AM et al: Prognostic significance of maternal urinary carbohydrate antigen 19-9 for antenatal diagnosis of posterior urethral valve associated with fetal hydronephrosis. Int Urol Nephrol. 51(6):909-15, 2019
                                          16. Lundar L et al: Prenatal extravasation of urine seems to preserve renal function in boys with posterior urethral valves. J Pediatr Urol. 15(3):241.e1-e7, 2019
                                          17. Mani S et al: Urinary ascites and transient intestinal obstruction in a preterm infant: an interesting case of posterior urethral valve. AJP Rep. 9(3):e209-12, 2019
                                          18. Nassr AA et al: Outcome of fetuses with lower urinary tract obstruction and normal amniotic fluid volume in second trimester of pregnancy. Ultrasound Obstet Gynecol. 54(4):500-5, 2019
                                          19. Vasconcelos MA et al: Posterior urethral valves: comparison of clinical outcomes between postnatal and antenatal cohorts. J Pediatr Urol. 15(2):167.e1-e8, 2019
                                          20. Woolf AS et al: Congenital disorders of the human urinary tract: recent insights from genetic and molecular studies. Front Pediatr. 7:136, 2019
                                          21. Clayton DB et al: Current state of fetal intervention for lower urinary tract obstruction. Curr Urol Rep. 19(1):12, 2018
                                          22. Fonseca EKUN et al: Keyhole sign in posterior urethral valve. Abdom Radiol (NY). 43(9):2517-8, 2018
                                          23. Jeong BD et al: Perinatal outcomes of fetal lower urinary tract obstruction after vesicoamniotic shunting using a double-basket catheter. J Ultrasound Med. 37(9):2147-56, 2018
                                          24. Johnson MP et al: Natural history of fetal lower urinary tract obstruction with normal amniotic fluid volume at initial diagnosis. Fetal Diagn Ther. 44(1):10-7, 2018
                                          25. Kim SJ et al: Long-term outcomes of kidney and bladder function in patients with a posterior urethral valve. Medicine (Baltimore). 97(23):e11033, 2018
                                          26. Moscardi PRM et al: Prenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction. J Pediatr Urol. 14(4):320.e1-320.e6, 2018
                                          27. Prat E et al: Neonate with urinary ascites but no hydronephrosis: unusual presentation of posterior urethral valves. BMJ Case Rep., 2018
                                          28. Rosenblatt J et al: Prenatal diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome by biochemical analysis of fetal urine. Prenat Diagn. 38(8): 585-90, 2018
                                          29. Farrugia MK et al: Report on The Society for Fetal Urology panel discussion on the selection criteria and intervention for fetal bladder outlet obstruction. J Pediatr Urol. 13(4):345-51, 2017
                                          30. Faure A et al: Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination. Prenat Diagn. 37(7):666-72, 2017
                                          31. Harper L et al: Can quantity of amniotic fluid reliably predict postnatal renal function in boys with posterior urethral valves: a decision curve analysis. Prenat Diagn. 37(9):931-34, 2017
                                          32. Spaggiari E et al: Sequential fetal serum β2-microglobulin to predict postnatal renal function in bilateral or low urinary tract obstruction. Ultrasound Obstet Gynecol. 49(5):617-22, 2017
                                          33. Taghavi K et al: Fetal megacystis: A systematic review. J Pediatr Urol. 13(1):7-15, 2017
                                          34. Vieira ÉLM et al: Posterior urethral valve in fetuses: evidence for the role of inflammatory molecules. Pediatr Nephrol. 32(8):1391-400, 2017
                                          35. Chitrit Y et al: Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? Prenat Diagn. 36(9):831-7, 2016
                                          36. Faure A et al: DNA copy number variants: a potentially useful predictor of early onset renal failure in boys with posterior urethral valves. J Pediatr Urol. 12(4):227.e1-7, 2016
                                          37. Ruano R et al: Fetal lower urinary tract obstruction: proposal for standardized multidisciplinary prenatal management based on disease severity. Ultrasound Obstet Gynecol. 48(4):476-82, 2016
                                          38. Smith-Harrison LI et al: Current applications of in utero intervention for lower urinary tract obstruction. J Pediatr Urol. 11(6): 341-7, 2015
                                          39. Sarhan OM et al: Did antenatal diagnosis protect against chronic kidney disease in patients with posterior urethral valves? A multicenter study. Urology. 82(6):1405-9, 2013
                                          40. Ansari MS et al: Risk factors for progression to end-stage renal disease in children with posterior urethral valves. J Pediatr Urol. 6(3):261-4, 2010
                                          41. Bernardes LS et al: Keyhole sign: how specific is it for the diagnosis of posterior urethral valves? Ultrasound Obstet Gynecol. 34(4):419-23, 2009
                                          42. Kousidis G et al: The long-term outcome of prenatally detected posterior urethral valves: a 10 to 23-year follow-up study. BJU Int. 102(8):1020-4, 2008
                                          43. Sarhan O et al: Long-term outcome of prenatally detected posterior urethral valves: single center study of 65 cases managed by primary valve ablation. J Urol. 179(1):307-12; discussion 312-3, 2008
                                          44. Robyr R et al: Correlation between ultrasound and anatomical findings in fetuses with lower urinary tract obstruction in the first half of pregnancy. Ultrasound Obstet Gynecol. 25(5):478-82, 2005
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Posterior urethral valves (PUV)
                                                        • Bladder outlet obstruction (BOO)
                                                      • Definitions

                                                        • Urethral membrane acts as valve, resulting in lower urinary tract obstruction (LUTO)
                                                          • Obstruction is usually partial
                                                          • Posterior urethra obstructed by valves
                                                          • Occurs exclusively in males

                                                      IMAGING

                                                      • General Features

                                                        • Ultrasonographic Findings

                                                          • MR Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Johal N et al: Contractile function of detrusor smooth muscle from children with posterior urethral valves - the role of fibrosis. J Pediatr Urol. 17(1):100.e1-e10, 2021
                                                                                  2. Buffin-Meyer B et al: A single-center study to evaluate the efficacy of a fetal urine peptide signature predicting postnatal renal outcome in fetuses with posterior urethral valves. Pediatr Nephrol. 35(3):469-75, 2020
                                                                                  3. Dębska M et al: Urethroplasty with balloon catheterization in fetal lower urinary tract obstruction: observational study of 10 fetuses. Ultrasound Obstet Gynecol. 56(6):916-20, 2020
                                                                                  4. Expert Panel on Pediatric Imaging et al: ACR Appropriateness Criteria® antenatal hydronephrosis-infant. J Am Coll Radiol. 17(11S):S367-79, 2020
                                                                                  5. Garel J et al: The perineal midsagittal view in male fetuses - pivotal for assessing genitourinary disorders. Pediatr Radiol. 50(4):575-82, 2020
                                                                                  6. Strizek B et al: Vesicoamniotic shunting for fetal megacystis in the first trimester with a Somatex® intrauterine shunt. Arch Gynecol Obstet. 302(1):133-40, 2020
                                                                                  7. Vinit N et al: Biometric and morphological features on magnetic resonance imaging of fetal bladder in lower urinary tract obstruction: new perspectives for fetal cystoscopy. Ultrasound Obstet Gynecol. 56(1):86-95, 2020
                                                                                  8. Vinit N et al: Fetal cystoscopy and vesicoamniotic shunting in lower urinary tract obstruction: long-term outcome and current technical limitations. Fetal Diagn Ther. 47(1):74-83, 2020
                                                                                  9. Brownlee E et al: Current epidemiology and antenatal presentation of posterior urethral valves: Outcome of BAPS CASS National Audit. J Pediatr Surg. 54(2):318-21, 2019
                                                                                  10. Cohen J et al: Fetal cardiac findings and hemodynamic changes associated with severe lower urinary tract obstruction in utero. Ultrasound Obstet Gynecol. 54(6):780-85, 2019
                                                                                  11. Fontanella F et al: Antenatal workup of early megacystis and selection of candidates for fetal therapy. Fetal Diagn Ther. 45(3):155-61, 2019
                                                                                  12. Fontanella F et al: Antenatal staging of congenital lower urinary tract obstruction. Ultrasound Obstet Gynecol. 53(4):520-4, 2019
                                                                                  13. Graupner O et al: Myocardial function in fetuses with lower urinary tract obstruction: is there a cardiac remodeling effect due to renal damage? Prenat Diagn. 39(7):495-504, 2019
                                                                                  14. Jain S et al: Developmental pathology of congenital kidney and urinary tract anomalies. Clin Kidney J. 12(3):382-99, 2019
                                                                                  15. Kajbafzadeh AM et al: Prognostic significance of maternal urinary carbohydrate antigen 19-9 for antenatal diagnosis of posterior urethral valve associated with fetal hydronephrosis. Int Urol Nephrol. 51(6):909-15, 2019
                                                                                  16. Lundar L et al: Prenatal extravasation of urine seems to preserve renal function in boys with posterior urethral valves. J Pediatr Urol. 15(3):241.e1-e7, 2019
                                                                                  17. Mani S et al: Urinary ascites and transient intestinal obstruction in a preterm infant: an interesting case of posterior urethral valve. AJP Rep. 9(3):e209-12, 2019
                                                                                  18. Nassr AA et al: Outcome of fetuses with lower urinary tract obstruction and normal amniotic fluid volume in second trimester of pregnancy. Ultrasound Obstet Gynecol. 54(4):500-5, 2019
                                                                                  19. Vasconcelos MA et al: Posterior urethral valves: comparison of clinical outcomes between postnatal and antenatal cohorts. J Pediatr Urol. 15(2):167.e1-e8, 2019
                                                                                  20. Woolf AS et al: Congenital disorders of the human urinary tract: recent insights from genetic and molecular studies. Front Pediatr. 7:136, 2019
                                                                                  21. Clayton DB et al: Current state of fetal intervention for lower urinary tract obstruction. Curr Urol Rep. 19(1):12, 2018
                                                                                  22. Fonseca EKUN et al: Keyhole sign in posterior urethral valve. Abdom Radiol (NY). 43(9):2517-8, 2018
                                                                                  23. Jeong BD et al: Perinatal outcomes of fetal lower urinary tract obstruction after vesicoamniotic shunting using a double-basket catheter. J Ultrasound Med. 37(9):2147-56, 2018
                                                                                  24. Johnson MP et al: Natural history of fetal lower urinary tract obstruction with normal amniotic fluid volume at initial diagnosis. Fetal Diagn Ther. 44(1):10-7, 2018
                                                                                  25. Kim SJ et al: Long-term outcomes of kidney and bladder function in patients with a posterior urethral valve. Medicine (Baltimore). 97(23):e11033, 2018
                                                                                  26. Moscardi PRM et al: Prenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction. J Pediatr Urol. 14(4):320.e1-320.e6, 2018
                                                                                  27. Prat E et al: Neonate with urinary ascites but no hydronephrosis: unusual presentation of posterior urethral valves. BMJ Case Rep., 2018
                                                                                  28. Rosenblatt J et al: Prenatal diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome by biochemical analysis of fetal urine. Prenat Diagn. 38(8): 585-90, 2018
                                                                                  29. Farrugia MK et al: Report on The Society for Fetal Urology panel discussion on the selection criteria and intervention for fetal bladder outlet obstruction. J Pediatr Urol. 13(4):345-51, 2017
                                                                                  30. Faure A et al: Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination. Prenat Diagn. 37(7):666-72, 2017
                                                                                  31. Harper L et al: Can quantity of amniotic fluid reliably predict postnatal renal function in boys with posterior urethral valves: a decision curve analysis. Prenat Diagn. 37(9):931-34, 2017
                                                                                  32. Spaggiari E et al: Sequential fetal serum β2-microglobulin to predict postnatal renal function in bilateral or low urinary tract obstruction. Ultrasound Obstet Gynecol. 49(5):617-22, 2017
                                                                                  33. Taghavi K et al: Fetal megacystis: A systematic review. J Pediatr Urol. 13(1):7-15, 2017
                                                                                  34. Vieira ÉLM et al: Posterior urethral valve in fetuses: evidence for the role of inflammatory molecules. Pediatr Nephrol. 32(8):1391-400, 2017
                                                                                  35. Chitrit Y et al: Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? Prenat Diagn. 36(9):831-7, 2016
                                                                                  36. Faure A et al: DNA copy number variants: a potentially useful predictor of early onset renal failure in boys with posterior urethral valves. J Pediatr Urol. 12(4):227.e1-7, 2016
                                                                                  37. Ruano R et al: Fetal lower urinary tract obstruction: proposal for standardized multidisciplinary prenatal management based on disease severity. Ultrasound Obstet Gynecol. 48(4):476-82, 2016
                                                                                  38. Smith-Harrison LI et al: Current applications of in utero intervention for lower urinary tract obstruction. J Pediatr Urol. 11(6): 341-7, 2015
                                                                                  39. Sarhan OM et al: Did antenatal diagnosis protect against chronic kidney disease in patients with posterior urethral valves? A multicenter study. Urology. 82(6):1405-9, 2013
                                                                                  40. Ansari MS et al: Risk factors for progression to end-stage renal disease in children with posterior urethral valves. J Pediatr Urol. 6(3):261-4, 2010
                                                                                  41. Bernardes LS et al: Keyhole sign: how specific is it for the diagnosis of posterior urethral valves? Ultrasound Obstet Gynecol. 34(4):419-23, 2009
                                                                                  42. Kousidis G et al: The long-term outcome of prenatally detected posterior urethral valves: a 10 to 23-year follow-up study. BJU Int. 102(8):1020-4, 2008
                                                                                  43. Sarhan O et al: Long-term outcome of prenatally detected posterior urethral valves: single center study of 65 cases managed by primary valve ablation. J Urol. 179(1):307-12; discussion 312-3, 2008
                                                                                  44. Robyr R et al: Correlation between ultrasound and anatomical findings in fetuses with lower urinary tract obstruction in the first half of pregnancy. Ultrasound Obstet Gynecol. 25(5):478-82, 2005