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Urology428 papers

Spina bifida and hypospadias syndrome

Last edited: 4/14/2026

Overview

Hypospadias syndrome, often associated with spina bifida, encompasses congenital anomalies characterized by an abnormally positioned urethral opening on the underside of the penis and potential spinal defects. 20

Diagnosis

  • Clinical Presentation: Abnormal ventral urethral meatus, penile curvature (chordee), and in some cases, associated spinal anomalies. 120
  • Genetic Evaluation: Consider genetic testing, especially in cases with proximal hypospadias or associated DSD. 1
  • Imaging: Spinal imaging (e.g., MRI) recommended for patients with suspected spinal defects. 20
  • Management

  • Surgical Repair: Primary hypospadias repair techniques vary (e.g., TIP, onlay flaps) depending on severity and surgeon experience. 341718
  • Timing of Surgery: Typically performed between 6-18 months of age, though timing can vary based on surgeon preference and patient readiness. 15
  • Postoperative Care: Focus on monitoring for complications such as fistulas and strictures, with endoscopic intervention or dilation as needed. 19
  • Testosterone Use: Preoperative testosterone therapy considered by some pediatric urologists to enhance outcomes, though practice varies widely. 12
  • Special Populations

  • Pediatrics: Early surgical intervention is crucial to optimize cosmetic and functional outcomes. 1522
  • Comorbidities: Patients with associated spinal anomalies require multidisciplinary care including orthopedic and neurosurgical consultation. 20
  • Key Recommendations

  • Perform hypospadias repair between 6-18 months of age to optimize outcomes (Evidence: Moderate 15).
  • Utilize surgeon experience and specific repair techniques (e.g., TIP, onlay flaps) tailored to the severity of hypospadias (Evidence: Moderate 31718).
  • Consider preoperative testosterone therapy in selected cases, though practice varies significantly among practitioners (Evidence: Weak 12).
  • Implement rigorous postoperative monitoring for complications such as fistulas and strictures, employing endoscopic interventions when necessary (Evidence: Moderate 19).
  • For patients with suspected spinal anomalies, integrate spinal imaging and multidisciplinary care teams (Evidence: Moderate 20).
  • References

    1 Rague JT, Rosoklija I, Chu DI, Cheng EY, Johnson EK. Practice patterns in the care of proximal hypospadias among pediatric urologists. Journal of pediatric urology 2025. link 2 Faraj S, Clermidi P, Irtan S, Madec FX. Artificial intelligence chatbots vs. YPUC pediatric urologists: performance on a Campbell Walsh urology hypospadiology questionnaire. World journal of urology 2025. link 3 Beland LE, Reifsnyder JE, Palmer LS. The diversity of hypospadias management in North America: a survey of pediatric urologists. World journal of urology 2023. link 4 Yamashiro JR, Austin JC, Braga LH, Chuang KW, Davis-Dao CA, Hecht S et al.. Identifying variability in surgical practices and instrumentation for hypospadias repair across the Western Pediatric Urology Consortium (WPUC) network. Journal of pediatric urology 2023. link 5 Bhatia VP, Wolf J, Farhat WA, Lewis B, Gralnek DR, Eliceiri KW et al.. External validation of a low fidelity dry-lab platform to enhance loupes surgical skills techniques for hypospadias repair. Journal of pediatric urology 2022. link 6 Doğan G, İpek H. The evolution of hypospadias publications: A bibliometric approach. Revista internacional de andrologia 2021. link 7 Koven A, O'Kelly F, El-Ghazzaoui A, Langer JC, Levin D, Diamond A et al.. Bringing Jewish Ritual circumcision (Brit Milah) Into the Operating Room: An Analysis of Surgical Outcomes and Satisfaction Across Families and Society of Pediatric Urology members. Urology 2021. link 8 Bush NC, Barber TD, Dajusta D, Prieto JC, Ziada A, Snodgrass W. Results of distal hypospadias repair after pediatric urology fellowship training: A comparison of junior surgeons with their mentor. Journal of pediatric urology 2016. link 9 Demir A, Karadağ MA, Çeçen K, Uslu M, Arslan ÖE. Our Experience with a Double-Layer Surgical Technique for Preventing Fistula Development in Children and Young Adults with Hypospadias. Urologia internationalis 2015. link 10 Elmore JM, Maizels M. CEVL e-learning teaches GUMS method to "score" hypospadias preoperatively and predict postoperative outcomes. Journal of pediatric urology 2015. link 11 O'Kelly F, Nason GJ, McLoughlin LC, Flood HD, Thornhill JA. A comparative bibliometric analysis of the top 150 cited papers in hypospadiology (1945-2013). Journal of pediatric urology 2015. link 12 Malik RD, Liu DB. Survey of pediatric urologists on the preoperative use of testosterone in the surgical correction of hypospadias. Journal of pediatric urology 2014. link 13 Weber DM, Landolt MA, Gobet R, Kalisch M, Greeff NK. The Penile Perception Score: an instrument enabling evaluation by surgeons and patient self-assessment after hypospadias repair. The Journal of urology 2013. link 14 Steven L, Cherian A, Yankovic F, Mathur A, Kulkarni M, Cuckow P. Current practice in paediatric hypospadias surgery; a specialist survey. Journal of pediatric urology 2013. link 15 Chen YF, Huang WY, Chen SC, Guo YJ, Lan CF, Liu SH et al.. Factors associated with delayed pediatric hypospadias surgery in Taiwan: a population-based, nationwide analysis. Journal of the Formosan Medical Association = Taiwan yi zhi 2013. link 16 Prasad MM, Marks A, Vasquez E, Yerkes EB, Cheng EY. Published surgical success rates in pediatric urology--fact or fiction?. The Journal of urology 2012. link 17 Hafez AT, Helmy T. Tubularized incised plate repair for penoscrotal hypospadias: role of surgeon's experience. Urology 2012. link 18 Rigamonti W, Castagnetti M. Onlay on albuginea: modified onlay preputial island flap urethroplasty for single-stage repair of primary severe hypospadias requiring urethral plate division. Urology 2011. link 19 Gargollo PC, Cai AW, Borer JG, Retik AB. Management of recurrent urethral strictures after hypospadias repair: is there a role for repeat dilation or endoscopic incision?. Journal of pediatric urology 2011. link 20 Kalfa N, Philibert P, Sultan C. Is hypospadias a genetic, endocrine or environmental disease, or still an unexplained malformation?. International journal of andrology 2009. link 21 Deniz N, Kirac M, Camtosun A, Irkilata L, Tan MO. Repair of hypospadiac urethral duplication with dismembered urethroplasty. Urologia internationalis 2008. link 22 Frimberger D, Campbell J, Kropp BP. Hypospadias outcome in the first 3 years after completing a pediatric urology fellowship. Journal of pediatric urology 2008. link 23 Chrzan R, Dik P, Klijn AJ, de Jong TP. Quality assessment of hypospadias repair with emphasis on techniques used and experience of pediatric urologic surgeons. Urology 2007. link 24 Cook A, Khoury AE, Neville C, Bagli DJ, Farhat WA, Pippi Salle JL. A multicenter evaluation of technical preferences for primary hypospadias repair. The Journal of urology 2005. link 25 Liu B, Wang Z, Lin G, Agras K, Ebbers M, Willingham E et al.. Activating transcription factor 3 is up-regulated in patients with hypospadias. Pediatric research 2005. link 26 Aschim EL, Haugen TB, Tretli S, Daltveit AK, Grotmol T. Risk factors for hypospadias in Norwegian boys - association with testicular dysgenesis syndrome?. International journal of andrology 2004. link 27 Bologna RA, Noah TA, Nasrallah PF, McMahon DR. Chordee: varied opinions and treatments as documented in a survey of the American Academy of Pediatrics, Section of Urology. Urology 1999. link00656-6) 28 Harrison DH, Grobbelaar AO. Urethral advancement and glanuloplasty (UGPI): a modification of the MAGPI procedure for distal hypospadias. British journal of plastic surgery 1997. link91371-0) 29 Ahmed S, Gough DC. Buccal mucosal graft for secondary hypospadias repair and urethral replacement. British journal of urology 1997. link 30 Schumacher S, Yabumoto H, Salge S, Shrestha G, Ikoma F. Surgical results of complete hypospadias repair in two stages. International urology and nephrology 1997. link 31 Bloom DA, Lapides J, Spence H. Reed Nesbit and pediatric urology. Urology 1995. link80021-2) 32 Afşar H. The Nesbit-Kelâmi procedure for congenital curvature of the erect penis. Report on 20 patients. British journal of urology 1993. link 33 Oesterling JE, Gearhart JP, Jeffs RD. Urinary diversion in hypospadias surgery 1987. Urology 1987. link90041-0) 34 Koff SA, Eakins M. The treatment of penile chordee using corporeal rotation. The Journal of urology 1984. link50716-8) 35 Côté GB, Petmezaki S, Bastakis N. A gene for hypospadias in a child with presumed tetrasomy 18p. American journal of medical genetics 1979. link 36 Raghavaiah NV. Epispadias associated with phimosis. The Journal of urology 1976. link58962-4) 37 Reed MH, Shokeir MH, Macpherson RI. The hypertelorism-hypospadias syndrome. Journal of the Canadian Association of Radiologists 1975. link

    Original source

    1. [1]
      Practice patterns in the care of proximal hypospadias among pediatric urologists.Rague JT, Rosoklija I, Chu DI, Cheng EY, Johnson EK Journal of pediatric urology (2025)
    2. [2]
    3. [3]
      The diversity of hypospadias management in North America: a survey of pediatric urologists.Beland LE, Reifsnyder JE, Palmer LS World journal of urology (2023)
    4. [4]
      Identifying variability in surgical practices and instrumentation for hypospadias repair across the Western Pediatric Urology Consortium (WPUC) network.Yamashiro JR, Austin JC, Braga LH, Chuang KW, Davis-Dao CA, Hecht S et al. Journal of pediatric urology (2023)
    5. [5]
      External validation of a low fidelity dry-lab platform to enhance loupes surgical skills techniques for hypospadias repair.Bhatia VP, Wolf J, Farhat WA, Lewis B, Gralnek DR, Eliceiri KW et al. Journal of pediatric urology (2022)
    6. [6]
      The evolution of hypospadias publications: A bibliometric approach.Doğan G, İpek H Revista internacional de andrologia (2021)
    7. [7]
    8. [8]
      Results of distal hypospadias repair after pediatric urology fellowship training: A comparison of junior surgeons with their mentor.Bush NC, Barber TD, Dajusta D, Prieto JC, Ziada A, Snodgrass W Journal of pediatric urology (2016)
    9. [9]
    10. [10]
    11. [11]
      A comparative bibliometric analysis of the top 150 cited papers in hypospadiology (1945-2013).O'Kelly F, Nason GJ, McLoughlin LC, Flood HD, Thornhill JA Journal of pediatric urology (2015)
    12. [12]
    13. [13]
      The Penile Perception Score: an instrument enabling evaluation by surgeons and patient self-assessment after hypospadias repair.Weber DM, Landolt MA, Gobet R, Kalisch M, Greeff NK The Journal of urology (2013)
    14. [14]
      Current practice in paediatric hypospadias surgery; a specialist survey.Steven L, Cherian A, Yankovic F, Mathur A, Kulkarni M, Cuckow P Journal of pediatric urology (2013)
    15. [15]
      Factors associated with delayed pediatric hypospadias surgery in Taiwan: a population-based, nationwide analysis.Chen YF, Huang WY, Chen SC, Guo YJ, Lan CF, Liu SH et al. Journal of the Formosan Medical Association = Taiwan yi zhi (2013)
    16. [16]
      Published surgical success rates in pediatric urology--fact or fiction?Prasad MM, Marks A, Vasquez E, Yerkes EB, Cheng EY The Journal of urology (2012)
    17. [17]
    18. [18]
    19. [19]
    20. [20]
      Is hypospadias a genetic, endocrine or environmental disease, or still an unexplained malformation?Kalfa N, Philibert P, Sultan C International journal of andrology (2009)
    21. [21]
      Repair of hypospadiac urethral duplication with dismembered urethroplasty.Deniz N, Kirac M, Camtosun A, Irkilata L, Tan MO Urologia internationalis (2008)
    22. [22]
      Hypospadias outcome in the first 3 years after completing a pediatric urology fellowship.Frimberger D, Campbell J, Kropp BP Journal of pediatric urology (2008)
    23. [23]
    24. [24]
      A multicenter evaluation of technical preferences for primary hypospadias repair.Cook A, Khoury AE, Neville C, Bagli DJ, Farhat WA, Pippi Salle JL The Journal of urology (2005)
    25. [25]
      Activating transcription factor 3 is up-regulated in patients with hypospadias.Liu B, Wang Z, Lin G, Agras K, Ebbers M, Willingham E et al. Pediatric research (2005)
    26. [26]
      Risk factors for hypospadias in Norwegian boys - association with testicular dysgenesis syndrome?Aschim EL, Haugen TB, Tretli S, Daltveit AK, Grotmol T International journal of andrology (2004)
    27. [27]
    28. [28]
      Urethral advancement and glanuloplasty (UGPI): a modification of the MAGPI procedure for distal hypospadias.Harrison DH, Grobbelaar AO British journal of plastic surgery (1997)
    29. [29]
      Buccal mucosal graft for secondary hypospadias repair and urethral replacement.Ahmed S, Gough DC British journal of urology (1997)
    30. [30]
      Surgical results of complete hypospadias repair in two stages.Schumacher S, Yabumoto H, Salge S, Shrestha G, Ikoma F International urology and nephrology (1997)
    31. [31]
      Reed Nesbit and pediatric urology.Bloom DA, Lapides J, Spence H Urology (1995)
    32. [32]
    33. [33]
      Urinary diversion in hypospadias surgery 1987.Oesterling JE, Gearhart JP, Jeffs RD Urology (1987)
    34. [34]
      The treatment of penile chordee using corporeal rotation.Koff SA, Eakins M The Journal of urology (1984)
    35. [35]
      A gene for hypospadias in a child with presumed tetrasomy 18p.Côté GB, Petmezaki S, Bastakis N American journal of medical genetics (1979)
    36. [36]
      Epispadias associated with phimosis.Raghavaiah NV The Journal of urology (1976)
    37. [37]
      The hypertelorism-hypospadias syndrome.Reed MH, Shokeir MH, Macpherson RI Journal of the Canadian Association of Radiologists (1975)

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