Skip to main content
SpringerLink
Log in

Hypospadias

  • Living reference work entry
  • First Online:
Pediatric Surgery

  • 37 Accesses

Abstract

The hypospadiac genital tubercle (GT) is the consequence of a development arrest of the tissues forming its ventral aspect, which occurs during the “masculinization window” (6–16 weeks of gestation). The causes of this common congenital malformation are essentially unknown and probably multifactorial. Four main factors may be responsible for hypospadias: (1) the child’s genes and hormonal system; (2) the placenta, which plays an essential role during the masculinization window; (3) the mother; and (4) the environment, with its potential hormonal disruptors. Assessment of the hypospadiac GT is a crucial step to explore the causes and plan surgery which, in some cases, can be preceded by hormonal stimulation. Surgery entails three main steps: (1) degloving the GT to identify the level of division of the corpus spongiosum, which is the main criteria to define hypospadias severity, i.e., the degree of dysplasia of the ventral tissues and consequently the degree of ventral curvature; (2) choosing the adequate technique of urethroplasty which is dependent on the quality of the urethral plate, the availability of preputial tissue, the length of urethra to be reconstructed, the size of the glans and depth of the glans groove; and (3) covering the straightened GT with a redistribution of the penile skin. Evaluation of the outcome is another critical step in this management and includes functional and cosmetic results until adulthood. One essential characteristic of pediatric urology/surgery is that reconstructions are performed on growing organs.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Abosena W, Talab SS, Hanna MK. Recurrent chordee in 59 adolescents and young adults following childhood hypospadias repair. J Pediatr Urol. 2020;16(2):162.e1–5.

    Article  PubMed  Google Scholar 

  • Akre O, Boyd HA, Ahlgren M, Wilbrand K, Westergaard T, Hjalgrim H, Nordenskjöld A, Ekbom A, Melbye M. Maternal and gestational risk factors for hypospadias. Environ Health Perspect. 2008;116:1071–6.

    Article  PubMed  PubMed Central  Google Scholar 

  • Asklund C, Jorgensen N, Skakkebaek NE, Jensen TK. Increased frequency of reproductive health problems among fathers of boys with hypospadias. Hum Reprod. 2007;22:2639–46.

    Article  CAS  PubMed  Google Scholar 

  • Beck C. Operative treatment of hypospadias. Ann Surg. 1899;30:536.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Belgorosky A, Guercio G, Pepe C, Saraco N, Rivarola MA. Genetic and clinical spectrum of aromatase deficiency in infancy, childhood and adolescence. Horm Res. 2009;72(6):321–30.

    CAS  PubMed  Google Scholar 

  • Boas M, Boisen KA, Virtanen HE, Kaleva M, Suomi AM, Schmidt IM, Damgaard IN, Kai CM, Chellakoory M, Skakkebaek NE, Toppari J, Main KM. Postnatal penile length and growth rate correlate to serum testosterone levels: a longitudinal study of 1962 normal boys. Eur J Endocrinol. 2006;154:125–9.

    Article  CAS  PubMed  Google Scholar 

  • Bougnères P, Porcher R, Esterle L, Baker D, de la Vaissière A, Meurisse S, Valtat S, Castell AL, Mouriquand P, Valleron AJ. Exploring the risk of hypospadias in children born from mothers living close to a vineyard. PLoS One. 2021;16(4):e0249800.

    Article  PubMed  PubMed Central  Google Scholar 

  • Bracka A. Hypospadias repair: the two-stage alternative. Br J Urol. 1995;76:31–41.

    Article  PubMed  Google Scholar 

  • Carmichael SL, Shaw GM, Laurent C, Olney RS, Lammer EJ. National Birth Defects Prevention Study. Maternal reproductive and demographic characteristics as risk factors for hypospadias. Paediatr Perinat Epidemiol. 2007;21:210–8.

    Article  PubMed  Google Scholar 

  • Carmichael SL, Cogswell ME, Ma C, Gonzalez-Feliciano A, Olney RS, Correa A, Shaw GM, National Brth Defects Prevention Study. Hypospadias and maternal intake of phytoestrogens. Am J Epidemiol. 2013;178:434–40.

    Article  PubMed  PubMed Central  Google Scholar 

  • Chalapathi G, Rao KL, Chowdhary SK, Narasimhan KL, Samuih R, Mahajan JK. Testosterone therapy in microphallic hypospadias: topical or parenteral? J Pediatr Surg. 2003;38(2):221–3.

    Article  CAS  PubMed  Google Scholar 

  • Chang C, Reifsnyder JE, Fahmy AG, Hanna MK. The adolescent glans penis: functional and aesthetic issues following childhood hypospadias repair. J Pediatr Urol. 2021;17(5):609.e1–8.

    Article  PubMed  Google Scholar 

  • Cohen-Kettenis PT. Gender change in 46, XY persons with 5alpha-reductase-2 deficiency and 17beta-hydroxysteroid dehydrogenase-3 deficiency. Arch Sex Behav. 2005;34:399–410.

    Article  PubMed  Google Scholar 

  • Cools M. Germ cell cancer risk in DSD patients. Ann Endocrinol. 2014;75(2):67–71.

    Article  Google Scholar 

  • Cox MJ, Coplen DE, Austin PF. The incidence of sexual differentiation and chrosomal abnormalities of cryptorchidism and hypospadias stratified by meatal location. J Urol. 2008;180:2649–52.

    Article  PubMed  Google Scholar 

  • de Mattos e Silva E, Gorduza DB, Catti M, Valmalle AF, Demède D, Hameury F, Mure PY, Mouriquand P. Outcome of severe hypospadias repair using three different techniques. J Pediatr Urol. 2009;5(3):205–11.

    Article  PubMed  Google Scholar 

  • Duckett JW. In: Frank JD, Gearhart JP, Snyder III HM, editors. Hypospadias repair. Operative pediatric; 2002.

    Google Scholar 

  • Duplay S. Sur le traitement chirurgical de l’hypospadias et de l’épispadias. Arch Gen Med. 1880;145:257–63.

    Google Scholar 

  • Ergun-Longmire B, Vinci G, Alonso L, Matthew S, Tansil S, Lin-Su K, McElreavey K, New MI. Clinical, hormonal and cytogenetic evaluation of 46,XX males and review of the literature. J Pediatr Endocrinol Metab. 2005;18(8):739–48.

    Article  CAS  PubMed  Google Scholar 

  • Fisch H, Lambert SM, Hensle TW, Hyun G. Hypospadias rates in New York state are not increasing. J Urol. 2009;181:2291–4.

    Article  PubMed  Google Scholar 

  • Forest MG, Cathiard AM, Bertrand JA. Evidence of testicular activity in early infancy. J Clin Endocrinol Metab. 1973;37:148–51.

    Article  CAS  PubMed  Google Scholar 

  • Fredell L, Lichtenstein P, Pedersen NL, Svensson J, Nordenskjold A. Hypospadias is related to birth weight in discordant monozygotic twins. J Urol. 1998;160:2197–9.

    Article  CAS  PubMed  Google Scholar 

  • Fujimoto T, Suwa T, Kabe K, Adachi T, Nakabayashi M, Amamiya T. Placental insufficiency in early gestation is associated with hypospadias. J Pediatr Surg. 2008;43:358–61.

    Article  PubMed  Google Scholar 

  • Gamidov S, Shatylko T, Gasanov N, Popova A, Dusmukhamedov R, Safiullin R, Sukhikh G. Sexual dysfunction in patients with late complications of hypospadias surgery. Andrologia. 2022:e14413.

    Google Scholar 

  • Gilliver SC, Ashworth JJ, Ashcroft GS. The hormonal regulation of cutaneous wound healing. Clin Dermatol. 2007;25:56–62.

    Article  PubMed  Google Scholar 

  • Gorduza DB, Gay CL, de Mattos E, Silva E, Demède D, Hameury F, Berthiller J, Mure PY, Mouriquand PD. Does androgen stimulation prior to hypospadias surgery increase the rate of healing complications? – a preliminary report. J Pediatr Urol. 2011;7(2):158–61.

    Article  PubMed  Google Scholar 

  • Grosos C, Bensaid R, Gorduza DB, Mouriquand P. Is it safe to solely use ventral penile tissues in hypospadias repair? Long-term outcome of 578 Duplay urethroplasties performed in one single institution over a period of 14 years. J Pediatr Urol. 2014;in press.

    Google Scholar 

  • Grumbach MM. A window of opportunity: the diagnosis of gonadotropin deficiency in the male infant. J Clin Endocrinol Metab. 2005;90:3122–7.

    Article  CAS  PubMed  Google Scholar 

  • Hughes IA, Houk C, Ahmed SF, Lee PA. Lawson Wilkins Pediatric Endocrine Society/European Society for Pediatric Endocrinology Consensus Group. Consensus statement on management of intersex disorders. J Pediatr Urol. 2006;2:148–62.

    Article  CAS  PubMed  Google Scholar 

  • Husmann DA, Cain MP. Microphallus: eventual phallic size is dependent on the timing of androgen administration. J Urol. 1994;152:734–9.

    Article  CAS  PubMed  Google Scholar 

  • Imperato-McGinley J, Gautier T, Peterson RE, Shacleton C. The prevalence of 5 alpha-reductase deficiency in children with ambiguous genitalia in the Dominican Republic. J Urol. 1986;136:867–73.

    Article  CAS  PubMed  Google Scholar 

  • J-P. Mettauer Am J Med Sci (ns) 1842;4:43.

    Google Scholar 

  • Josso N, Picard JY, Rey R, di Clemente N. Testicular anti-Mullerian hormone: history, genetics, regulation and clinical applications. Pediatr Endocrinol Rev. 2006;3(4):347–58.

    PubMed  Google Scholar 

  • Kalfa N, Philibert P, Sultan C. Is hypospadias a genetic, endocrine or environmental disease, or still an unexplained malformation? Int J Androl. 2009;32:187–97.

    Article  CAS  PubMed  Google Scholar 

  • Kaya C, Bektic J, Radmayr C, Schwentner C, Bartsch G, Oswald J. The efficacy of dihydrotestosterone transdermal gel before primary hypospadias surgery: a prospective, controlled, randomized study. J Urol. 2008;179(2):684–8.

    Article  CAS  PubMed  Google Scholar 

  • Koff SA. Mobilisation of the urethra in the surgical treatment of hypospadias. J Urol. 1981;125:394–7.

    Article  CAS  PubMed  Google Scholar 

  • Koff SA, Jayanthi VR. Preoperative treatment with human chorionic gonadotropin in infancy decreases the severity of proximal hypospadias and chordee. J Urol. 1999;162(4):1435–9.

    Article  CAS  PubMed  Google Scholar 

  • Koyanagi T, Matsuno T, Nonomura K, Sakakibara N. Complete repair of severe penoscrotal hypospadias in 1 stage: experience with urethral mobilization, wing flap-flipping urethroplasty and “glanulomeatoplasty”. J Urol. 1983;130:1150–4.

    Article  CAS  PubMed  Google Scholar 

  • Laron Z, Klinger B. Effect of insulin-like growth factor-I treatment on serum androgens and testicular and penile size in males with Laron syndrome (primary growth hormone resistance). Eur J Endocrinol. 1998;138(2):176–80.

    Article  CAS  PubMed  Google Scholar 

  • Looijenga LH, Hersmus R, Oosterhuis JW, Cools M, Drop SL, Wolffenbuttel KP. Tumor risk in disorders of sex development (DSD). Best Pract Res Clin Endocrinol Metab. 2007;21(3):480–95.

    Article  CAS  PubMed  Google Scholar 

  • Macleod DJ, Sharpe RM, Welsh M, Fisken M, Scott HM, Hutchison GR, Drake AJ, van den Driesche S. Androgen action in the masculinization programming window and development of male reproductive organs. Int J Androl. 2010;33(2):279–87.

    Article  CAS  PubMed  Google Scholar 

  • Main KM, Skakkebaek NE, Virtanene HE, Toppari J. Genital anomalies in boys and the environment. Best Pract Res Clin Endocrinol Metab. 2010;24:279–89.

    Article  PubMed  Google Scholar 

  • Malik RD, Liu DB. Survey of pediatric urologists on the preoperative use of testosterone in the surgical correction of hypospadias. J Pediatr Urol. 2014;pii: S1477–5131(14)00087-4. https://doi.org/10.1016/j.jpurol.2014.02.008. [Epub ahead of print].

  • Mathieu P. Traitement en un temps de l’hypospade balanique ou juxtabalanique. J Chir. 1932;39:481–4.

    Google Scholar 

  • Monfort G, Lucas C. Dihydrotestosterone penile stimulation in hypospadias surgery. Eur Urol. 1982;8(4):201–3.

    Article  CAS  PubMed  Google Scholar 

  • Morel Y, Plotton I, Mallet D, Nicolino M, Bertrand AM, David M, Nihoul-Fekete C, Chatelain P. Studies of a cohort of 46,XY with DSD including steroid biosynthesis deficiencies. Adv Exp Med Biol 2011;707:15–7.

    Google Scholar 

  • Morera AM, Valmalle AF, Asensio MJ, Chossegros L, Chauvin MA, Durand P, Mouriquand PD. A study of risk factors for hypospadias in the Rhône-Alpes region (France). J Pediatr Urol. 2006;2:169–77.

    Article  CAS  PubMed  Google Scholar 

  • Mouriquand PD. Etiological aspects of hypospadias. Dialogues Pediatr Urol. 2007;28:4.

    Google Scholar 

  • Mouriquand P, Snodgrass W, Canning D, Khoury A, Hayashi Y. Posterior hypospadias. Dialogues Pediatr Urol. 2008:29.

    Google Scholar 

  • Mouriquand PDE, Demede D, Gorduza S, Mure PY. Hypospadias. In: Gearhart, Rink, Mouriquand, editors. Pediatric urology. 2nd ed. Saunders Elsevier Publishers; 2009. p. 526–43.

    Google Scholar 

  • Mouriquand P, Gorduza DB, Noché ME, Targnion A. Long-term outcome of hypospadias surgery: current dilemmas. Curr Opin Urol. 2011;21:465–9.

    Article  PubMed  Google Scholar 

  • Nerli RB, Koura A, Prabha V, Reddy M. Comparison of topical versus parenteral testosterone in children with microphallic hypospadias. Pediatr Surg Int. 2009;25(1):57–9.

    Article  CAS  PubMed  Google Scholar 

  • Netto JM, Ferrarez CE, Schindler Leal AA, Tucci S Jr, Gomes CA, Barroso U Jr. Hormone therapy in hypospadias surgery: a systematic review. J Pediatr Urol. 2013;9:971–9.

    Article  PubMed  Google Scholar 

  • Nordenvall AS, Frisen L, Nordenstrom A, Lichtenstein P. Nordenskjold. Population based nationwide study of hypospadias in Sweden, 1973 to 2009: incidence and risk factors. J Urol. 2014;(191):783–9.

    Google Scholar 

  • Nørgaard M, Wogelius P, Pedersen L, Rothman KJ, Sørensen HT. Maternal use of oral contraceptives during early pregnancy and risk of hypospadias in male offspring. Urology. 2009;74:583–7.

    Article  PubMed  Google Scholar 

  • North K, Golding J. A maternal vegetarian diet in pregnancy is associated with hypospadias. The ALSPAC study team. Avon longitudinal study of pregnancy and childhood. BJU Int. 2000;85:107–13.

    Article  CAS  PubMed  Google Scholar 

  • Paulozzi LJ, Erickson JD, Jackson RJ. Hypospadias trends in two US surveillance systems. Pediatrics. 1997;100(5):831–4.

    Article  CAS  PubMed  Google Scholar 

  • Penington EC, Huston JM. The cloacal plate: the missing link in anorectal and urogenital development. BJU Int. 2002;89:726–32.

    Article  CAS  PubMed  Google Scholar 

  • Snodgrass W, Bush N. Recurrent ventral curvature after proximal TIP hypospadias repair. J Pediatr Urol. 2021;17(2):222.e1–5.

    Article  PubMed  Google Scholar 

  • Snodgrass WT, Lorenzo A. Tubularized incised-plate urethroplasty for proximal hypospadias. BJU Int. 2002;89:90–3.

    Article  CAS  PubMed  Google Scholar 

  • Thiry S, Gorduza D, Mouriquand P. Urethral advancement in hypospadias with a distal division of corpus spongiosum: outcome in 158 cases. J Pediatr Urol. 2014;10(3):451–4. https://doi.org/10.1016/j.jpurol.2013.10.019. Epub 2013 Nov 16

    Article  CAS  PubMed  Google Scholar 

  • Tosetti S, De Queiroz SM, Chassard D, Mouriquand P. Hypospadias surgery and locoregional anesthesia: vice or virtue? Paediatr Anaesth. 2013;23(1):100–1.

    Article  PubMed  Google Scholar 

  • Vidal I, Gorduza DB, Haraux E, Gay CL, Chatelain P, Nicolino M, Mure PY, Mouriquand P. Surgical options in disorders of sex development (dsd) with ambiguous genitalia. Best Pract Res Clin Endocrinol Metab. 2010;24(2):311–24.

    Article  PubMed  Google Scholar 

  • Welsh M, Saunders PT, Fisken M, Scott HM, Hutchison GR, Smith LB, Sharpe RM. Identification in rats of a programming window for reproductive tract masculinization, disruption of which leads to hypospadias and cryptorchidism. J Clin Invest. 2008;118:1479–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Université Claude-Bernard – Lyon 1, Villeurbanne, France

    Pierre Mouriquand & Pierre-Yves Mure

  2. Mère-Enfant – Groupe Hospitalier Est – Service d’Urologie Pédiatrique, Hospices Civils de Lyon, Bron, France

    Pierre Mouriquand, Daniela Brindusa Gorduza & Pierre-Yves Mure

Authors
  1. Pierre Mouriquand
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Daniela Brindusa Gorduza
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Pierre-Yves Mure
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Pierre Mouriquand .

Editor information

Editors and Affiliations

  1. Beacon Hospital, University College Dublin, Dublin, Ireland

    Prem Puri

Rights and permissions

Reprints and permissions

Copyright information

© 2022 Springer-Verlag GmbH Germany, part of Springer Nature

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Mouriquand, P., Gorduza, D.B., Mure, PY. (2022). Hypospadias. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_191-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-38482-0_191-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-38482-0

  • Online ISBN: 978-3-642-38482-0

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation