Surgical management of hypospadias in cases with concomitant disorders of sex development

  • Takanori OchiEmail author
  • Asuka Ishiyama
  • Yuta Yazaki
  • Hiroshi Murakami
  • Masahiro Takeda
  • Shogo Seo
  • Ryo Sueyoshi
  • Geoffrey J. Lane
  • Hidenori Haruna
  • Toshiaki Shimizu
  • Atsuyuki Yamataka
Original Article



To review the surgical treatment of hypospadias (HP) associated with disorders of sex development (DSD).

Patients and methods

HP cases were assessed for DSD by gross examination for atypical external genitalia, and assessment of hormone levels and karyotype. There were 58 HP cases with concomitant DSD treated between 1999 and 2017. DSD classification, type of HP, sex assignment, hormonal abnormality, surgical strategy, and post-urethroplasty complications (post-UPC) were reviewed.


DSD were sex chromosome abnormalities (n = 4), 46,XY (n = 51), 46,XX (n = 1), and 47,XY + 21 (n = 2). HP was perineal: (n = 26), scrotal: (n = 16), penoscrotal: (n = 15), and midshaft: (n = 1); repair was primary (n = 6) or staged (n = 52). Mean age at final urethroplasty (UP) was 4.12 ± 0.21 years; all cases had soft tissue interposition at UP. At mean follow-up 5.16 ± 0.56 years after final UP, observed post-UPC (n = 8; 13.8%) were urethral stenosis (n = 3), urethral diverticulum (n = 2), urethrocutaneous fistula (n = 2), and curvature (n = 1). Mean onset of post-UPC was 1.24 ± 0.77 years (range 0.1–6.3). The second half of our cases (n = 29; treated 2015 ~) had significantly less post-UPC (0/29; 0%) than the first half (8/29; 27.6%) (p = 0.0075).


Although UP for HP + DSD was formidably challenging, we achieved a significant decrease in post-UPC through a combination of surgical techniques and experience.


Hypospadias Disorders of sex development Urethroplasty 





Disorders of sex development




Post-UP complications


Urethrocutaneous fistula


Undescended testis


Steroidogenic factor-1



This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Conflict of interest

The authors have no financial conflicts of interest.


  1. 1.
    Hughes IA, Houk C, Ahmed SF et al (2006) Consensus statement on management of intersex disorders. J Pediatr Urol 2:148–162CrossRefPubMedGoogle Scholar
  2. 2.
    Lambert SM, Vilain EJ, Kolon TF (2010) A practical approach to ambiguous genitalia in the newborn period. Urol Clin N Am 37:195–205CrossRefGoogle Scholar
  3. 3.
    Baskin LS, Ebbers MB (2006) Hypospadias: anatomy, etiology, and technique. J Pediatr Surg 41:463–472CrossRefPubMedGoogle Scholar
  4. 4.
    Sircili MH, e Silva FA, Costa EM et al (2010) Long-term surgical outcome of masculinizing genitoplasty in large cohort of patients with disorders of sex development. J Urol 184:1122–1127CrossRefPubMedGoogle Scholar
  5. 5.
    Chertin B, Koulikov D, Hadas-Halpern I et al (2005) Masculinizing genitoplasty in intersex patients. J Urol 174:1683–1686CrossRefPubMedGoogle Scholar
  6. 6.
    Sueyoshi R, Seo S, Ochi T et al (2016) Reinforcing the ventral penile shaft with pedicled fat/connective tissues before urethroplasty lowers the risk for post-urethroplasty complications in hypospadias. J Pediatr Surg 51:2021–2024CrossRefPubMedGoogle Scholar
  7. 7.
    McNamara ER, Schaeffer AJ, Logvinenko T et al (2015) Management of proximal hypospadias with 2-stage repair: 20-year experience. J Urol 194:1080–1085CrossRefPubMedGoogle Scholar
  8. 8.
    Ishiyama A, Seo S, Murakami H et al (2015) Staged segmental urethroplasty for scrotal/perineal hypospadias: a new concept. Pediatr Surg Int 32:403–409CrossRefPubMedGoogle Scholar
  9. 9.
    Seo S, Ochi T, Yazaki Y et al (2015) Soft tissue interposition is effective for protecting the neourethra during hypospadias surgery and preventing postoperative urethrocutaneous fistula: a single surgeon’s experience of 243 cases. Pediatr Surg Int 31:297–303CrossRefPubMedGoogle Scholar
  10. 10.
    Yamataka A, Ando K, Lane GJ et al (1998) Pedicled external spermatic fascia flap for urethroplasty in hypospadias and closure of urethrocutaneous fistula. J Pediatr Surg 33:1788–1789CrossRefPubMedGoogle Scholar
  11. 11.
    Snodgrass WT, Nguyen MT (2002) Current technique of tubularized incised plate hypospadias repair. Urology 60:157–162CrossRefPubMedGoogle Scholar
  12. 12.
    Yamataka A, Shimotakahara A, Koga H et al (2012) Modified Snodgrass tubularized incised plate urethroplasty prevents fistula formation in hypospadias. Pediatr Surg Int 28:919–923CrossRefPubMedGoogle Scholar
  13. 13.
    Retik AB, Mandell J, Bauer SB et al (1994) Meatal based hypospadias repair with the use of a dorsal subcutaneous flap to prevent urethrocutaneous fistula. J Urol 152:1229–1231CrossRefPubMedGoogle Scholar
  14. 14.
    Pippi Salle JL, Sayed S, Salle A et al (2016) Proximal hypospadias: a persistent challenge. Single institution outcome analysis of three surgical techniques over a 10-year period. J Pediatr Urol 12:28 e21–e27CrossRefGoogle Scholar
  15. 15.
    Gupta D, Bhardwaj M, Sharma S et al (2010) Long-term psychosocial adjustments, satisfaction related to gender and the family equations in disorders of sexual differentiation with male sex assignment. Pediatr Surg Int 26:955–958CrossRefPubMedGoogle Scholar
  16. 16.
    Castagnetti M, El-Ghoneimi A (2011) The influence of perioperative factors on primary severe hypospadias repair. Nat Rev Urol 8:198–206CrossRefPubMedGoogle Scholar
  17. 17.
    No authors listed (1996) Timing of elective surgery on the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesthesia. American Academy of Pediatrics. Pediatrics 97:590–594Google Scholar
  18. 18.
    Migeon CJ, Wisniewski AB, Gearhart JP et al (2002) Ambiguous genitalia with perineoscrotal hypospadias in 46,XY individuals: long-term medical, surgical, and psychosexual outcome. Pediatrics 110:e31CrossRefPubMedGoogle Scholar
  19. 19.
    Garnier S, Maillet O, Cereda B et al (2017) Late surgical correction of hypospadias increases the risk of complications: a series of 501 consecutive patients. BJU Int 119:942–947CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Takanori Ochi
    • 1
    Email author
  • Asuka Ishiyama
    • 1
  • Yuta Yazaki
    • 1
  • Hiroshi Murakami
    • 1
  • Masahiro Takeda
    • 1
  • Shogo Seo
    • 1
  • Ryo Sueyoshi
    • 1
  • Geoffrey J. Lane
    • 1
  • Hidenori Haruna
    • 2
  • Toshiaki Shimizu
    • 2
  • Atsuyuki Yamataka
    • 1
  1. 1.Department of Pediatric General and Urogenital SurgeryJuntendo University School of MedicineTokyoJapan
  2. 2.Department of Pediatrics and Adolescent MedicineJuntendo University School of MedicineTokyoJapan

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