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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

Abstract

Introduction

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.

Results

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).

Conclusions

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

Keywords

Hypospadias Disorders of sex development Urethroplasty 

Abbreviations

HP

Hypospadias

DSD

Disorders of sex development

UP

Urethroplasty

Post-UPC

Post-UP complications

UCF

Urethrocutaneous fistula

UDT

Undescended testis

SF-1

Steroidogenic factor-1

Notes

Funding

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.

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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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