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.
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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
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Ochi, T., Ishiyama, A., Yazaki, Y. et al. Surgical management of hypospadias in cases with concomitant disorders of sex development. Pediatr Surg Int 35, 611–617 (2019). https://doi.org/10.1007/s00383-019-04457-6
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DOI: https://doi.org/10.1007/s00383-019-04457-6