Advertisement

Post-operative complications following masculinizing genitoplasty in moderate to severe genital atypia: results from a multicenter, observational prospective cohort study

Published:February 18, 2021DOI:https://doi.org/10.1016/j.jpurol.2021.02.006

      Summary

      Introduction

      Differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. A phenotype of severe genital atypia in patients raised as male is a relatively rare occurrence and standards for management are lacking. Decision making for early surgical planning in these rare cases includes, but is not limited to, degree of atypia, location of testes, and presence of Mϋllerian remnants. In this study we describe surgical approaches and short-term outcomes for masculinizing genitoplasty in moderate to severe genital atypia in young patients raised male, for whom parents opted for early surgery.

      Methods

      This NIH-sponsored study is an ongoing, observational, multicenter investigation assessing medical, surgical and psychological outcomes in children and their parents affected by atypical genitalia due to DSD. Participants were prospectively enrolled from 12 children's hospitals across the United States that specialize in DSD care. Criteria for child enrollment were a Quigley score of 3–6 in those with a 46, XY or 45,X/46, XY chromosome complement, age <3 years with no previous genitoplasty; patients were included independent of whether genitoplasty was performed. Cosmesis was graded according to a 4-point Likert scale and complications per the Clavian-Dindo classification.

      Results

      Of the 31 participants, 30 underwent hypospadias repair and 1 patient did not undergo a genitoplasty procedure. The majority of participants (22) received a staged hypospadias repair. Seventeen complications were identified in 12 of the 31 children (41%) at 12 months of follow up. Glans dehiscence and urethrocutaneous fistula were the most common complications. Orchiopexy was performed in 14 (44%) and streak gonads were removed in 4 (13%) participants. Both parents and surgeons reported improved cosmesis after surgery when compared to baseline.

      Conclusion

      Summary Figure
      Graphical AbstractRiver plot comparing Likert cosmesis score over time for mothers, fathers, and surgeons. Panel A includes all patients in the cohort.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Pediatric Urology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Sandberg D.E.
        • Pasterski V.
        • Callens N.
        Introduction to the special section: disorders of sex development.
        J Pediatr Psychol. 2017; 42: 487-495
        • Mouriquand P.D.
        • Gorduza D.B.
        • Gay C.L.
        • Meyer-Bahlburg H.F.
        • Baker L.
        • Baskin L.S.
        • et al.
        Surgery in disorders of sex development (DSD) with a gender issue: if (why), when, and how?.
        J Pediatr Urol. 2016; 12: 139-149
        • Sullivan K.J.
        • Hunter Z.
        • Andrioli V.
        • Guerra L.
        • Leonard M.
        • Klassen A.
        • et al.
        Assessing quality of life of patients with hypospadias: a systematic review of validated patient-reported outcome instruments.
        J Pediatr Urol. 2017; 13: 19-27
        • Ellens R.E.H.
        • Bakula D.M.
        • Mullins A.J.
        • Scott Reyes K.J.
        • Austin P.
        • Baskin L.
        • et al.
        Psychological adjustment of parents of children born with atypical genitalia 1 Year after genitoplasty.
        J Urol. 2017; 198: 914-920
        • Sharkey C.M.
        • Bakula D.M.
        • Wolfe-Christensen C.
        • Austin P.
        • Baskin L.
        • Bernabe K.J.
        • et al.
        Parent-rated severity of illness and anxiety among caregivers of children born with a disorder of sex development including ambiguous genitalia.
        Horm Res Paediatr. 2018; 90: 308-313
        • Finlayson C.
        • Rosoklija I.
        • Aston C.E.
        • Austin P.
        • Bakula D.
        • Baskin L.
        • et al.
        Baseline characteristics of infants with atypical genital development: phenotypes, diagnoses, and sex of rearing.
        J Endocr Soc. 2019; 3: 264-272
        • Baskin A.
        • Wisniewski A.B.
        • Aston C.E.
        • Austin P.
        • Chan Y.M.
        • Cheng E.Y.
        • et al.
        Post-operative complications following feminizing genitoplasty in moderate to severe genital atypia: results from a multicenter, observational prospective cohort study.
        J Pediatr Urol. 2020 Oct; 16 (Epub 2020 Jun 1.PMID: 32624410): 568-575https://doi.org/10.1016/j.jpurol.2020.05.166
        • Bernabe K.J.
        • Nokoff N.J.
        • Galan D.
        • Felsen D.
        • Aston C.E.
        • Austin P.
        • et al.
        Preliminary report: surgical outcomes following genitoplasty in children with moderate to severe genital atypia.
        J Pediatr Urol. 2018; 14: 157 e1-e8
        • Schultz J.R.
        • Klykylo W.M.
        • Wacksman J.
        Timing of elective hypospadias repair in children.
        Pediatrics. 1983; 71: 342-351
        • Perlmutter A.E.
        • Morabito R.
        • Tarry W.F.
        Impact of patient age on distal hypospadias repair: a surgical perspective.
        Urology. 2006; 68: 648-651
        • Hensle T.W.
        • Tennenbaum S.Y.
        • Reiley E.A.
        • Pollard J.
        Hypospadias repair in adults: adventures and misadventures.
        J Urol. 2001; 165: 77-79
        • Ortqvist L.
        • Andersson M.
        • Strandqvist A.
        • Nordenstrom A.
        • Frisen L.
        • Holmdahl G.
        • et al.
        Psychosocial outcome in adult men born with hypospadias.
        J Pediatr Urol. 2017; 13: 79 e1-e7
        • Ortqvist L.
        • Fossum M.
        • Andersson M.
        • Nordenstrom A.
        • Frisen L.
        • Holmdahl G.
        • et al.
        Long-term followup of men born with hypospadias: urological and cosmetic results.
        J Urol. 2015; 193: 975-981
        • Pippi Salle J.L.
        • Sayed S.
        • Salle A.
        • Bagli D.
        • Farhat W.
        • Koyle M.
        • et al.
        Proximal hypospadias: a persistent challenge. Single institution outcome analysis of three surgical techniques over a 10-year period.
        J Pediatr Urol. 2016; 12: 28.e1-28.e7
        • Long C.J.
        • Chu D.I.
        • Tenney R.W.
        • Morris A.R.
        • Weiss D.A.
        • Shukla A.R.
        • et al.
        Intermediate-term followup of proximal hypospadias repair reveals high complication rate.
        J Urol. 2017; 197: 852-858
        • Migeon C.J.
        • Wisniewski A.B.
        • Gearhart J.P.
        • Meyer-Bahlburg H.F.
        • Rock J.A.
        • Brown T.R.
        • et al.
        Ambiguous genitalia with perineoscrotal hypospadias in 46,XY individuals: long-term medical, surgical, and psychosexual outcome.
        Pediatrics. 2002; 110: e31
        • Lucas J.
        • Hightower T.
        • Weiss D.A.
        • Van Batavia J.
        • Coelho S.
        • Srinivasan A.K.
        • et al.
        Time to complication detection after primary pediatric hypospadias repair: a large, single-center, retrospective cohort analysis.
        J Urol. 2020; 204: 338-344https://doi.org/10.1097/JU.0000000000000762
        • Lucas-Herald A.
        • Bertelloni S.
        • Juul A.
        • Bryce J.
        • Jiang J.
        • Rodie M.
        • et al.
        The long-term outcome of boys with partial androgen insensitivity syndrome and a mutation in the androgen receptor gene.
        J Clin Endocrinol Metab. 2016; 101: 3959-3967
        • Kirsch A.J.
        • Escala J.
        • Duckett J.W.
        • Smith G.H.
        • Zderic S.A.
        • Canning D.A.
        • et al.
        Surgical management of the nonpalpable testis: the Children's Hospital of Philadelphia experience.
        J Urol. 1998; 159: 1340-1343
        • Kollin C.
        • Granholm T.
        • Nordenskjold A.
        • Ritzen E.M.
        Growth of spontaneously descended and surgically treated testes during early childhood.
        Pediatrics. 2013; 131: e1174-e1180
        • Tasian G.E.
        • Hittelman A.B.
        • Kim G.E.
        • DiSandro M.J.
        • Baskin L.S.
        Age at orchiopexy and testis palpability predict germ and Leydig cell loss: clinical predictors of adverse histological features of cryptorchidism.
        J Urol. 2009; 182: 704-709
        • Park K.H.
        • Lee J.H.
        • Han J.J.
        • Lee S.D.
        • Song S.Y.
        Histological evidences suggest recommending orchiopexy within the first year of life for children with unilateral inguinal cryptorchid testis.
        Int J Urol. 2007; 14: 616-621
        • McAleer I.M.
        • Packer M.G.
        • Kaplan G.W.
        • Scherz H.C.
        • Krous H.F.
        • Billman G.F.
        Fertility index analysis in cryptorchidism.
        J Urol. 1995; 153: 1255-1258
        • Cortes D.
        • Thorup J.M.
        • Visfeldt J.
        Cryptorchidism: aspects of fertility and neoplasms. A study including data of 1,335 consecutive boys who underwent testicular biopsy simultaneously with surgery for cryptorchidism.
        Horm Res. 2001; 55: 21-27
        • Hadziselimovic F.
        • Herzog B.
        Importance of early postnatal germ cell maturation for fertility of cryptorchid males.
        Horm Res. 2001; 55: 6-10
        • Rusnack S.L.
        • Wu H.Y.
        • Huff D.S.
        • Snyder 3rd, H.M.
        • Carr M.C.
        • Bellah R.D.
        • et al.
        Testis histopathology in boys with cryptorchidism correlates with future fertility potential.
        J Urol. 2003; 169: 659-662
        • Nistal M.
        • Riestra M.L.
        • Paniagua R.
        Correlation between testicular biopsies (prepubertal and postpubertal) and spermiogram in cryptorchid men.
        Hum Pathol. 2000; 31: 1022-1030
        • Hadziselimovic F.
        • Herzog B.
        • Buser M.
        Development of cryptorchid testes.
        Eur J Pediatr. 1987; 146: S8-S12
        • Coyle D.
        • Kutasy B.
        • Han Suyin K.
        • Antao B.
        • Lynch S.A.
        • McDermott M.B.
        • et al.
        Gonadoblastoma in patients with 45,X/46,XY mosaicism: a 16-year experience.
        J Pediatr Urol. 2016; 12: 283 e1-e7