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Surgery in disorders of sex development (DSD) with a gender issue : if (why), when, and how?

(2016) JOURNAL OF PEDIATRIC UROLOGY. 12(3). p.139-149
Author
Organization
Abstract
Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46, XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46, XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments.); gonosomic mosaicisms (45, X/46, XY patients); ovotesticular DSD; and "non-hormonal/non chromosomal" DSD. Questions are summarized for each DSD group with the support of literature and the feedback of several world experts. Given the complexity and heterogeneity of presentation there is no consensus regarding the indications, the timing, the procedure nor the evaluation of outcome of DSD surgery. There are, however, some issues on which most experts would agree: 1) The need for identifying centres of expertise with a multidisciplinary approach; 2) A conservative management of the gonads in complete androgen insensitivity syndrome at least until puberty although some studies expressed concerns about the heightened tumour risk in this group; 3) To avoid vaginal dilatation in children after surgical reconstruction; 4) To keep asymptomatic mullerian remnants during childhood; 5) To remove confirmed streak gonads when Y material is present; 6) It is likely that 46, XY cloacal exstrophy, aphallia and severe micropenis would do best raised as male although this is based on limited outcome data. There is general acknowledgement among experts that timing, the choice of the individual and irreversibility of surgical procedures are sources of concerns. There is, however, little evidence provided regarding the impact of non-treated DSD during childhood for the individual development, the parents, society and the risk of stigmatization. The low level of evidence should lead to design collaborative prospective studies involving all parties and using consensual protocols of evaluation.
Keywords
Disorders of sex development, DSD, Genital surgery in children, Congenital adrenal hyperplasia, CAH, Chromosomal anomalies, Gonadal dysgenesis, Gonadal dysplasia, 5 alpha reductase deficiency, 17 beta hydroxy steroid dehydrogenase, 17 beta HSD, Androgen insensitivity syndrome, AIS, Hypospadias, Micropenis, Mixed gonadal dysgenesis, Ovo-testicular DSD, CONGENITAL ADRENAL-HYPERPLASIA, ANDROGEN INSENSITIVITY SYNDROME, SPARING VENTRAL CLITOROPLASTY, MULLERIAN DUCT SYNDROME, TERM-FOLLOW-UP, AMBIGUOUS GENITALIA, 21-HYDROXYLASE DEFICIENCY, FEMINIZING GENITOPLASTY, SURGICAL-MANAGEMENT, CLOACAL EXSTROPHY

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Citation

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Chicago
Mouriquand, Pierre DE, Daniela Brindusa Gorduza, Claire-Lise Gay, Heino FL Meyer-Bahlburg, Linda Baker, Laurence S Baskin, Claire Bouvattier, et al. 2016. “Surgery in Disorders of Sex Development (DSD) with a Gender Issue : If (why), When, and How?” Journal of Pediatric Urology 12 (3): 139–149.
APA
Mouriquand, P. D., Gorduza, D. B., Gay, C.-L., Meyer-Bahlburg, H. F., Baker, L., Baskin, L. S., Bouvattier, C., et al. (2016). Surgery in disorders of sex development (DSD) with a gender issue : if (why), when, and how? JOURNAL OF PEDIATRIC UROLOGY, 12(3), 139–149.
Vancouver
1.
Mouriquand PD, Gorduza DB, Gay C-L, Meyer-Bahlburg HF, Baker L, Baskin LS, et al. Surgery in disorders of sex development (DSD) with a gender issue : if (why), when, and how? JOURNAL OF PEDIATRIC UROLOGY. 2016;12(3):139–49.
MLA
Mouriquand, Pierre DE et al. “Surgery in Disorders of Sex Development (DSD) with a Gender Issue : If (why), When, and How?” JOURNAL OF PEDIATRIC UROLOGY 12.3 (2016): 139–149. Print.
@article{8550175,
  abstract     = {Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46, XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46, XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments.); gonosomic mosaicisms (45, X/46, XY patients); ovotesticular DSD; and {\textacutedbl}non-hormonal/non chromosomal{\textacutedbl} DSD. Questions are summarized for each DSD group with the support of literature and the feedback of several world experts. 
Given the complexity and heterogeneity of presentation there is no consensus regarding the indications, the timing, the procedure nor the evaluation of outcome of DSD surgery. There are, however, some issues on which most experts would agree: 1) The need for identifying centres of expertise with a multidisciplinary approach; 2) A conservative management of the gonads in complete androgen insensitivity syndrome at least until puberty although some studies expressed concerns about the heightened tumour risk in this group; 3) To avoid vaginal dilatation in children after surgical reconstruction; 4) To keep asymptomatic mullerian remnants during childhood; 5) To remove confirmed streak gonads when Y material is present; 6) It is likely that 46, XY cloacal exstrophy, aphallia and severe micropenis would do best raised as male although this is based on limited outcome data. There is general acknowledgement among experts that timing, the choice of the individual and irreversibility of surgical procedures are sources of concerns. There is, however, little evidence provided regarding the impact of non-treated DSD during childhood for the individual development, the parents, society and the risk of stigmatization. The low level of evidence should lead to design collaborative prospective studies involving all parties and using consensual protocols of evaluation.},
  author       = {Mouriquand, Pierre DE and Gorduza, Daniela Brindusa and Gay, Claire-Lise and Meyer-Bahlburg, Heino FL and Baker, Linda and Baskin, Laurence S and Bouvattier, Claire and Braga, Luis H and Caldamone, Anthony C and Duranteau, Lise and El Ghoneimi, Alaa and Hensle, Terry W and Hoebeke, Piet and Kaefer, Martin and Kalfa, Nicolas and Kolon, Thomas F and Manzoni, Gianantonio and Mure, Pierre-Yves and Nordenskj{\"o}ld, Agneta and Pippi Salle, JL and Poppas, Dix Phillip and Ransley, Philip G and Rink, Richard C and Rodrigo, Romao and Sann, L{\'e}on and Schober, Justine and Sibai, Hisham and Wisniewski, Amy and Wolffenbuttel, Katja P and Lee, Peter},
  issn         = {1477-5131},
  journal      = {JOURNAL OF PEDIATRIC UROLOGY},
  language     = {eng},
  number       = {3},
  pages        = {139--149},
  title        = {Surgery in disorders of sex development (DSD) with a gender issue : if (why), when, and how?},
  url          = {http://dx.doi.org/10.1016/j.jpurol.2016.04.001},
  volume       = {12},
  year         = {2016},
}

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