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• W2322353032 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); ovo-testicular DSD; and “non-hormonal/non chromosomal” DSD. Questions are summarized for each DSD group with the support of literature and the feed-back 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. 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); ovo-testicular DSD; and “non-hormonal/non chromosomal” DSD. Questions are summarized for each DSD group with the support of literature and the feed-back 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. Ten years after the Chicago consensus meeting [[1]Hughes I.A. Houk C. Ahmed S.F. Lee P.A. Lawson Wilkins Pediatric Endocrine Society/European Society for Paediatric Endocrinology Consensus Group Consensus statement on management of intersex disorders.J Pediatr Urol. 2006; 2: 148-162Abstract Full Text Full Text PDF PubMed Scopus (424) Google Scholar], genital surgery continues to raise questions and criticisms concerning its indications, its timing, and its technical aspects [2Mouriquand P. Caldamone A. Malone P. Frank J.D. Hoebeke P. The ESPU/SPU standpoint on the surgical management of Disorders of Sex Development (DSD).J Pediatr Urol. 2014; 10: 8-10Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar, 3Wolffenbuttel K.P. Crouch N.S. Timing of feminising surgery in disorders of sex development.Endocr Dev. 2014; 27: 210-221Crossref PubMed Scopus (18) Google Scholar]. Opinions are more common than facts as the volume of patients in each group of disorders of sex development (DSD) is small, management is extraordinarily heterogeneous across centers, and pre- and post-treatment evaluations are mostly subjective, examiner-dependent, and culturally influenced. Hence, the classical methodology of evidence-based medicine meets major hurdles, which are responsible for several unanswered questions that we attempt to list in this standpoint article. The first major hurdle is the definition of the acronym DSD. Does it include all congenital developmental genito-sexual anomalies, and, if so, are undescended testicles, hypospadias, or even labial adhesions included? Or should the definition be limited to situations in which there is an inadequacy between genital anatomy (phenotype) and biological profile (biotype), which may raise questions about gender assignment? This restrictive definition of DSD does not identify genital anomalies with no detectable biological or chromosomal anomalies, which represent the vast majority of patients. The second hurdle is semantic as the terms “gender,” “sex,” “sexual,” have discordant interpretations. “Gender” is a social concept, which is the way the society mirrors the “individual identity.” It does not take into account the “individual identity” (“inside identity”) and the future “gender role” (“behavioral identity”), which are invisible at birth and the modalities of which are mostly unknown, that is multifactorial [[4]Cheikhelard A. Gapany C. Catti M. Mouriquand P. Potential determinant factors of sexual identity in ambiguous genitalia.J Pediatr Urol. 2005; 1: 383-388Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar]. The term “genital” has been avoided in the Chicago meeting, although atypical genito-sexual development should be the main focus of this discussion. Hence, it is essential to correlate phenotype and biotype as atypical anatomy is the first clinical sign from which suspicion of a DSD is raised in the newborn and will lead to a chain of investigations to define to which group of DSD the patient belongs. Using the Chicago canvas [[5]Lee P.A. Houk C.P. Ahmed S.F. Hughes I.A. International Consensus Conference on Intersex Organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology Consensus statement on management of intersex disorders. International consensus Conference on intersex.Pediatrics. 2006; 118: e488-e500Crossref PubMed Scopus (931) Google Scholar], five main groups of DSD patients may be identified, submitted to the gender assignment process, and may be considered for a surgical genital reconstruction.(1)In the 46,XX DSD group, classical congenital adrenal hyperplasia (CAH) represents the most common diagnosis. There is usually no gender issue in this group, except in case of late diagnosis and severely masculinized 46,XX individuals. Genital phenotype of prenatally non-treated 46,XX CAH patients at birth includes an increased development of the genital tubercle (GT) along with an increased length of the urethra, the opening of which is usually located on the ventrum of the GT, although in the most severely masculinized cases it may constitute a normal-looking phallus [[6]Lee P.A. Houk C.P. Review of outcome information in 46,XX patients with congenital adrenal hyperplasia assigned/reared male: what does it say about gender assignment?.Int J Pediatr Endocrinol. 2010; 2010: 982025Crossref PubMed Google Scholar]. These features are similar to those of a 46,XY hypospadiac GT with non-palpable testes. In 46,XX CAH, the vaginal cavity opens into the posterior wall of the urethra at a variable distance from the bladder neck but not higher than where the veru montanum (mullerian structure) is normally located in the male urethra. This confluence is also at variable distance from the perineum depending on the development of the urethra and the increased thickness of the pelvic floor muscles. The height of the urethro-vaginal confluence is not related to the degree of external masculinization, contrary to suggestions from the Prader classification [7Prader A. Genital findings in the female pseudo-hermaphroditism of the congenital adrenogenital syndrome; morphology, frequency, development and heredity of the different genital forms.Helv Paediatr Acta. 1954; 9: 231-248PubMed Google Scholar, 8Gorduza D. Tardy-Guidollet V. Robert E. Gay C.-L. Chatelain P. David M. et al.Late prenatal dexamethasone and phenotype variations in 46,XX CAH: concerns about current protocols and benefits for surgical procedures.J Pediatr Urol. 2014; 10: 941-947Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar]. The sagittal fusion of the genital folds is variable, from an almost feminine vulva to a complete scrotal-like appearance. In all cases, the gonads are not palpable in the genital folds. Recent evidence suggests that female classical CAH patients and caregivers do not wish to be considered to be DSD patients [[9]Lin-Su K. Lekarev O. Poppas D.P. Vogiatzi M.G. Congenital adrenal hyperplasia patient perception of “disorders of sex development” nomenclature.Int J Pediatr Endocrinol. 2015; 2015: 9Crossref PubMed Google Scholar].(2)The 46,XY DSD group is more heterogeneous, mostly including: abnormal androgen steroidogenesis, particularly 17β hydroxy steroid dehydrogenase (17β HSD) deficiency; and 5α reductase deficiency, which is more common in some areas in the world in which consanguinity is frequent (e.g. the Dominican Republic, New Guinea, and the Gaza strip). In these two first groups, the phenotype is often feminine at birth but will become virilized at puberty. The testicles are often palpable in the inguinal region. There are no mullerian structures as the AMH function remains intact. Internal genital organs are male. These are two situations for which controversies exist regarding gender assignment, sex of rearing, and surgery [[10]Cohen-Kettenis P.T. Gender change in 46,XY persons with 5alpha-reductase-2 deficiency and 17beta-hydroxysteroid dehydrogenase-3 deficiency.Arch Sex Behav. 2005; 34: 399-410Crossref PubMed Scopus (248) Google Scholar]. One critical issue is the fate of the testicles: Should they be kept in place until the hypothetical age of self-gender determination? Or if female sex rearing is decided on, should they be removed early to avoid pubertal virilization? If conservative management is chosen, temporarily blocking pubertal virilization with a GnRH analog until gender-identity development is settled is an option. The risk of gonadal tumor is small and probably equals the risk recorded with undescended testes. Gonadal dysplasia or dysgenesis is characterized by failed production of androgens and AMH responsible for a poorly developed genital tubercle (usually severe hypospadias and/or micropenis) and the persistence of a mullerian cavity (utricule) of variable size. These abnormally developed gonads can be undescended and present with a high tumor risk [11Cools M. Drop S.L.S. Wolffenbuttel K.P. Oosterhuis J.W. Looijenga L.H.J. Germ cell tumors in the intersex gonad: old paths, new directions, moving frontiers.Endocr Rev. 2006; 27: 468-484Crossref PubMed Scopus (352) Google Scholar, 12Cools M. Looijenga L.H.J. Wolffenbuttel K.P. T'Sjoen G. Managing the risk of germ cell tumourigenesis in disorders of sex development patients.Endocr Dev. 2014; 27: 185-196Crossref PubMed Scopus (62) Google Scholar]. Androgen receptor insensitivity can be complete or partial. With complete deficiency, the phenotype is feminine. There is a vaginal cupule which can be dilated at a later age to obtain a well-developed vaginal cavity. Testicles are usually intra-abdominal and sometimes found during surgery for inguinal hernias. The diagnosis is often delayed to puberty when the normal-appearing girl has primary amenorrhea. Breasts are often developed to some degree due to the aromatase activity and high circulating androgens. In this situation, gender assignment is usually not an issue and discussions of surgery involve removal of the gonads, which probably have a tumor risk similar to undescended testes, although some uncertainties remain about this [12Cools M. Looijenga L.H.J. Wolffenbuttel K.P. T'Sjoen G. Managing the risk of germ cell tumourigenesis in disorders of sex development patients.Endocr Dev. 2014; 27: 185-196Crossref PubMed Scopus (62) Google Scholar, 13Deans R. Creighton S.M. Liao L.-M. Conway G.S. Timing of gonadectomy in adult women with complete androgen insensitivity syndrome (CAIS): patient preferences and clinical evidence.Clin Endocrinol (Oxf). 2012; 76: 894-898Crossref PubMed Scopus (113) Google Scholar]. Vaginal surgery is sometimes necessary to create a vaginal cavity if vaginal dilatations are not successful. Testes are usually left in the abdomen until puberty to allow for breast development. In partial androgen receptor insensitivity, the phenotype is variable but often has the anatomical characteristics of hypospadias. Hypospadias is defined as a development arrest of the tissues forming the ventral aspect of the genital tubercle. Several degrees of severity are identified according to the level of division of the corpus spongiosum [[14]Mouriquand P. Demede D. Gorduza D. Mure P. Hypospadias.in: Gearhart Rink Mouriquand Pediatric urology. 2nd ed. 2009: 526-543Google Scholar]: The more proximal it is, the more severe is the hypospadias. This may lead to a ventral curvature of the genital tubercle, which is associated with the hypoplasia of the tissues forming the ventral aspect. The urethral meatus is ventral and opens distally to the division of the corpus spongiosum. The prepuce is incomplete with an excess of skin on the dorsum (preputial hood). The genital tubercle is usually smaller than unaffected male controls. Most patients are raised in the male gender [15Wisniewski A.B. Migeon C.J. Long-term perspectives for 46,XY patients affected by complete androgen insensitivity syndrome or congenital micropenis.Semin Reprod Med. 2002; 20: 297-304Crossref PubMed Scopus (49) Google Scholar, 16Migeon C.J. Wisniewski A.B. Gearhart J.P. Meyer-Bahlburg H.F.L. 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: e31Crossref PubMed Scopus (184) Google Scholar]. Questions regarding treatment are related to the hormonal stimulation of the genital tubercle to attempt to increase its size and the timing of hypospadias reconstruction, which is often complex and multi-stage in this group. Testicles can be undescended or not and likely have no additional tumor risk over isolated undescended testes, although some studies report a 15% risk of testicular tumor in PAIS [[11]Cools M. Drop S.L.S. Wolffenbuttel K.P. Oosterhuis J.W. Looijenga L.H.J. Germ cell tumors in the intersex gonad: old paths, new directions, moving frontiers.Endocr Rev. 2006; 27: 468-484Crossref PubMed Scopus (352) Google Scholar]. Infertility is likely in the AIS patients as many of the testes demonstrate poor germ cell maturation. Anti-mullerian hormone (AMH) deficiency is not associated with a gender assignment problem. The genital tubercle is normally developed but the child bears a fully developed uterus, fallopian tubes, and upper part of the vagina. The testicles are retained intra-abdominally and the difficulty is bringing them down into the scrotum without compromising their blood supply and the vas deferens, which are included in the uterine walls and possibly atretic [[17]Josso N. Belville C. di Clemente N. Picard J.-Y. AMH and AMH receptor defects in persistent Müllerian duct syndrome.Hum Reprod Update. 2005; 11: 351-356Crossref PubMed Scopus (213) Google Scholar]. Fertility is an issue as the germ cells display a poor second step of maturation of the spermatogonia into primary spermatocytes. It is also most improbable that sperm will follow a normal route in these patients. Medically assisted procreation is, therefore, indicated. Hypogonadotropic hypogonadism can result in variable degrees of hypovirilization. Surgery is not a central issue in this group.(3)There are multiple variants of sex-chromosome mosaicism. The most common situation for which gender assignment and surgery are discussed is mixed gonadal dysgenesis or 45,X/46,XY DSD [[18]Martinerie L. Morel Y. Gay C.-L. Pienkowski C. de Kerdanet M. Cabrol S. et al.Impaired puberty, fertility, and final stature in 45,X/46,XY mixed gonadal dysgenetic patients raised as boys.Eur J Endocrinol Eur Fed Endocr Soc. 2012; 166: 687-694Crossref PubMed Scopus (62) Google Scholar]. Although many of these patients have a normal male phenotype, those seen by the surgeons commonly present with asymmetrical genitalia: On one side a scrotum containing a gonad (commonly on the right side for unexplained reasons), on the other side a labia majora with a gonad not palpable in the genital fold, poorly palpable in the groin, or often non-palpable at all. This gonad is usually undifferentiated or a streak gonad, that is composed of stromal tissue without tubules or follicles. The intrascrotal gonad is either a sub-normal testis or a gonad containing testicular tissue, the rule being that any gonad palpable in the genital folds is either a testis or a mixture of testicular and ovarian tissues. The genital tubercle is severely hypospadiac with a perineal division of the corpus spongiosum, that is with significant ventral curvature. There is a mullerian cavity located at the level of the veru montanum behind the proximal urethra and opened into it. This cavity is often tubular, narrow, and rigid, quite different from the vaginal cavity seen in CAH. This situation raises the question of gender assignment, timing of surgery, and the fate of the gonads, as dysplastic gonads bear a significant risk of tumor.(4)Ovo-testicular DSD patients presents with both ovarian and testicular tissues and abnormally differentiated genital structures. Their great variability raises difficult questions regarding gender assignment and genital reconstruction.(5)The “non-hormonal/non chromosomal DSD” are mainly represented by anomalies of the caudal extremity mostly found in cloacal exstrophy patients or in aphallia, or in some severe micropenis. It is a failure of tubulization and cavitation of the caudal end of the body with a non-closure of the bladder, exposed abdominal organs in an unclosed pelvic ring [[19]Mouriquand P. Congenital disorders of the bladder and the urethra.in: Rink Textbook of genitourinary surgery. 1998Google Scholar]. Genitalia are considerably abnormal with a variable separation of the erectile structures. Internal organs, although often normal, may be duplicated. Gonads are usually normal but the genital tracts in the male are dysfunctional because of the abnormal anatomy of the posterior urethra and prostatic region. The genital tubercle is poorly developed and stretched by the separation of the two hemi-pelvises. Gender assignment and surgical reconstruction are difficult issues in this group. •Restore functional genital anatomy to allow future penetrative intercourse (as a male or a female),•Facilitate future reproduction (as a male or a female) when possible,•Reduce urological hazards related to abnormal genito-urinary anatomy, that is urinary tract infections, with potential upper urinary tract consequences and urinary incontinence,•Avoid fluid or blood retention in vaginal or uterine cavities,•Avoid late virilization at puberty in individuals raised as girls or breast development in individuals raised as boys,•Reduce the risk of gonadal cancers,•Foster development of “individual” and “social identities,”•Avoid stigmatization related to atypical anatomy,•To respond to the parents' desire to bring up a child in the best possible conditions The genital tubercle (clitoris and glans) can either be left intact or reduced in size in a female assigned patient or refashioned in a male assigned patient. Clitoral reduction consists of reducing the length of the genital tubercle while trying to preserve the nerves and vessels leading to the clitoris. A more precise description of the anatomy of these nerves in the late 1990s allows a much finer dissection of the afferent fibers leading to the glans/clitoris [20Baskin L.S. Fetal genital anatomy reconstructive implications.J Urol. 1999; 162: 527-529Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar, 21Kalfa N. Liu B. Cao M. Vilella M. Hsieh M. Baskin L.S. 3-dimensional neuroanatomy of the human fetal pelvis: anatomical support for partial urogenital mobilization in the treatment of urogenital sinus.J Urol. 2008; 180 (discussion 1714–5): 1709-1714Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar]. The same nerve preservation applies for the reconstruction of the epispadiac penis [[22]Ransley P. Duffy P. Wollin M. Bladder exstrophy closure and epispadias repair.in: Paediatric surgery. 4th ed. Butterworths, London1988: 620Google Scholar]. Most techniques of clitoral reduction remove a variable segment of the corpora cavernosa. As this is an irreversible step of the procedure, some surgeons attempted to preserve the full length of each corpora and bury them around the vaginal opening with the idea that they could be reused in the future should the patient later choose the male gender [[23]Pippi Salle J.L. Braga L.P. Macedo N. Rosito N. Bagli D. Corporeal sparing dismembered clitoroplasty: an alternative technique for feminizing genitoplasty.J Urol. 2007; 178 (discussion 1801): 1796-1800Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar]. However, such a reversion has not been reported to date. Once the corpora cavernosa have been removed or displaced, the clitoris is reattached to the corporeal stumps near the lower edge of the pelvis. The skin shaft of the genital tubercle is split vertically to refashion the labia minora. This is clearly delicate surgery. The potential damage to clitoral sensitivity and the irreversible character of this procedure are the two main criticisms made, although significant strides improving the surgery have been made. This is why clitoral reduction should be restricted to the significantly enlarged clitoris, knowing that in the CAH group, well conducted substitutive hormonal treatment permits a significant reduction in size. Attempts to avoid this surgery with prenatal hormonal substitution (dexamethasone) in the CAH group have been successfully reported as long as the treatment starts before week 6 of gestation, although potential side effects of steroids on the fetus and the mother during gestation are a source of discussion [24Forest M.G. David M. Morel Y. Prenatal diagnosis and treatment of 21-hydroxylase deficiency.J Steroid Biochem Mol Biol. 1993; 45: 75-82Crossref PubMed Scopus (144) Google Scholar, 25Tardy-Guidollet V. Menassa R. Costa J.-M. David M. Bouvattier-Morel C. Baumann C. et al.New management strategy of pregnancies at risk of congenital adrenal hyperplasia using fetal sex determination in maternal serum: French cohort of 258 cases (2002-2011).J Clin Endocrinol Metab. 2014; 99: 1180-1188Crossref PubMed Scopus (58) Google Scholar, 26Speiser P.W. Azziz R. Baskin L.S. Ghizzoni L. Hensle T.W. Merke D.P. et al.Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline.J Clin Endocrinol Metab. 2010; 95: 4133-4160Crossref PubMed Scopus (814) Google Scholar]. For reconstruction of the genital tubercle in the male, surgery is based on the principles of hypospadias surgery, which involves three main steps [[27]Renaux-Petel M. Mouriquand P. Mure P. Hypospadias.in: Puri P. Encyclopedia of pediatric surgery. 2016Google Scholar]:(1)Degloving of the genital tubercle to assess the severity of the hypospadias based on the level of division of the corpus spongiosum, the degree of hypoplasia of the ventral tissues and the subsequent ventral curvature, the position of the urethral meatus, the length of urethra to be reconstructed, the size of the genital tubercle, the size and shape of the glans, and the availability of foreskin tissue.(2)Refashioning of the missing urethra (urethroplasty) based on several different techniques, which can be divided into techniques solely using ventral tissues (Thiersch-Duplay, TIP, Mathieu), those combining ventral and dorsal tissues (Onlay, Duckett, Koyanagi-Hayashi) those using free grafts (i.e. buccal mucosa), those displacing the urethra (Beck-Koff), and two-stage procedures (Cloutier-Bracka) [28Djordjevik M. Djordjevik Hypospadias surgery challenges and limits. 2014Google Scholar, 29Gorduza D. Vidal I. Birraux J. Gay C.-L. Demède D. Mure P.-Y. et al.The surgical challenges of disorders of sex development (DSD).Arch Esp Urol. 2010; 63: 495-504Crossref PubMed Google Scholar]. Ventral curvature of the genital tubercle is commonly assessed in proximal hypospadias and can either be corrected by dorsal plication of the corpora cavernosa or by ventral grafting [28Djordjevik M. Djordjevik Hypospadias surgery challenges and limits. 2014Google Scholar, 30Braga L.H.P. Lorenzo A.J. Bägli D.J. Dave S. Eeg K. Farhat W.A. et al.Ventral penile lengthening versus dorsal plication for severe ventral curvature in children with proximal hypospadias.J Urol. 2008; 180 (discussion 1747–8): 1743-1747Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar].(3)Refashioning the penile skin shaft. This surgery is also difficult and complications are commonly reported, which can be divided into unsatisfactory cosmetic results, urethral healing failures (fistula, dehiscence), urine flow impairments (stenosis, urethrocele), persistent penile curvature, and ejaculation and erection disorders. Most patients will receive more than one procedure and long-term follow-up is mandatory [[31]Mouriquand P.D.E. Gorduza D.B. Noché M.-E. Targnion A. Long-term outcome of hypospadias surgery: current dilemmas.Curr Opin Urol. 2011; 21: 465-469Crossref PubMed Scopus (28) Google Scholar]. Phalloplasty is an option primarily in the post-pubertal patient who chooses a male gender. Some would discuss this surgery earlier in life [[32]De Castro R. Rondon A. Barroso U. Ortiz V. Macedo A. Phalloplasty and urethroplasty in a boy with penile agenesis.J Pediatr Urol. 2013; 9 (108.e1–2)Abstract Full Text Full Text PDF Scopus (9) Google Scholar]. It is a highly specialized surgery with a significant morbidity, and should be confined to some centers specialized in trans-sexual surgery [32De Castro R. Rondon A. Barroso U. Ortiz V. Macedo A. Phalloplasty and urethroplasty in a boy with penile agenesis.J Pediatr Urol. 2013; 9 (108.e1–2)Abstract Full Text Full Text PDF Scopus (9) Google Scholar, 33Callens N. De Cuypere G. T'Sjoen G. Monstrey S. Lumen N. Van Laecke E. et al.Sexual quality of life after total phalloplasty in men with penile deficiency: an exploratory study.World J Urol. 2015; 33: 137-143Crossref PubMed Scopus (32) Google Scholar, 34Terrier J.-É. Courtois F. Ruffion A. Morel Journel N. Surgical outcomes and patients' satisfaction with suprapubic phalloplasty.J Sex Med. 2014; 11: 288-298Abstract Full Text Full Text PDF PubMed Scopus (44) Google Scholar]. It is important to understand what happens during embryogenesis and more specifically during the delimitation process which divides and separates the cloacal cavity into three compartments – urological, genital, and intestinal – which will be individually connected to the outside with a proper opening and sphincter. This cavitation and separation process fails in most DSD situations described before. The vagina which represents the mid compartment is said to come from two different embryonic structures: Mullerian ducts for its cephalad two-thirds and ectoderm for its caudal third [[17]Josso N. Belville C. di Clemente N. Picard J.-Y. AMH and AMH receptor defects in persistent Müllerian duct syndrome.Hum Reprod Update. 2005; 11: 351-356Crossref PubMed Scopus (213) Google Scholar]. In the CAH group, the upper two-thirds need to be connected to the pelvic floor and separated from the urological compartment, incorrectly called uro-genital sinus. In the 45,X/46,XY DSD group, the retro-urethral cavity is narrower and more rigid than in the CAH group. When the female gender is chosen, this cavity also needs to be connected to the pelvic floor and separated from the urological path. In all other DSD groups, there is either no genital cavity (17βHSD), that is no mullerian structures or a vaginal “cupule”, which probably represents the lower third of the vagina. Complete absence of genital tract requires the creation of a new vagina using various techniques (intestine, peritoneum, buccal mucosa, etc.)." @default.
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• W2322353032 title "Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?" @default.
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