Intersex Disorders Research Articles

Approaches in care for people with variations of sex characteristics—focus groups in the European context on the strengths and challenges of multidisciplinary teams

Abstract Background New guidelines in the Consensus Statement on Management of Intersex Disorders by the Lawson Wilkins Pediatric Endocrine Society/European Society for Paediatric Endocrinology Consensus Group 2006 introduced multidisciplinary teams (MDTs) to provide care that involves collaboration between healthcare professionals, parents, and children with variations of sex characteristics (VSC). Aim The aim of this study was to examine a neglected but important field of collaboration among healthcare professionals and peer support groups who provide care for people with VSC. Outcomes The study outcome was the information obtained regarding the actual composition and collaboration of the multidisciplinary teams caring for children with VSC, including their collaboration with parents, peer support groups, and other care providers. Methods In this study we used an exploratory qualitative design based on mixed focus groups (in terms of professional background) and reflexive thematic analysis. Semi-structured focus group interview guides were used to obtain information about the participants’ viewpoints on the composition and challenges of, and collaboration between, peer support groups and members of multidisciplinary teams working to care for children with VSC and their parents. Seven focus groups were conducted with healthcare professionals and peer support groups in care teams in Central, Northern, and Western Europe. The data from the focus groups were examined using reflexive thematic analysis. Results The participants in the focus groups provided information regarding the use of multidisciplinary and interdisciplinary child- and family-oriented approaches and the strengths and challenges of collaboration and peer support groups. The results showed that the teams used a predominantly multidisciplinary approach and suffered from a lack of psychosocial providers, poor collaboration with peer support groups, and poor implementation of shared decision-making to address the clinical uncertainty of parents and people with VSC. Clinical Implications Clinicians should provide more psychosocial support and improve collaboration with peer support groups and nonmedical professionals. Strengths and Limitations This study is one of the first qualitative studies to provide information on the collaboration of multidisciplinary teams working to provide care for children with VSC and collaborate with their parents in the European context. However, due to language barriers, the dropout rate of participants, and the under-representation of peer support groups in the sample there was a lack of information on collaboration among healthcare professionals and peer support groups. Conclusions The collaboration between MDTs and parents does not involve adequate psychosocial and peer support or shared decision-making to address the uncertainty experienced by children and families when faced with information about variations of sex characteristics.

From Harmful Practices and Instrumentalisation, towards Legislative Protections and Community-Owned Healthcare Services: The Context and Goals of the Intersex Movement in Australia

People with innate variations of sex characteristics (also known as intersex traits or disorders or differences of sex development) have any of a wide range of innate physical traits that differ from medical and social norms for female and male bodies. Responses to these physical differences create experiences and risks of stigmatisation, discrimination, violence, and harmful medical practices intended to promote social and familial integration and conformity with gender stereotypes. As is evident globally, the Australian policy response to the existence and needs of people with innate variations of sex characteristics has been largely incoherent, variously framing the population as having disordered sex development in need of “fixing”, and a third sex/gender identity group in need of recognition, with only recent engagement by intersex community-controlled civil society organisations. This paper presents an overview of the context and goals of the intersex human rights movement in Australia. Australian intersex community organisations have sought to apply human rights norms and develop new infrastructure to address key health and human rights issues, and necessitating new ways of resolving policy incoherence. Together with human rights, mental health, and public health institutions, they have called for significant changes to medical models of care and reform to research and classification systems. Intersex community organising and resourcing have made a tangible difference. The Australian Capital Territory is the first jurisdiction in the country to move ahead with reforms to clinical practice, including a legislative prohibition of certain practices without personal informed consent, oversight of clinical decision-making, and investment in psychosocial support. A national community-controlled psychosocial support service has also commenced.

The Amalgamated Sophomore-Gonadoblastoma

Gonadoblastoma emerges as a benign gonadal neoplasm composed of germ cells and descendants of sex cord-stromal cells reminiscent of immature granulosa cells and Sertoli cells. Neoplasm is preponderantly associated with disorders of sexual development wherein majority (~80%) of neoplasms articulate phenotypic females. Tumefaction expounds primary amenorrhea and delayed sexual maturation with delayed development of genitalia and emergence of secondary sexual characters. Gonadoblastoma locus appears to be situated adjacent to Y centromere wherein TSPY gene is implicated in carcinogenesis. Commonly discerned distinct morphological configurations appear comprised of spherical or irregular clusters of immature Sertoli cells admixed with germ cells and circumscribing basement membrane. Intervening stroma exhibits enlarged, polygonal cells simulating Leydig cells admixed with focal calcification. Germ cells appear immune reactive to p53, placental alkaline phosphatase (PLAP), CD117/c-KIT and VASA protein or encoded testis specific Y protein. Testicular gonadoblastoma requires segregation from neoplasms such as Sertoli cell tumour. Genetic karyotyping is an essential investigation for distinguishing individuals with intersex disorders and potential occurrence of gonadoblastoma. Surgical extermination is a recommended mode of therapy for alleviating gonadoblastoma.

DIAGNOSTIC IMAGING OF DISORDERS OF SEX DEVELOPMENT (DSD) IN INFANTS AND CHILDREN

Various disorders of sex development (DSD), result in an abnormal development of genitalia formerly known as ambiguous genitalia or intersex disorders. It Is a complex social and medical emergency. The participation of a multidisciplinary team, including a pediatric radiologist, is needed to address the patient's medical need. The rst step in the management of DSD is to assign the sex of rearing, which should be based on multiple factors, such as genotype, the presence, location and appearance of the reproductive organs. various modalities of radiological imaging are used and shown to be of value in diagnosis. Ultrasound (US) is the primary tool for demonstrating internal organs, while genitography is used to assess the uterus, vagina and any stulas or Complex tracts. Magnetic resonance imaging (MRI) may provide additional and accurate information about the internal gonads and genitalia

Clinico-demographic Characteristics, Associated Anomalies and Treatment Profiles of Children presented with Undescended Testis at Medical University of Bangladesh

Background: The most common paediatric disorder among boy is undescended testis (UDT) which is usually identified during birth and these patients require proper treatment at the appropriate time due to increased risk of torsion, infertility, testicular cancer and associated inguinal hernia(>90%) as well as for cosmetic purposes.
 Objective: The purpose of this study was to determine side, palpability, age at presentation, associated anomalies and treatment plan in our institution.
 Methodology: This retrospective study was carried out in the department of Paediatric Surgery of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2017 to June 2019.
 Results: In this study 1.08% (213 children had UDT out of total paediatric outdoor 19803 patients) boys had UDT. They were aged from 7 months to 15 years (mean 5.61±3.81 years) at presentation. Only 16 (7.5%) patients presented within the 1 years of age in this study. 51.8% of patients presented at the age of >1yrs-5yrs. > 5yrs-10yrs of age was 33.5% cases and 13.6% of patients presented as late as 10yrs to 15 yrs. Unilateral UDT was found in 184 (86.4%) patients and bilateral was in 29(13.6%) patients. Right- sided unilateral UDT was in 112(52.6%) patients, left -sided unilateral UDT was in 72 (33.8%) patients. Palpable testis was found in 178(83.6%) patients and non-palpable found in 35(16.4%) patients. Associated anomalies of UDT were seen in 64(30%) patients. Hypospaedias was in 19(08%) patients and intersex disorders were in 15 (07%) patients. Orchidopexy was done in 192(90.1%) cases and orchidectomy was done only in 4(1.9%) cases. No testis was found in17 (08%) cases.
 Conclusion: UDT is one of the commonest disorders of paediatric age group. Very important issue is the early diagnosis and treatment of this anomaly. To treat the UDT in proper time help to optimize testicular function, earlier diagnosis of testicular malignancy, give cosmetic benefits and to prevent complications such as a clinical hernia or torsion.
 Journal of National Institute of Neurosciences Bangladesh, July 2021, Vol. 7, No. 2, pp. 173-176

True hermaphrodite of ovotestis in a 5-year-old child

Assigning possible and correct sex of individuals born with ambiguous genitalia, notably those with true hermaphroditism (TH) at their neonatal stage, is of paramount psychosocial advantage. A 5-year-old child with karyotype of 46, XX who was reared as male is herein presented. The right testicle was neither palpable in the scrotal sac nor in the inguinal canal. The left testicle was palpable in the scrotum and was of adequate size according to the child. A hormonal profile showed a testosterone level of 2.30 ng/dl (normal value: 30–50 ng/dl). Both testicular and ovarian tissues were found on one side (ovotestis), hence TH of ovotestis was confirmed. It is important to assign true sex for patients with an intersex disorder particularly those with TH so as to give positive psychosocial benefits for them as well as removing gonadal tissues which would lead to the development of gonadal malignancies.

Disorders of Sex Development.

Disorders of Sex Development.

Infected epidermal cyst of the clitoris in an infant

Clitoral enlargement in the pediatric population is a rare condition, usually related to problems of sexual differentiation, but malignant and benign clitoral lesions have also been described. We report the case of a newborn infant, investigated at birth for an intersex disorder because of clitoromegaly. Hormonal screening was normal and ultrasound (US) did not show a pelvic or abdominal mass. Three weeks later, the lesion was larger, tense and erythematous. An abscess was suspected. A drainage was then performed, and the bacteriological culture revealed the presence of Staphylococci aurei. A magnetic resonance imaging (MRI) performed to exclude a tumor of the soft tissue was normal. A diagnosis of infected epidermal cyst was confirmed by the pathology. Two months later, the external genital aspect was normal and the child asymptomatic.

Frequency of congenital cardiac anomalies in patients with anorectal malformations.

Objectives: To determine the frequency of congenital cardiac anomalies in patients with anorectal malformations. Study Design: Retrospective study. Setting: Department of Pediatric Surgery, Khyber Teaching Hospital, Peshawar. Period: Jan 2018 to June 2018. Material & Methods: All patients from 0 to 30 days of life both males and females (Inclusion criteria) with diagnosed anorectal malformation on clinical and radiological assessment, underwent cardiovascular work up to identify any cardiovascular disease. Patients with intersex disorders and those operated elsewhere were excluded. Mean and standard deviation were calculated for age. Frequency and percentage were calculated for gender, type of anorectal malformation, genital ambiguity, previous surgical intervention and congenital cardiac anomalies. Data was stratified for age, gender, lesion level, genital ambiguity and previous surgery to see the effect modification. Results: Out of 90 patients with anorectal malformation, there were 49 (54%) males and 41 (46%) females (M: F;1:1.19). with mean age of 7.65 ± 2.43 days. There were 39 (43.34%) patients with low anorectal malformation and 51 (56.67%) patients with high anorectal malformation. Genital ambiguity and previous surgical intervention was present in 8 (8.89%) and 11 (12.23%) patients, respectively. Congenital Cardiac Anomalies were diagnosed in 16 (17.78%) patients with anorectal malformation. Congenital Cardiac Anomalies were mostly seen in age group 1-10 days (21.95%), male children (22.45%) and high anorectal malformation (23. 52%). Conclusion: The frequency of congenital cardiac anomalies in patients with anorectal malformations was found lower than local figures. The majority of congenital cardiac anomalies associated with high anorectal malformation and male gender.

English

The disorder of the X chromosome causes defects in sex differentiation in the female phenotype. This work reports a six-month-old, Cocker Spaniel dog with intersex disorder diagnosed as pseudo-hermaphroditism. The dog was brought to the clinic with a swollen reddish penis-like protrusion, which was partially covered by the sheath. Clinical examination revealed the presence of raised nipple-like structures, resembling the nipples and extending from chest to groin region. However, the testes could not be detected. PCR analysis revealed the absence of the Y chromosome. A corrective surgery, including clitoridectomy, and removal of uterus and testicles-like structures, was performed. Histological examination of the removed organs showed underdeveloped testes and well-recognized endometrium. This report suggests that surgical correction may be used to overcome any possible complications in a six-month-old dog with pseudo-hermaphroditism

Нарушения половой дифференцировки: состояние проблемы через 15 лет после чикагского консенсуса

It is well known that the nomenclature and classification were changed in 2005 at the international consensus conference on intersex disorders, held in Chicago, where, among others, the following recommendations were proposed: (1) all children should be assigned a gender identity, and this should be done as quickly as possible, taking into account the time required for the examination. (2) all infants with congenital adrenal hyperplasia and 46,XX karyotype, including those with pronounced masculinization, must be raised as women. (3) Surgical treatment should be performed early and in cases of feminizing genitoplasty, clitoral reduction should be performed simultaneously with reconstruction of the urogenital sinus (separation of the vagina and urethra). An analysis of contemporary literature shows that all these theories, proposed 15 years ago at the Chicago meeting, failed to stand the test of time. New nomenclature and classification are constantly being revised. Currently, many groups of patients want to abolish the term «sexual maturity disorders.» Recommendations regarding gender reassignment and appropriate early surgical treatment have been completely ignored in some countries. All this was largely facilitated by the confrontational activities of a number of support groups.

Disorders of sex differentiation: state of the problem 15 years after the Chicago consensus

It is well known that the nomenclature and classification were changed in 2005 at the international consensus conference on intersex disorders, held in Chicago, where, among others, the following recommendations were proposed: (1) all children should be assigned a gender identity, and this should be done as quickly as possible, taking into account the time required for the examination. (2) all infants with congenital adrenal hyperplasia and 46,XX karyotype, including those with pronounced masculinization, must be raised as women. (3) Surgical treatment should be performed early and in cases of feminizing genitoplasty, clitoral reduction should be performed simultaneously with reconstruction of the urogenital sinus (separation of the vagina and urethra). An analysis of contemporary literature shows that all these theories, proposed 15 years ago at the Chicago meeting, failed to stand the test of time. New nomenclature and classification are constantly being revised. Currently, many groups of patients want to abolish the term «sexual maturity disorders.» Recommendations regarding gender reassignment and appropriate early surgical treatment have been completely ignored in some countries. All this was largely facilitated by the confrontational activities of a number of support groups.

37. The Impact of the 2006 Consensus Statement on Medical Discourse on Differences of Sex Development (DSD)

Published in 2006, the Consensus statement on management of intersex disorders sought to reform the medical care of people with Differences of Sex Development (DSD). The authors suggest changes in language to decrease the use of gendering (e.g. masculinized) and pathologizing (e.g. pseudohermaphrodite) language to describe patients. Despite this, people with DSD continue to express discomfort with the language their healthcare providers use. It is critical to address this gap to provide patient-centered care. Providers may struggle to use best language, and may learn the language they use through articles. In this study, a sample of peer-reviewed journal articles are analyzed to assess the impact of the Consensus Statement on the use of gendering and pathologizing language in medical discourse on DSD.

Long-term outcome in a case series of Denys-Drash syndrome.

BackgroundDenys–Drash syndrome (DDS) is a rare disease caused by mutations in exons 8 and 9 of the WT1 gene. It is characterized by the association of early onset steroid-resistant nephrotic syndrome (SRNS), Wilms’ tumour and, in some patients, intersex disorders, with increasing risk of gonadoblastoma. There are few published data concerning the long-term outcome of patients with DDS. The aim of this study was to report our experience. MethodsData were collected from five children (three boys) with confirmed DDS diagnosed from 1996 to 2017. The mean follow-up of these patients was 16 years.ResultsThe patients presented with SRNS and diffuse mesangial sclerosis at renal biopsy. All patients were hypertensive and progressed to end-stage kidney disease, initiating dialysis at a mean age of 28 months. Three patients developed Wilms’ tumour 9 months after the SRNS was identified, which was treated by nephrectomy and chemotherapy. All five patients received kidney transplantation. SRNS did not recur after transplantation in any of the patients and graft survival was similar to that of other kidney transplant recipients in our programme. All three boys had ambiguous genitalia and cryptorchidism but a confirmed male karyotype (46, XY). One girl presented with gonadal agenesis, whereas the other one had normal female ovarian tissue and external genitalia. Both girls had a female karyotype (46, XX). Gonadoblastoma was not observed at any case.ConclusionsEarly DDS recognition in patients with SRNS is crucial due to its low prevalence, the specific treatment approach required and early detection of Wilms’ tumour. Few data are available regarding long-term outcomes.

The Role of Patient Advocacy and the Declining Rate of Clitoroplasty in 46,XX Patients With Congenital Adrenal Hyperplasia.

Prior to the 1990s, most 46,XX infants with clitoromegaly secondary to congenital adrenal hyperplasia were treated with feminizing genitoplasty to make their cosmetic appearance congruent with their genotypic sex. A 2006 consensus statement for the management of intersex disorders accepted input from patient advocates and did not support purely cosmetic surgery for clitoromegaly. This study examined the extent to which the desired change was implemented in practice. Retrospective chart review was performed at a single Midwestern tertiary care medical center for patients born between 1979 and 2013. Of 45 virilized patients, 40 had clitoromegaly and 39 had urogenital sinus or posterior labial fusion. Twenty-seven (67.5%) patients underwent clitoroplasty and 33 (84.6%) underwent perineoplasty, including vaginoplasty, urethroplasty, imperforate vagina repair, and/or posterior labial fusion repair. There was a linear decline in the rate of clitoroplasty over time for the patient cohort. This study demonstrates the power of patient advocacy to improve medical practice.

Leukodystrophy with disorders of sex development due to WT1 mutations

BackgroundHypomyelinating leukodystrophies represent an expanding group of neurogenetic disorders characterized primarily by central nervous system hypomyelination and variable neurological and non-neurological involvement. Hypomyelinating disorders have been rarely associated with gonadal dysfunction, being mainly represented by hypogonadotrophic hypogonadism in 4H syndrome. WT1 gene-associated disorders are classically associated with complex phenotypes including early carcinogenic risk for gonadoblastoma and Wilms' tumor, chronic renal failure, nephrotic syndrome and sex developmental disorders in intersex disorders and ambiguous genitalia. MethodsThe authors describe three non-related Brazilian patients with hypomyelinating leukodystrophy associated with complex neurological and systemic dysfunction with WT1 gene mutations. ResultsAll described patients presented with similar neuroimaging features including thin corpus callosum, mild to moderate cerebellar atrophy and diffuse periventricular and profound hypomyelinating leukodystrophy involving supratentorial white matter with classical compromise linked to inherited non-somatic WT1 gene mutations in a similar pattern to Denys-Drash syndrome, including nephrotic syndrome with different glomerular disease, chronic renal failure, intersex disorder with ambiguous genitalia, and early occurrence of specific tumors, such as Wilms' tumor and gonadoblastoma. ConclusionsClinicians must include WT1 gene mutations in the differential diagnosis of hypomyelinating leukodystrophy with nephrotic syndrome, chronic renal failure, ambiguous genitalia or sex developmental disorders.

Shifting syndromes: Sex chromosome variations and intersex classifications.

The 2006 ‘Consensus statement on management of intersex disorders’ recommended moving to a new classification of intersex variations, framed in terms of ‘disorders of sex development’ or DSD. Part of the rationale for this change was to move away from associations with gender, and to increase clarity by grounding the classification system in genetics. While the medical community has largely accepted the move, some individuals from intersex activist communities have condemned it. In addition, people both inside and outside the medical community have disagreed about what should be covered by the classification system, in particular whether sex chromosome variations and the related diagnoses of Turner and Klinefelter’s syndromes should be included. This article explores initial descriptions of Turner and Klinefelter’s syndromes and their subsequent inclusion in intersex classifications, which were increasingly grounded in scientific understandings of sex chromosomes that emerged in the 1950s. The article questions the current drive to stabilize and ‘sort out’ intersex classifications through a grounding in genetics. Alternative social and historical definitions of intersex – such as those proposed by the intersex activists – have the potential to do more justice to the lived experience of those affected by such classifications and their consequences.

Interdisciplinary care in disorders/differences of sex development (DSD): The psychosocial component of the DSD-Translational research network.

Scientific discovery and clinical management strategies for Disorders/Differences of Sex Development (DSD) have advanced in recent years. The 2006 Consensus Statement on Management of Intersex Disorders stated that a mental health component to care is integral to promote positive adaptation, yet the parameters of this element have not been described. The objective of this paper is threefold: to describe the psychosocial screening protocol adopted by the clinical centers of the DSD-Translational Research Network; to summarize psychosocial data collected at 1 of the 10 network sites; and to suggest how systematic behavioral health screenings can be employed to tailor care in DSD that results in better health and quality of life outcomes. Steps taken in developing the largely "noncategorical" screening protocol are described. These preliminary findings suggest that DSD, as one category of pediatric chronic conditions, is not associated with marked disturbances of psychosocial adaptation, either for the family or the child; however, screening frequently uncovered "risk factors" for individual families or patients that can potentially be addressed in the context of ongoing clinical care. Administration of the DSD-TRN psychosocial screening protocol was demonstrated to be feasible in the context of interdisciplinary team care and was acceptable to families on a longitudinal basis. The ultimate value of systematic screening will be demonstrated through a tailoring of psychosocial, medical and surgical services, based on this information that enhances the quality of patient and family-centered care and subsequent outcomes.

FRI-17 “WALK LIKE A MAN”: SEX TESTING AND FEMALE GENDER IDENTITY IN INTERNATIONAL ATHLETIC COMPETITION

You have accessJournal of UrologyHistory of Urology: History Forum1 Apr 2017FRI-17 “WALK LIKE A MAN”: SEX TESTING AND FEMALE GENDER IDENTITY IN INTERNATIONAL ATHLETIC COMPETITION Unwanaobong Nseyo Unwanaobong NseyoUnwanaobong Nseyo More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2017.02.2482AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES During the 2016 Rio Summer Olympic Games, Caster Semenya participated in the 800m finals and won gold. Her win, while a personal accomplishment, also had historical implications. Seven years prior, questions began to be raised about her gender ultimately leading to her temporary ban from athletic competition while she underwent “sex verification testing.” The sex-testing to which she was subjected had a historical precedent. This paper seeks to explore the historical narrative surrounding intersex disorders as the presented in the form of female athletic competition. METHODS A literature review was conducted in PubMed which identified primary and secondary sources regarding “sex testing” and “femininity certificates.” These sources were reviewed in order to evaluate the sociocultural context of sex testing among female athletes in international competition. RESULTS Sex testing began in earnest in the 1950s with the mandatory sex testing of female athletes during the European Championships, although rumblings of questioning the ender of female athletes pre-dated the creation of this mandatory law. In the 1930s, as women became increasingly involved in athletic competition, women's bodies were scrutinized if they appeared too “male-like” or exhibited athletic exceptionalism. Early sprinters such as Dora Ratjen of Germany and Stella Walsh of Poland were driven away from the sport due to claims that they were in fact men. Once testing became official, it consisted of invasive physical examinations, heavily focused on the genitalia as women were asked to “parade nude before a panel of doctors.” Often as a direct response to these athletic community's inquiries into their sexual identity, these female athletes, who were branded as male and termed “hermaphrodite” subsequently underwent surgical reassignment. While testing transitioned to include genetic and hormone testing, the results remained relatively inconclusive and significantly altered the lives of the women on whom they were conducted. CONCLUSIONS Historically, dominant performances by women on the international athletic stage called into question their gender identity. Invasive sex testing was performed and many of the individuals who were investigated would be identified as intersex today. Contemporary discussions of transgender identity add a new layer of complexity to examinations of the intersection of gender and athletic competition as transgender athletes made history in the 2016 Rio Olympic Games by competing without having undergone gender reassignment surgery. © 2017FiguresReferencesRelatedDetails Volume 197Issue 4SApril 2017Page: e1066 Advertisement Copyright & Permissions© 2017MetricsAuthor Information Unwanaobong Nseyo More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Systematization of ambiguous genitalia

ABSTRACTSex assignment in newborns depends on the anatomy of the external genitalia, despite this stage being the final in embryogenesis. According to the current view, the genital tubercle is the embryonic precursor of penis and clitoris. It originates from mesenchymal tissue, but mesenchymal cells are arranged across the embryonal body and do not have specific androgen receptors. The nature of the signal that initiates early derivation of the indifferent genital tubercle is unknown at present. The aims of this article are to improve surgical management of intersex disorders and investigate the development of the genital tubercle. Clinical examination of 114 females with various forms of DSD revealed ambiguous (bisexual) external genitalia in 73 patients, and 51 of them underwent feminizing surgery. Intersexuality (ambiguity) in 46,XY patients results from disruptors in the pathways of sex steroid hormones or receptors; in 46,XX females arises from excessive levels of androgens. Systematization of intersex disorders distinguishes the karyotype, gonadal morphology, and genital anatomy to provide a differential diagnosis and guide appropriate surgical management. Modified feminizing clitoroplasty with preservation of the dorsal and ventral neurovascular bundles to retain erogenous sensitivity was performed in females with severe virilization (Prader degree III-V). The outgrowth of the genital tubercle and the fusion of the urethral fold proceed in an ordered fashion; but in some cases of ambiguity, there was discordance due to different pathways. Speculation about the derivation of the genital tubercle have discussed with a literature review. The genital tubercle derives from the following 3 layers: the ectodermal glans of the tubercle, the mesodermal corpora cavernosa and the endodermal urogenital groove. According to the new hypothesis, during the indifferent stages, the 5 sacral somites have to recede from their segmentation and disintegrate: the sclerotomes form the pelvic bones, the fused myotomes follow with their genuine neurotomes and the angiotomes join to the corpora cavernosa of the genital tubercle. Sexual differentiation of external genitalia is final in gender embryogenesis, but surprisingly derivation of the indifferent genital tubercle from 5 somites occurs before gonadal and internal organs development.