Thoughts on the nature of identity: how disorders of sex development inform clinical research about gender identity disorders.

Disorders of sex development (DSD), like gender dysphoria, are conditions with major effects on child sexuality and identity, as well as sexual orientation. Each may in some cases lead to change of gender from that assigned neonatally. These similarities-and the conditions' differences-provide a context for reviewing the articles in this issue about clinical approaches to children with gender dysphoria, in relation to assessment, intervention, and ethics.

A model of delivering multi-disciplinary care to people with 46 XY DSD.

In 2006, a consensus statement was jointly produced by the Lawson Wilkins Pediatric Endocrine Society (LWPES) and the European Society of Paediatric Endocrinology (ESPE) concerning the management of disorders of sex development (DSD) [1]. A recommendation provided by this consensus was that evaluation and long-term care for people affected by DSD should be performed at medical centers with multi-disciplinary teams experienced in such conditions. Here we provide our team's interpretation of the 2006 consensus statement recommendations and its translation into a clinical protocol for individuals affected by 46 XY DSD with either female, or ambiguous, genitalia at birth.

Thoughts on the nature of identity: disorders of sex development and gender identity.

Children with disorders of sex development have similarities to, but also marked contrasts with, children with normal anatomy but who have gender dysphoria. Understanding gender identity development in children with sex disorders will probably help us understand typical gender identity development more than in understanding gender development in children with gender identity disorder.

A brief primer for pediatric urologists and surgeons on developmental psychopathology in the exstrophy-epispadias complex.

Developmental psychopathology is common in children with exstrophy. It may be mild or severe, and it may persist or transform as the child grows. The pediatric urologist is ideally situated to identify signs or symptoms of early developmental psychopathology in these children.

Pediatric psychology in genitourinary anomalies.

Children with genitourinary anomalies are at risk for developmental and adjustment challenges. Pediatric psychologists can address the needs of these children within the urology clinic through focusing on assessment, intervention, and prevention of psychosocial problems associated with their urologic diagnosis. Care is optimized if surgeons and mental health providers work together to care for this challenging group of patients.

An Evidence-Based Model of Multidisciplinary Care for Patients and Families Affected by Classical Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency.

In 2002 a consensus statement pertaining to the management of classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency was jointly produced by the Lawson Wilkins Pediatric Endocrine Society and the European Society of Pediatric Endocrinology. One of the recommendations of this consensus was that centers should maintain multidisciplinary teams for providing care and support to these patients and their families. However, the specifics for how this should be accomplished were not addressed in the original consensus statement.

Successful removal of a penile constriction ring in a 14-year-old male.

Penile strangulation is a rarely described medical emergency, especially in the adolescent population. This case demonstrates the successful removal of a constricting metal ring from the penis of a 14-year-old male with a diamond blade equipped orthopedic oscillating saw while under ketamine anesthesia in the emergency department.

Anxiety disorders in children with epispadias-exstrophy.

Objectives: To evaluate the hypothesis that anxiety disorders are common comorbid conditions in children with the epispadias-exstrophy complex.

Methods: Twenty consecutive outpatient exstrophy subjects ranging in age from 5 to 22 years were assessed using a formalized semistructured psychiatric evaluation and were categorized according to the Diagnostic and Statistical Manual of Mental Disorders, version IV, criteria. Disorders were identified if subjects endured moderate to severe impairment in their home, academic, and social environments.

Gender identity and sex-of-rearing in children with disorders of sexual differentiation.

Aim: To compare declared sexual identity to sex-of-rearing in individuals with disorders of sexual differentiation.

Methods: All 84 patients > or =5 years old in a pediatric psychosexual development clinic were assessed for sex-of-rearing and sexual identity. Diagnoses included 1) male-typical prenatal androgen effects but an absent or severely inadequate penis - 45 patients with cloacal exstrophy or aphallia; 2) inadequate prenatal androgens and a Y-chromosome - 28 patients with partial androgen insensitivity (pAIS), mixed gonadal dysgenesis (MGD), hermaphroditism, or craniofacial anomalies with genital ambiguity; 3) inappropriate prenatal androgen effects and a 46,XX karyotype - 11 patients with congenital adrenal hyperplasia (CAH).

A 7-year experience of genetic males with severe phallic inadequacy assigned female.

Purpose: We examine the hypotheses underlying the clinical decision to assign female gender at birth in male neonates with severe phallic inadequacy.

Materials And Methods: A total of 18 genetic males with severe phallic inadequacy were assessed longitudinally for physical, social, psychological and sexual identity development. Diagnoses included cloacal (11) and classic (1) exstrophy, partial androgen resistance (3), mixed gonadal dysgenesis (2) and penile agenesis (1).

Psychosexual development in genetic males assigned female: the cloacal exstrophy experience.

Genetic males who have cloacal exstrophy, a rare, severe pelvic field defect that leads to aphallia, traditionally have been socially, legally, and surgically sex-assigned female at birth and reared female, despite a male karyotype and a typical male prenatal hormonal milieu. Psychosexual development in such children previously has been unknown. Studies of 29 children revealed that despite the absence of the typical postnatal and pubertal androgen surges and the presence of female genitalia, all female-assigned subjects displayed a marked male-typical shift in psychosocial and psychosexual development.

Situating unusual child and adolescent sexual behavior in context.

There is a lack of scientific knowledge concerning what constitutes normal versus unusual sexual behaviors among minors. Clinical judgements in these cases often are clouded by unfounded sociocultural assumptions, personal biases, legal issues, and moral considerations. Current diagnostic nomenclature that is used commonly for adult sexual activities is inappropriately applied to minors.

Discordant sexual identity in some genetic males with cloacal exstrophy assigned to female sex at birth.

Background: Cloacal exstrophy is a rare, complex defect of the entire pelvis and its contents that occurs during embryogenesis and is associated with severe phallic inadequacy or phallic absence in genetic males. For about 25 years, neonatal assignment to female sex has been advocated for affected males to overcome the issue of phallic inadequacy, but data on outcome remain sparse.

Methods: We assessed all 16 genetic males in our cloacal-exstrophy clinic at the ages of 5 to 16 years.