No abstract

Guidelines of psychosexual management for infants born with physical intersex conditions are intended to assist physicians and parents in making decisions about sex of assignment and rearing including the following: 1) sex assignment should be to the gender that carries the best prognosis for good reproductive function, good sexual function, normal-looking external genitalia and physical appearance, and a stable gender identity; 2) the decision regarding sex assignment should be made as early as possible, preferably during the newborn period, with an upper age limit for reversal of an initial sex assignment no later than 18 to 24 months; and 3) there should be minimal uncertainty and ambiguity on the part of parents and professionals regarding the final decision about sex assignment and rearing. J. Money used these guidelines in a case of a biologically normal male infant (one of a pair of monozygotic twins) whose penis was accidentally ablated during a circumcision at the age of 7 months. The decision to reassign the infant boy to the female sex and to rear him as a girl was made at 17 months, with surgical castration and initial genital reconstruction occurring at 21 months. Money reported follow-up data on this child through the age of 9 years. Although the girl was described as having many "tomboyish" behavioral traits, a female gender identity had apparently differentiated. Thus, it was concluded that gender identity is sufficiently incompletely differentiated at birth as to permit successful assignment of a genetic male as a girl, in keeping with the experiences of rearing. Subsequent follow-up by other investigators reported that by early adolescence the patient had rejected the female identity and began to live as a male at the age of 14 years. In adulthood, the patient recalled that he had never felt comfortable as a girl, and his mother reported similar recollections. At age 25, the patient married a woman and adopted her children. The patient reported exclusive sexual attraction to females. The present case report is a long-term psychosexual follow-up on a second case of ablatio penis in a 46 XY male. During an electrocautery circumcision at the age of 2 months, the patient sustained a burn of the skin of the entire penile shaft, and the penis eventually sloughed off. At age 7 months, the remainder of the penis and the testes were removed. By age 7 months, if not earlier, the decision was made to reassign the patient as a female and to raise the infant as a girl. The patient was interviewed on two occasions: at 16 years and twice while in the hospital for additional surgery at 26 years of age. At ages 16 and 26, the patient was living socially as a woman and denied any uncertainty about being a female. During childhood, the patient recalled that she self-identified as a "tomboy" and enjoyed stereotypically masculine toys and games; however, the patient also recalled that her favorite playmates were usually girls and that her best friend was always a girl. When seen at age 16, the patient had been admit...

As part of a larger study of psychosexual development and sexual functioning in women with congenital adrenal hyperplasia (CAH), we assessed self-reported sexual arousability with the Sexual Arousability Inventory-Short Form (SAI-SF; Hoon & Chambless, 1998). Compared to their unaffected sisters/female cousins (n = 15), women with CAH (n = 30) reported significantly lower sexual arousability on the SAI, with an effect size, using Cohen's d, of 1.16. For both the CAH women alone and combined with the controls, higher self-reported sexual arousability was significantly associated with (a) relationship status (married or cohabitating with a man versus being single or not in a relationship); (b) higher levels of sexual attraction to men in fantasy in the past 12 months on the Erotic Response and Orientation Scale (Storms, 1980); (c) higher Kinsey interview ratings of a heterosexual orientation in behavior in the past 12 months; and (d) more sexual experiences with men, according to a modified version of the Zuckerman (1973) Heterosexual Experience Scale (HES), in the past 12 months and lifetime (all ps < .001-.05). CAH women who were simple virilizers (versus salt-wasters) and those assigned female at birth (versus delayed or male) tended to report higher levels of sexual arousability (p < .10). Self-reported degree of satisfaction with genital surgery and genital function was also associated with higher levels of arousability. For CAH women and both groups combined, multiple regression analysis showed that the sole predictor of self-reported sexual arousability was HES lifetime sexual experiences with men. We discuss the results in the context of assessing sexual function and dysfunction in women with CAH.

MRI is a reliable method for evaluating paediatric uterovaginal anomalies, but should be analysed in conjunction with other imaging modalities (US and genitography). Previous surgery makes interpretation more difficult and, if possible, MRI should be carried out prior to any surgery. An accurate MRI examination can be extremely helpful prior to surgery and it is important for the radiologist to have knowledge of how these complex anomalies are managed and what pitfalls to avoid.

Vaginal agenesis is an uncommon congenital anomaly, and is often associated with agenesis of the uterus and fallopian tubes. Recent reports suggest that non-surgical methods to create a neovagina offer advantages when assisted reproductive treatments are used in the adult life of these women. We have reviewed the management of 39 women with congenital vaginal agenesis to assess the outcomes of neovaginal creation. In 25 women with total Müllerian agenesis, creation of a neovagina using progressive perineal dilatation alone was very successful in women motivated by a sexual relationship.