to the parents, often in conjunction with a psychologist, to decide when to disclose the diagnosis. management management of ais is currently limited to symptomatic management; methods to correct a malfunctioning androgen receptor protein that result from an ar gene mutation are not currently available. areas of management include sex assignment, genitoplasty, gonadectomy in relation to tumor risk, hormone replacement therapy, and genetic and psychological counseling. non-consensual interventions are still often performed, although general awareness on the resulting psychological traumatization is rising. sex assignment and sexuality most individuals with cais are raised as females. they are born phenotypically female and are usually heterosexual with a female gender identity; however, at least two case studies have reported male gender identity in individuals with cais. vaginal length in 8 women with cais before and after dilation therapy as first line treatment. the normal reference range (shaded) is derived from 20 control women. duration and extent of therapy varied; the median time to completion of treatment was 5.2 months, and the median number of 30-minute dilations per week was 5. dilation therapy most cases of vaginal hypoplasia associated with cais can be corrected using non-surgical pressure dilation methods. the elastic nature of vaginal tissue, as demonstrated by its ability to accommodate the differences in size between a tampon, a penis, and a baby's head, make dilation possible even in cases when the vaginal depth is significantly compromised. treatment compliance is thought to be critical to achieve satisfactory results. dilation can also be achieved via the vecchietti procedure, which stretches vaginal tissues into a functional vagina using a traction device that is anchored to the abdominal wall, subperitoneal sutures, and a mold that is placed against the vaginal dimple. vaginal stretching occurs by increasing the tension on the sutures, which is performed daily. the non-operative pressure dilation method is currently recommended as the first choice, since it is non-invasive, and highly successful. vaginal dilation should not be performed before puberty. gonadectomy while it is often recommended that women with cais eventually undergo gonadectomy to mitigate cancer risk, there are differing opinions regarding the necessity and timing of gonadectomy. the risk of malignant germ cell tumors in women with cais increases with age and has been estimated to be 3.6% at 25 years and 33% at 50 years. however, only three cases of malignant germ cell tumors in prepubescent girls with cais have been reported in the last 100 years. the youngest of these girls was 14 years old. if gonadectomy is performed early, then puberty must be artificially induced using gradually increasing doses of estrogen. if gonadectomy is performed late, then puberty will occur on its own, due to the aromatization of testosterone into estrogen. at least one organization, the australasian paediatric endocrine group, classifies the cancer risk associated with cais as low enough to recommend against gonadectomy, although it warns that the cancer risk is still elevated above the general population, and that ongoing cancer monitoring is essential. some choose to perform gonadectomy if and when inguinal hernia presents. estrogen replacement therapy is critical to minimize bone mineral density deficiencies later in life. hormone replacement therapy some have hypothesized that supraphysiological levels of estrogen may reduce the diminished bone mineral density associated with cais. data has been published that suggests affected women who were not compliant with estrogen replacement therapy, or who had a lapse in estrogen replacement, experienced a more significant loss of bone mineral density. progestin replacement therapy is seldom initiated, due to the absence of a uterus. androgen replacement has been reported to increase a sense of well-being in gonadectomized women with cais, although the mechanism by which this benefit is achieved is not well understood. counseling it is no longer common practice to hide a diagnosis of cais from the affected individual or her family. parents of children with cais need considerable support in planning and implementing disclosure for their child once the diagnosis has been established. for parents with young children, information disclosure is an ongoing, collaborative process requiring an individualized approach that evolves in concordance with the child's cognitive and psychological development. in all cases, the assistance of a psychologist experienced in the subject is recommended. neovaginal construction vaginal expander zsi 200 ns zsi 200 ns vaginal expander stretching the female vagina many surgical procedures have been developed to create a neovagina, as none of them is ideal. surgical intervention should only be considered after non-surgical pressure dilation methods have failed to produce a satisfactory result. neovaginoplasty can be performed using skin grafts, a segment of bowel, ileum, peritoneum, an absorbable adhesion barrier (intercede, made by johnson & johnson), buccal mucosa, amnion, dura mater. or with the support of vaginal stents/expanders. success of such methods should be determined by sexual function, and not just by vaginal length, as has been done in the past. ileal or cecal segments may be problematic because of a shorter mesentery, which may produce tension on the neovagina, leading to stenosis. the sigmoid neovagina is thought to be self-lubricating, without the excess mucus production associated with segments of small bowel. vaginoplasty may create scarring at the introitus (the vaginal opening), which requires additional surgery to correct. vaginal dilators are required postoperatively to prevent vaginal stenosis from scarring. inflatable vaginal stents are placed in the vagina deflated and then gently inflated. other complications include bladder and bowel injuries. yearly exams are required as neovaginoplasty carries a risk of carcinoma, although carcinoma of the neovagina is uncommon. neither neovaginoplasty nor vaginal dilation should be performed before puberty. prognosis challenges presented to people affected by this condition include: psychologically coming to terms with the condition, difficulties with sexual function, infertility. long-term studies indicate that with appropriate medical and psychological treatment, women with cais can be satisfied with their sexual function and psychosexual development. cais women can lead active lives and expect a normal lifespan. epidemiology it is estimated that cais occurs in 1 in 20,400 to 1 in 99,000 individuals with a 46,xy karyotype. nomenclature main article: other names for androgen insensitivity syndrome historically, cais has been referred to in the literature under a number of other names, including testicular feminization (deprecated) and morris syndrome. pais has also been referred to as reifenstein syndrome, which should not be confused with cais. history the first definitive description of cais was reported in 1817. the condition became more widely known after it was reviewed and named testicular feminization by american gynecologist john mclean morris in 1953. people with cais georgiann davis seven graham eden atwood