an absent or unsalvageable penis) they must be removed before puberty to prevent virilization from rising testosterone. Testes in androgen insensitivity are a special case: if there is any degree of responsiveness to testosterone, they should be removed before puberty. On the other hand, if androgen insensitivity is complete, the testes may be left to produce estradiol (via testosterone) to induce breast development, but there is a slowly increasing risk of cancer in adult life. Streak gonads without a Y chromosome cell line need not be removed but will not function. Finally, the gonads in true hermaphroditism must be directly examined; atypical gonads with Y line or potential testicular function should be removed but in rare instances a surgeon may try to preserve the ovarian part of an ovotestis. Potential surgical problems: A lifetime of hormone replacement will be required, to avoid osteoporosis and enable sexual functioning. Cloacal exstrophy and bladder exstrophy repair is needed regardless of the sex of assignment or rearing. Simple bladder exstrophy in a genetic female does not usually involve the vagina. Cloacal exstrophy in a genetic female usually requires major surgical reconstruction of the entire perineum, including bladder, clitoris, symphysis pubis, and both the vaginal introitus and urethra. However, the uterus and ovaries are normally formed. Severe bladder exstrophy or cloacal exstrophy in genetic males often renders the phallus widely split, small, and unsalvageable. The scrotum is also widely split, though testes themselves are usually normal. From the 1960s until the 1990s, many of these infants were assigned and raised as females, with fashioning of a vagina and gonadectomy as part of the perineal reconstruction. Potential surgical problems: Surgery for the more severe degrees of cloacal exstrophy is extensive and usually multistage. A variety of potential problems and complications can occur, including need for long-term colostomy or vesicostomy. Creating a functional urethra is difficult and poor healing, with scarring, stricture, or fistula can require a vesicostomy to prevent urinary incontinence. Construction of a functional internal and external anal sphincter can be equally difficult when this has been disrupted as well. Functional problems can warrant a temporary or long-term colostomy. The added challenge for the most severely affected genetic females, and for genetic males who are being raised as females, is construction of a neovagina. Scarring is extensive and the lower torso disfigured even with the best outcomes. Finally, it has become apparent that some XY