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HermaphroditismTrue hermaphroditism is an extremely rare form of intersex, accounting for fewer than 10% of all intersex disorders hermaphrodite, hermaphroditism, hermaprodite, hermafrodite. The gonadal tissue makeup is variable. They may have either an ovary on one side and testis on the other, or an ovary opposite an ovotestis. Bilateral ovotestes account for an additional 20% of true hermaphrodites, with 10% of patients having a testis-ovotestis arrangement. Fertility may occur in male and female patients, and sex of rearing, although historically as boys with reconstruction of hypospadias, now depends more on phallic size, ease of reconstruction, or Evaluation and ManagementEvaluation of infants with ambiguous genitalia requires a high index of suspicion that begins in the delivery room. It is imperative that members of the health-care team (i.e., obstetrics, pediatrics, nursing, and specialists including endocrinologists and pediatric urologists) work together and present a clear and uniform approach to the family. The hint of gender assignment in the delivery room, only to be changed later, can be disastrous and confusing to the family. Thus, gender-specific terms should be avoided by all until sex of rearing can be The simplest way of initiating a plan of evaluation is based on physical examination. Regardless of the phenotypic appearance, the presence or absence of gonads helps establish a working diagnosis. Although ultimate sex assignment may be based on phenotypic appearance, the gonadal sex helps the clinician rule in or out a clinically urgent situation, such as salt wasting congenital adrenal hyperplasia. It is safest to assume that phenotypic boys with bilateral nonpalpable testes have adrenogenital syndrome until proven otherwise. The presence of symmetry on examination of the genitalia helps one consider male or female pseudohermaphroditism whereas asymmetry suggests mixed gonadal dysgenesis or true hermaphroditism. The location and presence of a single urogenital opening versus separate urethra and vaginal openings and normal scrotal or labial development versus a bifid scrotum may help establish a A rectal examination shortly after birth may identify a uterus enlarged from maternal hormones. Imaging modalities greatly enhance the ability of further understanding the specific abnormality. Sonography can be a useful tool for imaging fetal genitalia and determining internal structures, such as a uterus or ovary. In addition, the adrenal glands can be assessed for enlargement in cases of AGS. A flush genitogram is performed by retrograde injection of contrast through the distal urogenital opening. This procedure can identify the urethra and level of entry of the vagina or other mullerian structures, such as a cervix or fallopian tube. More recently, magnetic resonance (MR) imaging has been useful for morphologic evaluation of Chromosomal analysis is essential for classification of the intersex abnormality, although it does not necessarily determine the sex of rearing or the type of surgical reconstruction required. Chromosomal findings certainly help in the decision-making regarding dysgenetic gonads and their risk for malignancy. In newborns with ambiguous genitalia, appropriate biochemical studies, including serum electrolytes, 17-hydroxy-progesterone, and testosterone, should be Sex assignment should be made as soon as possible after adequate information is obtained. A thorough discussion of the medical and psychosocial issues by a team of specialists is best pursued before making recommendations to the family. All too often, conflicting opinions are rendered prematurely, which may lead to confusion and distrust among A trial of depotestosterone may be warranted to determine whether penile enlargement may occur. In cases in which phallus size is inadequate, female assignment may be considered. In female hermaphrodites or true hermaphrodites with normal mullerian structures and a normal ovary on one side, female assignment is reasonable because these Sex assignment should be based on what would be the most appropriate sex of rearing. External appearance and sexual function after surgical reconstruction are the key factors in the The timing of surgery needs to be individualized based on medical (i.e., endocrine) considerations, familial acceptance of sex assignment, and technical feasibility. The general trend has been toward early reconstruction, with some patients requiring further reconstruction . For example, a girl with masculinized genitalia secondary to AGS may undergo clitoral recession or reduction, with or without vaginoplasty, early in life. Some patients may require further vaginal reconstruction at puberty. Internal genital structures that are incompatible with the assigned sex should be
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