What is true hermaphroditism? True hermaphroditism refers to the presence of both ovarian and testicular gonadal tissues in the body. True hermaphroditism gonadal abnormalities are manifested in different ways, while ovotestis refers to the presence of both testicular and ovarian tissues in the same gland. The most basic chromosomal karyotypes of patients with true hermaphroditism are 46, XX, 46, XY, 46, XX/46, XY chimerism, 45, XO/46, XY and so on. The proportion of various chromosomal karyotypes varies among reports. Clinical manifestations The external genitalia show different degrees of malformation, in male fondlers, most have penis and hypospadias, more than half have incomplete fusion of labia-scrotum, and few have normal male external genitalia; those with female phenotype have large clitoris, most have urogenital sinus, and most have dysplastic uterus. Pubertal manifestations: 70% have marked breast enlargement, a few have dysplasia or no development; half have menstrual flow and some have ovulation. In males, breast development, periodic “hematuria” and testicular pain; in females, enlarged clitoris, labia majora like scrotum, absence of vagina, amenorrhea or periodic abdominal pain, or a mass may be palpated in the abdomen or groin. The diagnosis of true hermaphroditism: true hermaphroditism, according to clinical manifestations and karyotype can not be diagnosed, often need to combine hormone level test, MRI, CT, B ultrasound and other examinations, if necessary, both sides of the gonadal probe and biopsy can confirm the diagnosis, the emergence of laparoscopic technology can be completed in the case of avoiding open surgery. The treatment of true hermaphroditism 1, gender re-determination In addition to referring to the results of the biological examination of the patient’s genetic, anatomical, physiological, etc., it must also be combined with the patient’s age, gender, external genital morphology and the wishes of himself and parents to decide. When choosing the gender, the patient’s own wishes should be fully respected. 2. gonadectomy and hormone supplementation The timing of gonadectomy and whether to remove or not should be considered: 1. The influence of gonad presence and removal on the formation of secondary sexual characteristics during puberty. If one side is testis and the other side is ovary, the opposite gonad should be removed as soon as possible; if it is ovotestis, the opposite gonad should be removed when the secondary sexual characteristics are more complete at puberty. 2. The site of gonads. Mainly refers to the site of the testicles. If the testicle is determined to be male, but cannot be moved to the scrotum, it should be removed. When the testicle is determined to be female, the testicle located in the groin or labia should also be removed. 3.Reconstruction of internal reproductive organs Structures that are not compatible with the identified sex, such as the fallopian tubes, uterus and vas deferens, are best removed at the time of dissection, and for some patients, this is a decisive step in sex selection. 4.Reconstruction of external genital organs The main purpose is to make the vulva have a morphology that is nearly compatible with the identified gender and have a good enough sexual function. 1.Penile reconstruction The inguinal flap, abdominal wall axial flap or forearm free flap can be used for penile reconstruction. Among them, forearm flap method of penile reconstruction because of its subcutaneous tissue is relatively thin, not bloated, realistic appearance, the effect is better. The urethra can be reconstructed using vaginal mucosa, labia minora skin or scrotal skin. At present, it is possible to perform mastectomy, hysterectomy, ovariectomy, vaginectomy, and at the same time apply vaginal mucosa or labia minora skin, scrotal skin to reconstruct the urethra, forearm flap or abdominal axial flap to reconstruct the penis in one stage. It is also possible to remove the breast, uterus, ovaries and vagina while preforming the urethra from the vaginal mucosa and burying it under the forearm or lower abdominal wall penile reconstruction flap, delaying the flap and forming the penis and urethra at the same time during the second stage surgery. 2.Vaginal reconstruction Vaginal reconstruction can be done with inguinal flap and scrotal flap, but there are disadvantages such as short reconstructed vagina, no secretory function, dryness during sex, etc. Sigmoid colon reconstructed vagina is odoriferous and easy to prolapse, currently the better choice is to use ileocecal segment with blood vessels to reconstruct vagina. With the application of laparoscopic assisted technology, there is almost no obvious scar on the abdomen and no scar on the perineum. The reconstructed vagina is of sufficient length and has mucus secreting function, with realistic appearance and satisfactory sexual life. The clitoris plays an important role in sexual behavior. Most of the clitoral corpus cavernosum should be removed, and the apical tissue should be made into a tissue flap with vascular nerve tip to be preserved, which has sensation and can be erected to ensure good sexual function after vaginal reconstruction. True hermaphroditism is a kind of gender dysphoria, which is not obvious in the early stage, but only becomes obvious after puberty, and in some cases, although it is obvious in early childhood, parents often do not want to publicize it, but decide the gender of their children according to their own likes and dislikes. Psychological and biological gender is seriously distorted. Therefore, for young children with abnormal sex organ development, we advocate early consultation and early decision on gender selection, so as to make their cultivated gender, biological gender, social gender and their own psychological gender orientation consistent and harmonious, in order to facilitate a healthy sexual orientation and psychosexual growth. In addition to facilitating a harmonious sexual orientation and psychosexual development, early and reasonable diagnosis and treatment may also help to preserve the possibility of fertility, as it has been reported that both male and female phenotypes have the possibility of fertility after removal of the oviduct in true hermaphroditism.