The identification of gender includes morphological criteria (karyotype, gonads, genital ducts, vulvae and secondary sexual characteristics) and psychological criteria (dependent sex, social sex). A contradiction between morphological and psychological criteria is considered as psychosexual abnormality, including transsexualism and transvestism. If there is a contradiction between morphological criteria, it is abnormal sexual differentiation or hermaphroditism, which can be divided into sex chromosome aberration, male pseudohermaphroditism, female pseudohermaphroditism and true hermaphroditism. Among them, male pseudohermaphroditism refers to chromosome 46XY, gonads are testes, but masculinization of genital ducts and/or external genitalia is incomplete, and it belongs to the most complicated kind of sexual differentiation anomalies in terms of etiology and type. 1. Materials and methods The cases in this group were from the departments of urology, endocrinology and obstetrics and gynecology of our hospital. The minimum age of the patients at the time of their first visit to our hospital was 2.33 years, the maximum age was 28 years, and the average age was 15.54 years; one was married and 46 were unmarried. There was one case of marriage between parents who were cousins, and four groups of “brothers” were found to be the same patients. The etiological diagnosis of 31 patients was further clarified by laboratory and imaging examinations after admission, and 16 patients did not have a clear etiological diagnosis. Table 1 Etiological classification of male pseudohermaphroditism patients admitted to our hospital Diagnosis Example Karyotype Vulvar phenotype Age Bone age Puberty Puberty Mullerian duct Endocrine examination Familial onset Number Vocal change, breast hair Structure and its laryngeal node, childbearing He pubic axillary hair Low Gonadotropic 2 46XY male phenotype, 15-24, more normal None None None None LH, FSH, T↓, vegetative gonadotropic penis, testes Average Age Small GnRH excitatory test, hypotropic Test small 19.5 6-9 years HCG excitatory T test diminished 46XY sexual 4 46XY female phenotype, 17-28, more normal None None None None LH, FSH↑, T↓, 2 “Sister” glandular hypoplasia small clitoris, not average small for same age E2↓,HCG excitation Patient 1 group, see testis,2 22.8 1-6 years T test absent Married 1 case blind end vaginal 17-alpha hydroxylation 3 46XY female infantile type 16-17, more normal none none none LH, FSH↑,T↓, enzyme defect ,slightly large clitoris E2 normal or ↓ in 2 cases with blind end of pubic tract 16.3-6 years old, 1 case with inguinal testis Complete androgenic hormone 1 46XY female phenotype, 22 none none none LH, FSH, T normal range with or without inguinal testis, E2 ↑ partial androgenic hormone (CAIS) 7 46XY female infantile type 5-23, more normal with different 2 cases with, none LH, FSH, T, E2 FSH, T, E2 insensitivity complex to hermaphroditism Average small for same age Change in degree 2 cases None Normal or ↑ syndrome (PAIS) with hypospadias, 14.7 1-3 years Testes can be found in labia majora or groin 5-α reductase 14 46XY Hermaphroditism, will 8-24, more normal Varies None None T normal or ↑, T/DHT ↑ Parental aunt phenotype or scrotum Average large for same age Change in degree The degree of deficiency was greater than 30 times greater than that of parental marriage 1 case of hypospadias 14.6, 4 years old to 2 “sisters”, labia majora or abdominal 4 years old 3 “sisters” testes could be found in the groin 1 group each Note: 1. For patients older than 18 years old, bone age was calculated as 18 years old. 2. Among the male pseudohermaphroditism patients without etiological diagnosis, there were 3 “brothers” patients in one group, but they had a sister with no abnormality. Among the 47 patients in this group, 5 patients who received surgery outside the hospital and then came to our hospital for a second consultation, and 42 patients who came to our hospital for the first consultation. During the consultation and treatment in our hospital, 42 patients were treated surgically, 2 were treated non-surgically, and 3 were discharged for other reasons. The longest follow-up period was 23 years. Of the 42 patients who underwent surgery at our hospital, 8 were preoperative dependent gender males and 34 were preoperative dependent gender females. The 8 patients who were raised male before surgery maintained their male gender after surgery (among them, 1 patient underwent bilateral cryptorchidopexy in an outside hospital at the age of 16, but his psychological gender was male, and he came to our hospital at the age of 24 and received androgen replacement therapy); the 34 patients who were raised female before surgery changed their gender to male 16 and maintained female 18 after surgery; therefore, the total gender of patients after surgery was male During the first inpatient surgery, 24 patients with male gender were discharged from the hospital with good recovery after the first surgery in 7 cases and poor recovery after the first surgery in 17 cases (4 cases with cystostomy tube, 6 cases with urethral fistula, 2 cases with urethral stricture, 1 case with infection, and 4 cases with poor appearance); 17 patients underwent 2 or more surgeries in our hospital. (4 cases of urethral fistula repair, 3 cases of urethral stricture expansion, 1 case of urethrotomy, 5 cases of urethroplasty, 1 case of penile deformity correction, 2 cases of penile lengthening, and 1 case of penile prosthesis implantation). All 18 patients of postoperative gender female were discharged with good recovery after the 1st operation, and 12 of them were recommended to undergo vaginoplasty again before marriage. Two non-operatively treated patients, both diagnosed with hypogonadotropic hypogonadism, will receive supplemental androgen replacement therapy for life. Three patients were discharged automatically without treatment, one with a diagnosis of complete androgen insensitivity syndrome (CAIS), who was discharged automatically because the testes were not found by ultrasound; two patients were discharged automatically for financial reasons, as the cause of the disease was not clearly diagnosed. CAIS) Partial androgen insensitivity 0 7 2 2 0 5 3 2 Sensory syndrome (PAIS) 5-α reductase deficiency 0 14 1 1 0 13 7 6 Male pseudohermaphroditism 2 14 5 5 0 9 6 3 Table 3 Patients undergoing first surgery Postoperative sex male 24 patients Postoperative sex female 18 patients Postoperative sex female Type of surgery Male external genital reconstruction and/or cryptorchidism Transvaginal bilaterally Transabdominal bilaterally Gonadal and/or clitorovaginoplasty Clitoroplasty Cryptorchid fixation and/or vagotomy Excision Gonadectomy or internal genital resection Number of surgeries 23 1 12 5 4 2 Table 4 Classification of male pseudohermaphroditism Diagnosis Definition Testicular (1) Idiopathic hypogonadotropic hypogonadism Hypogonadism due to decreased FSH and LH secretion, inability to synthesize sufficient testosterone Production (2) Leydig cells unresponsive to LH Leydig cells are unresponsive to LH and cannot synthesize testosterone due to hypoplastic Leydig cells or defective LH/HCG receptors on Leydig cells. It is autosomal recessive. (3) 46XY gonadal dysgenesis, also known as embryonic testicular degeneration syndrome and testicular disappearance syndrome, can be familial or sporadic, the cause of which is unknown. The synthesis of testosterone requires the participation of five enzyme systems, which are involved in the synthesis and metabolism of testosterone. (5) Defects in 3-beta hydroxysteroid dehydrogenase affect the synthesis of testosterone, and defects in the first three enzymes also affect the synthesis of cortisol and aldosterone. (7) Defective 17-20 carbon chain cleavage enzyme (8) Defective 17-β hydroxysteroid dehydrogenase (9) Complete androgen insensitivity syndrome (CAIS) refers to a qualitative or quantitative change in the androgen receptor that prevents androgens from exerting their masculinizing effects. CAIS has a completely female phenotype and may or may not have pubic and axillary hair at puberty. (12) Reifenstein’s syndrome is also known as familial incomplete male pseudohermaphroditism type I (PAIS). (13) Rosewater syndrome (14) 5-alpha reductase deficiency Testosterone is converted to dihydrotestosterone by 5-alpha reductase, the main androgen that differentiates the undifferentiated vulva into the male vulva. 5-alpha reductase deficiency results in pseudohermaphroditism. It is also known as pseudovaginal perineal scrotal hypospadias and familial incomplete male pseudohermaphroditism type II. (15) Mullerian duct persistence syndrome Testicular Sertoli cells do not secrete MIS or Mullerian ducts are insensitive to MIS, Mullerian ducts do not degenerate and differentiate into fallopian tubes and uterus Due to different laboratory conditions in each hospital, it may be difficult to make a definitive etiologic diagnosis in some patients, for example, if the laboratory is not able to detect blood DHT or 5-alpha reductase activity, it is not possible to make a definitive etiologic diagnosis by T/DHT >35 or low 5-alpha reductase activity to diagnose 5-alpha reductase deficiency. However, we believe that although the etiological diagnosis is important, it is not a decisive factor in determining the treatment plan; after the diagnosis of male pseudohermaphroditism is confirmed, treatment can be carried out while following the principles of male pseudohermaphroditism treatment. The purpose of surgery is to enable the patient to participate in social and sexual life in a healthier way. The key to surgery is the choice of postoperative gender and the treatment of gonads. The principles of surgery include: 1. Early surgery. It is best to treat in the neonatal period so that the postoperative gender is consistent with the patient’s dependent gender and social gender to avoid psychopathy.2. The choice of postoperative gender [8] should be based on the advantages of the external genitalia, reproductive ducts, gonads, the patient’s and family’s wishes, age at the time of surgery, and the patient’s preoperative dependent gender and social gender, but doctors should focus more on the surgical conditions of the external genitalia and reproductive ducts and the The patient’s preoperative gender, and if possible, the postoperative gender is preferred to female. In this group of patients, there were 8 cases in which the preoperative gender was male, and all the family members requested to keep the male gender after surgery; there were 34 cases in which the preoperative gender was female, and 16 cases in which the postoperative gender was changed to male. However, it can be seen from the above surgical outcome statistics that the proportion of patients discharged after complete healing from surgery is not satisfactory due to the complexity of the surgery to make the patient male and the high requirements for the patient’s own surgical conditions, the surgeon’s surgical level, and postoperative care. The chances of various postoperative complications are high; moreover, often a single operation is not satisfactory and even multiple operations are required. One patient in our group with long-term follow-up underwent multiple surgeries in our hospital during the 18 years from the age of 12 to 30, including penile deformity correction, vagotomy, ductal urethral orthoplasty, cystostomy, testicular descent, scrotoplasty, penile lengthening, and penile prosthesis implantation, and the patient had poor postoperative sexual performance, a heavy psychological burden, and a heavy financial burden. Therefore, based on our follow-up experience, we believe that the postoperative gender is preferred for women, which is consistent with many reports from home and abroad. However, for patients who are older at the time of consultation, whose social gender is already male and who strongly request to maintain male gender after surgery, their opinions should be fully respected, because for these patients, even though they cannot have normal sexual life after surgery, their postoperative morphological gender is consistent with their psychosexual and sexual behavior tendencies, so that they do not develop psychosexual perversions and can participate in social and labor life in a healthier way, which is also more in line with “physiological-psychosocial medical model”; however, they should be preoperatively explained in detail about the complexity of the operation, the possibility of many postoperative complications, and the poor male sexual function after the operation, and then perform the operation after obtaining full understanding and approval. In addition, for patients with 5-alpha reductase deficiency, some scholars believe that they often experience gender reversal in adulthood if they are raised as females, so it is advisable to raise them as males and perform male external genital orthopedic surgery before puberty.3. Gonads and genital ducts that contradict the fostering gender should be removed, especially ectopic or dysplastic gonads, to prevent the possibility of malignant transformation. It has been reported in the literature that germ cell tumors occur in about 30% to 60% of patients with simple XY gonadal hypoplasia, which is the most tumor-prone type of sexual development abnormalities. 4. Long-term postoperative hormone supplementation is required to promote and maintain the corresponding secondary sexual characteristics. The treatment of male pseudohermaphroditism patients not only includes surgery, but also involves psychological and social factors, which is a long-term problem. What is the quality of life about them after surgery? Are they able to participate in social work normally? Do they have a satisfactory sexual life? Are they married and are they happily married? And so on, we will continue to follow up, observe and count, hoping to come up with a more comprehensive treatment and care plan.