Overview.
The use of progestins, estrogens, and androgens during pregnancy, especially early pregnancy, may masculinize the female embryo or fetus. The effects of estrogens on the masculinization of the female fetus are inconsistent when taken at different times in pregnancy. If sex hormones are taken before the 12th week of pregnancy, the closure of the labial sacs is more pronounced; if sex hormones are used after the 12th week of pregnancy, the enlargement of the clitoris is more pronounced, and sometimes the vaginal opening opens into the urethra, but this is less common.
Etiology
Abnormalities of sexual differentiation caused by the mother and increased exogenous androgens, including early pregnancy application of progestins and androgen preparations with potential androgenic activity, causing masculinization; mid-pregnancy application causes only clitoral enlargement and urogenital sinus malformations. Maternal presence of masculinizing tumors during pregnancy including male cell tumors, metastatic ovarian cancer, luteoma, lipoid tumors, mesenchymal cell tumors, and mesenchymal cell hyperplasia can also cause masculinization of the female fetus.
Symptoms
The effects of taking estrogen at different times of pregnancy on female fetal masculinization are inconsistent. If sex hormones are taken before the 12th week of pregnancy, the closure of the labial sacs is more obvious; if sex hormones are used after the 12th week of pregnancy, the enlargement of the clitoris is more obvious, and sometimes the opening of the vaginal canal opens into the urethra, but this is less common. The degree of masculinization of the female fetus appears to be less in the case of androgens, and the enlarged clitoris can be gradually reduced in size after birth.
Examination
Sex chromatin is positive, with chromosome grouping 46,XX. 24-hour urine 17-ketosteroids and pregnanetriol are elevated. ultrasound and CT sometimes show bilateral adrenal enlargement or space occupying the adrenal glands.
Diagnosis.
Diagnostic criteria:
1. female infant born with hermaphroditic malformation of external genitalia.
2. mother has a history of application of sex hormone preparations during pregnancy.
3. mother with or without manifestations of masculinization.
4. imaging studies of the ovaries or adrenal glands reveal tumors.
5. The female infant has normal postnatal growth and development, with normal pubertal development and no physical or metabolic abnormalities caused by E2 synthesis disorders.
Treatment
1. Preoperative preparation
(1) Diagnostic tests are required before surgery ① urine 17-ketosteroid; ② blood 17-hydroxyprogesterone basal value test; ③ chromosome examination; ④ X-ray examination; ⑤ ultrasound or CT examination; ⑥ genetic diagnosis.
(2) Preoperative psychological care Communicate with the patients and explain the successful cases of surgery to eliminate their fear, reduce their unfamiliarity with the hospital environment and medical personnel, and make them actively cooperate with the surgical treatment.
(3) Preoperative preparation: Remove the pubic hair around the clitoris and take metronidazole tablets orally as prescribed by the doctor.
(4) For children, those who need general anesthesia need to fast for 6 to 8 hours before surgery.
(5) The operation should be avoided during menstruation. Wash the vulva every night for three days before the operation.
2. Surgical methods
Clitoral reduction surgery, bilateral labiaplasty.
3. Postoperative treatment and care
(1) Strictly aseptic operation, input anti-inflammatory and hemostatic drugs as prescribed by the doctor, closely observe the changes of vital signs, and notify the doctor in time if there is any abnormality.
(2) The environment of the ward should be spacious and bright, quiet and clean.
(3) Nurses should inspect the patients diligently, keep the dressings dry and clean, observe whether the dressings are loose and oozing, and notify the doctor in time to deal with any abnormalities.
(4) If there is an indwelling urinary catheter should be kept open, do not fold or twisted pressure, guide the patient to drink more water to achieve the role of flushing the urethra, so as to avoid the formation of urinary tract infection caused by discomfort. Encourage and help the patient to turn over, lateral fixation of the urinary catheter side, so as to avoid long-term localized pressure on the body and lead to pressure sores.
(5) If the patient is in pain, the nurse should guide the patient to self-regulation and distraction. If the pain is intolerable, notify the doctor and apply medication for pain relief as prescribed by the doctor.
(6) Three days after the operation, the patient should be fed with fluids, and on the fourth day, the patient should be fed with semi-fluids, and a high-protein and easy-to-digest diet should be fed to the patient.