Symptoms of testicular feminization syndrome

Testicular feminization syndrome is a rare syndrome of fetal developmental disorder, which is a 46,XY male. Although the testicles secrete normal amounts of testosterone, the fetal tissue cells are insensitive to the secreted testosterone, causing the male external genitalia to develop in the female direction during the fetal period, forming the labia, vagina and clitoris. So, what are the symptoms of testicular feminization syndrome?

Symptoms of testicular feminization syndrome:

① Patients often come for consultation due to primary amenorrhea and infertility.

②Female body shape and fat distribution, with female habits.

③ Having normal female breasts, but with less breast tissue and slightly smaller or normal nipples.

④ Armpit and pubic hair are sparse or absent.

⑤ There are female external genitalia, the labia minora is poorly developed, the clitoris is normal or small, and the vagina is blind sac-like.

⑥The female internal reproductive organs are absent or underdeveloped.

⑦ The gonads are undescended testicles, and their tissue morphology is similar to that of testicles. The gonads of patients are male gonads, namely testicles. In the embryo, testicles, but due to the lack of testosterone in the target organ cells, they do not respond to testosterone, which hinders the external genitalia and males. After puberty, the testicles develop and can secrete estrogen at the same time, which produces a feminizing effect, causing breast development and external genitalia to develop into a female type. This makes the development of the vulva and the gonads opposite in gender. So it is called.

The patient has a female appearance, with female-shaped external genitalia, a relatively tall stature, long arms, and giant hands and feet. After puberty, secondary sexual characteristics appear, breasts develop, pubic hair and axillary hair are sparse or absent, the uterus and fallopian tubes have not formed, the labia minora are poorly developed, the vagina is short, and the upper part is fertile. The gonads are often in the inguinal groove or in the abdominal cavity, and sometimes they can descend to the labia majora. The testicles are still normal in appearance, but they no longer mature after puberty and become immature. They are absolutely infertile.

The above are the symptoms of testicular feminization syndrome. The treatment of testicular feminization syndrome varies with age. It is not suitable to remove the testicles for young patients because part of the testosterone secreted by the testicles during puberty is converted into estrogen in the body, which can promote the development of breasts and body shape into female type. Therefore, bilateral testicles should be removed in late puberty to prevent testicular deterioration. After the operation, appropriate amount of female hormones are given to maintain normal sexual characteristics. Vaginoplasty or other plastic surgery can be performed on the external genitalia if necessary.