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Anorchism | Definition, Caused, Signs, History and Treatment

Anorchism | Definition, Caused, Signs, History and Treatment

Diseases Info

Anorchism | Definition, Caused, Signs, History and Treatment

Anorchism | Definition, Caused, Signs, History and Treatment

It is a congenital disease found in males, which marks the absence of one or more testes or testicles. It is quite rare and restricts the development of male secondary sexual characteristics like facial hair and division of fats according to male physiology during puberty. Testes, also called testicles or balls are defined as the set of organs which play a central role in the male reproductive system. They produce sperm cells and the testosterone which is a male sex hormone. Their absence kills libido and potency.

Anorchism may also be associated with the absence of various other structures of the spermatic trhact. Anorchism is also called as Anorchidism or Anorchia.

How it is caused:

In anorchism a person born with XY karyotype lacks one or both testes by birth. It is a defect by birth. After fertilization, the foetus develops rudimentary gonads within few weeks which produce more hormones for further development of the reproductive system. If gonads are not developed then female genitals are developed by the embryo in the coming few weeks.

However, if they are formed but they fail to develop or function within eight to ten weeks then a person is born with undefined genitalia. If the rudimentary testes decline after fourteen weeks then the person will have partial male genitalia with the notable absence of gonads. This is how a person suffers from anorchidism or anorchism.

Clinical signs:

Various experiments have been performed on the people suffering from anorchism. Signs vary in patients belonging to different ages. It is not observable in children. D. Tzvetkov et al say, “In every case, the patients are genotypically male with negative sex chromatin, the scrotum is empty and badly formed, the micro-penis is hypospadic, and there is no prostatic tissue. The presence of exterior genitals suggests that during somato-sexual differentiation until the scrotum is shaped, foetal testicular secretion exists [lo]. Later pathological changes appear as a result of foetal testis regression. In the past, it has been necessary to perform a laparotomy to diagnose the disease.”

Clinical history:

JAMES F. GLENN and HARRY T. McPherson in their research regarding the history of anorchism write,

“Complete absence of the gonads and failure of secondary masculine development are rarely seen. In 1933 Counsellor and ·Walker reported on 2 male patients with gynaecoid fat distribution and failure of secondary sexual development with scanty beard and incomplete maturation of the genitalia and noted 9 similar case reports.’ In 1949 Hepburn reported on surgical findings in a 19-year-old eunuchoid male patient who exhibited the characteristic findings of increased span as related to height, scanty hair and beard, small phallus and prostate and absence of ejaculation. At exploration, the right vas was rudimentary and the left vas was absent with no evidence of testicular tissue.

Wilkins indicates that bilateral anorchism is occasionally noted in “individuals with entirely normal male differentiation but the absence of secondary sexual development. Vasa differentia is normally formed but end blindly in a rudimentary epididymis. In these cases, the urinary gonadotropins are increased.”

Treatment:

In Florida Hospital, America, endocrinologist share working with patients suffering from anorchism. They have developed various strategies to overcome this congenital disease. Ranging from a variety of topics from surgical procedures to psychological therapies experts perform a variety of procedures on patients suffering from this. It involves an effective treatment plan which consists of implanting prosthetic testicle into the scrotum, hormone replacement therapy and finally psychological reassurance which enables a person suffering from anorchism to adapt to the new positive change. Before puberty, it cannot be treated because it is not diagnosed at an earlier stage.

Uni-lateral anorchia does not require any therapy or surgical intervention as long as the levels of testosterone are up till a certain level. If anorchsim is not treated well till puberty it promotes eunecoidism. Sonographies, Computer Tomography, MRT are other procedure to cure this. It is another fact that there is no cure for bi-lateral anorchia as it is sterile.

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