Dr. med. Dirk Manski

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Bilateral Anorchia: Vanishing Testis Syndrome

Definition of the Vanishing Testis Syndrome

Bilateral anorchia or the vanishing testis syndrome is a loss of testicular function during fetal development. It leads, depending on the time of the damage, to more or less pronounced male pseudohermaphroditism. The maximum variation is the loss of testicular function before the 8th week of fetal development, with female differentiation of the internal and external sex organs. The internal female sex organs may be missing, if Anti-Müllerian hormone was secreted before loss of the testes. The minimal variation is the male newborns with normal genitalia, but without detectable testicular function (after HCG stimulation).

Diagnosis of the Vanishing Testis Syndrome

The testosterone concentration after HCG stimulation does not show an adequate increase. Concentrations of Anti-Müllerian hormone and inhibin are low. Diagnostic laparoscopy confirms the absence of testicular tissue (McEachern, 2004).

Treatment of Bilateral Anorchia

Normally, the patient is assigned to the male gender role with lifelong testosterone replacement treatment.

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McEachern, R.; Houle, A.; Garel, L. & Vliet, G. V. Lost and found testes: the importance of the hCG stimulation test and other testicular markers to confirm a surgical declaration of anorchia.
Horm Res, 2004, 62, 124-128

  Deutsche Version: Anorchie