|
Anorchidism
|
- Monorchidism: one testis is absent,
Usually the left testis is absent and the remaining one undergoes compensatory hypertrophy. Hormonal patterns remain normal; histologically, the diagnosis is made by finding blindly-ending spermatic vessels. If hypoplastic epididymis is seen, the diagnosis cannot be made.
- Testicular regression syndrome: both testes absent; several etiologies
- True agonadism (46 XY gonadal agenesis syndrome): ambiguous genitalia (shown in photograph at right); regression occurs at 8-12 weeks gestation
- Rudimentary testes syndrome: normal male phenotype with small cryptorchid tests; regression occurs at 14-20 weeks gestation
- Congenital bilateral anorchidism: normal external male genitalia; regression after 20 weeks gestation
- Vanishing testes syndrome: patients develop hypergonadotropic hypogonadism after puberty with infantile phallus, hypoplastic scrotum, and impalpable prostate (shown); regression between birth and puberty
- Leydig cell only syndrome: patients are anorchid but not eunuchoid, due to clusters of Leydig cells producing testosterone
|
|