Anorchia is the absence of both testes at birth.
The embryo develops early sex organs in the first several weeks after the egg is fertilized. If early testes do not develop in males before 8 weeks into the pregnancy, the baby will have female genitals.
If the testes disappear between 8 and 10 weeks, the baby will be born with ambiguous genitalia. This means the child will have parts of both male and female genitals.
However, if the testes disappear between 12 and 14 weeks, the baby will have normal male genitals (penis and scrotum), but no testes. This is known as congenital anorchia, or the "vanishing testes syndrome."
The cause is unknown. Genetic factors may be involved in some cases. .
This condition should not be confused with bilateral undescended testes, in which the testes are located in the abdomen or groin.
The outlook is good with treatment.
Call your health care provider if a male child:
Vanishing testes - anorchia; Empty scrotum - anorchia; Scrotum - empty (anorchia)
Achermann JC, Hughes JA. Disorders of sex development. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 23.
Lambert SM, Vilain EJ, Kolon TF. A practical approach to ambiguous genitalia in the newborn period. Urol Clin North Am. 2010 May;37(2):195-205. PMID: 20569798
Updated by: Scott Miller, MD, urologist in private practice in Atlanta, GA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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