Search Encyclopedia:    
List of Topics Print This Page
 

Anorchia


Definition:

Anorchia is the absence of both testes at birth.

Alternative Names:

Vanishing testes - anorchia; Empty scrotum - anorchia; Scrotum - empty (anorchia)

Causes:

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.

Symptoms:

Symptoms include:

  • Normal outside genitals before puberty
  • Failure to start puberty at the correct time
Exams and Tests:

Signs include:

  • Empty scrotum
  • Lack of male sex characteristics (penis and pubic hair growth, deepening of the voice, and increase in muscle mass)

Tests include:

Treatment:

Treatment includes:

  • Artificial (prosthetic) testicle implants
  • Male hormones (androgens)
  • Psychological support
Outlook (Prognosis):

The outlook is good with treatment.

Possible Complications:

Complications include:

  • Face, neck, or back abnormalities in some cases
  • Infertility
  • Psychological problems due to gender
When to Contact a Medical Professional:

Call your health care provider if a male child:

  • Appears to have extremely small or absent testicles
  • Does not seem to be starting puberty during his early teens
References:

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


Review Date: 9/29/2014
Reviewed 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.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

   View History
  Anorchia

Related Links
Request an Appointment Online or call
1-800-789-PENN (7366)
Ambiguous genitalia
Infertility
Testes
   
   

 

About UPHS   Contact Us   Site Map   Privacy Statement   Legal Disclaimer   Terms of Use

The University of Pennsylvania Health System, Philadelphia, PA 1-800-789-PENN © 2014, The Trustees of the University of Pennsylvania