Penile agenesis

Jump to: navigation, search
Penile agenesis
ICD-9 752.69

WikiDoc Resources for Penile agenesis

Articles

Most recent articles on Penile agenesis

Most cited articles on Penile agenesis

Review articles on Penile agenesis

Articles on Penile agenesis in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Penile agenesis

Images of Penile agenesis

Photos of Penile agenesis

Podcasts & MP3s on Penile agenesis

Videos on Penile agenesis

Evidence Based Medicine

Cochrane Collaboration on Penile agenesis

Bandolier on Penile agenesis

TRIP on Penile agenesis

Clinical Trials

Ongoing Trials on Penile agenesis at Clinical Trials.gov

Trial results on Penile agenesis

Clinical Trials on Penile agenesis at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Penile agenesis

NICE Guidance on Penile agenesis

NHS PRODIGY Guidance

FDA on Penile agenesis

CDC on Penile agenesis

Books

Books on Penile agenesis

News

Penile agenesis in the news

Be alerted to news on Penile agenesis

News trends on Penile agenesis

Commentary

Blogs on Penile agenesis

Definitions

Definitions of Penile agenesis

Patient Resources / Community

Patient resources on Penile agenesis

Discussion groups on Penile agenesis

Patient Handouts on Penile agenesis

Directions to Hospitals Treating Penile agenesis

Risk calculators and risk factors for Penile agenesis

Healthcare Provider Resources

Symptoms of Penile agenesis

Causes & Risk Factors for Penile agenesis

Diagnostic studies for Penile agenesis

Treatment of Penile agenesis

Continuing Medical Education (CME)

CME Programs on Penile agenesis

International

Penile agenesis en Espanol

Penile agenesis en Francais

Business

Penile agenesis in the Marketplace

Patents on Penile agenesis

Experimental / Informatics

List of terms related to Penile agenesis


Overview

Penile agenesis is a very rare birth defect in humans, occurring about once in 5-10 million births, where a male child is born without a penis.[1] There were recently thought to be fewer but the condition is remarkably more frequent in India and South-east Asia. It is also known as aphallia [from the Greek, "a" for negative or no, and "phallia" for penis]. Most patients have no known family history of aphallia, and often have a normal male anatomy, save for the absence of the penis.

A partner condition is testicular or gonadal agenesis. This is where the male child is born without gonads and consequently develops no testes. The function of Leydig cells in the embryo is to convert the gonads into testicles in males-to-be and into ovaries in females. In this condition, the process fails.

Penile agenesis can exist independently after full testicular development, though the causes of this in particular are uncertain. Testes are the sole producer of 5-alpha dihydrotestosterone (5aDHT) in the male body. One cause of the gonads failing to metamorphose into testes is the absence of 5aDHT. Therefore the masculizing process to allow for the development of the genital tubercle which eventually becomes the penis is halted. In this scenario, the child is born with penile and testicular agenesis. Another slang term for this condition is "nullo".

The sufferers of just one of the conditions usually continue as males throughout their lives. With very few exceptions the penile and testicular agenetic is brought up as a female and eventually undergoes http://www.wikidoc.org/index.php/Sex_reassignment_surgery. This is despite being 46xy karyotype male and having no female sexual characteristics. This is an unclear part of anatomy. The organs that prevent the development of the embryo into a female are called Anti-Mullerian hormones. They are commonly believed to be created in the testes, but male embryos without testes still appear to produce these hormones.

Complications

One complication of the full condition is the absence of a urinary outlet. This can be approached as follows. Before genital metamorphosis, the urethra runs down the anal wall, to be pulled away by the genital tubercle during male development. It remains there in the agenetic. In one case, 45 years ago, the surgeons redirected the urethra to the rim of the anus immediately after birth.

Patients with penile and testicular agenesis are impotent.

Treatment

Largely, treatment is psychological and surgical reconstruction.

See also

References

  1. Bangroo, A. K. (2005). "Penile agenesis" (PDF). J. Indian Assoc. Pediatr. Surg. 10 (4). Unknown parameter |coauthors= ignored (help)

Linked-in.jpg