Wednesday, September 12, 2007

Absent Penis

Introduction





"It's a boy!"

When we first glimpse our children in the delivery room, their sex is one of the immediate things we notice. It's also one of the first things we report to family and friends. In the last generation, many of us have seen evidence of our children's sex on ultrasound even before they were born, but still at the birth, we look to see.

How disconcerting to parents when a boy's penis seems to disappear, either at birth or shortly thereafter. The good news is that the outlook is bright.






What is it?





When a penis appears absent or too small, we call the condition inconspicuous penis. I've seen this in a great many boys. Several very different situations are lumped into this category. I will describe webbed penis, concealed penis, trapped penis, micropenis, and absent penis







Who gets it?





Boys can be born with a webbed penis, or the condition can result from an overexuberant circumcision where adhesions form between the scrotal skin and the penile skin. Webbed penis usually causes no problems (unless a routine circumcision is later performed).

Some children are born with a concealed penis (also known as buried penis or hidden penis), and for some it happens after circumcision. It is common in infants and toddlers, and occasionally seen in older children and obese adolescents.




Children are not born with trapped penis; circumcision causes it. Routine circumcision of a webbed penis or circumcision when there is significant scrotal swelling (from a hydrocele or hernia) can lead to trapped penis.



Micropenis is a hormonal problem that takes place sometime after 14 weeks' gestation when the penis has already formed.

Absent penis, or penile agenesis, is very rare, occurring in fewer than one in 20 million boys.






What are the symptoms?







A webbed penis is a normal-size penis where the skin of the scrotal sac extends partway up the shaft of the penis.

A concealed penis is a normal-size penis that lies hidden in the pubic fat pad. This condition is also called buried penis or hidden penis. Often the penis can be easily exposed by gently pulling on it or by pressing down on the surrounding fat pad.




A trapped penis is a normal-size penis that is partially stuck in the pubic fat pad. Scarring or adhesions trap the recessed penis in the fat pad. This condition can predispose children to urinary tract infections or urinary retention. Surgery is usually wise.



All of the above conditions have a penis of normal size. In determining size, the stretched penile length is far more important than the relaxed length. To evaluate penis size, stretch the penis gently and measure from the bone at the base all the way to the tip. Be sure to depress the surrounding fat pad to get all the way to the base. Here are the normal values:



Average Stretched Penile Length

(Adapted from Feldman KW, Smith DW. Journal of Pediatrics. 1975; 86:395)





:Age



Mean +/- 1 SD (inches)

Mean - 2.5 SD (inches)





0-5 months

1.5 +/- 0.3

0.75





6-12 months

1.7 +/- 0.3

0.9





1-2 years

1.9 +/- 0.3

1.0





2-3 years

2.0 +/- 0.4

1.1





3-4 years

2.2 +/- 0.4

1.3





4-5 years

2.2 +/- 0.4

1.4





5-8 years

2.4 +/- 0.4

1.5





8-11 years

2.5 +/- 0.4

1.5



Adult

5.2 +/- 0.6

3.7



Micropenis is a penis that is more than 2.5 standard deviations below the average size for age. In a newborn, a stretched penile length less than 3/4 inch (1.9 cm) is considered a micropenis.

In absent penis, or penile agenesis, the scrotum and testicles usually form normally, but the penis doesn't form at all.





How long does it last?





Without treatment, a webbed penis is unlikely to get better as the child grows.

If the concealed penis can be easily exposed by gently pulling on it or by pressing down on the surrounding fat pad, the situation will usually correct itself over time.

In a trapped penis, scarring or adhesions trap the recessed penis in the fat pad. This condition can predispose children to urinary tract infections or urinary retention.

In micropenis, if the penis grows when a three-month trial of testosterone is given, the outlook is good for normal adult penis size and function.

Absent penis is a permanent condition, although treatment can greatly improve the situation





How is it diagnosed?







When a boy has an inconspicuous penis, the parents' concerns are quite understandable. The penis should be promptly examined and measured, and the parents should be clearly told whether it is normally formed and of normal size. Whenever any question remains, a pediatric urologist is the best person to evaluate the penis and recommend a plan. Consulting a urologist is all the more appropriate when new concerns arise as the boy grows.





How is it treated?





Sometimes surgery is needed for inconspicuous penis, sometimes medical treatments, and often nothing at all.



A webbed penis sometimes requires surgery. Results with surgery, however, are excellent.

Sometimes surgery is needed for concealed penis. Either way, social, urinary and erection results are excellent.



Surgery is usually wise for a trapped penis.



In micropenis, hormone levels need to be checked. A boy's chromosomes should also be checked to see if there is an underlying genetic syndrome. An MRI may be needed to look at the hormone-secreting glands. A trial of testosterone treatment may solve the problem.



If micropenis does not respond to testosterone stimulation, the difficult question of reassigning gender arises. This decision has been made even more difficult by the conflicting and changing recommendations of experts in the field.



At one time, gender reassignment was the routine choice, even though it involves castration, surgical reconstruction, estrogen supplements, and huge emotional issues for the child and family. Many people base their opinions on the case of a baby boy whose penis was accidentally amputated during circumcision. When he was 22 months old, he was castrated and reassigned a female sex. He had surgery to make his genitals appear female. He was raised as a girl. At puberty, he was given estrogens to promote breast growth. Reports said that he had a very good adjustment to the female sex, but it later became clear that from an early age he had rejected the idea that he was a girl. During his teen years, he insisted on switching back to living as a man. He later married as a man.



Recent long-term studies of micropenis have found that even if the penis remains small, most boys raised as boys end up as sexually active, heterosexual males who stand to urinate, have a strong male identity, normal erectile function, and who enjoy sex. Their partners also report sexual satisfaction. Unfortunately, teasing by peers can be a real problem. A penile prosthesis may help.

Adults who have themselves had intersex issues argue strongly against reassignment before the child is old enough to choose. Their opinion should be listened to carefully.



Treating absent penis presents many challenges. A team including a geneticist, endocrinologist, urologist, pediatrician, and mental health experts should be involved.





How can it be prevented?





Often inconspicuous penis cannot be prevented. Avoiding inappropriate circumcisions can reduce the risk of an inconspicuous penis.

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