Epispadias: Incontinence

Achievement of continence is problematic with exstrophy, as none of the normal structures to control the bladder exist, and must be created through surgical intervention. Some techniques include vesicostomies, Mitrofanoff diversions, augmentation of bladder volume with sections of sigmoid colon, and so forth. In some cases, normal voiding through the penile urethra can occur, but in others a diversion of the urinary flow through means of clean intermittent catheterization through a stoma, or surgically created port is necessary. Artificial bladder neck devices to control the bladder are available.

By the way, people with hypospadias can have difficulty with incontinence, too, although it’s often more a leaking situation rather than a reduction in bladder voiding control. Lots of other conditions can cause incontinence, such as spinal cord damage or weakness of the pelvic floor muscles. Incontinence issues can be difficult to handle emotionally, but these issues can be “right sized” as the person accepts that incontinence is just another physical problem that we learn to deal with.

Note: Incontinence pads and briefs for men and women are available (you can buy them online if you’re shy) and they work pretty well, as I’ve learned from experience. –Betty

Epispadias: Fertility and Pregnancy

Fertility in men with isolated epispadias is often lower than fertiity in the normal population. Abnormal ejaculation, lower sperm counts or azoospermia is seen in roughly half to three quarters of men with exstrophy epispadias complex. Theoretically, if normal sperm are present in the testicles of these men, the potential for fatherhood exists, but assisted fertility techniques may be required to overcome poor sperm quality.

Women with epispadias who are pregnant may have more difficulty carrying their baby to term than other women because of differences in their pelvises and other organs. The gynecologist/obstetrician may decide to monitor pregnant women with epispadias more often and more carefully.

Epispadias: Treatment

Treatment is surgical in all cases. The main goal of surgery for the exstrophy is closure of the abdominal wall and formation of a bladder, if one can be salvaged. The spacing or width of the pelvis can can be abnormal in cases of exstrophy, called diastasis, and surgery to rotate and join the halves of the pelvis often takes place in infancy.

Epispadias is corrected with techniques like the Cantwell-Ransey approach to close the urethra and the Kelly procedure. The main difference in epispadias is that the penis is very wide, and very short. The erectile chambers or cavernosa in the epispadic penis are separated and are not connected to each other, which is not the case in the typical penis or hypospadic penis. The aim of the Kelly procedure is to bring the portions of the penis anchored to the pelvis together to conserve and liberate as much length as possible so the proportions of the penis are more typical. Correction of chordee follows standard techniques practiced in hypospadias repair.

Epispadias: Causes & Incidence

Epispadias is caused by malformation of the embryonic genital turbercle and cloacal membrane sometime in the 5th or 6th week of pregnancy. It is not related to Hypospadias, which is thought to be a hormonally moderated defect of penile formation and closure of the urethal plate which takes place about 8 weeks after conception.

Estimates are that epispadias with exstrophy occurs about once every 30,000 births, isolated epispadias without exstrophy 1 in 120,000, and isolated epispadias in females once in 500.000.

Epispadias: Description

Epispadias is a malformation of the male genitalia in which the urethra ends or opens on the upper surface of the penis. This birth defect is most commonly observed as a part of the exstrophy epispadias complex, which is a spectrum of failures of midline fusion affecting the pelvis, abdominal wall and bladder. Characteristics of epispadias include a very short and wide penis often classified as complete epispadias when the entire urethra is exposed from the abdominal wall to the tip of the penis. There is usually upward bending or chordee associated with epispadias.

Epispadias can also occur in females, where it is manifested as a split clitoris and displacement of the urinary meatus.

Epispadias is always a part of the exstrophy complex, but can also occur as an isolated birth difference in a child with an otherwise normal bladder, pelvis and abdominal wall.

Adult Surgical Re-Repair for Hypospadias

So, something is not feeling quite right down there. . . . Maybe you are noticing your stream decreasing when you void, or pain in your testicle or your side or lower back. It might hurt a bit when you void or you find you have to strain to empty your bladder. Maybe you are feeling lumps or hard spot in your penis, discomfort during sexual activity, or maybe you are having a lot of urinary tract infections. What does it mean?

You try to ignore it but it won’t go away. You may start having flashbacks to surgeries you had down there as a boy. You want to do anything to avoid having to admit that something might be wrong, something that might require surgery.

Unfortunately this is a very common situation. Most urologists don’t do any type of long term follow up studies on all the boys with hypospadias or epispadias that they successfully “fix.” If they did the actual “success rates” would likely be very low, because as we age there is a good chance we may need some type of re-repair.

The unfortunate reality is that there is no perfect solution when a doctor tries to rebuild, recreate or repair part of our body, especially if foreign tissue is introduced or tissue not made for that purpose is given a new form (i.e. re-building a urethra using foreskin or skin from the inner arm, thigh, or bladder). There is a good chance such repairs will have a shelf life and you will need to consider having a re-repair later in life.

From experience, we hear story after story of guys who were “fixed” as children and then, if they are lucky, they hit their mid 30’s or early 40’s and start to realize that something is going wrong. This can bring up all sorts of psychological issues and past trauma for the person.

The following is a list of some of the issues or complications that can happen:

  • Strictures (this seems to be the most frequently occurring issue by far)
  • Narrowing of the meatus (opening of the urethra)
  • Blockages such as those resulting from hair growing in the urethra
  • Stones in the urethra (possibly due to hair growth in the urethra)
  • Epididymitis (pain in that passage way from the testicle to the urethra)
  • Fistulas (an extra hole that fluids can pass through—like springing a leak)
  • Diverticulum (a widening of an area along the urethra that pouches out when filled with fluid)
  • Chronic urinary tract infections

The most common issue is urethral stricture disease. A stricture is a narrowing of the urethra that causes the stream pressure to reduce when voiding. It also causes pressure on the systems located behind the stricture. Things can back up and cause problems eventually for the prostate, the bladder, the kidneys and the testicles. Strictures can be the result of scar tissue building up inside a re-created urethra. It could be the result of the foreign tissue that was used to recreate or patch up the urethra breaking down, or problems with the area where the newer tissue meets the original tissue. Sometimes the tissue doesn’t have good blood flow or it may not respond to sex hormones the same way the original tissue does.

The most common initial solution for strictures seems to be to dilate the urethra. This involves inserting a tool to stretch the strictured part of the urethra to make it wider. This can sometime provide some temporary relief, but it is never really a solution to the problem. Dilating a strictured urethra just serves to re-traumatize the already scared tissue and it is likely that this will eventually make the situation worse.

Usually, at some point you will have to face the possibility that you will indeed need some sort of re-repair. This can be a very hard reality to face. You may have gone years since ever being in the hospital because of your penis. People around you might not know about your issues with hypospadias or epispadias and will wonder what medical problems you are having, especially if you have to miss work for surgery.

  • How do you tell your loved ones, family members, or close friends about the situation?
  • What do you say to your employers and co-workers?
  • How do you find the right doctor?
  • What about health insurance?

Many of the traumatizing emotional feelings that may have been locked up for years can start to come flooding back to the surface as you realize you may need further surgery.

Even if you have a supportive spouse, partner or close family members it can be very difficult to really feel supported or feel that they understand the emotions attached to this situation. In reality they can’t truly understand, as much as they might want to. This is why it is so important to find supports that know what you are going through—supports that you can only really get from other guys with HS or ES. With the connections available through the internet and social networking systems you no longer need to feel alone or isolated when facing adult re-repairs for your HS or ES. Groups like HEA (the Hypospadias and Epispadias Association: www.heainfo.org) can provide an opportunity for you to learn through articles, online chats and message boards, in person connections through their network of members and annual conferences, and more.

This psychosocial support is probably one of the most important factors to consider when facing an adult re-repair. It is much easier to deal with when you know you have connections with people who really understand and have been there.

By Chris in BC

Mother of Boy with Hypospadias

Hi I am Ky and am the founder of the yahoo club Mums with hypospadias kids.

My son was born in 1992 with moderate hypospadias, back then I had no access to computers and didn’t do any research into the topic, I was just told that it had to be fixed (and who was I to argue I’m not a doctor), boy if I only knew the things I know now. Don’t get me wrong I would have still had it corrected but I would have had the power of knowledge at my side to fight inexperienced doctors.

My son was 7 months old when he was operated on, and all went well although at postop appointment a doctor stated to me “Oh! he has made a bit of a mess with that one” this was all that was ever said.

When my son was 2 years old I noticed that he looked like he only had one testicle, this had to be repaired by surgery, not one doctor had picked up on it before I noticed it.

As my son grew I noticed that he was very embarrassed by the way his penis looked, mind you there was so much skin around the head of the penis it almost looked like a hammer, so I understood his embarrassment.

When my third son was born in 1999 he was born with an undescended testicle, so since I had to go to a specialist anyway I decided to take my first son along too and ask the doctors opinion. While she was having a look we found three fistulas, what were these holes from, I had no idea ( I have since learnt that fistulas are like blow outs in the uretha ), now it wasn’t a question of whether to have him neatened up or not he had to have surgery anyway. Both sons were operated on the same day Feb 2000, what a horrible day that was.

My first sons catheter dropped out twice and was reinserted twice, at the postop appointment we found that one of the fistulas was still there and leaking, it was devastating to think that he had to be operated on yet again. It has been 5 months and we have recently discovered that the fistula has closed over, YEAH !!!!

I now have a club where I help other mothers learn through my experience and try and help them by offering support, knowledge and most importantly friendship.

Healthy Little Boy with No Physical Signs of His Condition

Hi my name is Rebecca and my son’s name is Will. Will was born with severe hypospadias. At birth the doctors could not tell a gender because of the placement of the urethra and the undescended testicles. X-rays confirmed that he had no girl parts but we still had to wait for the chromosome test to come back. This was an awful experience for a new mom who was sick for 9 months of her pregnancy. The day after Will’s birth we saw a pediatric Urologist. He confirmed hypospadias and told us about the surgery options. We decided to start surgery as soon as possible. We did not want Will to have any memories of this as it was very traumatic. We will of course tell him about it, but we did not want him to remember any pain or trauma. The first surgery was done about 9 months old and they straighten the penis, descended one testicle and laid the groundwork for the new urethra. The second surgery was when he was about 15 months old. During that surgery they rebuilt the new urethra to extend to the end of the penis, brought down the other testicle and fixed a hernia. All in all Will is a healthy little boy, with no physical signs of his condition. When he looks at himself he will not know that he ever had a problem and all he will know of it is what I tell him. I hope this give encouragement to all anticipating surgery that things can go well and we wish everyone well. Thank you.


Glad Postponed Surgery Until Son Was Older

My son was diagnosed with severe penoscrotal hypospadias with chordee at birth. He was never able to stand to void; he had to sit down. All the doctors told me it would be better for him to have the surgery at a young age before he turned one. We saw three or four pediatric urologists before we found one we felt comfortable with. I was very scared and uneducated about the whole procedure and the outcomes. We actually had surgery set for several different times and I always backed out of them. Then one day my son came to me and said, “Mommy, why can’t I stand and pee like daddy?” It broke my heart and I explained to him and asked him if he wanted to have the surgery. I explained to him that it would hurt of a little while, but the doctor would give him medicine for the owie… So with his help we decided to do the surgery. My son was five when he had the procedure done. The surgery took about 5 long, long hours. The first few hours after surgery were ok until it came time for him to go potty for the first time. They wouldn’t let him leave the hospital till he went, and it took hours before he would go.. He had to go so bad and he would start to go a little bit and then would just freeze up and scream and cry; it was a horrible feeling and then I thought I made the wrong decision on the surgery but not for long. It took him about 6 hours till he finally went to the bathroom.. The first 5 times where very very painful for him but by the next day he was back to his normal self. The only time it would hurt was the first time going to the bathroom in the morning. His surgery was only about four months ago so we really don’t know the outcome yet, but his urologist says it looks great and he is optimistic about having to have anymore surgeries. One thing though is it took my son about three months to even try going to the bathroom standing up.. He was afraid to…. He thought if he went potty standing up that if he wanted to ever go sitting down again he would have to have another surgery… About the right time to do surgery is when you feel ready to, don’t let nobody pressure you into it if it don’t feel right…. I am glad I waited to do my son’s when he was older… He at least can understand a little better what was going on and what was going to happen. For who ever reads this I want to say Good Luck and God Bless you and your children.. Children are so fragile please be kind to them….


Our Son Looks Perfect

My son was born with a hypospadias. When the pediatrician that examined him the morning after his birth told me, she briefly explained what it was and said, “But don’t worry. After surgery he’ll have a beautiful penis!” (My husband was not there — he had gone home to get some well-deserved sleep.)

When he was three months old, we took my son to see a pediatric urologist, who I did not like. He had no bedside manner, which led me to have no confidence in him. My husband has been in the health care industry for quite some time. He asked around and the guy had a good reputation. However, we moved a few months later and prepared ourselves for finding another doctor.

We both work for a hospital system with its own health plan. There is not a pediatric urologist on staff, but there is one urologist who handles all the hypospadias corrections. Regardless, we requested that our son be referred to a pediatric urologist, but our request was denied. Off we went to see the guy on staff…

The doctor was confident that he could correct our son’s level 1 hypospadias, so when our son turned 11 months old, he had the surgery.

I couldn’t believe my eyes when I removed his diaper for the first time! Everything was black and blue! And because we had to double-diaper a squirmy little boy, I lined up people to come and help me that week he was recovering. (Just to have someone entertain him while I changed him.) As the week wore on, everything started to look much better. After eight days, the catheter was removed with no complications.

On his follow-up visit this April, the doctor told us that the surgery was so successful, we would not even have to tell him he had a hypospadias — that he looks no different than other boys. My husband and I were, quite frankly, relieved. We will make that decision as he gets older. Our concern is that he NOT grow up with the stigma that he is different from other boys.

I must admit — I read a lot of horror stories about corrections gone bad and was very worried. However, this guy did a wonderful job — and our son looks perfect!