Category Archives: Hypospadias

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

Hypospadias Repair Surgery

Informed Consent

In this section, we would like to present information of importance to anyone who is considering a surgery for hypospadias repair for their child, or themselves.

First off, HEA does not have an official position on surgery, whatsoever. We feel that the need for surgery is a personal determination and one of our organizational missions is to insure that all the information is available to the patient, and his decision is based on sound and solid advice and fact. No one can truly make an informed decision without all the possible outcomes of the procedure described and discussed–this is known as informed consent. In just about every legal system, informed consent is a patient right and the responsibilities to the patient on the part of the health care provider is codified in civil law.

Risks

All surgical procedures carry a degree of risk, it does not matter if the procedure is circumcision or open heart surgery. Most of the risks that a patient takes in surgery are the common ones, like bleeding, infection, adverse reaction to anesthesia, failure to heal, scarring and the like. Hypospadias surgeries will range from the very simple “touch up” of a glanular hypospadias without chordee, to the most severe case of penoscrotal hypospadias with severe chordee, requiring creation of a complete urethra in multiple staged surgeries. No one would argue that the child with the most severe case of hypospadias will require surgery, often to allow basic sexual and normal urinary function where none existed previously. For this child the risk of surgery is clearly a sensible undertaking compared to the benefit of undergoing procedures.

As the hypospadias becomes more distal, or less severe, the functional impact on daily life and sexual activity is not as great, and in the vast majority of mild coronal cases, no surgery is often chosen. Surgery in the mildest cases is cosmetic in benefit and will not confer much functional improvement to the patient, if at all. In this case, the risk is relatively higher, since the benefit of the process is not as great as it was for the first patient with severe impairment of function. The basic risks are still the same. In this case, the patient has more choices and options, since he can choose to do nothing.

Choosing No Treatment

About 60 to 65% of all cases of hypospadias are of the coronal or glanular type, which means the defect is confined to the glans or head of the penis or just at the junction of glans and shaft. For the most part, these children can urinate standing, and the erection is straight enough for intercourse in adulthood. A great majority of men and boys in this classification of hypospadias do not have corrective surgery, so this is a completely valid option that many parents have chosen.

Complications Normally Associated with Hypospadias Repair

Note that there is no guarantee of success, nor does the possibility of having a complication mean that any particular patient will have complications…the following is presented as a description of the complications of surgery we have experienced as a collective group of patients.

Wound Breakdown and Fistulae

The most frequent complication of hypospadias surgery is wound breakdown during the healing process, or surgeries that “fall apart” or grafts and flaps that fail to “take.” A re-do session is often the only recourse in these cases. Fistulae are passages from the urethra to the surface of the penis that leak or spray urine. These are usually always caused by failure of the healing process along a suture line, and most modern repair techniques avoid allowing suture holes in the various tissue layers to line up, creating paths for leaks. Separate closure of layers and good technique are the best defense against a fistula. It is possible for a fistula to open months or years after a surgery, when it is thought that the penis had completely healed.

Strictures

All incisions in tissue will incur some degree of scarring during healing, including internal structures like the lining of the urethra. A stricture is a local scar formation in the urethra which restricts or stops the flow of urine. Stricturing is the main cause for revision of childhood surgeries in adult patients. It seems that there is a definitely lifespan to hypospadias surgeries performed using earlier techniques, and we advise men in this situation to find a specialist in adult hypospadias revision surgery. Adult and pediatric hypospadias is not the same thing, and adult hypospadias repair is a growing specialty in urologic surgery. Newer techniques like buccal mucosa grafts that use the lining of the mouth to fashion new urethra have far less risk of stricture formation, since buccal mucosa is very scar resistant. The downside is that two surgical sites are created to recuperate from. Buccal Mucosa surgeries are still fairly new, so long term longevity of these repairs is not known.

Hair Growth in Urethra

Older surgeries often grafted hair bearing skin for urethroplasties, and when the child became an adolescent, hair would grow in the urethra causing discomfort and urinary dysfunction. The use of hair bearing skin is avoided in current practice. Laser ablatement of the hair can bring some relief, but often a repeat urethroplasty is necessary.

Scarring

Scarring of the skin of the penis and glans can result in loss of sensation, or change in existing sensation that may be positive or negative. It is very uncommon for sensation to be completely impaired, but extensive scarring from multiple procedures in the past may make it necessary to remove all scarred tissue and begin with fresh grafts. Scarring can be very upsetting to the patient anxious about the appearance of his penis.

Expectations for the outcome of surgery is something you must discuss with your surgeon. In the past, ability to urinate standing and a straight erection for intercourse was the yardstick by which hypospadias surgery was judged. To be charitable, some of the aesthetics of surgeries following this doctrine were less than optimal. It was almost a universal given that the penis would be circumcised in the process. Now, the appearance of the penis has become very important, and foreskin reconstruction is practiced for those who wish to remain intact. As a patient and consumer of your surgeons’ services, it is up to you to discuss your expectations of the outcome of the surgery and make sure you understand the goals your surgeon is working to achieve in the procedure. Although the technique now is very good and aesthetics are much better than in the past, the surgeon must still work with what the patient brings to the operating table; the basic proportions and size of the penis cannot be changed.

Psychological Considerations

The discussion so far has centered on physical and functional concerns of hypospadias. Psychologically, living with hypospadias can be very complicated, and while one man with a mild case has no concerns or problems whatsoever, the next may find his mild hypospadias a huge burden in life, and surgery becomes an obsession. Simply having surgery is not going to change a life pattern and correct all the issues one faces in life overnight. This is why an honest discussion of the mental aspects of surgery is very important, with the assistance of a mental health professional or other trusted persons as a support mechanism for the patient moving forward.

Hypospadias Treatment

The treatment for hypospadias is surgery. There are over 200 published techniques and surgical procedures that correct hypospadias and it isn’t possible to delve into much detail here. The need for surgery ranges from optional in the mild forms, to becoming necessary to allow function in the more severe cases. Each case of hypospadias must be evaluated on its own merits. Frequently, chordee must be corrected before hypospadias can be addressed, since chordee can affect the degree of hypospadias. While single stage surgeries are becoming more routine, it is likely that a two-stage procedure will be chosen in cases of severe chordee, or severe hypospadias and chordee. Chordee repair will occur in the first stage, and typically six months later a second surgery is scheduled to revise the location of the urinary opening.

HEA recommendations regarding surgery:

  • Do not rush into a decision. Hypospadias is not life threatening and will not change or get worse in time. There is no emergency, and no need for haste. Take your time.
  • Gather as much information as you need to make a decision you are comfortable with. Choose a surgeon who is familiar with the degree of hypospadias you or your child has, and one who has experience in that severity of hypospadias.
  • Parents should consider delaying surgery until the child is old enough to take part in decision making.
  • Adults should seek out specialists in adult hypospadias repair, or revision of childhood surgeries. Pediatric hypospadias repair is not the same as adult surgery. Adult hypospadias repair is a recognized specialty in urology. In addition, some plastic surgeons specialize in hypospadias repair and re-repair.
  • Ensure that you communicate your expectations of the outcome to the surgeon and that you understand that certain things are possible and others are not. For years the standard of care was to create a straight erection for intercourse and a good stream to allow urination while standing. Cosmetics were not given a high priority. This is changing and the appearance of the penis after repair is good with modern surgeries. What cannot be done is to create a penis that is larger than it was to begin with. While the penis may appear longer after chordee repair, it is simply a straightening of the penis the patient had to begin with.
  • Consider doing nothing. Surgery always leads to scarring, which can develop into strictures, which can require further surgery to repair. Surgery always leads to a loss of some sensation. This loss of sensation may range from slight to highly problematic. Surgery frequently involves loss of some tissue (even when tissue from the foreskin, buccal mucosa, or other areas is added to re-form problematic structures). Repeated surgeries on the penis can mean loss of more and more tissue, which in some cases can mean reduction or loss of function.

by Douglas J

Causes and Rates of Occurrence

The causes and rates of incidence of hypospadias have not yet been definitively defined by researchers. Past studies have been small and samples have not been representative of the general population. Official reporting on hypospadias has been spotty. The news media has tended to pick up these poorly-done studies and mis-state their conclusions while exaggerating their importance. That said, we’re pretty confident that the following information is correct.

Hypospadias can occur in generations of families in a classic hereditary pattern and also as isolated cases. Most hypospadias is isolated, so while it can be passed down, it is not necessary to have a relative with hypospadias for there to be a possibility it will occur. Estimates are that there is an 8% increased risk of hypospadias if the father is affected, and 12% increased risk if an older sibling is also hypospadic. Please note that this is NOT an 8 or 12 percent risk of hypospadias in any random birth. It is an estimate of the increase in the overall background risk of bearing a child with hypospadias.

Overall estimates of the rate of occurence of hypospadias range from 1 in 300, to 1 in 125 live births, and like any other human demographic, the numbers change by location, accuracy of record keeping, consistency in classification of the difference and so on. While there is some difference in rates by race, the differences are not statistically significant. Hypospadias occurs in all races and ethnicities. Some researchers have concluded that hypospadias is increasing. Others have not reached that conclusion, pointing to inconsistent reporting of hypospadias in the past, and natural fluctuations in annual statistics.

Some things we don’t know about hypospadias:

  • There is a lot of internet content on pesticides, pthalates, phytoestrogens, DES, prescription drugs, industrial pollution and so on, and the hypothetical effects on birth defects. While we do know that certain prescription drugs can cause hypospadias, the links between hypospadias and other substances in the environment have not been proven. At this time, there is no proven single cause for hypospadias, and since hypospadias is highly variable in form and occurence, it is likely that it is caused by multiple factors.

Hypospadias (HS), What Is It

Having been born with HS at a time before the internet, it was a condition that did not have a name in my case until I was in my twenties. Thinking it was just me with the condition, I never went to the reference library to research it and my parents, sibling, doctor, etc. never brought up the subject.

There are various forms of hypospadias from mild, where the meatus (outlet of the urethra where the urine and semen come out) is on the glans, through more severe where the opening is on the shaft of the penis, to quite severe where the opening can be between the scrotum and anus. All the variation may have chordee, which is a bend in the penis, and this may cause problems when the penis is erect so that sexual penetration may be difficult. With HS there may also be a hooded foreskin which covers half of the glans; this tissue may be used in any subsequent surgical procedure.

It may be that a severe case of HS needs some surgical procedure to try to improve the chordee and/or bring the meatus nearer the tip of the penis. There can be some failures in these procedures; strictures (a narrowing of the urethra) in the newly formed urethra or a fistula (an extra hole through which urine leaks out) in the scar line may present. Mild cases may be left as they are at birth since the functions of the penis are working well.

There is the psychological aspect to consider, however, since the male individual tends to be aware of his penis from an early age. Inevitably there will be comparisons made at school or in showering after sport and just how informed the individual was made at home may well help to cope with this situation. In other words, the young person should be aware that he is not alone in this HS business and need not be ashamed of his differently-shaped penis.

I would say that two or three young adults with HS have recently told me that they have made immediate friends with people at school, both male and female, who are aware of their HS. These people have been treated with respect and understanding. This is something I did not dare do as a young adult but just shows that knowledge, and the proper use of it, can be a great help in coming to terms with hypospadias.

by an HEA Member

Hypospadias: An Overview

Stated simply, hypospadias is a mislocation of the urinary opening, below the usual place on the tip of the penis in males, or when the bladder empties through the front vaginal wall in females. This is the end of the simple part, as hypospadias is one of the most variable of birth differences and is the most common birth difference in boys. The location of the opening can vary from just below the tip of the penis, to the most severe variety in which the bladder empties just forward of the rectum. Hypospadias occurs as a part of over 100 known genetic syndromes, and can be accompanied by other birth differences such as inguinal hernia and undecended testicles.

Chordee, a bending of the penis, is frequently a fundamental part of hypospadias, although it is possible for both conditions to occur without the other. Boys with hypospadias will often have a hooded foreskin, which is a foreskin that is incomplete on the bottom of the penis. Circumcision should not be performed on boys with hypospadias, as foreskin often provides tissue necessary for hypospadias repair surgery. It is still possible to perform hypospadias repair on a circumcised child, but it may be necessary to harvest grafts from less ideal locations if foreskin is not available. Some specialists can preserve or improve the appearance or function of the foreskin during hypospadias repair, if circumcision is not desired. Chordee is commonly repaired with hypospadias in single stage procedures, but more complicated cases may done in staged surgeries, with chordee repair being first.

Hypospadias is a term some specialists prefer to reserve to describe the male condition; in females, vesico-vaginal fistula (a passage from the bladder to the vagina) is the preferred diagnosis. In rare cases, this passage is the urethra. Diagnosis would depend on whether there is a normally located native urethra in addition to the fistula, the presence of which would exclude hypospadias. Girls with fistulae or hypospadias will urinate from the vagina, often with discomfort due to the irritating properties of urine. Children of both genders may have frequent or recurring urinary tract infections and in these cases follow-up investigation is often indicated to determine if urinary tract anomalies exist, or if reflux is present at the kidneys.

Hypospadias can occur in generations of families in a classic hereditary pattern and also as isolated cases. Most hypospadias is isolated, so while it can be passed down, it is not necessary to have a relative with hypospadias for there to be a possibility it will occur. Estimates are that there is an 8% increased risk of hypospadias if the father is affected, and 12% increased risk if an older sibling is also hypospadic. Please note that this is NOT an 8 or 12 percent risk of hypospadias in any random birth. It is an estimate of the increase in the overall background risk of bearing a child with hypospadias.

Overall estimates of the rate of occurence of hypospadias range from 1 in 300, to 1 in 125 live births, and like any other human demographic, the numbers change by location, accuracy of record keeping, consistency in classification of the difference and so on. While there is some difference in rates by race, the differences are not statistically significant. Hypospadias occurs in all races and ethnicities. Some researchers have concluded that hypospadias is increasing. Others have not reached that conclusion, pointing to inconsistent reporting of hypospadias in the past, and natural fluctuations in annual statistics.

Some things we don’t know about hypospadias:

  • There is a lot of internet content on pesticides, pthalates, phytoestrogens, DES, prescription drugs, industrial pollution and so on, and the hypothetical effects on birth defects. While we do know that certain prescription drugs can cause hypospadias, the links between hypospadias and other substances in the environment have not been proven. At this time, there is no proven single cause for hypospadias, and since hypospadias is highly variable in form and occurence, it is likely that it is caused by multiple factors.

Arthur: Douglas J