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Hypospadias

Most boys are born with a fully functioning penis. But a congenital condition called hypospadias can produce a penis that not only performs inefficiently but also looks different. Luckily, pediatric urologists have various surgical techniques at their disposal to repair the ill-placed urethral opening, hooded foreskin and curvature associated with this condition. The following information should help you speak to your son's urologist.

What happens under normal conditions?

The penis serves a dual role in males by providing a pathway for urine to exit the bladder and for semen to enter the vagina. Both tasks are facilitated when the urethral opening (meatus) is positioned normally at the tip of the head (glans) of the penis.

At birth, the foreskin usually cannot be pulled back to expose the head of the penis. In most boys the foreskin becomes retractable in the first few years of life, although in others it may not be able to be pulled back until as late as puberty.

What is hypospadias?

The most crucial steps in the development of the penis take place between weeks nine and 12 of pregnancy. During this time, male hormones act to stimulate formation of the urinary channel and foreskin. Various problems with hormone action may result in a congenital condition called hypospadias.

Hypospadias is a common birth defect, occurring in one out of every 150 to 300 boys. Most often it is the only problem in these infants and does not imply there are other defects in the urinary system or other organs. It is a condition in which the urethral opening does not form completely to the tip of the penis. Instead, the opening may be located anywhere along the underside of the penis. While the urethral opening is most often found near the head, a position referred to as distal, it also may be located from the middle of the penile shaft to the base of the penis or even behind the scrotum, a position called proximal. About 70 percent of boys with this condition have distal hypospadias. In 15 percent of those cases, it is associated with mild downward curvature of the penis. In contrast, when the urethral opening is located more proximally, curvature occurs in more than 50 percent of patients.

Usually hypospadias is apparent at birth. Not only is the urethral opening in the wrong position, but the foreskin typically is also incompletely developed, resulting in what is called a dorsal hood that leaves the tip of the penis exposed. In fact, it is the appearance of the foreskin that most often calls attention to the problem. However, some male newborns have an abnormal foreskin with a normally positioned urethral opening, while in others, a complete foreskin may hide an abnormal urethral opening. About 8 percent of boys with hypospadias, also have a testicle that is not fully descended into the scrotum.

How is hypospadias treated?

Physicians have been correcting hypospadias with surgery since the late 1800s. But while more than 200 operations have been described, only a handful of techniques have been used by pediatric urologists since the modern era of hypospadias reconstruction began in the 1980s.

Regardless of the approach, the goal is to create a normal straight penis with a urinary channel that ends at the tip of the head of the penis. The operation usually involves four steps: straightening the shaft; creating the channel; positioning the urethral opening in the head and either circumcising or reconstructing the foreskin.

Hypospadias repair is usually accomplished in a ninety-minute to three-hour same-day surgery. In a few instances, however, it is done in stages, usually when a pediatric urologist wants to separately straighten the shaft before constructing the urinary channel.

For a variety of reasons doctors prefer to do hypospadias surgery in full-term and otherwise healthy boys, between the ages of three and 18 months. Yet the repair can be corrected at any age in childhood and even into adulthood. Occasionally, when the opening is proximal, treatment with the male hormone testosterone previous to surgery may be recommended.

What can be expected after treatment for hypospadias?

Many surgeons prefer that their patient not urinate through the fresh repair during the first few days after surgery. So they leave a small catheter in the penis that drains into the diaper. Antibiotics, and sometimes bladder antispasmodics, are given while the catheter is in use.

In general, the younger the child, the less discomfort following repair. In fact, when the operation is done, as most pediatric urologists recommend, in boys three months to one year old they have no memory of it. Yet, even older boys tolerate this surgery well, especially with the availability of current pain medications.

Modern hypospadias surgery creates a penis with good function that looks normal or nearly normal. The complication rate in boys with distal hypospadias repair is less than 10 percent. Problems can occur more often after a proximal correction.

The most common dilemma after surgery is the development of a hole — or fistula — from the urinary channel to the skin. Scarring within the channel or urethral opening also can occur, interfering with urination. In addition, the wound may fail to heal. If your child complains of urine leakage from a second opening or a slow urinary stream after hypospadias repair, he should see his pediatric urologist.

Most complications surface within the first few months after surgery, although fistulas or blockages may not be found for years. Otherwise a successful repair should last a lifetime, including during rapid penis growth spurt at puberty. Also, most complications are easily repaired with additional surgery after the tissue has healed from the first operation. Further repairs should not interfere with a surgeon's ultimate goal of creating a functional and normal appearing penis.

Physicians differ in opinion as to how long after surgery patients should continue returning for check-ups. Some pediatric urologists believe the risk for problems after the first few months is so low that routine office appointments are not necessary thereafter. Others think boys should be seen throughout childhood until after puberty. The final decision will be up to you and your son's doctor.

Frequently asked questions:

Can genetic factors contribute to the likelihood of hypospadias?

In approximately 7 percent of sufferers, the father is also affected. The chance that a second son will be born with hypospadias is about 12 percent. If both father and brother are affected, the risk in a second boy increases to 21 percent.

Is it necessary to correct distal hypospadias?

Many parents ask if surgery is needed for mild degrees of hypospadias. While it is difficult to predict problems later in life by evaluating an infant, there are several valid reasons for recommending routine correction, regardless of the severity.

First, as many as 15 percent of boys with this condition will have a noticeable downward curvature, a chordee, that may interfere with his ability to achieve an effective erection in adulthood.

Second, while the urethral opening may be in a nearly correct position, it is often misshapen or enlarged. Or it may have a web of skin just behind the opening. Those factors can disturb the urinary stream. Consequently some boys will notice spraying to the sides or a downward deflection. The penis functions but these problems can be embarrassing.

Similarly, a partially formed foreskin that is not corrected will always appear abnormal, even resembling the hood of a cobra, which can call unwanted attention to the defect. Any potential problem must be weighed against the likelihood of a successful surgery. But most pediatric urologists today recommend the procedure for all but the most minor degrees of hypospadias.

What kind of anesthesia is used? Is it safe to put infants to sleep?

Hypospadias repair is done while the patient is asleep, under general anesthesia. Many anesthesiologists also administer nerve blocks near the penis or in the back to minimize discomfort when the child awakens after the operation. These forms of anesthesia are very safe, especially when given by anesthesiologists who specialize in the care of children. Today, it is considered safe to do surgeries such as hypospadias repair in otherwise healthy infants.

Which repair is best for my son?

The procedure your son's urologist chooses will depend on a number of factors, including the degree of hypospadias and extent of penile curvature. Since his doctor will not be able to analyze the situation until the operation is underway, he or she must be familiar with a range of approaches. What may appear as an easily repaired distal hypospadias may turn out, on closer examination, to be a more complex problem. Consequently, pediatric urologists with special training and expertise with this condition perform hypospadias repair.

How do I care for my son's wound after surgery? How long will the healing take?

Studies consistently show that hypospadias repair wounds do not require special attention to heal properly. While your son's surgeon can choose from several types of bandages, he or she may not apply any at all. You will receive specific instructions regarding bandages and routine bathing.

If your son has a catheter, it may be left to drain into diapers, which can be changed as usual. If your son is older, it will be connected to a bag, which you will learn how to empty. Catheters are usually kept in place for five days to two weeks.

Wound healing from a hypospadias repair begins immediately, and lasts for many months. Early on there may be swelling and bruising, which improves over a few weeks. Sometimes the skin of the penis heals with an unsightly bump or there are complications. Recommendations for additional surgery will not be made for at least six months, allowing the tissues to recover. Many slight imperfections will resolve during this time.

If my child still has problems after several operations, can his hypospadias still be repaired?

Yes. Fortunately, the majority of operations are successful the first time. Yet, a few children require re-operation because of complications. Most of them will have a good outcome the second time, while a few will have lingering problems leading to even more surgery. This small group of patients is sometimes referred to as "hypospadias cripples," implying that their problems cannot be fixed.

While it is difficult to consider more surgery in these unusual circumstances, there are options available that offer hope for success. For example, scarring from prior operations can be removed and replaced with fresh tissue from other areas, most often from inside the cheek, to create a urinary channel and still achieve good cosmetic results.

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