Severe hypospadias

What is hypospadias?

This is a condition where the urethra (hole where pee comes out) is not in the correct place. It is instead further down and on the underside of the penis. The type of hypospadias is described by where the opening is. The mildest type (glanular) is where the opening is on the head of the penis.

In moderate hypospadias the opening comes where the glans meets the body of the penis (coronal and sub-coronal). Openings farther back (on the penis itself or at the base of the penis) are uncommon.

In addition to the hole being in the wrong place, the foreskin is often incompletely formed on the under-surface. This leads to the foreskin looking ‘hooded’. Sometimes the penis is bent downwards, usually because of the tight skin but sometimes happens because of an abnormality of the body of the penis.

Is this common?

Yes it is common. It happens to around between 0.3 percent to 0.4 percent of children, but severe forms are not common.

Is it associated with any other abnormalities?

Severe hypospadias can be associated with kidney problems. In these cases kidney scans are done to check this out, but scans are not needed in most cases.

Why does it happen?

The urethra (hole where urine comes out) forms from a strip of special skin forming itself into a tube on the under side of the penis. It closes up like a zip fastener pulling closed from the back end to the tip of the penis. For some reason the end part of the tube fails to form and remains as a flat plate. The underlying cause is unknown in most cases.

There are several theories why this happens including an increased female hormone-like substances in the environment causing mild forms of hypospadias. Very occasionally it seems to run in families.

What are the symptoms?

Usually the penis or foreskin may not look ‘right’. The urine stream may be angled downwards, and the penis may not stand out straight when erect.

How is it diagnosed?

Hypospadias is usually easy to diagnose when examined by a specialist.

Is surgery required?

Surgery may be needed for 2 reasons:

1. To make everything work properly (make the pee comes out straight and to make the penis straight).
2. For cosmetic reasons.

Unlike more mild forms of hypospadias, with severe hypospadias the appearance is usually quite abnormal.

When is surgery done?

Technically surgery can be done at any age. We prefer to operate around 9 to 18 months for a few reasons:

• the operation is less upsetting at this age.
• they are not potty trained and cannot control their pee (older children may refuse to pee after such operations causing trouble).
• the penis is a reasonable size on which to operate.

What happens before the operation?

No special preparation is required. It will be sore after the operation so it is a good idea to have paracetamol and ibuprofen at home.

What does surgery involve?

There are many types of operations designed to repair hypospadias.

The operations we use try to bring the hole up to the correct position on the ‘head’ of the penis (glans), make sure that the penis is straight and repair or remove the foreskin all at the same time. Sometimes it is possible to do this in 1 operation but sometimes 2 operations (stages) are needed.

In stage 1 a free area of tissue (graft) is taken from either:

• the foreskin
• the lining of the mouth

This flap of tissue is put on to the underside of the penis. In the second stage this new tissue is used to make the new urethra tube 6 to 9 months later.

Your child will probably be in hospital overnight or longer and have a tube (stent) draining the urine for a few days. Your doctor will explain the type of surgery planned for your child. If the operation is less complicated than expected your child may only need to stay for a few hours after surgery.

What is a stent?

The surgeon may decide to leave a tube (stent) into the bladder to drain the urine. This is left in place for 1 to 7 days depending on the operation. It usually drains urine into the nappy. A bag will be attached for older children who no longer use nappies. If a stent is used for more than 2 days, then antibiotics are prescribed to stop an infection in the urine.

The tube is used to stop urine running over the internal stitches so that in the first 24 hours there is not so much stinging. When it is removed your child may still find passing urine sore but this gets better over 24 hours.

For bigger operations a catheter is used to keep urine from coming through the stitches for a longer period to help healing. This catheter may irritate the bladder causing spasms in about 10 percent of cases. If this happens it is easily treated by giving a medicine to stop the spasm.

How long does the operation take?

The operation takes between about 2 to 4 hours. Anaesthesia is needed for this operation, so we need some time to let your child wake up afterwards. This can take quite some time and depends on the the individual.

What complications can occur with surgery?

Children at the age of 9 to 18 months usually get over surgery very quickly and easily. Hypospadias operations are not particularly uncomfortable although weeing for the first few days may be a bit difficult.

There can be some bleeding into the dressing or the wound may get a mild infection.

The penis always swells and bruises. Sometimes this can look quite worrying, but as long as your child is weeing easily there should be no reason to worry. Bruising may take a couple of weeks to go down (especially with the larger operations) and the swelling should go down after a few months.

In a 2 stage procedure the tissue put on the under-surface to remake the tube later, may not take or may shrink. If this happens, the graft or flap needs to be re-done involving another operation. When grafted, this happens in around 10 percent to 15 percent of operations.

The stent may come out partially or completely before the operation by mistake. If this happens it will cause discomfort and may affect the repair. Your doctor or nurse will explain what to look out for and what to do if there are any problems with the stent or dressing.

There can also be a partial or complete breakdown. This is where the hole is on the head of the penis will be followed by the hole opening not quite as far up the glans as it should. This is more common the more severe the hypospadias. It also depends on how good the tissues are. The 2 stage operation is often used when there is not enough tissue to do a one-stage repair. Even after a 2 stage repair the repair may breakdown

Sometimes the new opening of the urethra becomes narrow (a stricture) which may be treated by stretching or by a further adjustment operation.

In operations for moderate hypospadias, the most common thing to go wrong is the new tube may spring a leak called a fistula. This happens from around 10 percent to 20 percent of cases. If this happens it is usually quite easy to put a few stitches in the hole at a second small operation 6 to 8 months later.

When the foreskin is reconstructed to make it complete, the operation is done to deliberately leave the foreskin ‘loose’. This is to stop the foreskin being too tight and causing problems later. The foreskin repair can break down leaving the foreskin looking hooded again. This happens in around 10 percent of operations. The foreskin can then be left, removed or reconstructed again later.

What happens after the operation?

Children at the age of 9 to 18 months usually get over surgery very quickly and easily.

Hypospadias operations are not particularly uncomfortable although weeing for the first few days may be.

Food and drink are usually allowed as soon as your child has woken up.

There may be a little spotting of blood from the wound or moistness that dries within a day.

The area will be sore for a couple of days but these can be helped with paracetamol or ibuprofen are useful.

For most patients there will be a dressing that stays on for 1 to 7 days. This may be a gauze pad holding the penis onto the tummy wall to keep it still, or a see-through sterile waterproof dressing with antibiotic ointment on the penis to stop the dressing from sticking to the operation site. This also helps to keep the penis still and to hold the stent in place. This dressing can be wiped down but you should not put your child in the bath until the dressing and stent are removed

With any operation on the penis the delicate skin can become swollen and very bruised looking. This does not mean the operation is not going to be successful. This swelling sometimes happens several days after the operation, even after the dressing is removed.

If the staged operation is used then the appearance can be very strange after the first stage. This is because in order to get the graft into the correct place and for it to be big enough to make a new urethra at the second stage the under side of the penis must be cut right back to the opening of the urethra. It may look like the condition has gotten worse but this will not be the case.

Will we need to be seen after the operation?

Your child will be seen a few weeks after the operation to make sure that the repair is healing well and to plan the second stage of operation if needed. We will then keep an eye on your child at least until they are potty trained so that we can measure how fast they wee and to make sure that the tube is a good size.

Are there any long term effects after operation?

Once the area is healed it is rare for there to be long term effects. If the foreskin has been reformed, this sometimes does not stretch and needs another treatment later. Sometimes the urethra may be a bit small and give problems weeing.

If there is a bend in the penis that is corrected, the bend may become more obvious after puberty again.

It is rare for there to be problems with erections later in life. Although we try our best to make the penis look as normal as possible, it may still look a bit different to others.