Introduction
Hypospadias is one of the most common birth defects, affecting 1 in 200 boys. In this condition, the urethra (which is the tube through which urine leaves the body) opens on the underside of the penis instead of at its tip. Very often there is also a curvature or a downward bend of the penis which becomes more pronounced when the penis is erect. This curvature is called chordee. Sometimes, the penis appears to be rotated to one side resulting in what is called torsion. Torsion and chordee can occur without hypospadias too.
Why does hypospadias occur?
We’re not sure why hypospadias happens. When the fetus develops inside the mother, the genitals form under the influence of hormones. It has been postulated that inadequate hormonal action during this time results in hypospadias. However, the vast majority of boys with hypospadias have normal hormones. There are also hypotheses about various substances in the environment increasing the risk of hypospadias in the fetus, but again, the evidence is not clear. Boys who have a brother or a father affected by hypospadias are more likely to have the condition although such familial cases account for less than 10% of all cases. As far as we know, there are no foods that if consumed during pregnancy would lead to hypospadias in the baby.
How is hypospadias diagnosed?
Most often, hypospadias is diagnosed at birth or shortly after, simply by physical examination. No tests are required to identify hypospadias. We classify hypospadias depending on where the urethra opens along the penis. When it opens closer to the tip, we call it distal hypospadias. When it opens closer to the scrotum, we call it proximal hypospadias. Mid-penile hypospadias is the type in which the urethra opens somewhere along the middle of the penile shaft. Sometimes, the urethra may open at the scrotum or on the perineum. The foreskin in hypospadias has a distinct hooded appearance too.
What are the symptoms of hypospadias?
An altered appearance of the penis is the most obvious symptom. The urinary stream can also be seen emerging from a site other than the tip of the penis. Distal hypospadias may be noticed when the child is a little older. In boys with curvature, the urinary stream is deflected, making it difficult for older boys to use the toilet. If the urethral opening is very narrow, the stream may be very thin or splayed. When the urethra opens at the perineum or very close to the scrotum, the child may have to squat or sit to pass urine.
However, hypospadias in itself does not lead to infections, kidney problems, or growth failure in a child. A child with hypospadias is otherwise healthy.
How is hypospadias treated?
Reconstructive surgery is required to correct hypospadias. There are many different types of surgeries to correct hypospadias, and no particular method has been found to be superior to the others. Depending on the type of hypospadias, the reconstruction may require a single surgery or two surgeries. The choice of surgery to be performed depends on the anatomy of the hypospadias. Most of the time, a tube is left in the newly constructed urethra for a few days to allow it to heal. The urine is allowed to drain through this tube. Pain medications are given to ease any discomfort. Thorough local hygiene is paramount to ensuring that the surgical wound heals well. The goals of surgery for hypospadias are:
- To restore the normal appearance of the penis
- To straighten any curvature of the penis
- To create a urethra that opens at the tip of the penis
- To ensure a normal stream of urine
What are the potential complications of hypospadias surgery?
Fistula formation and recurrent curvature are the two main reasons why a hypospadias repair could potentially need reoperation. While fistula formation will become apparent within a few days after surgery, recurrent curvature becomes obvious only when the boy grows, sometimes becoming apparent only at puberty.
A fistula is a tiny opening in the new urethra which occurs when the wound does not heal well. Urine may dribble through this opening, along with a good stream from the new surgically created opening at the tip of the penis. While a tiny fistula may close on its own, a larger one needs the surgeon to repair it.
At what age must repair be performed?
It is recommended to complete all stages of reconstruction between 6 and 18 months of age.
Can I circumcise my baby if he has hypospadias?
If your child has hypospadias, it is recommended not to circumcise him as the foreskin can be used in reconstructive surgery. Reconstructive surgery can be performed in such a way as to give a circumcised appearance.
If my baby has hypospadias, does it mean that he has other problems too?
In most cases, hypospadias occurs as an isolated defect and all the other organs in the body, including the reproductive organs, are absolutely normal. Infrequently, hypospadias may be a part of a cluster of anomalies, called a syndrome (e.g. WAGR syndrome, Denys-Drash syndrome). In about 10% of cases, hypospadias is associated with undescended testes. If one or both testes are undescended, then there is a possibility of a disorder of sexual differentiation. This possibility is highest when the hypospadias is proximal and both testes are undescended. Such children need to be investigated before any reconstructive surgery is performed. Tests to identify or rule out a disorder of sexual differentiation are also advisable in cases where the penis is abnormally small for the age of the child or the scrotum is bifid and the urethra opens at the perineum or between the two halves of the scrotum. It is important that any such disorder is correctly identified before embarking on reconstructive surgery.
Can my child have a normal adult life after hypospadias surgery?
After successful hypospadias repair, sexual and reproductive functions in adulthood is normal in the absence of any other abnormality.
Dr. Geeta Kekre is a well-known Paediatric Surgeon in Pune. Along with her team, she has successfully treated many children with hypospadias. If you or someone you know is seeking treatment for hypospadias, please visit our center for further assistance.
