Hypospadias: Understanding this birth defect in male children

Hypospadias is a birth defect in male children in which the opening of the urethra, which is the tube that carries urine from the bladder out of the body, is under the penis instead of at the tip. Depending on the exact point where it is located, this condition can vary in severity. In addition to affecting the appearance of the penis, this abnormal opening of the urethra can lead to urination problems in children. The disease can have other psychological and emotional consequences for children as they grow older. Early diagnosis and treatment are critical in resolving cases of this birth defect. In most cases, the condition requires surgical correction. Although the condition can be challenging for both children and their families, it is important to remember that hypospadias can be successfully treated and most children with the condition grow up to live normal, healthy lives.

What is hypospadias?

Hypospadias is a birth defect in male children in which the urethral opening is on the underside of the penis instead of the tip. This is usually associated with a downward curvature of the penis known as Chordee. This is a common condition of varying severity and occurs in approximately 1 in 200 male children.

While the disease is more often an isolated defect without other serious systemic problems. But in some cases, it can be seen that it is related to disorders of sexual development (DSD), hormonal, endocrine or genetic disorders. The presence of an undescended testicle, a severe form of the disease, and other systemic signs should warn of this possibility.

Types of hypospadias

Here are the main types of disease depending on the location of the opening of the urethra:

• Deaf: Here the opening of the urethra is located on the head of the penis and not on the tip. This is the mildest form of the disease.
• Coronal: In this case, the opening of the urethra is located just below the head of the penis, where the head meets the shaft.
• Sub-crown: Here the opening of the urethra is located just below the shaft of the penis.
• Middle shaft: In this type of hypospadias, the opening of the urethra is located along the middle of the shaft of the penis.
• Foamy scrotum: If the opening of the urethra is at the junction of the penis with the scrotum, it is a penoscrotal hypospadias.
• Scrotum: In this case, the opening of the urethra is on the scrotum.
• Perineum: The most severe form, where the opening of the urethra is in the perineum (the area between the scrotum and the anus).

Is hypospadias a serious problem?

Yes, untreated hypospadias will lead to urinary flow problems, penile curvature, erectile dysfunction, painful erections, an apparently small penis, and the inability to have normal intercourse. This is true even in some milder forms of the disease.

Treatment of hypospadias

Any child with abnormal (ambiguous) genitalia at birth should receive immediate medical attention, as it can even be part of a life-threatening hormonal disorder, as well as to determine the true gender of the child. If other medical problems are ruled out and isolated hypospadias is diagnosed in a male child, only elective therapy is necessary. Even apparently milder varieties of the condition require surgical correction because of the associated penile curvature and abnormal penile skin. Patients with milder forms of the disease usually undergo a one-stage operation (called urethroplasty), while severe varieties are performed in stages. The ideal time for surgery is about 1 year.

The second stage, if necessary, is carried out after about 6 months. Some children may need preoperative hormone therapy. Complications associated with surgery increase as the child grows. Moreover, the technical aspects of surgery (in the hands of specialists), wound healing, and postoperative care are much easier in late childhood. The mental disorder of the child and guardians during the operation at an older age is much greater. Uncorrected hypospadias also appears to be associated with severe psychological distress and group/social problems in older children and their parents.

Therefore, the key to the best outcome is to perform the right surgery at the right age in the right hands. With the advent of better sutures and instruments, greater understanding of anatomy, and specialization in hypospadias surgery, outcomes are markedly better than in the previous era.

Treatment of untreated hypospadias in older children

Even in elderly patients, the operation is possible with a good postoperative result, provided that proper surgical correction is performed by specialists. Detailed preoperative planning, use of advanced surgical techniques, interdepartmental collaboration, and patient cooperation are key to success in this scenario. The patient and family need adequate education, psychological support and guidance.