Hypospadias: Understanding this birth defect in male babies

Hypospadias is a birth defect in male babies wherein the opening of the urethra, which is the tube that carries urine from the bladder out of the body, is located under the penis instead of at the tip. Depending on the exact point where it is located, this condition can vary in severity. Besides impacting the appearance of the penis, this abnormal urethral opening can lead to challenges with urination in babies. The condition may have other psychological and emotional consequences on children as they grow older. Early diagnosis and treatment are crucial while dealing with cases of this birth defect. In most cases, the condition requires surgical correction. While the condition can be challenging for both babies and their families, it’s 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, where the urinary opening is on the undersurface of the penis, instead of being at the tip. It is usually associated with a downward curvature of the penis, known as Chordee. This is a common condition of variable severity and occurs in about 1 in 200 male children.

While, the condition is more commonly an isolated defect without other major systemic issues. But in a few cases, it can be seen to be associated with disorders of sex development (DSD), hormonal, endocrine or genetic disorders. The presence of undescended testis, severe form of the condition, and other systemic signs should alert to this possibility.

Types of hypospadias

Here are the main types of the condition, depending on the location of the urethral opening:

• Glanular: Here, the urethral opening is located on the head of the penis, but not at the tip. This is the mildest form of the condition.
• Coronal: In this case, the urethral opening is situated just below the head of the penis, where the head meets the shaft.
• Subcoronal: Here, the urethral opening is located slightly further down the shaft of the penis.
• Midshaft: In this variation of hypospadias, the urethral opening is located along the middle of the shaft of the penis.
• Penoscrotal: When the urethral opening is located at the point where the penis joins the scrotum, it is a case of penoscrotal hypospadias.
• Scrotal: In this case, the urethral opening is located on the scrotum.
• Perineal: The most severe form, where the urethral opening is located on the perineum (the area between the scrotum and the anus).

Is hypospadias a serious problem?

Yes, untreated hypospadias will lead to issues with urinary stream, penile curvature, erectile dysfunction, painful erection, apparently small penile size and inability to have normal sexual relationship. This is true even with some of the milder varieties of the condition.

Treatment of hypospadias

Any child with abnormal (ambiguous) genitalia at birth should receive immediate medical attention, as this could even be part of life threatening hormonal disorders and also to ascertain the actual sex of the child. If other medical issues are ruled out, and a diagnosis of isolated hypospadias in a male child is ascertained, only elective therapy is necessary. Even apparently milder varieties of the condition require surgical correction, due to the associated penile curvature and abnormal penile skin. Patients with milder varieties of the condition generally undergo a single stage surgery (referred to as Urethroplasty), while the severe varieties undergo a staged surgery. The ideal time for surgery is about 1 year of age.

The second stage, when necessary, is performed about 6 months later. Some children may require preoperative hormonal therapy. The complications associated with the surgery are seen to increase as the child gets older. Moreover, the technical aspects of performance of the surgery (at expert hands), wound healing and post operative management are much easier in late infancy. The mental distress to the child and caretakers, when operating at an older age, is significantly more. It is also seen that uncorrected hypospadias is associated with severe mental anguish and peer group/ social issues in older children and their parents.

Therefore, the key to the best possible result lies in the performance of the right surgery at the right age at the right hands. With the advent of better sutures and instrumentation, greater understanding of the anatomy and specialisation in hypospadias surgery, results are markedly better than in the previous era.

Treatment of untreated hypospadias in older children

Even in an older patient, surgery is possible with a good postoperative outcome, provided the proper surgical correction is performed by expert hands. Detailed preoperative planning, use of advanced surgical techniques, interdepartmental collaboration and ensuring the co-operation of the patient are key to success in this scenario. Proper education, psychological support and guidance is required for the patient and family.