Hypospadias: types, symptoms, causes and diagnosis

Hypospadias: types, symptoms, causes, diagnosis, care and treatment

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Overview

Hypospadias is a relatively rare congenital condition where the opening of the penis is on the underside of the organ. This condition is more common in infants with a family history of hypospadias.
The penis may curve down in an infant and the baby may spray while urinating.
It usually requires surgical correction to restore the proper flow of urine. This usually takes place before 18 months of age.

What is hypospadias?

In hypospadias, the opening of a boy’s urethra (through which both urine and semen pass) is located on the underside of the penis rather than at the tip. This condition, which is present at birth, affects about 1 in 200 boys. While very mild forms of hypospadias may not interfere with urinary or reproductive function, most boys with the condition will require surgery. The outlook for infants who undergo this operation is extremely good: In most instances, they make a full recovery and have a normal-looking, fully functional penis within about six months.

Symptoms of hypospadias?

In hypospadias, the urethral opening can be located at any point along the underside of the penis. The location of the opening determines the severity of the condition. In anterior or distal hypospadias, the urethral opening is located near the tip of the penis. This is the mildest form of hypospadias, occurring in about 50 percent of cases. Middle hypospadias means that the opening is located midway up the penis and accounts for about 30 percent of cases. In the most severe form of the condition, known as posterior or proximal hypospadias, the opening develops at a boy’s scrotum or perineum and occurs in 20 percent of cases.

Other signs of include:

  • downward urinary spray (in older boys with more severe hypospadias, this may mean they have to sit down to urinate)
  • downward curve of the penis, called chordee
  • “hooded” appearance to the penis, caused by extra foreskin along the top side
  • abnormal appearance of the tip of the penis (the glans)

In some cases, boys born with hypospadias may also have undescended testicles, inguinal hernias, or both.

Causes of hypospadias?

Hypospadias is a congenital condition, meaning that it happens while the baby is developing in the mother’s womb. As the fetus develops, the tissue on the underside of the penis that forms the urethra doesn’t close completely, shortening the passageway. In many cases, the foreskin — the fold of skin covering the penis tip, or glans — also doesn’t develop properly, resulting in extra foreskin on the top side of the penis and none on the underside.

There is no known cause of hypospadias. Researchers do know, however, that it appears to run in families: Hypospadias is slightly more common in boys whose father or brother also had the condition.

On examination:

A dorsal hood of foreskin and glanular groove are evident, but, upon closer inspection, the prepuce is incomplete ventrally and the urethral meatus is noted in a proximally ectopic position. Rarely, the foreskin may be complete, and the hypospadias is revealed at the time of circumcision. If it is encountered during neonatal circumcision, after the dorsal slit has been performed, the procedure should be halted, and the patient should be referred for urologic evaluation.
The most simple classification of Hypospadias is mild, moderate and severe, which can also be called first, second and third degrees of severity.
 

This classification system is based on the location of the external opening for urine and semen (the urethral meatus). In mild or first degree hypospadias, the opening is on the underside of the head of the penis or where the head and the shaft meet. This accounts for about 80% of the cases of hypospadias.

Moderate or second degree hypospadias (15%) occurs when the hole is actually on the shaft of the penis, somewhere between the junction of head and shaft, and the lower part of the shaft. Severe or third degree this  occurs when the hole is located on the lower part of the shaft just in front of the testicles or is located behind the testicles, between them and the anus.

Note the deficient ventral foreskin, blind urethral pit at the glanular level, and lighter pigmented urethral plate extending to the true meatus at the proximal shaft level.
The location of the urethral meatus was described if visible, and hypospadias was graded as glandular, coronal, penile, penoscrotal, scrotal, or perineal according to the anatomical position

How we care for hypospadias

The clinicians at Boston Children’s Hospital have extensive experience in caring for boys with such disease and see more than 300 children with this condition every year. We combine compassionate patient care with the latest techniques and surgical techniques to treat children with hypospadia.

How is hypospadias diagnosed?

Hypospadias can only rarely be seen on fetal ultrasound, so doctors typically diagnose it just after the baby is born, during his first physical examination. Because the mildest forms of it can be overlooked, you should contact your doctor if you notice that your son’s urethral opening is not at the tip of his penis, that his penis curves downward, or his foreskin is not fully developed. Upon diagnosing such disease, your doctor should refer your son to a pediatric urologic surgeon.

How is hypospadias treated?

Surgery remains the best and only way to correct all but the mildest cases of hypospadias.
A pediatric urological surgeon can perform the procedure, ideally when your son is between 6 to 12 months old. Although the technique may differ depending on your son’s individual case, the goals of surgery are usually the same:

  • to reposition the opening of urethra at the tip of the penis (urethroplasty)
  • to straighten the penis, if chordee is present (orthoplasty)
  • to improve the outward appearance of the penis (removing the hooded foreskin)

Surgery typically lasts one to two hours and occurs under general anesthesia. Unless the hypospadias is severe, the surgeon will complete the repair in one stage. However, some more severe cases will require two stages, but that is relatively rare. Depending on the extent of surgery, your child may either go home the same day or stay in the hospital overnight.

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