| Hypospadias is a common anomaly of the external genitalia in newborns, occurring in about 1 out of every 150 to 250 live births. It is defined as a congenital defect of the penis, in which the urethral meatus (opening of the urethra) is incompletely developed. This can cause the opening of the urethra to be located anywhere on the ventral (bottom) surface of the penis. In mild cases it may be at the base of the glans (the head of the penis) or just a little bit off center, but it more severe cases it may be anywhere along the bottom shaft of the penis or on the scrotum.
Hypospadias, in addition to the cosmetic effects, can cause a child's urinary stream to be deformed because the meatus is not centered. This can range from a mildly downward deflected stream and difficulty urinating while standing up for children with a mild hypospadias to more serious difficulties if the meatus is on the shaft of the penis or on the scrotum. It may also cause sexual dysfunction if there is any curvature to the penis and it is not corrected.
While hypospadias my be genetically inherited, and is more common in children in which his father and a sibling also have hypospadias, it is usually an isolated abnormality, although it may be associated with undescended testicles and/or inguinal hernias in some children. The incidence of hypospadias does seem to be increasing.
Hypospadias is usually easy to notice in a newborn because it is commonly associated with a hooded foreskin, making the opening of the urethra easy to see. Children with a hooded foreskin have a foreskin that is developed normally on the top and sides of the tip of the penis, but with an absent foreskin on the bottom. In about 5% of cases of hypospadias, the foreskin is completely formed and normal appearing and will cover up the hypospadias. In these cases, unless the child is being circumcised, the hypospadias will probably not be discovered until much later in life when his foreskin begins to naturally retract.
A hypospadias may also be associated with a chordee, or a ventral (downward) curvature of the penis.
Hypospadias is usually classified by the location of the meatus either before chordee release (Smith's classification) or after chordee release (Schaeffer and Erbes classification), because in many cases, release of the chordee may alter the position of the meatus lower. Classifications include first degree or glanular hypospadias, where the meatus is at the level of the glans, second degree, where the meatus is at the level of the penile shaft, either coronal (the base of the glans), subcoronal (below the base of the glans) or midpenile (in the middle of the shaft), or a third degree, which includes a meatus opening on the scrotum or below, either a penoscrotal, scrotal or perineal hypospadias.
First degree or glanular hypospadias are the most common, occurring in 60% of children with hypospadias. Second degree hypospadias is less common (25%) and third degree are the least common (15%). Children with a third degree hypospadias may need further evaluation, including a voiding cystourethrogram (VCUG) to look for associated abnormalities.
Children with a hypospadias should not be circumcised, and in cases with a complete foreskin, where the hypospadias is not discovered until the circumcision is begun, then the circumcision should not be completed until your child is evaluated by a Pediatric Urologist. The reason for not performing a circumcision and removing the foreskin in children with a hypospadias, is that the foreskin will probably be used as part of the repair during surgery to correct the hypospadias.
While very mild cases of hypospadias in which the opening of the meatus is just a little bit off center may not need to be repaired, more severe cases, especially if the meatus is on the shaft of the penis or scrotum, will need to be surgically repaired.
The surgical repair is usually performed early, when your child is between six months and 12 months of age. Surgery will help to release a chordee and bring the meatus up to the tip of the glans, so that your child can have a normal urinary stream. Except for severe cases, most repairs are in a one stage procedure. More severe cases may need two stages to completely repair the hypospadias, and may also involve the use of suprapubic tubes or urinary drip stents as a urinary diversion.
Most children with hypospadias should see a Pediatric Urologist shortly after they are born for an evaluation and to discuss the need for treatment. It is also important to defer a circumcision until this evaluation is complete. For more severe cases of hypospadias, especially if there are undescended testes and a concern for ambiguous genitalia, the evaluation should occur as soon as possible and while he is still in the nursery.
Internet Resources:
- Hypospadias: a description of hypospadias and its surgical correction from the University of Michigan Medical Center Section of Urology
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