Hypospadias is a birth defect of the urethra in the male that involves an abnormally placed urethral meatus (opening). Instead of opening at the tip of the penis, a hypospadic urethra opens anywhere along a line running from the tip along the underside of the shaft to the junction of the penis and scrotum or perineum. A distal hypospadias may be suspected even in an uncircumcised boy from an abnormally formed foreskin and downward tilt of the glans.
The urethral meatus opens on the glans penis in about 50-75% of cases; these are categorized as glanular hypospadias. Hypospadias of the shaft (when the urethra opens on the shaft), and peroneum (when the urethra opens on the perineum) occur in up to 20 and 30% of cases respectively. The more severe degrees are more likely to be associated with chordee or curvature. Undescended testes and hernias can be associated with hypospadias.
Hypospadias are among the most common birth defects of the male genitalia but widely varying incidences have been reported from different countries, from as low as 1 in 4000 to as high as 1 in 125 boys.There has been some evidence that the incidence of hypospadias around the world has been increasing in recent decades.
Most hypospadias are sporadic, without inheritance or family recurrence. For most cases, no cause can be identified.
Genetic factors are likely involved in at least some cases, as there is about a 7% familial recurrence risk.
Glanular hypospadias are primarily a cosmetic defect and have little effect on function except for direction of the urinary stream. If uncorrected, hypospadias can make male urination messy, necessitate that it be performed sitting, impair delivery of semen into the vagina (possibly creating problems with fertility), or interfere with erections. Surgical repair of hypospadias is nearly always successful in one procedure and can usually be performed in the first year of life or at age 3. When the hypospadias is very proximal, or there are associated birth defects such as chordee, the best management can be a more complicated decision. It often requires a 2 stage repair. The foreskin is often used for grafting and circumcision should be avoided prior to repair.
Currently, I operate in the first year of life and the majority of children have 1 operation. The more severe cases have 2 procedures. The majority require a catheter or stent for 1 week but usually go home after approximately 3 days to return after 1 week for removal of the stent. They are then seen in the clinic 6 weeks later.
In a minority of patients surgery produces unsatisfactory results, such as scarring, curvature, or formation of urethral fistulas (leak) or strictures. A fistula is an unwanted opening through the skin along the course of the urethra, and can result in urinary leakage or an abnormal stream. A stricture is a narrowing of the urethra severe enough to obstruct flow. In my hands, fistula occurs in approximately 5% of cases and strictures very rarely. They usually require further surgery.