Hypospadias
Definition
Hypospadias is a deformity of the male genitals, which is
present at birth. The urethra (urine tube) has not grown
right to the tip of the penis; instead it opens on the underside
further back than normal.
Incidence
The incidence of hypospadias is somewhere between 1 in 125
and 1 in 300 live male births.
Causes/preventions
The cause is unknown. Hypospadias occurs during the first
few months of life in the womb when the genitals are developing.
In hypospadias the penis does not complete normal development.
Hypospadias can be inherited, but most cases are an isolated
finding. If the father or one son has hypospadias there
is about a 1 in 10 chance of another son being affected;
if both father and son have hypospadias the chance for a
further son is about 1 in 4.
Signs
& Symptoms
The appearance of a penis with hypospadias varies with severity.
The severity of the deformity is classified by the position
of the urethral opening (meatus) on the underside of the
penis. The meatus can be anywhere in a line from the glans
to the area between the anus and scrotum. The more severe
the deformity the nearer the opening to the anus; fortunately
in over 90% of cases the opening is in the glans or last
third of the penile shaft. In a young baby it is not always
easy to see where the urine is coming from as the misplaced
opening is small and difficult to see. Usually a hooded
foreskin is the most obvious sign of hypospadias. A hooded
foreskin forms a floppy, wrinkled hood over the back of
the glans; some of the foreskin is missing on the underside.
The penis often has a downward bend (chordee) when it is
erect.
Hypospadias may be associated with undescended testicles,
hernias and other abnormalities of urinary tract. The more
severe the hypospadias the greater the likelihood of associated
anomalies.
Complications
of disorder
During early childhood the less severe forms of hypospadias
usually cause no symptoms. However with time most boys with
untreated hypospadias develop spraying or misdirection of
their urine. The abnormal appearance may cause social embarrassment
or psycho-sexual difficulties once they realise their penis
is different. Except in severe untreated deformities sexual
function is usually normal.
Tests
Tests are not usually necessary. In those cases with severe
deformities then imaging tests of the urinary tract should
be performed. In very severe deformities it may be difficult
to decide if the child is male or female; in this distressing
situation urgent specialist opinion is necessary.
Treatment:
Surgical
There is some discussion about the ideal age to operate;
many surgeons prefer to operate around the age of one year;
however, most British plastic surgeons prefer to wait until
the age of 3, when most boys are out of nappies and are
more cooperative and easy to manage.
A huge
number of operations have been used for hypospadias. Broadly
speaking there are two sub-groups of operation; one-stage
and two-stage surgery. One-stage surgery is popular in North
America and with many paediatric urologists. Many British
plastic surgeons prefer the two-stage approach.
Whatever technique is used the goal is the same: a straight
penis, with a slit-shaped opening on the tip of the glans.
Mostly it is not possible to create a normal foreskin, so
the penis will look circumcised.
The principle of surgery for hypospadias is to straighten
abnormal curvature before creating a new urethra which opens
at the end of the glans. One-stage techniques use flaps
of penile skin to create the new urethra during the first
operation. Two-stage techniques use a skin graft from the
abnormal foreskin to lay the foundations for creating the
new urethra at a second operation.
Hypospadias surgery is difficult and in inexperienced hands
complications are common. Complications include: failure
to straighten the penis, development of leaks (fistulae)
and development of narrowings in the new urethra (strictures).
In experienced hands the incidence of these complications
should be less than 10% and preferably less than 5%. Multiple
complications and procedures for hypospadias, once common
and the resulting “hypospadias cripple” should
should be a thing of the past.
Outcome
Parents of boys with hypospadias should expect their surgeons
to correct the deformity and produce a near normal looking
penis in, at most two-stages, by the time their son is ready
to attend school. In very severe deformities staged surgery
is more often needed; complication rates are also higher.
