
Title:       Hypospadias.

Authors:     O'Brien, Walter M.;  Gibbons, M. David
Citation:    American Family Physician, April 1989 v39 n4
             p183(9)

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Subjects:    Boys_Surgery
             Hypospadias_Surgery
             Penis_Surgery

Reference #: A7609833

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Full Text COPYRIGHT American Academy of Family Physicians
1989

Hypospadias Hypospadias is a common congenital problem in
boys. Neonatal recognition is essential, and circumcision
must be avoided. Although there is no increase in risk of
other genitourinary anomalies, hypospadias, when associated
with cryptorchidism, warrants further evaluation to exclude
an intersex disorder. Surgical reconstruction ideally should
be performed when the child is between six and nine months of
age.  The management of hypospadias has undergone a major
evolution during the past decade.  At one time, many degrees
of hypospadias were left untreated.  When attempted, surgical
repair was usually performed when the child was three to four
years of age.  The procedures required a "staged" approach
and prolonged hospitalizations involving catheter and tube
care. Recently, we have seen a movement toward outpatient,
single-stage hypospadias repair in infancy, without routine
urinary diversion.(1,2)

In this article, the embryology and treatment of hypospadias
will be discussed to assist the family physician in
counseling the parents of an infant born with this condition
and to facilitate early and coordinated referral to
specialists experienced in hypospadias reconstruction.

Definition and Embryologic Features

Hypospadias is a congenital defect of the penis that results
in incomplete development of the urethra.  It is a common
disorder, affecting approximately one in 300 live-born male
infants.(3) As a result of incomplete development, the
urethra terminates proximal to the expected position at the
tip of the glans and may also be terminally deficient in its
development.  The urethral meatus may be located anywhere
along the ventral course of the urethral plate--from an
almost normal position at or just below the corona (75
percent of cases) to a position on the perineum.

Embryologically, the urethral groove forms along the ventral
surface of the phallus during elongation of the urogenital
sinus.(3) As the phallus elongates, the groove extends to the
level of the corona.  The sides of the groove roll up and
come together in the midline to form a floor for the urethra.
The glanular portion of the urethra probably forms in the
same way, although some investigators think that this portion
of the urethra develops as a core within the glans that
subsequently connects with the remainder of the urethra.(3)
Failure of normal urethral development results in a meatus
located proximal to the glans.

Diagnosis

Usually, hypospadias is easily noted during examination of
the newborn in the hospital nursery.  A more proximal meatus
may not be initially apparent on a cursory examination, and a
dimple on the glans may be confused with a normal glanular
meatus.  However, a dorsal preputial hood is nearly always
present in a boy with hypospadias.  The dorsal hood
represents the partially formed foreskin.  The embryologic
malformation that leads to incomplete urethral development
also causes failure of the ventral portion of the foreskin to
develop.

It must be strongly emphasized that a newborn male with a
dorsal preputial hood should be considered to have
hypospadias and should not undergo neonatal circumcision. 
The skin of the dorsal prepuce must be left intact in the
event that it is necessary for subsequent surgical repair of
the urethral defect.

Boys with hypospadias may also have chordee, an abnormal
ventral curvature of the penis.  The deficiency in
development of the ventral urethral plate tethers the penis
and causes ventral angulation. Chordee is typically present
with more proximal degrees of hypospadias.

Pure hypospadias is an isolated anomaly.  There is a 14
percent incidence of hypospadias in siblings of index cases. 
Fathers have a history of hypospadias in 8 percent of
cases.(3) Since boys with hypospadias do not have an
increased incidence of genitourinary tract anomalies compared
with the general population, radiographic procedures to
search for other anatomic problems are not indicated.
However, an infant with both hypospadias and cryptorchidism
(especially when the testes are impalpable) should undergo
full urologic and endocrine evaluation to exclude such
conditions as congenital adrenal hyperplasia, mixed gonadal
dysgenesis or hermaphroditism.

Treatment

At one time, surgical repair was reserved for severe degrees
of hypospadias.  The sole indication for surgery in boys with
proximal hypospadias was to enhance penile function.  Boys
with proximal hypospadias are usually unable to urinate while
standing.  In addition, the ability to deposit sperm in the
vagina is impaired when the urethral meatus is in a proximal
position.  Since distal hypospadias in many boys is not
accompanied by significant functional problems (urination and
ejaculation are normal), it was argued in the past that
repair of distal hypospadias was for "cosmesis" only.  Also,
at that time, the risks and complications of the surgical
procedure, including multiple anesthetics and psychologic
problems in older patients, outweighed the potential
benefits.  Thus, the prevailing attitude was to "leave well
enough alone."

Todya, however, significant improvements in operative
techniques, the development of reliable pediatric anesthesia
and marked reduction in complication rates have made surgical
treatment acceptable for all degrees of hypospadias.  Strong
evidence supports the major psychologic importance of a
normal-looking penis.  We have seen boys with hypospadias
whose fathers had uncorrected hypospadias.  The overwhelming
majority of these fathers wanted their sons to receive the
psychologic and functional benefits of early hypospadias
repair.

OPTIMAL AGE FOR REPAIR

In the past, hypospadias reconstruction was deferred until
the child reached three to four years of age.  Currently, the
optimal time for hypospadias repair is between six and nine
months of age. At this age, the child has the physiologic
maturity to undergo general anesthesia, administered by a
skilled pediatric anesthesiologist, without any increased
risk.  In addition, there is no memory of the procedure at
this very early age.  With the combination of optical
magnification and microscopic surgical instruments and
suture, repair is technically feasible in all boys at this
age.

Pediatric psychologic theory suggests that the optimal time
for genital surgery is under the age of 18 months,(4) when
there is no specific genital awareness.  In addition,
separation anxiety is minimized by the presence of the
parents at the hospital and the outpatient nature of nearly
all of these procedures.

Another advantage to performing hypospadias repair in early
infancy is that parental concern over the abnormality is
minimized. When repair is delayed, parental anxiety may
heighten and the older child may perceive this parental
concern.  Furthermore, boys over two and one-half to three
years of age may experience intense concern over penile
loss--the so-called "castration anxiety." It is most
important to emphasize to parents that surgery at six to nine
months of age is not associated with any increased risk or
complication rate and has major psychologic advantages for
both the child and the family.

HOSPITALIZATION AND ANESTHESIA

In our center, the vast majority of hypospadias procedures
are performed on an outpatient basis or, occasionally, with
an overnight hospital stay.  When hospitalization is
necessary, parents are encouraged to remain overnight with
their child.  Infants can nurse or have a bottle immediatelyith
an overnight hospital stay.  When hospitalization is
necessary, parents are encouraged to remain overnight with
their child.  Infants can nurse or have a bottle immediately
after the procedure.  Although temporary urinary diversion,
with either a suprapubic tube or a urethral catheter, was
routine in the past, this is now seldom if ever employed. 
Urinary diversion, when necessary, is far simpler today than
in the past.  The new techniques not only are tolerated well
by the infant but also are easily cared for by the parents.

Hypospadias repair is always performed with general
anesthesia and a regional (caudal) block.  The caudal block,
which can be thought of as a very low type of epidural
anesthesia, produces regional anesthesia in the genital and
inguinal areas and minimizes the amount of general anesthesia
required.

There is surprisingly little postoperative discomfort, and
patients generally void without difficulty.  Acetaminophen
(10 mg per kg every four hours as necessary) is all that is
necessary for analgesia.  In children with temporary urinary
diversion, oxybutynin (Ditropan) may occasionally be needed
to prevent bladder spasms due to the soft urethral diaper
catheter.

OPERATIVE PROCEDURES

Historically, over 200 procedures have been described for
hypospadias reconstruction.  Selection of the appropriate
technique depends on many factors, including meatal position
and configuration, as well as the presence or absence of
chordee.  The three principal procedures we use for
single-stage repairs are the advancement techniques
(meatoplasty and glanuloplasty [MAGPI]),(5) the urethral
extension techniques (Mathieu)(6) and the urethral
replacement techniques (transverse preputial island
flap--tube or onlay).(7,8)

The MAGPI procedure is chosen for boys who have a compliant
distal meaturs without chordee.  The meatus is advanced
distally to the tip of the glans and the lateral glans edges
are used to cover the distal urethra, resulting in a conical
shaped glans with a meatus at the tip.  Skin coverage of the
penile shaft is achieved by rotating halves of the dorsal
hood ventrally.  Urinary diversion is not necessary, and the
procedure is performed on an outpatient basis.  On the third
postoperative day, the dressing is removed at home in a tub
of warm water.

The Mathieu procedure reconstructs the distal urethra using a
ventral meatal-based skin flap.  This type of reconstruction
is selected for boys who have a noncompliant distal meatus
without chordee.  The flap is raised from the skin of the
ventral surface of the penis and is then hinged over to form
the terminal urethra (so-called "flip-flap" procedure).  As
in the MAGPI procedure, lateral glans wings are used to cover
the distal urethra, resulting in a meatus at the glans tip. 
The penile shaft is covered using the dorsal foreskin.  We
perform the Mathieu repair on an outpatient basis without
urinary diversion.  As with the MAGPI procedure, the dressing
can be easily removed by the parents in a warm tub bath on
the third postoperative day.

The transverse preputial island flap procedures are used for
boys with more proximal hypospadias or those with more severe
degrees of chordee.  The inner aspect of the dorsal foreskin
is isolated as a skin flap, then rotated ventrally on its own
vascular pedicle to reconstruct the urethra.  In the
tubularized method, a tube can be created, substituting for
the distal urethra.

Alternatively, when the onlay island flap technique is used,
a flat, nontubularized, vascularized skin flap can be rotated
into position and laid in as the "floor" of the distal
urethra, keeping the dorsal wall intact.

In patients undergoing a transverse preputial island flap
urethral reconstruction, temporary urinary diversion with a
urethral stent is required for six to eight days to ensure
proper urethral healing.  To accomplish this, a small, soft 6
F Silastic tube is placed across the reconstructed urethra,
into the bladder.  The proximal end coils in the bladder and
the distal end drains directly into the diaper. Procedures
are usually performed on an outpatient, "sameday" basis or as
an overnight admission, especially if the child lives far
from our center.  The dressing can be removed in three to
four days, and the soft Silastic catheter is removed in the
office in one week.

COMPLICATIONS

As with any operative procedure, complications can be avoided
when hypospadias repair is performed by an experienced
surgeon and when the patient has been properly selected. 
Potential major complications include infection, graft
necrosis, meatal stenosis and retraction, all of which are
uncommon.  Urethral stricture and urethrocutaneous fistula
may occur more frequently and occasionally necessitate a
second, less extensive procedure.  In general, the very low
rate of complications with the one-stage techniques supports
their continued use.

Recently, we reviewed our experience with more than 130 boys
undergoing complete, one-stage hypospadias repair.  Results
were excellent in more than 95 percent of this group.  The
complication rate was extraordinarily low in the boys having
MAGPI, Mathieu or onlay island flap repairs.  In those having
tubularized island flap repairs (the most complex procedure),
major complications (fistula or stricture) occurred in only
five of 23 boys.  Each patient was managed with a single
extra procedure, with excellent results.

Nearly all boys undergoing hypospadias reconstruction within
the last two years have had outpatient surgery or overnight
stays.  Fifty percent of the boys were six to nine months of
age, and 80 percent were 18 months or younger.

Final Comment

The ultimate goal in the infant or young boy with hypospadias
is to achieve a penis that is functionally and cosmetically
normal--straight with erections, conical-shaped glans with a
central meatus, circumcised appearance and no fistula.  This
should be accomplished at an early age, when there is no
chance for psychologic trauma.

The state-of-the-art approach to hypospadias reconstruction
involves the surgeon's complete familiarity with all
reconstructive techniques, safe pediatric anesthesia, an
outpatient or overnight surgical setting, and elimination of
unnecessary urinary diversion. REFERENCES (1)Gibbons MD. 
Nuances of distal hypospadias.  Urol Clin North Am
1985;12:169-74. (2)Gibbons MD.  Hypospadias.  In: Resnick MI,
Caldamone AA, Spirnak JP, eds.  Decision making in urology.
Philadelphia: Decker, 1985:168-9. (3)Duckett JW. 
Hypospadias.  In: Walsh PC, ed.  Campbell's Urology.  5th ed.
Vol 2. Philadelphia: Saunders, 1986:1969-99. (4)Manley CB,
Epstein ES.  Early hypospadias repair.  J Urol
1981;125:698-700. (5)Duckett JW.  MAGPI (meatoplasty and
glanuloplasty): a procedure for subcoronal hypospadias.  Urol
Clin North Am 1981;8:513-9. (6)Gonzales ET Jr, Veeraraghavan
KA, Delaune J. The management of distal hypospadias with
meatal-based, vascularized flaps.  J Urol 1983;129:119-20.
(7)Duckett JW.  The island flap technique for hypospadias
repair.  Urol Clin North Am 1981;8:503-11. (8)Elder JS,
Duckett JW, Synder HM.  Onlay island flap in the repair of
mid and distal penile hypospadias without chordee. J Urol
1987;138:376-9.

Title:       Hypospadias: a treatable birth defect.

Authors:     Furino, Trish
Citation:    Pediatrics for Parents, July-Aug 1988 v9 n7_
             p9(1)

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Subjects:    Infants_Surgery
             Birth defects_Surgery
             Penis_Abnormalities
             Hypospadias_Surgery

Reference #: A6682550

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Full Text COPYRIGHT Pediatrics for Parents 1988

Hypospadias: A Treatable Birth Defect Approximately one out
of every 300 male babies will be born with hypospadias, a
congenital defect of the penis, each year.  (Female
hypospadias, which is extremely rare, occurs about once in
every 1 million births.)  It is the third most common birth
defect, after heart defects and clubfoot.  Hypospadias is a
genetic disorder that seems to run in families.  While it is
by no means a life-threatening disorder, it should be
surgically repaired for both functional and cosmetic reasons.

When a fetus' penis is forming normally, the urethra extends
from the bladder to the tip of the penis.  In hypospadias,
the urethra does not develop fully and may end along the
shaft, placing the urinary opening on the underside of the
penis.  In more severe cases, the opening may even be near
the scrotum or anus.

Hypospadias in itself does not cause major urinary problems,
although, depending on the placement of the opening, boys who
have not had it repaired might have to endure the
embarrassment of having to sit to urinate.  Left unrepaired,
hypospadias may also cause a curvature of the penis (called
chordee) which, if severe enough, can make intercourse
painful or even impossible.

If you are told at your baby's birth that he has hypospadias,
it may be best to delay circumcision since the extra skin can
often be used by a surgeon for repairs.  You may be referred
to a pediatric urologist -- if there is one in your area --
or to a urologist.  If you live in or near a big city, it
will be easier to find a pediatric urologist.

When choosing a surgeon, Howard Snyder, III, M.D., Associate
Director of Urology at the Children's Hospital in
Philadelphia, suggests you "ask how many repairs he or she
does regularly.  It should be at least one every two weeks,"
he advises.  However, if you live in a small town, this may
not be realistic.  If you're seeing a specialist in a more
rural area, be sure this physician is knowledgeable about
hypospadias.  Hypospadias repair, although a relatively
low-risk, routine procedure, is quite delicate, combining
plastic surgery and urology, and should be done by a
physician who is well-experienced with it.

Most surgeons prefer to do hypospadias repair when a baby is
nine or ten months old.  While there are many reasons for
this, one of the major ones is because at this age, babies
aren't yet aware of their genitalia.  Once they become aware
of their genitalia, at about 18 months, any intrusion into
that area could be traumatic.

Many hospitals, particularly very advanced or specialized
institutions, will do the repair on a same-day surgery basis.
You bring your child in early in the morning, he is operated
on, and if, everything looks fine, he can go home by early
evening.

The surgery can last anywhere from one to four hours,
depending on the degree of repair required.  In very
uncomplicated cases, all that is needed is for a new opening
to be formed at the tipof the penis and the old one to be
closed off.  In more involved situations, a thin tube is
inserted to carry urine from the bladder to the tip of the
penis, where a new opening is made.  All of this is done
under general anesthesia.

After the operation, your child's penis will be well-bandaged
and he will probably have an IV in his arm.  In the recovery
room, he will be watched for signs of fever and vomiting.  He
will be given some fluids, the IV may be removed, and he'll
have his penis checked.  He should be expected to urinate at
least once before being allowed to leave the recovery room. 
This will be quite painful for him, and he may scream; the
pain will subside in a day or two.

If you are able to take him home soon after the surgery, keep
a close eye on him.  He's likely to be fussy, sleepy and
lethargic from the anesthesia.  Watch for fever, excessive
crying and lethargy, and be sure he is urinating.  If you use
ultra-absorbent disposable diapers, it would be a good idea
to temporarily switch to regular ones, since the
ultra-absorbent ones may make it difficult for you to tell if
he's urinating.

Your son will very likely be back to normal within a few days
of the surgery, and you can rest assured he will look and
function just like all the other boys in the restroom.

Trish Furino has a 20 month old son who was born with
hypospadias.  She is a freelance writer and is director of
publications and literature for the Childbirth Education
Association of Greater Philadelphia.
