Hypospadia

Hypospadia
Congenital defect
results frm incomplete fusion of urethral plate
during development of the male penis.
Approx 70% of hypospadias occur on the corona or
distal shaft of the penis.
Neonates with hypopadias are not at increased risk
for having other congenital abnormalities of the
urinary tract. However, penoscrotal or perineal
hypospadias may represent an intersex disorder
and sevaluation should include a karyotype. An
intersex work-up is also indicated if a hypospadias
and an undescended testicle are noted.

Definition
Hypospadia
Proximal urethra which may be located
on the ventral side at any position
between the tip of the glans and the
perineum.
A ventral penile shaft deviation.
A typical dorsal winged prepuce, by a
lack of circular ventral union of the
prepuce.

Hypospadias are classified by the

location of the urethral opening (Fig.
39-38).

Classification
Duckett (1996)
anterior (50%), middle (30%) and posterior
(20%)
These are also classified according to the
location of the meatus.
The anterior form: glandular, coronal and distal
penile.
The middle form: "midshaft and proximal penile.
The posterior form: penoskrotal, scrotal and
perineal.

Other classifications :

epidemiology
occurs in 1 in 300 males.
risk for hypospadias is increased by
history of maternal estrogen or progestin
use during pregnancy.
incidence of hypospadias is higher in
whites than in blacks, and the condition
is more common in those of Jewish and
Italian descent. A genetic component
may be present in certain families; the
familial rate of hypospadias is about 7%.

Pathogenesis
Urethra emerges from the urethral folds, which fuse
ventrally under theinfluence of androgens. The fusion
begins proximally in the 11th week of gestation and
proceeds distally. The fusion involves endodermal and
ectodermal tissue. The androgen effect is mediated by
the 5-reductase.
In hypospadias, malformations concern the endodermal
and ectodermal tissue. An example of an ectodermal
abnormality is the deficient ventral foreskin with a
dorsal "hood". Endodermal abnormalities include the
position of the meatus, a deficient urethra distal to the
meatus and chordee formation (urethral plate).

Etiology

Androgen Deficiency:major cause

low concentration or decreased sensitivity of the target
tissue. Many enzyme deficiencies which cause hypospadias
are known, such as5-reductase deficiencyor defects of
the androgen receptor.
Genetics:
multifactorial etiology involving several known genes
(polygenic disease). This can be concluded from the family
history and twin studies. In addition to the known enzyme
defects, most genes involved in the etiology of hypospadias
are still unknown.
Environmental Factors:
A variety of substances with estrogenic activity
contaminates the environment and is enriched through the
food chain. Substances with estrogenic activity are
insecticides, natural estrogens from plants and chemicals
from the plastics industry.

Diagnosis
Clinical examination :
Should be diagnosed
shortly after birth
Description :
1. position shape and width
of orrifice
2. Presence of antretic
urethra and division of
corpus spongiosum
3. Appearance of preputial
hood and scrotum
4. Penile size
5. Curvature of penis on
erection
. Symptomatology

Symptomatology :

Stenotic meatus :weak

urinary flow
Proximal hypoadia
with penile curvature
might not be able to
void standing

 Investigation:
Karyotyping : detect gonadal DSD,
cryptorchidism
Radiological study , uteroscopy ,
cystoscopy

Classification and severity

assesment (gms scoring )

Mild 3-6
Moderate
7-9
Severe
10-12

Management & Treatment

Surgery is the treatment of choice for most hypospadias.
Goals of surgical treatment of hypospadias are as follows:
To create a straight penis by repairing any curvature
(orthoplasty)
To create a urethra with its meatus at the tip of the penis
(urethroplasty)
To re-form the glans into a more natural conical
configuration (glansplasty)
To achieve cosmetically acceptable penile skin coverage
To create a normal-appearing scrotum

 Circumcision should be delayed so as to

preserve the foreskin for use in surgical
intervention.
AAP recommends surgery prior to 18
months of age when gender identity is
established. In the two-staged approach
for the correction of severe hypospadias,
the first surgery is performed by 6 months
and the second procedure by 1 year of
life.

 For psychologic reasons, the hypospadias should be

repaired before 2 years of age.
A very effective procedure for correction of distal
hypospadias is tabularized incised plate urethroplasty
(Fig. 39-39).
The two critical steps to this procedure involve a
"relaxing" incision of the urethral plate distal to the
hypospadias opening and tubularization of the
"relaxed" urethral plate.
Repair of more proximal hypospadias defects may
require use of skin grafts. For example, the penile
skin or the foreskin can be mobilized on a pedicle of
dartos fascia and used either as an onlay or a
tubularized graft.

Tabularized incised plate urethroplasty. A and B. The

two critical steps to this procedure involve a
"relaxing" incision of the urethral plate distal to the
hypospadias opening and (C, D) tubularization of the
"relaxed" urethral plate.

 MAGPI is Meatal advancement and glanuloplasty (

Duckett, 1981b). The MAGPI-technique is only
suitable for distal hypospadias.
Tubularized incised plate (TIP) Urethroplasty
suitable for distal and proximal penile hypospadias (
Snodgrass, 1994). Considered technically simple and
has a low complication rate, cosmetic result of glans
and the meatus is good.
Useful option for re-operations with preserved
urethral plate. The urethral plate is not removed but
deeply and longitudinally incised. After mobilization
and tubularization, the urethral plate is closed around
a catheter. A ventral curvature is corrected using the
Nesbit technique.

Mathieu Hypospadia Repair

Good option for distal penile hypospadias (Mathieu, 1932).
An rectangle of skin over the proximal urethra is raised and
folded distally. To avoid a horizontal meatus, a modification
of the original technique with V-incision of the flap exists
(MAVIS = Mathieu and V incision sutured).
Most common complications are unfavorable meatal
cosmetics, skin flap necrosis with fistula or stricture of the
urethral meatus.
Island Flaps Hypospadia Repair
Suitable for distal and middle penile hypospadias (
Duckett, 1981a). The island flap is raised from the prepuce:
the pedicled flap consists of the inner leaf of the prepuce
with Tunica dartos. The flap is rotated around the penis and
used in onlay technique with a preserved urethral plate. If a
resection of a chordee has been necessary, a tubular island
flap is necessary.

complications
general risks
Inflammation
wound healing
disorders,
blood loss during
and
after surgery,
urinary tract
infection and
disturbances of
sensation or
scarring

Urethral fistula (5specific

15%)
Scar narrowing of
the urethra
(stricture)
Recurrence of
penile curvature by
growth in size of the
penis with
incomplete
chordeectomy
Urethral stenosis
Urethral diverticula
Scar deformation

 Schwartz principles of surgery 8th

edition
http://www.pediatricurologybook.com
/hypospadias.html
http://www.urology-textbook.com/hyp
ospadias.html
http://emedicine.medscape.com/artic
le/1015227-overview