NUR 2744

Congenital : Hypospadias, Phimasis,

Undescent testis.

PN. MAYA CHERUNI

 Introduction
• Hypospadias
• Phimasis
• Undescent testis
Hypospadias
 Definition
• A birth defect of the penis
involving the urethra.
• The urethra normally travels in the
male through the full length of the
penis so that the stream of urine
comes from the urethral opening at
the tip of the penis.
 Types
Mild type urethral opening is below the tip
●

(first degree): nearby. (gland)

Moderate open closer to the body on the

●

(second degree): underside of the shaft of the penis

Severe opening below the penis on the skin

●

(third degree): (perinal)

 Etiology
• Most common are not fully
understood.
1. Embryology of the Male Urethra
2. Androgen Deficiency
3. Genetic factor are most likely in some
cases.
4. Environmental Factors
5. Penis Curvature in Hypospadias
Pathophysiology
Predisposing Factors:
Congenital

The genitals develop a masculine phenotype primarily

under the testosterone

As the phallus grow, the open urethral groove extends

from its base to the level of the corona

Urethral folds coalesce in the midline from base to tip

forming
Tubularized penile urethra Median scrotal raphe
Account for

Posterior and middle urethra

The anterior or granular urethra develops in a proximal direction with

an ectodermal core forming at the tip of the glans penis

Canalizes to join with the more proximal urethra at the level of the corona
 Sign and symptom
• Opening of the urethra at a location
other than the tip of the penis
• Downward curve of the penis
(chordee)
• Hooded appearance of the penis
because only the top half of the
penis is covered by foreskin
• Abnormal spraying during urination
 Risk factor
• Family history: about 7% of patients
with hypospadias have children with
hypospadias and 14% of male
siblings of the index patient with
hypospadias.
• Further risk factors: increased
maternal age, low birth weight, in-
vitro fertilization.
 Treatment
• Surgical
– Orthoplasty
– Urethroplasty
– Neourethral coverage
– Meatoplasty and glanuloplasty
 Treatment
• Surgical
– Skin closure
– MAGPI Hypospadia Operation
– Tubularized incised plate (TIP)
Urethroplasty
– Mathieu Hypospadia Repair
– Island Flaps Hypospadia Repair
 Diagnosis test
• Diagnosis is made by a physical
examination.
 Complication
• The most common complication from
Hypospadias surgery is occurrence of
a fistula. A second operation may be
required in such cases.
• Include:
– Complete disruption of the repair
– Hematoma (large blood clot) formation
– Urethral stricture (stenosis) formation 
Phimosis
 Definition
• “Phimosis” Greek “Phimos” (the muzzle
on an animal and the noseband on the
bridle of horse.)
• Is a condition whereby the foreskin of
the penis is too tight.
• Phimosis is defined as “stenosis”
(tightness)
• The condition is usually congenital but it
may be the result of an infection.
 Etiology
• Begins with an infection of the foreskin.
• Repeated infection such as balanitis. The
delicate tissues undergo a process of
partial healing/partial flare-up/partial
healing again.
• The scar tissue becomes fibrous.
• Nearby healthy tissue strives to form
new scabs and becomes infected. This
adds to spread of germs.
 Pathophysiology
• The uncircumcised male penis comprises
the penile shaft, the glans penis, the
coronal sulcus, and the foreskin/prepuce.
• Poor hygiene and recurrent episodes
of balanitis or balanoposthitis.
• Forceful retraction of the foreskin leads
to microtears at the preputial orifice
that also leads to scarring and phimosis.
 Sign and symptom
• Phimosis is usually painless condition.
• Infection may result from an inability
to carry out effective cleaning of the
area in which swelling, redness and
discharge may all be present making
the area tender and painful.
• A very tight foreskin can cause
problems during intercourse, urination.
 Treatment
• Antibiotics may control the
infection. Hot soaks may help
separate the foreskin from the
glans. (Circumcision)
• Unscarred foreskins, a preputial
stretch may be used.
(preputioplasty).
 Diagnosis test
• Diagnosis is made by a physical
examination.
Undescent testis
 Definition
• Known as cryptorchidism, is
characterized by testicles that do
not follow the normal developmental
pattern of moving into the scrotum
before birth.
• The testicle (testis) is responsible
for the production of male hormone
and also sperm.
Normal scrotal position can be
1. Along the "path of descent" from high in
the posterior (retroperitoneal) abdomen,
just below the kidney, to the inguinal ring
2. Found in the inguinal canal
3. ectopic, found to have "wandered“ usually
outside the inguinal canal
4. found to be undeveloped (hypoplastic) or
severely abnormal (dysgenetic)
5. found to have vanished (anorchia)
 Etiology
• Before the child is born.
• Testicle migrates down from high in the
abdomen and passes through abdominal wall
and groin to take its normal position in the
scrotum.
• In 4% of boys at birth, and there is an even
higher incidence in premature infants.
• ¾ of undescended testicles will descend
within the 1st 3 months of life.
 Pathophysiology
• In the fetus, the testes are in the abdomen.
• They migrate downward through the groin
and into the scrotum.
• Late in fetal development, during the
8thmonth of gestation.
• In some newborn boys the testes are not
present in the scrotum, either because the
testes did not descend or because the
testes never developed in the fetus
 Sign and symptom
• An undescended testicle is not
located within the scrotum. The
condition may be associated with
other abnormalities of the
genitourinary system.
 Risk factor
• Low birth weight (less than 2500 g)
• Maternal exposure to estrogen
during the first trimester
• Multiple birth (e.g., twin, triplet)
• Premature birth (before 37 weeks
gestation)
• Small size for gestational age
 Treatment
• The goals of treatment include the following:
– Improve fertility
– Promote easier examination for testicular cancer
(earlier detection)
– Correct associated abnormalities (e.g., hernia)
– Prevent testicular torsion.
– Alleviate psychological concerns regarding body
image
– Reduce risk for injury (especially if the testis is
positioned near the pubic bone)
 Treatment
• Can be hormonal, surgical or both.
• Hormonal
– Descent sometimes can be induced
with hCG
– GnRH appears to be comparable to hCG
in achieving testicular descent but is
not approved for use in the US
 Treatment
• Surgical
1. Orchiopexy
• If testicle is palpable.
• The testicle is manipulated into the
scrotum and sutured in place.
• The most significant complication of
orchiopexy is testicular atrophy (rare).
 Treatment
• Surgical
2. Exploration
• If testicle is non-palpable.
• May approach openly through groin or
laparoscopically.
• This procedure determines whether or not
the testis is present, positions and fixes
viable testes within the scrotum; and
removes nonviable testicular remnants.
 Diagnosis test
• Can be done in 3 way physical, lab &
imaging.
• Physical
– General: look for syndromic features
 Diagnosis test
– Genital:
• Note the testicular position, consistency,
and size in relation to the opposite testis
• Hypoplastic or poorly rugated scrotum or
hemiscrotum
• Inguinal fullness
• Bimanual digital rectal examination (under
general anesthesia)
• Hypospadias + cryptorchidism = think
intersex states
 Diagnosis test
• Labs
– In infants:
• Karyotype
• Electrolytes
• LH, FSH, testosterone, muillerian inhibiting
factor
• Adrenal hormones and metabolites (eg, 17-
hydroxyprogesterone)
– In older children: testosterone, LH,
FSH, and MIS
 Diagnosis test
• Imaging
– Imaging usually lacks sensitivity in
detecting nonpalpable testis
– Consider ultrasound:
• To look for gonads and exclude the
presence of a uterus
• In obese boys (testes may be difficult to
feel on exam)
 Complication
• Minimized by prompt recognition
and timely referral for treatment
1. Testicular germ cell cancers
– NOTE: surgical correction of
malposition (orchiopexy) reduces but
does not eliminate the risk of having
testicular cancer
 Complication
2. Subfertility
– Related to the effect of testicular
temperature on spermatogenesis
– Associated with lower sperm counts,
sperm of poorer quality, and lower
fertility rates
 Complication
3. Testicular torsion
– Seen predominantly in neonates and
postpubertal boys
– Often occurs in association with the
development of a testicular tumor
(growing mass twists on itself)
 Complication
4. Inguinal hernia
– 90% of undescended testes are
associated with patent processus
vaginalis
– May present with an incarcerated or
strangulated inguinal hernia
 Prevention
• These include:
– Infertility or testicular cancer in adulthood.
– Injury to the undescended testes.
– Emotional stress—While surgery usually
results in a normal appearing scrotum, the
undescended testis is sometimes smaller
than the normal one. If your son becomes
concerned about this as an older child or
adolescent, a prosthesis (artificial
replacement) can be placed in the scrotum.