CONGENITAL ANOMALY OF GENITOURINARY SYSTEM, BY MR. DINABANDHU BARAD

1. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

2. CONGENITAL
ANOMALY OF
GENITOURINARY
SYSTEM
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU
BY:
Dinabandhu Barad
MSC TUTOR, SNC,SOA,DTU

3. HYPOSPADIAS

4. HYPOSPADIAS
• The term hypospadias refers to a urethral opening that is on the
ventral surface of the penile shaft, proximal to the end of the
glans.
• The meatus may be located anywhere along the shaft of the
penis, from the glans to the scrotum, or even in the perineum.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

5. • Some boys with hypospadias,
particularly those with
proximal hypospadias, have
chordee, in which there is
ventral penile curvature
during erection.
HYPOSPADIAS
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

6. INCIDENCE
• Hypospadias occurs in up to 4 in 1,000 newborn boys.
• The reason why the penis does not develop properly is still not clear.
• The development of the penis whilst the baby is growing in the womb
is partly dependent on the male sex hormones such as testosterone.
• The effects of testosterone on the growing penis may be blocked in
some way.
• Although it is not a genetic condition, hypospadias can run in some
families
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

7. PATHOPHYSIOLOGY
• Hypospadias is a congenital defect that is thought to occur during the
embryologic development of the urethra, between 8 and 20 weeks'
gestation.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

8. PATHOPHYSIOLOGY
• The external genital structures are identical in males and females
until 8 weeks, after which time the genital structures develop a
masculine phenotype in males, primarily under the influence of
testosterone and its by product dihydrotestosterone.
• As the phallus grows, the open urethral groove extends from its base
to the level of the corona.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

9. ETIOLOGY
GENETIC FACTORS:
• A genetic predisposition has been suggested by the eightfold
increase in incidence of hypospadias among monozygotic twins as
compared with singletons.
• This finding may relate to the demand of two fetuses for human
chorionic gonadotropin (HCG) produced by a single placenta, with an
inadequate supply during critical periods of urethral development.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

10. ETIOLOGY
GENETIC FACTORS:
• A familial trend has been noted with hypospadias. The prevalence of
hypospadias in male children of fathers with hypospadias has been
reported as 8%, and 14% of brothers of children with hypospadias
are also affected. The inheritance is likely polygenic.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

11. ETIOLOGY
ENDOCRINE FACTORS
• A decrease in available androgen or an inability to use available
androgen appropriately may result in hypospadias.
• In a 1997 report by Aaronson et al, 66% of boys with mild
hypospadias and 40% with severe hypospadias were found to have a
defect in testicular testosterone biosynthesis
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

12. ETIOLOGY
ENDOCRINE FACTORS
• Mutations in the 5-alpha reductase enzyme, which converts
testosterone (T) to the more potent dihydrotestosterone (DHT), have
been associated with hypospadias.
• A 1999 report by Silver et al found that nearly 10% of boys with
isolated hypospadias had at least one affected allele with a 5-alpha
reductase mutation.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

13. ETIOLOGY
ENDOCRINE FACTORS:
• A fivefold increased risk of hypospadias appears to exist in males
born through in-vitro fertilization (IVF) in comparison with a control
group.
• This may reflect maternal exposure to progesterone, which is
commonly administered in IVF protocols.
• Progesterone is a substrate for 5-alpha reductase and acts as a
competitive inhibitor of the T-to-DHT conversion.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

14. ETIOLOGY
ENVIRONMENTAL FACTORS
• Estrogens have been implicated in abnormal penile development in
many animal models.
• Environmental substances with significant estrogenic activity are
ubiquitous in industrialized society and are ingested as pesticides on
fruits and vegetables, endogenous plant estrogens, in milk from
lactating pregnant dairy cows, and in pharmaceuticals such as
phthalates.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

15. ETIOLOGY
COMBINATION THEORY
A growing body of evidence suggests that the development of
hypospadias has a two-hit etiology involving a genetic predisposition
coupled with fetal exposure to an environmental disruptor
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

16. CLASSIFICATION
According to Duckett classification (1996) there are anterior (50%),
middle (30%) and posterior (20%) hypospadias.
– The anterior form: glandular, coronal and distal penile.
– The middle form: "midshaft” and proximal penile.
– The posterior form: penoscrotal, scrotal and perinea
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

17. Glanular hypospadias
: the urethra is within
the head of his penis
(glans).
Coronal: The opening
of the urethra is just
below the head of his
penis.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU
CLASSIFICATION

18. • Distal penile: the opening
of the urethra is in the
distal portion of the shaft of
the penis
• Midshaft: The opening of
the urethra is located along
the shaft of the penis.
• Proximal penile: the
opening of the urethra is
in the proximal portion of
the shaft of the penis
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU
CLASSIFICATION

19. • Penoscrotal: The opening of
the urethra is located where
the penis and scrotum meet.
• Scrotal
hypospadias:hypospadias
with the urethral opening on
the scrotal surface.
• perineal hypospadias: the
urethral meatus opens in the
perineum near the anus; the
scrotum is usually cleft.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU
CLASSIFICATION

20. CLINICAL MANIFESTATIONS
• 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
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

21. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

22. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

23. DIAGNOSTIC MEASURES
• Prenatal ultrasound
• Physical exam of a newborn: upon examination, the foreskin is
usually incomplete and the misplaced urethral opening is located.
• Excretory urogram: This test uses X-rays to provide pictures of the
urinary tract, and other congenital anomalies
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

24. TREATMENT
Medical management
• Minor cases of hypospadias, in which the meatus is located
up toward the tip of the glans, may not require surgical
repair and may simply be managed with observation.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

25. ADJUVANT HORMONAL THERAPY
• Presurgical treatment with testosterone injections or creams, as well as
HCG injections, to promote penile growth, and for the
improvement in chordee with lessening in the severity of the
hypospadias.
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

26. SURGICAL MANAGEMENT
Management begins in the newborn period.
Circumcision should be avoided, because the foreskin often is used in
the repair.
The ideal age for repair in a healthy infant is 6–12 months
–There is no greater risk of general anesthesia at this age
compared to 2–3 yr
–Penile growth over the next several years is slow
–The child does not remember the surgical procedure
–Postoperative analgesic needs are less than in older children
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

27. DIFFERENT SURGERIES
• Glandular hypospadias requires a glandular meatotomy
• Coronal hypospadias requires a meatal advancement and
glanduloplasty (MAGPI operation)
• Proximal hypospadias without a chordee can be treated by a skin flap
advancement
• If chordee present it should be excised and flap urethroplasty
performed
Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU