2. DEFINITION
• Hypospadias is defined as hypoplasia of
the tissues forming the ventral aspect of
the penis beyond the division of the
corpus spongiosum.
• Hypospadias is believed to result from
arrested penile development, leaving a
proximal urethral meatus.
3. • Incidence- 1/250 male newborns
• Association of 3 anomalies
Abnormal ventral opening of urethral
meatus
Abnormal ventral curvature of the penis
Abnormal distribution of foreskin with a
dorsal hood
4. DIAGNOSIS
• Hypospadias is diagnosed by physical examination, first
suspected by the ventrally deficient prepuce and confirmed
by the proximal meatus.
5. • Abnormal ventral findings potentially
include
1. Downward glans tilt,
2. Deviation of the median penile raphe,
3. Ventral Curvature,
4. Scrotal encroachment onto the penile
shaft,
5. Midline scrotal cleft, and
6. penoscrotal transposition.
6. NORMAL PENILE ANATOMY
2 Corpora Cavernosa, Corpus Spongiosum
Enclosed In A Fascial Sheath- T.Albuginea
Bucks Fascia, Thick Fibrous Envelope
7. Embryology of Penile Development
• The external genital anlage is initially indifferent
and develops the female phenotype unless
exposed to androgens during the critical
gestational time period of 8 to 12 weeks.
• The urethral plate develops as an extension of
endoderm from the cloaca along the ventral
midline of the genital tubercle.
9. • Proliferating mesenchyme to either side creates
urethral folds and establishes the urethral groove.
• Fusion of the urethral folds begins proximally and
continues distally at least to the glans.
• Two theories are proposed for glanular urethra
development: ectodermal ingrowth cannulating the
glans to the urethral plate versus urethral plate
tubularization to the tip of the glans.
10.
11.
12. ETIOLOGY
Genetic Factors
• Familial aggregation is found in 4% to 10% of
hypospadias cases, including first-, second-, and third-
degree relatives.
Gene Mutations
• Murine studies indicating androgen receptor activity
regulates Fgf8, Fgf10, and Fgfr2 involved in urethral
development have led to screening for defects in these
candidate genes in patients with hypospadias.
13. ETIOLOGY
In most cases, the cause of this congenital defect is not
fully understood.
Treatment with hormones such as progesterone during
pregnancy may increase the risk of hypospadias.
Certain hormonal fluctuations, such as failure of the
fetal testes to produce enough testosterone or the
failure of the body to respond to testosterone, increase
the risk of hypospadias and other genetic problems.
17. The cosmetic indications, which are strongly linked
patient’s future psychology, are:
1. Abnormally located meatus
2. Cleft glans
3. Rotated penis with abnormal cutaneous raphe
4. Preputial hood
5. Penoscrotal transposition
6. Split scrotum.
18. Pre operative Consideration
Timing of surgery:
• Performed between 6 and 12 months.
• Healing seems to occur more quickly and with fewer
scars, and young infants overcome the stress of surgery
more easily.
• This age seems to insulate most children form the
psychologic, physiologic, and anaesthetic trauma
associated with hypospadias surgery.
19.
20. Preoperative Hormonal Stimulation:
HCG 250-500 U sc twice a week for 3 weeks. Increase in penile
size and length
Decrease in hypospadias and chordee severity
Increased vascularity and thickness of corpus spongiosum
Allowance of more simple repairs
IM testosterone enanthate – 2mg/kg/dose given for a total of 2
or 3 doses before hypospadias repair
Testosterone propionate cream – 2% three times daily for 3
weeks
23. Serafeddin (15TH century) was a surgeon from central Antolia
during the Ottoman period.
• In one of his books he describes the fine scalpel “mibza”
used for the treatment of meatal stenosis in hypospadias.
24. ORTHOPLASTY
• Correction of ventral curvature
• Ventral tissues—including shaft skin, dartos,
corpus spongiosum, urethral plate, and
overlying tunics of the corpora cavernosa may
be shortened relative to the dorsal surface.
• VC occurred in 11% of primary distal cases,
30% midshaft, and 81% proximal hypospadias.
25. • Preoperative assessment cannot accurately predict
either the extent of curvature or the means required for
straightening.
• Intraoperative assessment of penile curvature by either
artificial or pharmacologic methods is a critical step in
hypospadias repair.
• Performed after degloving of penile shaft skin.
26.
27.
28. • Artificial erection induced by saline injection remains
the most commonly used means to assess presence and
severity of VC.
• Pharmacologic erection allows for a more accurate and
continued assessment of penile curvature before,
during, and after its correction.
• Intracorporal injection of the arterial vasodilator
prostaglandin E1.
29. • Curvature up to 30
degrees can be corrected
by Midline Dorsal
Plication into the tunica
albuginea of the corpora
cavernosa directly
opposite the area of
greatest bending.
30. Nesbit technique :
• Excision of diamond
shaped wedge/s at the
point of maximum
curvature and closing the
tunica transversely with
absorbable sutures.
33. URETHROPLASTY
Distal hypospadias :
• TIP repair
• Others like MAGPI, Mathieu flip-flap, and Urethral
advancement.
Midshaft hypospadias :
• TIP repair
• Onlay preputial flap
34. Proximal hypospadias :
• TIP repair
• Onlay preputial flap
• Single stage urethroplasty with preputial flap or the
Koyanagi flap.
• Two stage repair with Byars flaps or grafts.
35.
36. Distal Hypospadias
Tubularized incised plate (TIP) repair:
• Circumscribing incision is made approximately 2 mm
below meatus
• Ventral V incision
• Penis degloved
• Midline incision of the urethral plate
• Urethral plate tubularization begins distally
approximately 3 mm from the end of the plate, ensuring
an oval, not rounded, meatus.
37.
38. • Dartos flap is dissected from the dorsal prepuce and shaft
skin, buttonholed, and transposed ventrally to cover the
neourethra.
• Glansplasty begins distally, and a 7-0 polyglactin suture.
39. Midshaft Hypospadias
TIP repair
Onlay preputial flap :
• Thin skin proximal to the urethral meatus is incised to the
midline convergence of corpus spongiosum wings.
• Then inner prepuce is harvested on its vascular pedicle from
either the dorsal hood or dartos flap.
• The flap should be gently stretched to fit the urethral plate
without redundancy.
40.
41.
42. Proximal Hypospadias
• The greatest controversy in primary hypospadias surgery
concerns decision making for proximal cases.
• Options depend on whether the urethral plate is available for
urethroplasty after associated VC is straightened.
• If so, then either TIP repair or an onlay preputial flap can be
used.
• When the urethral plate is transected a one-stage
urethroplasty can be accomplished by tubularized preputial
flaps or the Koyanagi flap or a two-stage repair done with
Byars flaps or preputial grafts.
43. Proximal tubularized incised plate repair :
• Circumscribing incision preserves urethral plate in patient desiring
circumcision.
• After degloving, glans wings are separated from the urethral
plate.
• Corpus spongiosum is dissected from the cavernosal bodies
• Midline urethral plate incision.
• Spongioplasty over the neourethra.
46. Koyanagi flap
• Proposed lines of incisions to create flap
• The flap can be divided into two wings as shown or
maintained in one piece with a central buttonhole to
transpose it ventrally.
47. • The urethral plate in the center of the flap is dissected from
the corpora to near the meatus, and the glanular portion of
the plate is excised as glans wings are made.
48. • Inner flap margins are
reapproximated, and excess
flap skin is excised.
• The outer margins are closed
to complete tubularization
49. Byars flap
• After degloving and release of ventral dartos,
persisting ventral curvature greater than 30
degrees led to excision of the urethral plate.
• The dorsal preputial hood is incised in the
midline and the two flaps transposed
ventrally on either side of the penis.
• The prepuce is advanced into the glans;
alternatively, the urethral plate can be
maintained within the glans.
50. • Flap edges are
approximated in the
midline.
• Six months later a U-
shaped incision is made
approximately 10 mm
wide.
55. • Epispadias is a congenital malformation in
which the opening of the urethra is on the
dorsum of the penis.
• In boys with epispadias, the urethra
generally opens on the top or side of the
penis rather than the tip. However, it is
possible for the urethra to be open along
the entire length of the penis.
• In girls, the opening is usually between the
clitoris and the labia, but may be in the
belly area.
57. Causes
• Unknown
• Related to improper development of the pubic
bone
• Failures of abdominal and pelvic fusion in the
first months of embryogenesis
• Epispadias can be associated with bladder
exstrophy, an uncommon birth defect in which
the bladder is inside out, and sticks through the
abdominal wall
• Also occur with other defects
58. Classification
Classification of epispadias is based on the
location of the meatus the penis. It can be
positioned:
• On the glans (glanular)
• Along the shaft of the penis (penile)
• Near the pubic bone (penopubic).
59. • The position of the meatus is important
because it predicts the degree to which
the bladder can store urine (continence).
The closer the meatus is to the base of the
penis, the more likely the bladder will not
hold urine
60. Symptoms
In males:
• Abnormal opening from the joint between the
pubic bones to the area above the tip of the
penis
• Backward flow of urine into the kidney (reflux
nephropathy)
• Short, widened penis with an abnormal
curvature
• Urinary tract infections
• Widened pubic bone
61. In females:
• Abnormal clitoris and labia
• Abnormal opening where the from the bladder
neck to the area above the normal urethral
opening
• Backward flow of urine into the kidney (reflux
nephropathy)
• Widened pubic bone
• Urinary incontinence
• Urinary tract infection
62. Diagnostic measures
• Prenatal diagnosis - rare
• Blood test to check electrolyte levels
• Intravenous pyelogram (IVP), a special x-
ray of the kidneys, bladder, and ureters
• MRI and CT scans, depending on the
condition
• Pelvic x-ray
• Ultrasound of the urogenital system
63. Surgical technique in males:
• The modified Cantwell technique
It involves partial disassembly of the
penis and placement of the urethra in a
more normal position.
64. Treatment
The primary goals of treatment of epispadias are to:
• maximize penile length and function by correcting
dorsal bend and chordee; and
• create functionality and cosmetically acceptable
external genitalia with as few surgical procedures as
possible.
• If the bladder and bladder neck are also involved,
surgical treatment is required to establish urinary
continence and preserve fertility.
65. • The second technique is the Mitchell technique.
»It involves complete disassembly of
the penis into its three separate
components.
»Following disassembly, the three
components are reassembled such
that the urethra is in the most
functional and normal position
and dorsal chordee is corrected.
66. Surgical technique in females
• The urethra and vagina may be short and near
the front of the body and the clitoris is in two
parts.
• If diagnosed at birth, the two parts of the clitoris
can be brought together and the urethra can be
placed into the normal position.
• If repaired early enough, lack of urinary control
(incontinence) may not be a problem.
67. • If the diagnosis is missed or if early repair
is not performed, then incontinence can be
surgically corrected at the time of
diagnosis.
• If the vaginal opening is narrow in older
girls or younger women, reconstruction
can be performed after puberty.
68. Take home message
• Hypospadias – ectopic ventral opening of the
meatus
• Associations- chordee and hernia
• Dorsal hood of foreskin, glanular groove with
ventral incomplete prepuce
• Circumcision contraindicated
• Surgery- 4 to 18 months
Dihydrotestosterone derived from 5α-reduced testosterone mediates the key steps in penis formation: elongation of the genital tubercle and fusion of urethral folds.
