Professional Documents
Culture Documents
Hypospadias
Hypospadias
Testosterone
Penis
DEVELOPMENT
• Undescended testes- 9%
• Inguinal hernias- 9%
• Upper urinary tract anomalies 1-3%
• Utriculus masculinus- Incomplete mullerian duct regression
• Cryptorchidism- intersexuality needs to be ruled out especially in cases of non
palpable testis
TIMELINE
• 100-200 AD
• Heliodorus and Antyllus
• Amputation distal to meatus
• 1838
• Dieffenbach
• Pierced glans to meatus with stenting
• 1869
• Thiersch
• Local tissue flaps
• Hook
• Multistage repairs
• One stage repair
SYNDROMES
•ANTERIOR
MIDDLE POSTERIOR
• Diagnosis is clinical
• Imaging not required in cases of isolated hypospadias, regardless of severity
• Surgery is the treatment of choice
• Performed at the age of 3 months or older
ALGORITHM FOR HYPOSPADIAS
URETHROPLASTY
TIP
Urethral plate
maintained, no gross scar
VC<30˚
• BXO
VC>30˚ Sufficient previously • GROSS
skin, no excised skin SCARRING
BXO strip • VC>30˚
without • HAIR IN
scar URETHRA
2 STAGE
GRAFT 2 STAGE ORAL GRAFT
INLAY ORAL GRAFT
PREPUCIAL FLAP
TUBULARISED INCISED
PLATE (TIP) REPAIR FOR
DISTAL HYPOSPADIAS
• Rectangular flap is outlined proximally on the ventral penile shaft, elevated and
sutured distally to the urethral plate
PROXIMAL HYPOSPADIAS REPAIR
• Choice of repair depends upon the extent of ventral curvature after degloving and
excision of scrotal extensions onto the penile shaft
• VC<30- TIP, preputial flap
• VC>30- single stage tubularised preputial flaps, 2 stage preputial flaps or 2 stage
preputial graft repair
TIP IN PROXIMAL
HYPOSPADIAS
• A: Circumscribing incision preserves
urethral plate in patient desiring
circumcision.
• B: After degloving, glans wings are separated
from the urethral plate. Corpus spongiosum is
dissected from the cavernosal bodies and
released distally from the glans wings for
later spongioplasty. At this point artificial
erection is performed and ventral curvature
straightened as discussed in the text.
• C: Midline urethral plate incision.
• D: Two-layer urethral plate tubularization
using interrupted subepithelial 7-0
polyglactin followed by running 7-0
polydioxanone.
• E: Spongioplasty approximates divergent
corpus spongiosum over the neourethra,
before a tunica vaginalis barrier flap is added.
2 STAGE GRAFT
• Indication- VC>30˚
• Choice of flap depends whether patient undergoes circumcision or urethroplasty
• Urethral plate is transected as a part of straightening procedures followed by
preputial or buccal mucosa graft
• If circumcision is preferred, then discarded prepuce is used for graft
• If urethroplasty is desired, then oral mucosa from lower lip is preferred
1st stage consists of transecting the urethral plate to correct the ventral curvature followed by substitution of urethral bed
by using free preputial graft and creation of proximal urethrostomy.
A. Defect to be grafted after the urethral plate has been excised, glans wings developed, and proximal urethrostomy
created.
B. Graft secured to glans, shaft skin, and scrotum at the perimeter of the defect and then quilted to the corpora first along
the midline and then to either side at approximately 1-cm intervals.
C. Detail showing the graft extending to either side of the urethrostomy before quilting.
D. Tie-over dressing gently compresses the graft to reduce possibility for seroma or hematoma to accumulate beneath it.
E. Healed graft.
• 2nd stage consists of creating a
neomeatus by tubularising the
artificially created urethral bed over a
feeding tube and creating a distal
meatus.
• 2nd stage is usually carried out after 6
months of 1st stage
• Tunica vaginalis flap is placed over the
newly created urethra.
Tunica vaginalis flap covering the neourethra
PREPUTIAL FLAPS
• Onlay and tubularised flaps are single stage alternatived to TIP and 2 stage grafts.
ONLAY PREPUTIAL FLAP
• A. Lines of incision to create the preputial flap and preserve the urethral plate.
• B. Preputial flap mobilized on its vascular pedicle.
• C. Flap sewn to the urethral plate
TUBULARISED PREPUTIAL
FLAPS
• After degloving and release of ventral
dartos, persisting ventral curvature
greater than 30 degrees led to excision of
the urethral plate. An inner preputial flap
approximately 10 mm wide is dissected on
its dartos vascular pedicle and transposed
ventrally. This flap can be tubularized,
with the proximal end anastomosed to the
spatulated native urethra and the distal
end to the glans wings.
• Complications include:
• Fistula
• Glans dehiscence
• Meatal stenosis
• Neourethral stricture
• Diverticulum at the flap site
• BXO
• Graft contractures
• Obstructive urinary symtoms
• Sexual dysfunction
EPISPADIAS
• Defect in the dorsal wall of urethra
• Normal urethra is replaced by a strip of mucosa
• Associated Defects:
• Dorsally placed meatus
• Dorsal chordee
• Sphincter incontinence
• Pubic bone diastasis
• Unilateral renal agenesis
• VUR in cases of complete epispadias
• Male: Female =3-5:1
• Incidence is 1:1,00,000
TREATMENT
• Treatment goals include:
• Cosmetically acceptable genitalia
• Preservation of urinary continence
• Prevention of reflux
• Urethroplasty and penile elongation procedures are carried out before bladder neck
reconstruction
• Bladder capacity is the eventual indicator of continence and therefore is increased upto 95cc
in 18 months post epispadias repair before carrying out bladder neck reconstruction
procedures to have better functional outcomes
• Yound-dees-leadbetter and Marshall-Marchetti-Krantz suspension and ureteric
reimplantation are performed usually between 4-5 years of age
• Meticulous repair of urethra, appropriate selection of patients and surgical expertise form the
cornerstone for success
FEMALE EPISPADIAS
• Rare anpomaly
• Incidence 1;480,000
• Davis classification used for severity
• Mild: urethral orifice patulous
• Moderate: urethra dorsally split along most of the urethra
• Severe: cleft along entire urethra + incontinence
• Genital defect includes bifid clitoris, depressed mons with underdeveloped labia minora
• Vagina and internal genitalia usually normal
• Deficient ureterovesical junction wity
resultant reflux
• Small and thin bladder with limited capacity
• A. Normal female anatomy.
• B. Vesicular epispadias—urethra mainly normal but relation to clitoris altered.
• C. Subsymphyseal epispadias—defect in anterior wall of urethra for about one half its length.
• D. Retrosymphyseal epispadias—defect in anterior wall of entire urethra. Sphincter is usually involved
TREATMENT
• Objectives:
• Reconstruction of functionally and cosmetically acceptable genitalia
• Achievement of continence
• Preservation of upper urinary tract
A. Typical appearance of female epispadias with initial incisions outlined.
B. Excision of the glabrous skin of the mons.
C. Tapering of the urethra with a dorsal resection of a wedge of tissue.
D. Reconstruction of the urethra over a catheter with running suture.
E. and F, Medial aspect of the labia minora and clitoris.
G. Initial layer of mons closure.
H. Approximation of the labia minora over the urethral reconstruction.
I. Second layer of mons closure.
J. Creation of clitoral hood.
K. Completion of mons closure.
BLADDER EXSTROPHY
EXSTROPHY-EPISPADIAS COMPLEX
Allantoic duct
Midgut Midgut The theory of embryonic maldevelopment in
Genital tubercle
Urorectal exstrophy held by Marshall and Muecke (1968) is
Cloacal
septum
that the basic defect is an abnormal
membrane Bladder
anlage
Hindgut overdevelopment of the cloacal membrane during
Tailgut
Cloacal the 4th week of gestation, which prevents medial
membrane Incompletely
divided cloaca migration of the mesenchymal tissue and proper
lower abdominal wall development.
4th Week 8th Week
Peritoneum
Peritoneum Hindgut
Hindgut Urachus
Rectum
Bladder Urorectal
Bladder
septum
Genital Anal
tubercle canal
Urogenital Anal
sinus membrane
Cloacal Urogenital
membrane membrane
• Classical exstrophy >50% of this complex
• Closure of lateral body wall folds results in exstrophy
• If failure of fusion in abdominal and pelvic regions both Cloacal exstrophy
• If failure of fusion in pelvis alone Bladder exstrophy
• Other theories:
• Abnormal development of genital hillocks
• Abnormal caudal insertion of body stalk with failure of mesenchyme interposition
• Maldevelopment of bony pelvis
CLASSICAL EXSTROPHY
• Pelvic floor muscles particularly levator ani group is positioned more posteriorly and
outwards leading to a more flattened puborectal ling leading to exstrophy
Pubococcygeus
Puborectalis Pubococcygeus
• Premature rupture of cloaca causes triangular defect being taken up by bladder and
posterior urethra
• Associated indirect inguinal hernias can be seen owing to persistent processus
vaginalis, large superficial and deep rings, and lack of obliquity of inguinal canal
• The perineum is short and broad and the anus is situated directly behind the
urogenital diaphragm. Patient has varying degrees of anal incontinence and rectal
prolapse due to deficient anterior pelvic musculature
MALE GENITAL DEFECTS
• Main role of osteotomy to be relaxation of tension on the bladder, posterior urethra, and abdominal
wall repair during healing.
BLADDER REPAIR TECHNIQUES
• The primary objective in functional closure is to convert the bladder exstrophy into a complete
epispadias with the urethra well up onto the proximal shaft or midshaft of the penis
A. Initial incision around the bladder plate in the male.
B. Initial incision around the bladder plate in the female.
C. Incision follows the bladder template and includes the umbilicus.
D. Dissection of the lateral aspect of the bladder from the abdominal wall.
E. Dissection of the crura from the symphysis pubis.
F. Division of the urogenital (UG) diaphragm fibers.
G. Initial layer of the bladder closure. Urethral meatus is calibrated to 14-Fr size. Stents are
brought out of the bladder.
H. Pubic approximation is performed with No. 2 nylon mattress suture (see inset). Abdominal wall closure
is completed. Suprapubic tube and stents are brought out through the neoumbilicus.