Hypospadias is a congenital deformity where the opening of the urethra (the meatus) is sited on the underside (ventral) part of the penis , anywhere from the glans to the perineum. Hypospadias is one of the most common congenital anomalies in boys, occurring in 1 in 150 to 300 live births

1. Hypospadias Cripple -
Staged Reconstruction
DR. AHMAD JUNAID
Post Graduate Resident
DEPARTMENT OF UROLOGY & RENAL TRANSPLANT

2. A 19 yrs. boy Ghulam Mustafa , resident of Jhang admitted through
OPD on 31stjanuary,2020 as a follow up case of crippled hypospadias
for which buccal mucosal graft was done six months back as a first
stage procedure.
Patient previously had history of hypospadias , was operated multiple
times and repair was unsuccessful.
After the base line investigations & evaluation of the previous graft
he underwent the second stage procedure i-e urethroplasty ( tube
formation)

3. CRIPPLED HYPOSPADIAS

4. 6 Weeks after Buccal Mucosal
Graft incorporation
Buccal Mucosa Incorporated after Crippled
Urethral Plate Excision

5.  Foley's Catheter of 12Fr is
passed.
 Neo urethral plate formed by the
BMG is incised from the margins
to raise the healthy, vascularized
flaps for tube formation

6.  Urethral Tube formation
using absorbable suture.
 A sub cuticular technique is
employed during longitudinal
closure of the neourethra.

7.  Urethral tube is completed
 Intermittent use of a tourniquet
at the base of the penis during
urethroplasty
Important aspect of closure of the
neo urethra is that the edge of the
epithelial surface is inverted and
the raw surface of the sub-
epithelial tissue is approximated.

8.  Before skin cover , neo urethra
is covered by a second layer of
bucks fascia

9.  Skin is closed using absorbable
suture.
 Foley’s Catheter is secured will be
kept for 2 weeks.

10. Follow up – After 6 weeks

11. Per Urethral voiding with good Stream

12. LITRATURE REVIEW

13. Hypospadias is a congenital deformity where the opening of
the urethra (the meatus) is sited on the underside (ventral)
part of the penis , anywhere from the glans to the perineum.
 Hypospadias is one of the most common congenital
anomalies in boys, occurring in 1 in 150 to 300 live births
(elliott et al., 2011; springer et al., 2015).
DEFINITION

14. Embryology
6 weeks:
 Mesenchymal ingrowth at the cloacal
membrane forms the foundation for anal and
genital anatomy development.
 Migrating cells coalesce over the cloacal
membrane to form the cloacal folds, which
merge to form the genital tubercle.
7 weeks :
 Urogenital diaphragm forms below the
genital tubercle at 7 weeks of gestation.
 The urogenital folds and labio scrotal folds
emerge lateral to the urogenital membrane
 In males the urogenital folds fuse while the
genital tubercle elongates forming the penis
and glans

15. Embryology cont.
 Expression of the SRY gene product
between 7 and 8 weeks of gestation results
in masculinization of the undifferentiated
gonad, testosterone production, regression
of female structures, and elongation of the
genital tubercle
8 16 Weeks
 Penile urethral plate is formed by the
invagination of the endoderm bounded
laterally by the urethral folds . Penile
urethra is formed as a result of antegrade
fusion of the urethral folds

16. Embryology cont.
 Stratified squamous lining of the glanular
urethra originates via retrograde
ectodermal ingrowth causing the
cannulation of the glans. (“Ectodermal
ingrowth theory”)
 The glanular urethra then fuses at the
coronal margin with the Penile Urethra.
 Male urethra closure is an androgen-
dependent process & disruption of this
process results in the large spectrum of
variants of hypospadias.

17. Embryology cont.
 Leydig cell testosterone converted by 5α-reductase
Dihydrotestosterone (8 -12 week of gestational period)
development of the penis by cells in the developing external genitalia
 Growth factor Sonic Hedgehog (Shh) Genital tubercle & Urethral plate formation
 Androgen receptors penile skin, urethra, corpus spongiosum
Androgens mediation in urethral development
 Lack of androgen receptor stimulation
Failure of midline fusion of the scrotal and/or urethral folds.

18. RISK FACTORS
 Defective Androgen receptor quality and/or quantity.
 3β-hydroxysteroid dehydrogenase enzyme (microsomal enzyme
essential in biosynthesis of active steroid hormone) deficiency.
 Insufficient testosterone and/or dihydrotestosterone synthesis.

19. RISK FACTORS cont.
 Premature birth.
 Use of assisted reproduction techniques are also associated with
higher rates of hypospadias
 Wilms tumor 1, Sox9, DMRT1, and GATA4 affect gonad
development and have been associated with hypospadias.

20. Associated Anomalies
 Cryptorchidism (in up to 10% of cases of hypospadias).
 Open processus vaginalis or inguinal hernia (in 9-15%).
 A chromosomal abnormality will be present in approximately 22%
of individuals with hypospadias and cryptorchidism.

21. DIAGNOSIS & CLASSIFICATION
Includes three criteria:
 An ectopic, ventrally located urethral meatus;
 Ventral penile curvature;
 An incomplete, dorsally hooded foreskin.

22. Hypospadias Cripple
 Multiple procedures due to complications such as urethra cutaneous
fistulas or tube disruption leaves the penis progressively scarred &
changes the established blood supply. This can result in a significant
defect with poor function , a so-called Hypospadias Cripple.
 This condition is a challenge to reconstructive surgeons because of
the complexity of the problem and limited options for
reconstruction.

23. Goals Of Surgical Reconstruction
 Correction of penile curvature to ensure a long, straight erection.
 Advancement of the urethra to ensure normal passage of urine and
semen through the glans (projectile stream and normal erection)
 Creation of a cosmetically pleasing penis.
 Assessment of the long-term significance of the defect and an
objective discussion with the boy's parents, whether a surgical repair
should be performed.

24. Hypospadias Cripple - Staged Reconstruction
Hypospadias Cripple is managed by Staged Urethroplasties
 Bracka ( Graft) repair
 Byar’s ( Flap) repair

25. BRACKA I
 Correction of curvature.
 Excision of the urethral plate.
 Harvesting and Incorporating
graft to create a neo urethral
plate.
Bracka (Graft) Repair

26. BRACKA II
Involves tubularization of the
graft that was placed during the
first stage.
Bracka (Graft) Repair

27. INDICATIONS
 Hypospadias Cripples
 Penoscrotal Hypospadias
 Balanitis Xerotica
 Ventral Chordae > 30 degrees

28. ADVANTAGES
 Can be used when preputial skin is deficient
 Salvage procedure in Hypospadias cripples
 Better cosmesis
 Low complication rates

29. Byar’s ( Flap) Repair
Byar’s I
the penis is degloved,
VC corrected,
urethral plate is excised
glans cleft is created, and the
Prepucial flaps are sutured to the ventral
penile shaft.

30. Byar’s ( Flap) Repair
Byar’s II
Byars flaps are tubularized in 2-
layers to bring the meatus to the
glans

31. BUCCAL MUCOSAL GRAFT
 It is easy to harvest
 It is tough and resilient
 Easy to handle
 There is (usually) enough of it
 It ‘takes’ well (a thick epithelium and a thin dermis with a dense sub
dermal vascular plexus allows early inosculation)

32. OTHER GRAFTS
 Preputial skin
 Penile skin
 Post auricular skin
 Lingual mucosa
 Scrotal skin

33. FOLLOW UP
 Pericatheter urethrogram
 Removal of stent at 3 weeks
 Uroflowmetry
 RUG or flexible urethroscopy