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LECTURE 33_HYPOSPADIAS.pptx
1. INTRODUCTION
1st credited to Galenus
Derived from 2 greek word: hypo – “under” & spadon –
“rent”.
DEFINITION: opening of urethral on the ventral surface
of the penis proximal to the normal site of external
urethral meatus (at the tip of glans) ± ventral curvature of
penile shaft (ventral chordee)
2. Characteristic
Usually 3 features
ventral meatus
ventral curvature (chordee)
Dorsal "hood“; deficient
foreskin ventrally
3. Incidence
1:300 live male births
6000 boys each year in the US
Some genetic component
8% of patients have father with hypospadias
14% of patients have male siblings with
hypospadias
If child with hypospadias, risk to next child
12% risk with negative family history
19% if cousin or uncle with hypospadias
26% if father or sibling
More common in Caucasians (Jews and
Italians)
4. Associated anomalies
Undescended testes- in 7–9% generally, and 22% of patients with
severe hypospadias
Inguinal hernia-in 9–16%
Prostatic utricle
Upper urinary tract abnormalities
Disorder of sexual differentiation (DSD)-
about30% of patients with severe hypospadias
50% of patients with severe hypospadias and bilateral UDT
Malformation syndrome:
Opitz synd.: X-linked, AD
hypertelorism, hypospadia, mild to moderate mental
retardation, swallowing problem
5. HISTORY
Galen (130–199 AD) used the term hypospadias
. Matthieu 1932 – meatal based flap.
John Duckett
Tubularized transverse preputial island flap – 1980
Meatal advancement and glansplasty inco-operated(MAGPI) -
1981
Onlay preputial flap
Warren Snodgras – Tubularized Incised Plate in 1994
Bracka A – staged repair using preputial graft – 1995
6. EMBRYOLOGY
Differentiation of the undifferentiated external genitalia
into the male pattern starts at 8 weeks
Urethralization starts proximal and advances distally (9-16
weeks)
At 12 weeks, coronal sulcus is seen
At 16 weeks, glanular urethra via endodermal cellular
differentiation
7. Embryology
Prepuce forms as ridge of skin
from corona
Hypospadias
Failure of ventral aspect to form
Dorsal hood
Chordee
Differential growth between
normally developed dorsal tissue
and underdeveloped ventral
corporal tissue
Fibrous tissue distal to
hypospadiac meatus
8. AETIOLOGICAL FACTORS
Cause is yet unknown in the vast majority of patients
GENETIC MUTATIONS
Testosterone-dependent sonic hegdehog
Homeobox A (A9, A10, A11 and A13– hand-foot-genital syndrome)
and D
FGF 10
ATF3
and MAMLD1 (Cxorf6)
ENZYME DEFICIENCIES
5α- reductase
3a-hydroxysteroid dehydrogenase, 17a-hydroxylase, and 17,20-
lyase
9. HORMONES
Extrinsic: preconceptual oestrogens and progesterones in
pills, artificial reproductive techniques(ART)
Intrinsic: steroid secreting tumours, obesity
ENVIRONMENTAL
Endocrine disruptors: Biphenol A, polyesters,vinyl
Canned foods, plastics, herbicides and pesticides
BPAs better transported in microwaved foods, hand
washes…
Vegetarian mothers
Phytoestrogens especially from soya
Herbicides and pesticides
Birth weight: noticed more in small for gestational age babies
13. PRE-OPERATIVE CARE
Investigations
FBC
Serum electrolyes, urea and creatinine
Urine MCS
Children with proximal hypospadias and UDT should be
evaluated for DSD
Serum biochemistry
Karyotype
Gonadal biopsy
Urethrocystoscopy/laparoscopy
14. PARENTAL COUNSELING AND CONSENT
DO NOT CIRCUMCISE
To operate or not (cosmesis)
To circumcise or not to (preputial flaps,
grafts)
Operative technique is subject to
intraoperative findings
May need staged repair
Diversion or not- SPC
16. Goals
good calibre urethra with a slit-like urethral
meatus at the tip of the glans and a straight
penis
Boys with hypospadias should grow up as self-
confident young men with a normal body image.
Voiding and sexual activity should not be
impaired by hypospadias or its corrective
procedure
17. PRINCIPLES
Timing
Good illumination
Magnification – loupes/microscopes
Atraumatic fine sutures
Hemostasis: Tourniquets, diathermy and adrenalin
Fine/microsurgical instruments
Tissue handling with minimal trauma
28. Conclusions
Common
Genetic component exists
Evaluation for associated anomolies with
severe proximal hypospadias
Rule out intersex, especially with
cryptorchidism
Multiple repairs exist, tailor to the patient,
anatomy, and previous repairs