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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)
Characteristic
 Usually 3 features
 ventral meatus
 ventral curvature (chordee)
 Dorsal "hood“; deficient
foreskin ventrally
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)
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
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
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
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
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
 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
Classification
Hypospadia
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
 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
Algorithm proposed by EAU
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
PRINCIPLES
 Timing
 Good illumination
 Magnification – loupes/microscopes
 Atraumatic fine sutures
 Hemostasis: Tourniquets, diathermy and adrenalin
 Fine/microsurgical instruments
 Tissue handling with minimal trauma
COMPONENTS OF REPAIR
 Orthoplasty
 Urethroplasty
 Glanoplasty
 Meatoplasty
 Skin cover
Urethroplasty
Urethral advancement
Tubularizing the urethral plate
Vascularized pedicled flap
Two stage free flap repair
SNODGRASS’ TIP
-Tubularised incised Plate
MATHIEU’S –Flip flap
Island
Onlay
Staged repair using graft
•Buccal mucosal graft
•Post-auricular graft
•Buccal mucosal graft
Technical Aspects/Postoperative
care
 Magnification
 Dressing
 Immobilzation and prevention of hematoma
and edema
 Bladder spasms
 Oxybutinin
 Analgesia
 Local penile block
 Caudal block
 Diversions
Factors for Technical Success
 Use of vascularized tissues
 Careful tissue handling
 Tension-free anastomosis
 Non-overlapping suture lines
 Meticulous hemostasis
 Fine suture material
 Adequate urinary diversion
OUTCOME
 IMMEDIATE
 Complication rates below 10%
 haematoma, dehiscence, fistula, meatal
stenosis, recurrent chordee
 Depend of degree /location of meatus
Complication
 Fistula
 Wound dehiscence
 Urethrocutaneous fistula
 Meatal stenosis
 Urethral diverticulum
 Residual chordee
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

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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
  • 11.
  • 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
  • 18. COMPONENTS OF REPAIR  Orthoplasty  Urethroplasty  Glanoplasty  Meatoplasty  Skin cover
  • 19. Urethroplasty Urethral advancement Tubularizing the urethral plate Vascularized pedicled flap Two stage free flap repair
  • 23. Staged repair using graft •Buccal mucosal graft •Post-auricular graft •Buccal mucosal graft
  • 24. Technical Aspects/Postoperative care  Magnification  Dressing  Immobilzation and prevention of hematoma and edema  Bladder spasms  Oxybutinin  Analgesia  Local penile block  Caudal block  Diversions
  • 25. Factors for Technical Success  Use of vascularized tissues  Careful tissue handling  Tension-free anastomosis  Non-overlapping suture lines  Meticulous hemostasis  Fine suture material  Adequate urinary diversion
  • 26. OUTCOME  IMMEDIATE  Complication rates below 10%  haematoma, dehiscence, fistula, meatal stenosis, recurrent chordee  Depend of degree /location of meatus
  • 27. Complication  Fistula  Wound dehiscence  Urethrocutaneous fistula  Meatal stenosis  Urethral diverticulum  Residual chordee
  • 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