Hypospadias

Stephen Confer, MD
Ben O. Donovan, MD
Brad Kropp, MD
Dominic Frimberger, MD
University of Oklahoma
Department of Urology
Section of Pediatric
Urology

Hypospadias
Any condition in which the meatus
occurs on the undersurface of the
penis
Usually 3 features
ventral meatus
ventral curvature (chordee)
Dorsal "hood; deficient
foreskin ventrally
Classification
Embryology
Genital tubercle fuses
in midline
Mesodermal folds
create the urethral and
genital folds
coalesce in midline as
phallus elongates
Distal glans channel
tunnels to proximal
urethra as solid core
then undergoes
canalization
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

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



Variations of Hypospadia
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)
Higher incidence in monozygotic twins (8.5x)
Associated Anomalies
Undescended testes 9% and inguinal
hernia 9%
Upper tract anomalies rare (1-3%)
Utriculus masculinus
10 to 15% in perineal or penoscrotal
hypospadias
Incomplete mullerian duct regression

Associated Anomalies
Rule out intersex, especially with
cryptorchidism
Adrenogenital syndrome
Mixed gonadal dysgenesis
Incomplete pseudohermaphroditism
True hermaphrotidism
Associated Anomalies
hypospadias and cryptorchidism
high index of suspicion for an intersex state
Walsh reported the incidence of intersexuality in
children with cryptorchidism, hypospadias, and
otherwise nonambiguous genitalia to be 27%
nonpalpable testis were at least threefold more likely
to have an intersex condition than those with a
palpable undescended testis (50% versus 15% )
Associated Anomalies
The idea that evaluation for an endocrine
abnormality and/or intersex state should be
undertaken in those with posterior hypospadias,
regardless of gonadal position or palpability, is
controversial but is supported in the literature,
because significant, identifiable, and treatable
abnormalities are common

Further Evaluation
Only with severe hypospadias and sexual
ambiguity
Includes testicular abnormalities
Up to 25% of these patients have enlarged utricles or
other female structures
The incidence of abnormalities with other forms
of hypospadias approximates that of the general
population
Therefore no further evaluation is indicated
History of Procedures
First in 100 to 200 A.D.
Heliodorus and Antyllus
Amputation distal to meatus
Dieffenbach, 1838
Pierced glans to meatus and
leave stent in place
Thiersch, 1869
Local tissue flaps
Hook
Vascularized preputial flaps
History of Procedures
Multistage repairs
Release chordee
Urethroplasty
One stage repairs
More feasible since the
introduction of
artificial erection,
which has nearly
eliminated inadequate
chordee
Treatment
Meatoplasty and glanuloplasty
Multiple techniques
Orthoplasty
Utilize artificial erection
Release urethra from fibrous tissue
Plicate dorsal tunica albuguinea
Ventral graft if needed
Treatment
Urethroplasty
Onlay vascularized flap
Tubularized flap
Free graft
Skin cover
Mobilized dorsal prepuce and penile skin
Double faced island flap
Scrotoplasty

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
Technical Aspects
Instruments
Fine instruments for delicate tissue handling
Suture
Chromic- absorbs rapidly
6-0 or 7-0 polyglycolic for buried sutures
Hemostasis
Tourniquet
Lidocaine with epinephrine
Low current Bovie, bipolar sticks to tissue
Technical Aspects
Magnification
Dressing
Immobilzation and prevention of hematoma
and edema
Diversions
Stent secured to glans with open drainage into a
diaper

Technical Aspects
Bladder spasms
Oxybutinin
Analgesia
Local penile block
Caudal block
Age at repair
6 to 18 months
Testosterone cream
May or may not be beneficial
considerable controversy surrounding the use
of hormonal stimulation
whether to administer any adjunctive
gonadotropins or hormones and, if so, which
agent, route, dose, dosing schedule, and timing
of treatment is to be employed
Gearhart and Jeffs (1987) administered
testosterone enanthate intramuscularly (2
mg/kg body weight), 5 and 2 weeks before
reconstructive penile surgery. They noted a
50% increase in penile size and an increase in
available skin and local vascularity in all
patients.
Acute Complications
Wound infection
Poor wound healing 2 to ischemia of flaps
Edema
Drain tubes if free graft is used
Erections
Chronic Complications
Urethrocutaneous fistula
Urethral diverticulum
Residual chordee
Persistent hypospadias
Urethral stricture
Hair bearing skin
Meatal stenosis
Excess skin
Balanitis xerotica obliterans
Hypospadias Repair
Over 150 operations have be described
Distal hypospadias
Tubulization of the incised urethral plate (Snodgrass)
Meatal advancement (MAGPI)
Meatal-based flaps (Mathieu)
Proximal hypospadias
Onlay grafts
Vascularized inner preputial transfer flaps (Duckett)
Free grafts (skin, buccal mucosa)
MAGPI
Mathieu
Redman and
Barcat
Island
Onlay
Buccal Mucosal Graft
Hypospadias - 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