Transcript Hypospadias - OU Medicine

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
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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
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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
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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
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Wound infection
Poor wound healing 2 to ischemia of flaps
Edema
Drain tubes if free graft is used
Erections
Chronic Complications
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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