hipospadia

hipospadia
• Setiap kondisi di mana meatus
terjadi pada permukaan bawah
penis
• Biasanya 3 fitur
– meatus ventral
– kelengkungan ventral (chordee)
– Punggung "hood“; kulup
kekurangan bagian perut
Klasifikasi
Embriologi
• tuberkulum genital
sekering di garis
tengah
• lipatan mesoderm
menciptakan uretra
dan genital lipatan
• menyatu di garis
tengah sebagai
memanjang lingga
• Distal saluran kelenjar
terowongan untuk
uretra proksimal
Embriologi
• bentuk prepusium sebagai
punggung kulit dari
korona
• hipospadia
– Kegagalan aspek ventral
membentuk
– punggung hood
• chordee
– Pertumbuhan diferensial
antara biasanya
dikembangkan punggung
jaringan dan jaringan fisik
terbelakang ventral
– distal jaringan fibrosa untuk
hypospadiac meatus
 Embriologi
• bentuk prepusium sebagai
punggung kulit dari
korona
• hipospadia
– Kegagalan aspek ventral
membentuk
– punggung hood
• chordee
– Pertumbuhan diferensial
antara biasanya
dikembangkan punggung
jaringan dan jaringan fisik
terbelakang ventral
– distal jaringan fibrosa untuk
hypospadiac meatus
Variasi Hipospadias
 Insidensi
• 1: 300 kelahiran laki-laki hidup
• 6000 anak laki-laki setiap tahun di AS
• Beberapa komponen genetik
– 8% pasien memiliki ayah dengan hipospadia
– 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)
– Gratis cangkok (kulit, mukosa bukal)
MAGPI
Mathieu
Redman dan
Barcat
Pulau
Hiasan
Bukal mukosa Graft
 Hipospadia - Kesimpulan
• Umum
• komponen genetik ada
• Evaluasi untuk anomali terkait dengan
hipospadia proksimal yang parah
• Mengesampingkan interseks, terutama
dengan kriptorkismus
• Beberapa perbaikan ada, penjahit kepada
pasien, anatomi, dan perbaikan sebelumnya