27/04/2015

Fimosis, Parafimosis,
Hipospadia dan Epispadia
(Kuliah Mahasiswa Sm 04 FK Unsrat, 28 & 30 April 2015)

dr. Harsali F. Lampus, MHSM, SpBA

Divisi Bedah Anak
Bagian Ilmu Bedah FK Unsrat /
RSUP Prof. Dr. R.D. Kandou Manado
email: drharsali@gmail.com

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Referensi
1.

2.

3.

4.

5.

6.

7.

Palmer JS. Abnormalities of the external genetalia in boys. In: Wein AJ.
Kavoussi LR. Novick AC. Partin AW. Peters CA. Editors. Campbell-Walsh
Urology, 10th ed. Elsevier Saunders: Philadelphia. 2012. p.3537-56.
Snodgrass WT. Hypospadias. In: Wein AJ. Kavoussi LR. Novick AC. Partin AW.
Peters CA. Editors. Campbell-Walsh Urology, 10th ed. Elsevier Saunders:
Philadelphia. 2012. p.3503-36.
Baskin LS. Hypospadias. In: Coran AG. Adzick NS. Krummel TM. Laberge JM.
Shamberger RC. Caldamone AA. Editors. Pediatric Surgery. 7th Ed. Elsevier
Saunders: Philadelphia. 2012. p.1531-54.
Borer JG. Retik AB. Hypospadias. In: Wein AJ. Kavoussi LR. Novick AC. Partin
AW. Peters CA. Editors. Campbell-Walsh Urology, 9th ed. Elsevier Saunders:
Philadelphia. 2007. ebook.
Gearhart JP. Mathews RI. Extrophy-Epispadias Complex. In: Wein AJ.
Kavoussi LR. Novick AC. Partin AW. Peters CA. Editors. Campbell-Walsh
Urology, 10th ed. Elsevier Saunders: Philadelphia. 2012. p.3325-78.
Woo LL. Thomas JC. Brock III JW. Bladder and Cloacal Exstrophy. In: Coran
AG. Adzick NS. Krummel TM. Laberge JM. Shamberger RC. Caldamone AA.
Editors. Pediatric Surgery. 7th Ed. Elsevier Saunders: Philadelphia. 2012.
p.1515-30.
Journal of Pediatric Urology, Journal of Pediatric Surgery, Journal of Urology,
British Journal of Urology, Seminar in Pediatric Surgery.

Fimosis

Fimosis fisiologis (Primer)
Fisiologis pada usia 3-4 tahun
90% anak yang tidak disirkumsisi, saat usia 3 tahun
akan dapat meretraksikan preputiumnya secara komplit
Kurang dari 1% sampai usia 17 tahun
Retraksi paksa tidak direkomendasikan

Fimosis patologis (Sekunder)

Urinary tract infection

Balanitis,
Balanoposthitis,
Balanitis xerotica obliterans
Sikatrix akibat retraksi paksa

Parafimosis
 Children have a
tendency to neglect
to reduce the
foreskin following
retraction at the time
of micturition
 An iatrogenic
paraphimosis by
inadvertently leaving
the foreskin
retracted following
insertion of an
indwelling urethral
catheter.

Fimosis
- Preputium tidak dapat
diretraksikan secara
parsial atau komplit.
- Etiologi:
1. Adhesi alamiah kulit bagian
dalam preputium dengan
glans
2. Preputial ring

- Pemisahan kulit bgn

dalam preputium dari
glans tergantung pada:
1. Debris epitel (smegma)
2. Ereksi penis intermitent

Fimosis
Patologis
 Tanda-tanda infeksi
 Penebalan/pelebaran
phymotic band/
preputial ring
 Indurasi dan fibrosis
Penatalaksanaan:
 Topical steroid cream
 Circumcision

Management:

Manual reduction:
Use sustained gradual pressure
on the glans by both thumbs
while the first and second digits
reduce the edematous foreskin

Manual decompression:
Wrapping the penis in gauze
and applying a hypertonic
solution (such as 3% saline) for
2030 min will cause a
dramatic reduction in the
amount of edema

Dorsal slit:
Incise the tight preputial ring in
order to reduce the foreskin
and relieve the venous
congestion.

Circumcision:
Can be performed at a later
date, if necessary.

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Circumcision

Buried Penis

Hipospadia
A. Trapped
Penis akibat
skar sikatriks
pasca
sirkumsisi
B. Urine pooling
akibat skar
sikatriks
C. Penoskrotal
web (webbed
penis)
D. Webbed
penis dengan
hipospadia

Hypospadias, is defined as an association of three

anomalies of the penis:
(1) an abnormal ventral opening of the urethral meatus
that may be located anywhere from the ventral
aspect of the glans penis to the perineum,
(2) an abnormal ventral curvature of the penis
(chordee), and
(3) an abnormal distribution of foreskin with a hood
present dorsally and deficient foreskin ventrally
(Mouriquand et al, 1995 )
The second and third characteristics are not present in
all cases.
Borer JG. Retik AB. Hypospadias. In: Wein AJ. Kavoussi LR.
Novick AC. Partin AW. Peters CA. Editors. Campbell-Walsh
Urology, 9th ed. Elsevier Saunders: Philadelphia. 2007. ebook.

Hipospadia

Hipospadia
Ventral
meatus

Dorsal
Hood
Chordee

Chordee

Hypospadias can be defined as an

arrest in normal development of the
urethra, foreskin, and ventral aspect
of the penis.
This results in a wide range of
abnormalities; the urethral opening
can be anywhere along the ventral
shaft of the penis, within the scrotum,
or even in the perineum.
Baskin LS. Hypospadias. In: Coran AG. Adzick NS. Krummel TM.
Laberge JM. Shamberger RC. Caldamone AA. Editors. Pediatric
Surgery. 7th Ed. Elsevier Saunders: Philadelphia. 2012. p.1531-54.

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Insidensi

Insiden 1 : 300 males (Beck, 1917)

US, 1 : 150 (Paulozzi et al, 1997)
1 : 250 newborns atau
1 : 125 live male births (Baskin, 2012)

Embriologi
Kuliah Pakar khusus
Theory of urethral development
Theory of ectodermal intrusion
Theory of endodermal differentiation

Hormone receptor impairment

Abnormalitas metabolisme androgen
atau androgen reseptor (AR)

Gearhart et al, tidak menemukan defisiensi AR
atau 5-reductase pada pasien hipospadia

Allera et al, menemukan defek pada openreading frame AR tapi hanya pada 1 dari 9
pasien hipospadia berat
Mutasi pada gen AR

Sutherland et al, Mutasi AR gen jarang
berhubungan dengan hipospadia

Defek AR gen hipospadia

Enzyme impairment
Defek pada 3 enzim utama dalam jalur
biosintesis testosteron

3-hydroxysteroid dehydrogenase

17-hydroxylase

17, 20-lyase
Defek pada enzim steroidogenik yang
berperan pada jalur cholesterol ke
dihydrotestosterone tidak umum
menyebabkan hipospadia

Etiologi
UNKNOWN
Predisposisi genetik saja tidak dapat
mencetuskan hipospadia
Etilogi hipospadia multicausalitas
Berhubungan dengan:
1. Hormone receptor impairment
2. Genetic impairment
3. Enzyme impairment
4. Environmental factors and Endocrine
disrupters

Genetic impairment
Hox gen (A13)
ATF3
MAMLD1(Cxorf6)
ACT3
Cyr61
CTGF
CADD45
Ekspresi gen pada masa embrionic
dipengaruhi oleh faktor hormonal
(Estrogen dan sintetiknya)

Environmental factors and

Endocrine disrupters
 Eksposur dengan progestin dan kontrasepsi
oral pada trimester pertama kehamilan
 Maternal vegetarian diet (phytoestrogen)
 Pestisida dan fertilizer (endocrine
disrupters)
 Androgen defisiensi
 Estrogen dan prednison tikus hamil
mencetuskan hipospadia
 Assisted reproduction (hormonal
manipulation)

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Klasifikasi Hipospadia

Spektrum Hipospadia

Penile chordee tanpa Hipospadia

Normal
Meatal
opening

Dorsal
Hood

Chordee

Variasi penyerta Hipospadia

Normal Preputium
Mega meatal
Megameatus intact
prepuce (MIP) variant
of hypospadias

Micropenis
Transposisi Penoscrotal

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Diagnosis
Ditegakkan dengan Pemeriksaan Fisik

Defisiensi preputium ventralis

Ventral meatal opening

Dorsal hood

Congenital ventral curvature (chordee)

Apperent downward bending of penile (ventral
bowing)

Penile torsion

Megameatus intact prepuce (MIP)

Penoscrotal transposition (PST)

Menilai
Panjang Penis

Penilaian
Hipospadia
dan
variasinya

Associated Anomalies
Undescended testis (9,3%)

32% pada posterior hipospadia

6% pada moderate hipospadia

5% pada distal hipospadia
Inguinal Hernia (9%)

17% pada posterior hipospadia
Utricle (utriculus masculinus) (11%)
Remember: Hipospadia berat (posterior/
proximal) yang disertai dengan Undescended
testis harus dicurigai sebagai DSD (Disorder
of Sexual Development)

Penatalaksanaan

Specific
Medical
Records for
Hypospadias
Patients

Kompetensi Dokter Umum

Kognitif, Psikomotor, dan Afektif
Pembedahan

Rujukan ke Pediatric surgeon, Urologist, atau
Plastic surgeon

Terapi hormonal pra pembedahan
Timing of operation

6-18 bulan
Complications

Fistula, Meatal stenosis, Stricture,
Divertikulum, Infeksi, criple hipospadia

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Tubularized Incised Plate

Urethroplasty (TIP) -Snodgrass-

Lateral based flap

Epispadia

Ekstrofi Bladder Epispadia

Kompleks

 Epispadias varies from a mild glanular defect in a

covered penis to the penopubic variety with complete
incontinence in males or females.
 It is most commonly noted as a component of bladder
and cloacal exstrophy.

Ekstrofi Bladder vs Cloacal Ekstrofi

Insidensi
Cloacal Ekstrofi 1 : 200.000-400.000
live births
Bladder ekstrofi 1 : 10.000-50.000 live
births
Isolated epispadias 1 : 117.000 males
70% kasus isolated epispadias pada
laki-laki

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Penatalaksanaan

Diagnosis
Inspeksi:

Tampak phallus dengan
panjang 1 cm yang terbelah
simetris dengan urethral plate
ditengahnya.

Tampak kulit scrotum kanan
dan kiri di bawah phallus

Testis kanan dan kiri tidak
teraba dalam scrotum

Anus berada lebih ke anterior
mengeluarkan feses
kekuningan

Pembedahan

Rujukan ke Pediatric
surgeon dan Urologist

Kerjasama Tim

Melindungi
permukaan
mukosa buli
Timing of
operation

EB/CE Segera

IE elektif