keabnormalan kongenital (hipospadias, epispadias, fimosis, parafimosis, undescended testis

MAGS 1213Nazri b. Aiwan @ Ismail

KPPUK

UNIT 2.3.4:UNDESCENDED TESTIS,UNIT 2.3.4:UNDESCENDED TESTIS, KEABNORMALAN KONGENITAL KEABNORMALAN KONGENITAL

(PHIMOSIS, HIPOSPADIAS, (PHIMOSIS, HIPOSPADIAS, EPISPADIAS)EPISPADIAS)

KandunganKandungan

Undescended TestisUndescended Testis PhimosisPhimosis ParaphimosisParaphimosis EpispadiasEpispadias HipospadiasHipospadias

Anatomi Sis.Pembiakan LelakiAnatomi Sis.Pembiakan Lelaki

UNDESCENDED TESTESUNDESCENDED TESTES(Cryptorchidism)(Cryptorchidism)

DEFINISIDEFINISI Ketiadaan satu atau kedua-dua testes di Ketiadaan satu atau kedua-dua testes di

dalam scrotumdalam scrotum 30 % bilateral30 % bilateral


Most undescended testicles are present at Most undescended testicles are present at birth. birth.

Up to one third of premature male newborns Up to one third of premature male newborns are born with an undescended testicle, and 3 are born with an undescended testicle, and 3 to 5 % of term male infants are affected. to 5 % of term male infants are affected.

By three months of age, the incidence is By three months of age, the incidence is reduced to 0.8 %; between three months of reduced to 0.8 %; between three months of age and adulthood, the incidence does not age and adulthood, the incidence does not change.change.

True undescended testicles rarely descend True undescended testicles rarely descend spontaneously after three months of age. spontaneously after three months of age.


ETIOLOGIETIOLOGI AgenesisAgenesis Intraabdominal arrestIntraabdominal arrest Incomplete descent (intracanalicular)Incomplete descent (intracanalicular) Delayed descentDelayed descent Halangan – lesi mekanikalHalangan – lesi mekanikal Penyakit endokrina - hipoganadismPenyakit endokrina - hipoganadism

Normal Descended TestisNormal Descended Testis

Selepas 6 minggu kandungan, gen Selepas 6 minggu kandungan, gen testis-testis-determining SRYdetermining SRY pada kromosom Y memberi pada kromosom Y memberi kesan secara terus terhadap kesan secara terus terhadap indifferent gonadindifferent gonad untuk membentuk testis. untuk membentuk testis.

Sel Germ terletak pada germinal ridge berdekatan Sel Germ terletak pada germinal ridge berdekatan dengan ginjal dalam retroperitoneum. dengan ginjal dalam retroperitoneum.

Dlam 6-7 minggu kandungan, Sel Sertoli terbentuk Dlam 6-7 minggu kandungan, Sel Sertoli terbentuk dan merembeskan dan merembeskan Müllerian inhibitory substanceMüllerian inhibitory substance (MIS), yang merencatkan pembentukan organ (MIS), yang merencatkan pembentukan organ genitalia perempuan. genitalia perempuan.

Dalam 9 minggu kandungan, Sel Leydig mula Dalam 9 minggu kandungan, Sel Leydig mula menghasilkan testosterone yang menyebabkan menghasilkan testosterone yang menyebabkan pertumbuhan wolffian duct kepada bahagian trek pertumbuhan wolffian duct kepada bahagian trek genitalia lelaki. genitalia lelaki.

Normal Descended TestisNormal Descended Testis

Serentak itu, testis terbentuk sebagai organ Serentak itu, testis terbentuk sebagai organ bersama seminiferous tubules yang diseliputi bersama seminiferous tubules yang diseliputi salurdarah dan encapsulated oleh tunica salurdarah dan encapsulated oleh tunica albuginea. Seterusnya testis bergerak turun ke albuginea. Seterusnya testis bergerak turun ke pelvis, berdekatan dengan pelvis, berdekatan dengan internal ringinternal ring..

Testis terus berada dalam retroperitoneal Testis terus berada dalam retroperitoneal sehingga 28 minggu kandungan, kemudian sehingga 28 minggu kandungan, kemudian testis bergerak turun ke inguinal. testis bergerak turun ke inguinal.

Kebanyakan penurunan testis ke skrotum Kebanyakan penurunan testis ke skrotum sempurna pada 40 minggu kandungan.sempurna pada 40 minggu kandungan.

Embryonic Sexual Development Embryonic Sexual Development

PatofisiologiPatofisiologi

Mekanisma yang mempengaruhi Mekanisma yang mempengaruhi undescended testis – undescended testis –

a)a) gubernacular abnormalitiesgubernacular abnormalities

b)b) reduced intra-abdominal pressuresreduced intra-abdominal pressures

c)c) intrinsic testicular and/or epididymal intrinsic testicular and/or epididymal abnormalitiesendocrine abnormalitiesabnormalitiesendocrine abnormalities

d)d) anatomic anomalies (eg, fibrous bands anatomic anomalies (eg, fibrous bands within the inguinal canal or abnormal within the inguinal canal or abnormal arrangement of the cremasteric muscle arrangement of the cremasteric muscle fibers). fibers).


Kategori;Kategori; True undescended testiclesTrue undescended testicles (including (including

intra-abdominal, peeping at the internal intra-abdominal, peeping at the internal ring and canalicular testes), which exist ring and canalicular testes), which exist along the normal path of descent and along the normal path of descent and have a normally inserted gubernaculum; have a normally inserted gubernaculum;

Ectopic testiclesEctopic testicles, which have an , which have an abnormal gubernacular insertion; abnormal gubernacular insertion;

Retractile testiclesRetractile testicles, which are not truly , which are not truly undescended.undescended.

Lokasi True Undescended TestisLokasi True Undescended Testis


MANIFESTASI KLINIKALMANIFESTASI KLINIKAL Dikesan semasa pemeriksaan fizikal Dikesan semasa pemeriksaan fizikal

selepas lahirselepas lahir Cremasteric retraction reflex inactive.Cremasteric retraction reflex inactive. Non-palpable testis – uni/bilateral.Non-palpable testis – uni/bilateral. Inguinal herniaInguinal hernia Low sperm count (adult)Low sperm count (adult)

Lt. Lt. CryptorchidismCryptorchidism


PENYIASATANPENYIASATAN UltrasoundUltrasound CT ScanCT Scan MRIMRI LaparoscopyLaparoscopy Serum studies should include testosterone, Serum studies should include testosterone,

luteinizing hormone (LH), follicle-stimulating luteinizing hormone (LH), follicle-stimulating hormone (FSH) and müllerian-inhibiting hormone (FSH) and müllerian-inhibiting substance (MIS). Elevations in LH and FSH, as substance (MIS). Elevations in LH and FSH, as well as the absence of detectable MIS, suggest well as the absence of detectable MIS, suggest testicular absence.testicular absence.


KOMPLIKASIKOMPLIKASI InfertilityInfertility: sperm production takes place at a lower : sperm production takes place at a lower

temperature than body temperature. When testis is not temperature than body temperature. When testis is not descended, it is exposed to higher body temperature, and descended, it is exposed to higher body temperature, and sperm production is affected. In later life these patients have sperm production is affected. In later life these patients have much reduced sperm count and chance to father (50-70% much reduced sperm count and chance to father (50-70% less than normal).less than normal).

Twisting of Testis (Twisting of Testis (TorsionTorsion): When the testis is not ): When the testis is not descended, it is very free to move around itself. This makes it descended, it is very free to move around itself. This makes it easy to twist and cut off its own blood supply; the result easy to twist and cut off its own blood supply; the result being loss of testis.being loss of testis.

Testicular TumorTesticular Tumor: There is a small risk of tumor development : There is a small risk of tumor development in Undescended Testis. By doing an operation to bring it in Undescended Testis. By doing an operation to bring it down this can be easily checked out. If the testis is not down this can be easily checked out. If the testis is not brought down, tumor can develop without being noticed and brought down, tumor can develop without being noticed and can become advanced before treatment.can become advanced before treatment.

Inguinal HerniaInguinal Hernia


PENGURUSANPENGURUSAN Orchidopexy – menempatkan semula Orchidopexy – menempatkan semula

testis pada scrotal sac secara testis pada scrotal sac secara pembedahan.pembedahan.

Terapi hormon untuk bilat. Undescended Terapi hormon untuk bilat. Undescended testis bagi merangsang penghasilan testis bagi merangsang penghasilan testesterone;testesterone; hCG atau/dan LH-releasing hormones (LHRH)hCG atau/dan LH-releasing hormones (LHRH)


PEMBEDAHAN ORCHIOPEXY ;PEMBEDAHAN ORCHIOPEXY ;1.1. Open inguinalOpen inguinal

2.2. LaparoscopicLaparoscopic

Open Inguinal OrchiopexyOpen Inguinal Orchiopexy

Laparoscopic OrchiopexyLaparoscopic Orchiopexy

PHIMOSISPHIMOSIS

FIMOSISFIMOSIS

DEFINISIDEFINISI Keadaan penyempitan bukaan kulit Keadaan penyempitan bukaan kulit

prepus, yang menghalangnya tertarik ke prepus, yang menghalangnya tertarik ke belakang melepasi gland penis.belakang melepasi gland penis.

FIMOSISFIMOSIS

FIMOSISFIMOSIS

ETIOLOGIETIOLOGI KongenitalKongenital Balanitis (Inflamasi glans penis dan Balanitis (Inflamasi glans penis dan

prepus)prepus) Parut diprepus akibat kecederaan Parut diprepus akibat kecederaan

atau jangkitan di prepusatau jangkitan di prepus

FIMOSISFIMOSIS

PATOFISIOLOGIPATOFISIOLOGI Pembukaan prepus yang sempit, ia tidak Pembukaan prepus yang sempit, ia tidak

boleh di tarik ke belakang melepasi glans boleh di tarik ke belakang melepasi glans penis.penis.

Penyempitan disebabkan secara Penyempitan disebabkan secara kongenital atau trauma.kongenital atau trauma.

Jika prepus tidak dapat di tarik secara Jika prepus tidak dapat di tarik secara berterusan, smegma akan berkumpul. berterusan, smegma akan berkumpul.

Ini akan menyebabkan jangkitan pada Ini akan menyebabkan jangkitan pada glans penis(balanitis). Air kencing sukar glans penis(balanitis). Air kencing sukar di keluarkan.di keluarkan.

FIMOSISFIMOSIS

MANIFESTASI KLINIKALMANIFESTASI KLINIKAL KetidakselesaanKetidakselesaan KesakitanKesakitan DisuriaDisuria Distensi pundi kencingDistensi pundi kencing Prepus yang sempitPrepus yang sempit Jangkitan glans penis atau prepusJangkitan glans penis atau prepus

FIMOSISFIMOSIS

KOMPLIKASIKOMPLIKASI Retensi urine Retensi urine BalanitisBalanitis UTI – sistisis, pielonephritisUTI – sistisis, pielonephritis

RAWATANRAWATAN AnalgesikAnalgesik AntibiotikAntibiotik CircumcisionCircumcision

PARAFIMOSISPARAFIMOSIS

DEFINISIDEFINISI Keadaan penarikan Keadaan penarikan dan penyempitan kulit dan penyempitan kulit prepus pada corona prepus pada corona

glans penis glans penis disebabkan kegagalan disebabkan kegagalan prepus ditarik balik ke prepus ditarik balik ke

hadapan melepasi hadapan melepasi glans penisglans penis

Gland PenisCorona

Prepuce


ETIOLOGIETIOLOGI KongenitalKongenital Balanitis (Inflamasi glans penis dan Balanitis (Inflamasi glans penis dan

prepus)prepus) Parut diprepus akibat kecederaan atau Parut diprepus akibat kecederaan atau

jangkitan di prepusjangkitan di prepus Iatrogenic – i.e. catheterizationIatrogenic – i.e. catheterization Self inflicted - piercing with a penile ring Self inflicted - piercing with a penile ring

into the glansinto the glans Secondary to erectionsSecondary to erections


PATOFISIOLOGIPATOFISIOLOGI When the foreskin becomes trapped behind the When the foreskin becomes trapped behind the

corona for a prolonged period, a tight band of corona for a prolonged period, a tight band of tissue forms around the penis. tissue forms around the penis.

This constricting ring initially impairs venous This constricting ring initially impairs venous blood and lymphatic flow from the glans penis blood and lymphatic flow from the glans penis and prepuce, in turn causing edema of the glans.and prepuce, in turn causing edema of the glans.

As the edema worsens, arterial blood flow As the edema worsens, arterial blood flow becomes compromised. The ensuing tissue becomes compromised. The ensuing tissue ischemia and vascular engorgement cause ischemia and vascular engorgement cause painful swelling of the glans and prepuce and painful swelling of the glans and prepuce and may eventually lead to gangrene or may eventually lead to gangrene or autoamputation of the distal penis.autoamputation of the distal penis.


KEJADIAN:KEJADIAN: Melibatkan golongan remaja dan lelaki mudaMelibatkan golongan remaja dan lelaki muda

MANIFESTASI KLINIKALMANIFESTASI KLINIKAL Sakit glans penisSakit glans penis Kencing tersekatKencing tersekat DisuriaDisuria penis is enlarged and congested with a collar of penis is enlarged and congested with a collar of

edematous foreskinedematous foreskin EdemaEdema JangkitanJangkitan


KOMPLIKASIKOMPLIKASI Retensi urine Retensi urine BalanitisBalanitis UTI – sistisis, pielonephritisUTI – sistisis, pielonephritis GengreneGengrene

PARAFIMOSISPARAFIMOSISRAWATANRAWATAN Tenangkan pesakitTenangkan pesakit Berikan Analgesik – lignocaine jellyBerikan Analgesik – lignocaine jelly Berikan Antibiotik – kes infeksiBerikan Antibiotik – kes infeksi Pelepasan parafimosis;Pelepasan parafimosis;

Manual - lakukan tekanan perlahan-lahan Manual - lakukan tekanan perlahan-lahan menggunakan hujung jari ke atas glans penis menggunakan hujung jari ke atas glans penis dan tolak glans penis ke belakang dalam masa dan tolak glans penis ke belakang dalam masa yang sama tarik prepus yang ketat balik yang sama tarik prepus yang ketat balik melepasi koronamelepasi korona

Dekompresi (Perth-Dundee method)Dekompresi (Perth-Dundee method) swelling swelling dengan jarum suntikandengan jarum suntikan

Dorsal slitDorsal slit Rawatan definitif - CircumcisionRawatan definitif - Circumcision

Manual Reduction ParaphimosisManual Reduction Paraphimosis

Dorsal Slit & CircumcissionDorsal Slit & Circumcission

Paraphimosis

Dorsal slit

Circumcission

EPISPADIASEPISPADIAS

Normal Embryonic Sexual Development Normal Embryonic Sexual Development

Embryonic Sexual Development Embryonic Sexual Development (Undifferentiated Stage)(Undifferentiated Stage)

MaleMale FemaleFemale

9 week old embryo - 11 weeks Pregnant

1. Anus, 2. Labio scrotal folds, 3. Legs ,4. Genital tubercle7. Urethral groove, 8. Urogenital folds

Embryonic Sexual Development Embryonic Sexual Development (differentiated Stage)(differentiated Stage)

MaleMale FemaleFemale

Male - 11 week old fetus - 13 weeks Pregnant

Pembentukan genitalia fetus Pembentukan genitalia fetus (Male)(Male)

Bergantung dihydrotestosterone yg dihasilkan Bergantung dihydrotestosterone yg dihasilkan testes. testes.

Genital tubercle memanjang dan membentuk penis Genital tubercle memanjang dan membentuk penis Lipatan urogenital yg terletak dikedua-dua belah Lipatan urogenital yg terletak dikedua-dua belah

urogenital membrane bercantum & membentuk urogenital membrane bercantum & membentuk urethral groove. Lipatan urogenital melitupi bhgn urethral groove. Lipatan urogenital melitupi bhgn spongy urethra. spongy urethra.

Genital tuber membentuk glans penis (4). Genital tuber membentuk glans penis (4). Badan penis terbentuk melalui percantuman Badan penis terbentuk melalui percantuman

lipatan urogenital, percantuman masih belum lipatan urogenital, percantuman masih belum sempurna (7).sempurna (7).

Skrotum (6) terbentuk melalui percantuman lipatan Skrotum (6) terbentuk melalui percantuman lipatan labio scrotal (2). labio scrotal (2).

Raphe pada skcrotum (5) mewakili zon Raphe pada skcrotum (5) mewakili zon percantuman lipatan labio scrotal. percantuman lipatan labio scrotal.


DEFINISIDEFINISI Pembukaan urethral pada bahagian Pembukaan urethral pada bahagian

superior/dorsum penis.superior/dorsum penis.


KLASIFIKASIKLASIFIKASI

c) Phenopubicc) Phenopubic(Complete)(Complete)

a) Glandulara) Glandular b) Penileb) Penile

Epispadias & exstrophy of the bladder


Epispadias can be explained by defective Epispadias can be explained by defective migration of the paired primordia of the migration of the paired primordia of the genital tubercle that fuse on the midline genital tubercle that fuse on the midline to form the genital tubercle at the fifth to form the genital tubercle at the fifth week of embryologic development. week of embryologic development.

PathophsiologyPathophsiology In males, epispadias causes impotentia In males, epispadias causes impotentia

coeundi (kegagalan ereksi), which results from coeundi (kegagalan ereksi), which results from the dorsal curvature of the penile shaft, and the dorsal curvature of the penile shaft, and impotentia generandi (sterility), which results impotentia generandi (sterility), which results from the incomplete urethra.from the incomplete urethra.

frequent ascending infections to the prostate or frequent ascending infections to the prostate or bladder and kidneys bladder and kidneys

psychological problems related to the deformity. psychological problems related to the deformity. If epispadias is distal to the bladder neck, If epispadias is distal to the bladder neck,

urinary continence may not be present.urinary continence may not be present.


MANIFESTASI KLINIKALMANIFESTASI KLINIKAL Normal urethra digantikan oleh broad mucosal Normal urethra digantikan oleh broad mucosal

strip pada dorsum corpora cavernosa; bukaan strip pada dorsum corpora cavernosa; bukaan meatus pada dorsum penis antara glans meatus pada dorsum penis antara glans hingga pubis, hingga pubis,

Shaf penis membengkok keatas (dorsal) Shaf penis membengkok keatas (dorsal) dengan ketiadaan preputial apron, & cleft dengan ketiadaan preputial apron, & cleft terdapat pada permukaan atas penis.terdapat pada permukaan atas penis.

Penis –leper, pendek dan bengkok ke atas Penis –leper, pendek dan bengkok ke atas IncontinenceIncontinence


PENGENDALIANPENGENDALIAN Sokongan moralSokongan moral Pembedahan ‘corrective’Pembedahan ‘corrective’a)a) Glandular epispadias - reposition of the Glandular epispadias - reposition of the

distal urethra and glanuloplasty.distal urethra and glanuloplasty. b) Penile epispadias - resection of the

chordee and urethroplastyc) Penopubic - close the abdominal wall and

the bladder exstrophy

HIPOSPADIASHIPOSPADIAS

DEFINISIDEFINISI

Pembukaan Inferior bahagian meatus Pembukaan Inferior bahagian meatus (Incomplete formation of the distal part (Incomplete formation of the distal part of the urethra)of the urethra)

Berlaku 3 dalam 1 000 kelahiranBerlaku 3 dalam 1 000 kelahiran

HIPOSPADIASHIPOSPADIASJENISJENIS

1.1. Glandular hypospadiasGlandular hypospadias• Paling kerapPaling kerap

• Pembukaan meatus Pembukaan meatus external adalah sedikit di external adalah sedikit di belakang pembukaan belakang pembukaan biasa.biasa.

• Tiada kecacatan, chordee Tiada kecacatan, chordee dan masalah sexdan masalah sex

2.2. Coronal hypospadiasCoronal hypospadias• Ada chordeeAda chordee


JENISJENIS

3.3. Penoscrotal/PerinealPenoscrotal/Perineal

• Marked chordeeMarked chordee

• Penis terlalu pendek, leper & membengkok Penis terlalu pendek, leper & membengkok keataskeatas

• Undescended testesUndescended testes


PENGENDALIANPENGENDALIAN

1.1. Tiada rawatan untuk mild hipospadiasTiada rawatan untuk mild hipospadias2.2. Indikasi PembedahanIndikasi Pembedahan

• Gross cordeeGross cordee

• Meatus membuka terlalu jauh ke belakangMeatus membuka terlalu jauh ke belakang

3.3. Pembedahan – corrective surgeryPembedahan – corrective surgery 6-12 months6-12 months

keabnormalan kongenital (hipospadias, epispadias, fimosis, parafimosis, undescended testis

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