Embriologi sistem

urogenital
YOGA PAMUNGKAS SUSANI
 The ten most frequently reported birth defects in
Victoria between 2003-2004.
1.Hypospadias
2.Obstructive Defects of the Renal Pelvis or
Obstructive Genitourinary Defects
3.Ventricular Septal Defect
4.Congenital Dislocated Hip
5.Trisomy 21 or Down syndrome
6.Hydrocephalus
7.Cleft Palate
8.Trisomy 18 or Edward Syndrome - multiple
abnormalities of the heart, diaphragm, lungs,
kidneys, ureters and palate 86% discontinued.
9.Renal Agenesis/Dysgenesis - reduction in neonatal
death and stillbirth since 1993 may be due to the
more severe cases being identified in utero and
being represented amongst the increased
proportion of terminations (approximately 31%).
10.Cleft Lip and Palate - occur with another defect in
33.7% of cases.
Learning objective
- memahami proses perkembangan sistem
urinarius pada janin
- memahami kelainan kongenital terkait
embriologi sistem urinarius
Masih ingat anatomi sistem
urinarius?
Perkembangan ginjal
 primary kidneys system, muncul pada minggu ke-4
Pronephros - tidak fungsional dan regresi sempurna pada akhir minggu ke-4

Fungsional antara mg ke-6-10, degenerasi pada akhir trimester

1
Ginjal mesonehric terdiri atas 40 glomerulus dangan tubulus
Mesonephros mesonephricus yang berumuara ke ductus mesonephricus
menuju ke cloaca
Pronephric duct → mesonephric duct (ductus Wolfian)
Tubulus dan ductus menjadi beberapa struktur pada laki-laki
- Primordia of the permanent kidneys
Metanephros - Berkembang dari 2:
Gemma ureterica/ diverticulum metanephricum/ The
ureteric bud (tunas ureter)
Blastema metanephrogenicum
•Gemma ureterica/The ureteric bud merupakan
primordium dari ureter, pelvis renalis, kaliks, dan tubulus
collectivus
•Perpanjangan tunas akan penetrasi ke blastema
•metanephrogenicum yang merupakan bakal nephron
•Perkembangan ginjal berlangsung hingga minggu ke-36
•Maturasi fungsi terjadi setelah lahir
Perubahan posisi ginjal
Di dalam pelvis → seperti posisi dewasa dicapai pada
minggu ke-9 → kontak dengan glandula suprarenal
Dikarenakan pertumbuhan relatif embrio kaudal
terhadap ginjal. Ginjal juga berotasi ke medial 90
derajat.
 Pada awalnya arteri renalis adalah cabang dari arteri iliaca communis,
selanjutnya a. renalis dicabangkan oleh aorta abdominalis.
a. Renalis accessoria merupakan variasi yang sering terjadi (sekitar 25%)
b. A. renalis polar dapat menyilang di anterior ureter dan menyebabkan
obstruksi ureter → hidronefrosis
c. A. renalis accessoria adalah end artery → jika rusak → iskemia
 The ten most frequently reported birth defects in Victoria between 2003-2004.
1.Hypospadias
2.Obstructive Defects of the Renal Pelvis or Obstructive Genitourinary Defects
3.Ventricular Septal Defect
4.Congenital Dislocated Hip
5.Trisomy 21 or Down syndrome
6.Hydrocephalus
7.Cleft Palate
8.Trisomy 18 or Edward Syndrome - multiple abnormalities of the heart, diaphragm,
lungs, kidneys, ureters and palate 86% discontinued.
9.Renal Agenesis/Dysgenesis - reduction in neonatal death and stillbirth since 1993 may
be due to the more severe cases being identified in utero and being represented
amongst the increased proportion of terminations (approximately 31%).
10.Cleft Lip and Palate - occur with another defect in 33.7% of cases.
Obstructive Renal Pelvis Defect
- ureter memuntir, membelok
- kompresi ureter oleh arteri
- malformasi dinding abdomen
Penyumbatan bisa terjadi pada UPJ, UVJ
Akibat:
- hidronefrosis
- oligohidramnion
- megaureter
Unilateral Renal Agenesis
▪lebih banyak pada bayi laki-laki
▪Masih bisa asimtomatik
▪Lebih sering ginjal kiri yang tidak ada
▪Ginjal yang tersisa dapat mengalami
hipertrofi kompensatoris
Bilateral renal agenesis
- lebih banyak pada laki-laki (3x)
- oligohidramnion
- gangguan perkembangan paru
- deformitas pada wajah
Kegagalan penetrasi ureteric bud ke
metanefrogenik blastemal → kegagalan
pembentukan nefron
Potter syndrome (sloped forehead, parrot
beak nose, shortened fingers,hypoplasia
internal organ: gut and lungs)
Anomali fusi
Crossed fused ectopia Horseshoe kidney
Ginjal yang menyatu terhalang naik oleh a.
mesenterica inferior
Bisa asimtomatik
Risiko untuk batu dan infeksi naik
15% sindroma Turner mengalami horseshoe
kidney
Nephroblastoma (Wilms Tumor)

found in infants from 0-24 months of age

consists of blastemal, epithelial, and stromal cell types
associated with mutations in genes related to kidney development
(PAX2, WT1, etc.)
essentially due to incomplete mesenchymal-to-epithelial
transformation (i.e. the cells fail to fully differentiate and transform
into cancerous cells).
Polycystic kidney disease

can arise due to a variety of factors:

◦ Overproliferation: excessive growth of tubule epithelium can occlude the
lumen causing blockage.
Perkembangan vesica,
uretra
Sinus urogenital:
- pars vesical → vesical urinaria
Pars pelvica → uretra pada collum vesical, uretra pars prostatica,
seluruh uretra pada wanita
Pars phallic → bakal penis/klitoris
Ductus mesonefrikus berkontribusi membentuk dinding belakang
vesical → Trigonum vesicae
Ureter ektopik
Ureter tidak bermuara ke vesica urinaria
Pada pria bisa langsung bermuara ke pars prostatica uretra atau bisa
ke ductus deferens, utriculus prostaticus, glandula seminalis
Pada wanita bisa ke collum vesical, uretra, vagina, vestibulum vagina
Terjadi karena ureter dibawa terlalu ke kaudal oleh ductus
mesonefrikus
INKONTINENSIA
Anomali urakal
Extrophia vesica
Defisiensi dinding anterior vesical dan abdomen
Lebih sering laki-laki
Diakibatkan oleh kegagalan migrasi sel mesenkimal antara
ectoderm dan endoderm dinding abdomen (membrane
kloaka) selama minggu ke-4.
Epitel uretra spongiosa berasal
dari 2:
Endoderm pars phallic sinus
urogenital dan
Ectoderm permukaan yang
membentuk epitel uretra distal
(fossa navicularis)
Urogenital
Gonad berasal dari
mesoderm intermediate
Ductus genital berasal dari
sepasang mesonephric dan
paramesonephric ducts
◦The mesonephric ducts →
MALE genital ducts
◦The paramesonephric ducts →
FEMALE genital ducts
Under the influence of SRY, the gonad develops
into a TESTIS containing spermatogonia, Leydig
cells, and Sertoli cells.
Leydig cells produce TESTOSTERONE, which
support growth of the mesonephric ducts. NOTE:
without testosterone, the mesonephric ducts will
REGRESS.
Some testosterone is converted into
Dihyroxytestosterone (DHT), which supports
development of the prostate gland, penis, and
scrotum.
Sertoli cells produce ANTI-MÜLLERIAN
HORMONE (aka Müllerian Inhibiting Substance, or
MIS), which induces regression of the
paramesonephric ducts. NOTE: in the absence of
MIS, the paramesonephric ducts will PERSIST.
Ureteric bud: ureter
Mesonephric ducts: rete testis, efferent ducts,
epididymis, vas deferens, seminal vesicle,
trigone of bladder
Urogenital sinus: bladder (except trigone),
prostate gland, bulbourethral gland, urethra
In the absence of SRY, the gonad develops into
an ovary with oogonia and stromal cells.
Since no testosterone is produced, the
mesonephric ducts regress.
Since there is also no MIS, the
paramesonephric ducts persist to give rise to
the oviducts, uterus, and upper 1/3 of the
vagina
The urogenital sinus contributes to the
formation of the bulbourethral glands and the
lower 2/3 of the vagina
the uterus, cervix, and upper 1/3 of the
vagina (derived from paramesonephric ducts)
are shown in red and the lower 2/3 of the
vagina, bulbourethral glands (purple buds
appearing at the end of the movie), and
vestibule (derived from urogenital sinus)
Ureteric bud: ureter
Mesonephric ducts: trigone of bladder
Paramesonephric ducts: oviduct, uterus, upper
1/3 of vagina
Urogenital sinus: bladder (except trigone),
bulbourethral gland, urethra, lower 2/3 of
vagina
Chriptorchidimus
Processus vaginalis
Genitalia eksterna
HIPOSPADIA
Glanular
Penile
Penoscrotal
Perineal

Inadekuat produksi androgen oleh testis fetus

Epispadia
Seringkali terkait dengan ekstrofia vesica
Inadekuat interaksi ectodermal-mesenkimal selama
perkembangan tuberculum genital.
T/F?

The bladder (except the trigone) is derived from

tissue associated with the mesonephric duct.
T/F
The glomeruli of the kidney are derived from the
ureteric bud
TERIMAKASIH