normal and abnormal development of the urogenital tract...extrophia: epispadiasis complete...
TRANSCRIPT
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Normal and abnormal development
of the urogenital tract
Péter Nyirády
Semmelweis University
Department of Urology
EBU Certified Clinic
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Normal development of the urogenital tract
• anatomy of development
• regulating genes involved in the development of the urogenital tract
• research of the congenital anomalies
• to get known and understand the embiogenesis
• stopped development in mice by driven „nil” mutation
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• intrauterine life (human fertilization)
• human gestation (fusion of spermatozoon, oocyta)
• development of organs and form of the foetus (10 weeks)
• expansion of the embryo, enlargement, maturation(40th week)
• role of the upper urinary tract
• regulate secretion of nitrogenous waste products
• homeostasis of water and electrolytes
• acid base balance
• the production of hormones
Beginning of life and role of the urological tract
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
Human embryonic disc
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
Development of head and buccopharyngeal, tail and
cloacal membrane
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Embriogenesis of the kidney (nephrogenesis) I.
• three paired ‘kidneys’ in the mammalian embryo: (intermediate
mesoderm of the dorsal body wall) arises sequently
• the pronephros (22nd day)
• the pronephros and mesonephros degenerate during
mammalian fetal life
• whilst the metanephros develops into the adult kidney
• in contrast, the pronephros: functioning kidney in the adult
hagfish
• in some amphibians the mesonephros is the excretory organ in
adult lampreys, some fishes and amphibians
AGENESIA, HYPOPLASIA RENIS
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• 26th day pronephric duct arises and elongates (caudally) and
reaches cloacal membrane arises
• this duct is renamed as Wolffian duct
• at 24th day of gestation mesonephros develops
• mesonephric tubules develops with „mesenchymal to
epithel” transformation (40 tubules); glomeruli, proximal and
distal collecting tubules (up to 6-10. weeks)
• cranial part (epigenital), caudal part (paragenital)
Embriogenesis of the kidney (nephrogenesis) II.
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
5th week
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• metanephros (consist of only 2 cell types)
• ureteric bud (epithelial cells)
• metanephric mesenchym (mesenchymal cells)
• at 28th day ureteric bud arises (Wolffian duct’s postero-medial
aspect of mesonephric duct as a diverticulum)
• 32nd day reaches the metanephric blastema (elongates and tip)
• series of reciprocal interactions to branch multiple times
HYPOPLASIA RENIS, REN MULTICYSTICA
Embriogenesis of the kidney (nephrogenesis) III.
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• induction of the ureteric bud: 4 major calyces, 6-8 generation
fuse to form minor calyces, final 8 definitive collecting duct system
• mesenchymal induction: proximal convoluted tubes, loop of
Henle, distal convoluted tubes (glomerulogenesis: 8-9. week)
• nephrogenesis 40. week
• angio-, vasculogenesis (arteria iliaca)
HYPOPLASIA, REN DEGENERATIONEM MULTICYSTICA
VASCULARIS ATYPIA
Embriogenesis of the kidney (nephrogenesis) IV.
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
5. hét
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Ascend of the kidney and filtration
• lobulated kidney ascends from its pelvic position during the 6-9.
weeks
• blood supply from the aorta (lower branches degenerates)
• pelvic kidney faces anteriorly and rotates medially
• foetal urine is produced from the 10th week (plasma filtrations
little modified)
• tubular function develops after the 14th week
• the foetal kidneys provide over 90% of the amniotic fluid
(adequate volume to move freely, lung and skeletal development)
UNROTATED KIDNEY, DYSTOPIC KIDNEY, OLIGOHYDRAMNION
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
8th week
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
Stephen- Mackie rule
HYPOPLASIA v. DYSPLASIA
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of
the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
Weigert-
Meyer rule
REN DUPLEX
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Hypoplasia Ren dystopicus
sacralis
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Ren arcuatus
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Pyeloureteral obstruction
Praehydronephrosis
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Normal development of the urinary bladder I.
• ingrowth of mesoderm point of confluence of the allantois and the hindgut
• partitioning of the cloaca: (around 28th day) by an advancing septum
• septum aided by the in-growth of lateral Rathke’s folds
• cloaca is divided into an anterior primitive urogenital sinus that receives
the mesonephric ducts, and a posterior rectum
• septation is completed urorectal septum reaches cloacal membrane (6.w)
• urogenital membrane breaks down (7th week)
• continuity between the developing urinary tract and the amniotic cavity.
• primitive urogenital sinus: between the allantois and the mesonephric
ducts called the vesicourethral canal form the definitive bladder.
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
5th week
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• inductive interactions between endoderm of the urogenital sinus
•endoderma: urothelium
• mesenchyma: detrusor smooth muscle and adventitia
• Growth of the anterior abdominal wall between the allantois and
urogenital membrane is accompanied by an increase in size and capacity
• at 13th week circural and longitudinal muscles are visible on trigone
• at 16thweek: continentia? (inner and outer longitudinal and circular
detrusor smooth muscle)
• 21st week urothelium is developed
Normal development of the urinary bladder II.
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P.M. Cuckow, P. Nyirády: Embryology and Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
8th week
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Development of the ureterovesical junction
• ureteric orifices are incorporated into the developing bladder
• become separated from the mesonephric duct orifices
• further incorporation of more proximal mesonephric ducts the ureteric
orifices move superolaterally relative to the mesonephric duct orifices
• These stay close to the midline and descend into the developing posterior
urethra
• 9th week smooth muscle of the ureters develop (urine production)
• 18th week PU and UV obstruction
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Ureterocele VUR
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Malformations of the urinary bladder
US: bladeer 11-12nd week Dg.: (extrophia 20th week)
Agenesia: hydronephr., dysplasia
Hypoplasia:
Megacysta - megaureter VUR
Duplex: obstruction?
complete: separate urethra (penis, uterus, vagina)
partial: 1 urethra
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Urachus: opened, cysta, sinus, diverticulum.
Diverticulum: obstructioon? (valve)
Malformations of the urinary bladder II.
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Extrophia: epispadiasis complete reconstruction.
symphysealysis
fecal incontinenece
tersticular retention
Malformations of the urinary bladder III.
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Exstrophia vesicae urinariae
bladder exstrophia Cloacal exstrophia
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Circumcision piramis from
Sakara (b.c. around 2200)
Medici Katalin, II. Henrik és Diana de Poietiers
Development of the external male genitals
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Chordee
• Galen (130-199) doctor of the gladiators
• „Hypospadias”: infertility, curvature of the penis (1500 years)
• Oribasus (325-403) penile curvature sexual intercourse is
impossible
• Mettauer (1842) U.S. : „succesion of subcutaneous incisions
until the organ is liberated” - 1967 D.R. Smith
Urethral reconstruction
• Antyl (I. sz.) és Paul of Aegina (625-690) amputation
• (1000 years)
• more than 300 methods
E. Durham Smith: The history of hypospadias, Pediatr Surg Int (1997) 12: 81-85
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Development of the external male genitals
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Development of the penis
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Anatomy of the normal penis
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Meatal stenosis: obstruction: acute indication for operation
Frenulum breve:
Phimosis: - obstruction
- after the age of 2 years, opus
Penile curvatura:
hypospadiasis sine hypospadia
Prostaglandin probe fascia-dyspl. chordectomia
corporoplastica
short urethra?
Penile congenital anomalies
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Hypospadiasis penis
- place of the meatus
- chordee (adolescent)
- estetical lookeing
Acute operation: meatal
stenosis!
Dg.: curvature
Time: 1/2 - 3 years
Urethral problems II.
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Hypospadiasis
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Duplex urethra
Megalourethra: no corpus spongiosa
Paraurethr. cysta:
Cowper, utriculus prost. Endoscopic incision
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Urethra duplex
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Epispadiasis: gland episp.
1:100.000
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P.M. Cuckow, P. Nyirády: Embryology and
Pathophysiology of the Kidneys and Urinary Tracts
in Pediatric Urology, W.B. Saunders, 2001.
- agenesia
- aplasia
- hypoplasia
- retentio testis
intraabdominal
abdominoinguin.
inguinal
inguinoscrotal
ingahere
- fusional disorder of
testis and epididymis
Developmental disorder of the testes I.
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Developmental disorder of the testes II.
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THANK YOU FOR YOUR
ATTENTION!