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PEDI GU REVIEW embryology i

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  • 1. EMBRYOLOGY Pediatric GU Review UCSD Pediatric Urology George Chiang MD Sara Marietti MD Outlined from The Kelalis-King-Belman Textbook of Clinical Pediatric Urology 2007 (not for reproduction, distribution, or sale without consent)
  • 2. Timeline Quiz I Anterior Abdominal Wall Renal Vasculature Pronephros Mesonephros Kidney Ureter Calices 0 4 8 12 16 20 24 28 32 36
  • 3.
    • What is Pronephros?
    Transitory, nonfunctional kidney, analogous to that of primitive fish. First evidence seen in the 3rd week and completely degenerates by the 5th week
  • 4.
    • What is Mesonephros?
    Second kidney. Transient but serves as the excretory organ for the embryo while the definitive kidney the metanephros begins its development
  • 5. Timeline Quiz II Cloaca UG Sinus Bladder Primitive Gonads Gonadal Ridge Ovary/Testes 0 4 8 12 16 20 24 28 32 36
  • 6.
    • What is cloaca?
    clo·a·ca (klō-ā'kə) pronunciation n., pl. -cae (-sē'). 1. A sewer or latrine. 2. Zoology. 1. The common cavity into which the intestinal, genital, and urinary tracts open in vertebrates such as fish, reptiles, birds, and some primitive mammals. 2. The posterior part of the intestinal tract in various invertebrates.
  • 7. Timeline Quiz III Phallus Vagina/Uterus/Fallopian tubes Prostate, SVs, Vas Deferens 0 4 8 12 16 20 24 28 32 36
  • 8. Match the Teratogen Rubella Virus Cocaine Oral Contraceptives ACE Inhibitors Anticonvulsants Maternal Hyperthermia Ethanol Progestational Agents Eagle-Barrett Syndrome,hypospadias, hydronephrosis Micropenis Hypospadias, cryptorchidism Hypospadias,hydronephrosis Renal Dysplasia Hypospadias, ambiguous genitalia, cryptorchidism Ambiguous genitalia Hypospadias
  • 9. Adrenal Development
    • Adrenal Medulla or Adrenal Cortex
    Derived from migrating ectodermal neural crest cells
  • 10.
    • Where is adrenal cortex derived from?
    Primitive mesoderm medial to the urogenital ridge; 4th gestational week
  • 11.
    • Cortex develops 2 distinct areas by 8th week
      • Central area(fetal zone)
      • Outer Rind (adult cortex) GFR
    • Adrenal glands increase in size from 2nd/3rd month from 5 mg-->80 mg but lose 1/3 weight in first 2 weeks of life secondary to fetal zone involution
  • 12. Adrenal Development
    • Adrenal Medulla or Adrenal Cortex
    Cells may migrate to celiac plexus, broad ligament, ovarian/spermatic vessels or around kidneys/ueterus
  • 13. Hypothalamic-pituitary-adrenal axis Placental Estrogens Fetal Cortisol Inactive Cortisone POMC mRNA ACTH Adrenal Cortisol Fetal HPA Axis ? ?
  • 14. Hypothalamic-pituitary-adrenal Axis
    • Hypothalamus releases CRH 8-12 weeks
    • Fetal glucocorticoid stimulates release of placental CRH
    • Overall upregulation of fetal cortisol is essential for survival
  • 15. Adrenal Development
    • Adrenal Medulla or Adrenal Cortex
    In fetus produces large amounts of DHEA after 1st trimester
  • 16.
    • Adrenal medulla
      • Chromaffin cells can be found throughout medulla and sympathetic chain
      • Organ of Zuckerkandl
      • Go over pg 238 of adrenal handout
  • 17.
    • Who is Zuckerkandl?
    Emil Zuckerkandl (September 1, 1849 in Raab, Hungary – May 28, 1910 in Vienna) was a Hungarian-Austrian anatomist. Educated at the University of Vienna (M.D. 1874). In 1875 he became privat-docent of anatomy at the University of Utrecht, and he was appointed assistant professor at the University of Vienna in 1879, being made professor at Graz in 1882. Since 1888 he has been professor of descriptive and topographical anatomy at the University of Vienna. Zuckerkandl has contributed many monographs to medical journals. Among his works the following may be mentioned: "Zur Morphologie des Gesichtschädels" (Stuttgart, 1877); "Über eine Bisher noch Nicht Beschriebene Drüse der Regio Suprahyoidea" (ib. 1879); "Über das Riechcentrum" (ib. 1887); and "Normale und Pathologische Anatomie der Nasenhöhle und Ihrer Pneumatischen Anhänge" (Vienna, 1892).
  • 18. Match the Condition Retrocaval ureter Renal Agenesis ADPKD ARPKD Multicystic dysplastic kidneys Pelvic Ectopia Horeshoe Kidney Supernumerary Kidney Pronephros fails to develop and mesonephros fails to develop Failure of renal ascent Persistence of R subcardinal vein as IVC Ascent arrested by IMA Enlargement of collecting ducts with “sunburst pattern” Early ureteral obstruction or faulty ureteral bud 1 in 3000 pregnancies Division of ipsilateral ureteral bud Cysts form thru abnormal collecting duct branching Renal Vasculature and Kidney
  • 19. Anterior wall/Bladder/Urethra
    • Exstrophy
      • Failure of secondary mesoderm to cover infraumbilical abdominal wall
      • Premature rupture of the cloacal membrane
    Cloacal Exstrophy week Classic Exstrophy week 5th 7th
  • 20.
    • When does the cloaca develop?
    First 3 weeks
  • 21. Notch is filled with mesenchyme to form urorectal septum This pushes caudally and divides cloaca into primitive rectum and UG sinus 5th week
  • 22.
    • 7th week ureters empty into bladder
    • Mesonephric ducts shift caudad in UG sinus and lie close to each other
    • Ureters shift cephalad and laterally into bladder
  • 23.
    • 8th week bladder muscle begins to appear
    • 9th week bladder expands into sac
    • 12th week bladder epithelium becomes transitional
    • Urachal anaomalies can occur from mesodermal failure
    • Bladder duplications are usually caudal duplication
  • 24.
    • What is Eagle Barrett Syndrome?
      • A) Triad syndrome
      • B) Prune Belly Syndrome
      • C) Deficient abdominal musculature, UDT, urinary tract anomalies
      • D) All of the above
  • 25.
    • What are the theories behind Prune Belly?
    1) Faulty mesodermal development during the 6th to 12th week 2) Deformation of abdominal wall by distended viscera or increased pressure 3) Primary abdominal wall defect resulting in decreased pressure
  • 26. Ureteral Development
    • How does ureteral atresia develop?
    • How does duplication occur?
    • Where does the ureteral bud develop?
    Relative or total ischemia during kidney migration Multiple ureteral buds develop Distal end of mesonphric duct or Wolffian duct
  • 27. Ureteral Development
    • How does the trigone develop?
    Formed by integrating the upper portions of the common excretory ducts and a small portion of the mesonphric ducts
  • 28. Gonads, genital ducts,genitalia Week Male Female 6th Indifferent Gonad Indifferent Gonad 10th Ext genitalia are becoming more visible 11th Prostate arises Urethral Glands 12th Littre’s glands Secondary ovarian cortex
  • 29. Gonads, genital ducts,genitalia Week Male Female 13th SVs start to develop Mesonephric ducts regress 14th Penile shaft elongates 16th Uterus forms 20th Prepuce completely formed Vaginal lumen completely formed
  • 30. Gonads, genital ducts,genitalia Week Male Female 24th Efferent ductules of epididymis Tunical albuginea of ovary forms 28th PV has herniated thru abdomen Gub fuses with lateral uterus
  • 31. Embryological Remnants
    • Male Mullerian Remnants?
    Appendix Testes, Prostatic Utricle
    • Female Wolffian Remnants?
    Epoophoron, Paraoophoron, Gartner’s cyst
  • 32.
    • What is a utricle?
    sau·tri·cle 1 (ytr-kl) n. 1. A membranous sac contained within the labyrinth of the inner ear and connected with the semicircular canals. 2. Botany A small bladderlike one-seeded indehiscent fruit, as in the amaranth.
  • 33. Embryological Homologues
    • UG Sinus
    • Genital Tubercle
    • Genital Folds
    • Genital Swellings
    Clitoris Labia Minora Urethra/Upper Vagina Labia Majora Glans Penis Scrotum Urethra/Penile Shaft Prostatic Urethra/Prostate
  • 34. MORE QUESTIONS
  • 35.
    • Classic features of prune-belly syndrome include dilated tortuous ureters, atretic anterior abdominal wall musculature, and undescended testes. In addition, there is usually hypoplasia of the:
    • Radius
    • Tibia
    • Prostate
    • Vas deferens
    • epididymis
  • 36.
    • In a patient with an absent right kidney and a left pelvic kidney, the right adrenal is:
    • Absent and the left is adjacent to the upper pole of the kidney
    • Absent and the left is in the normal anatomic position
    • In the normal anatomic position and the left is adjacent to the upper pole of the kidney
    • In the normal anatomic position and the left is in the normal anatomic position
    • In the normal anatomic position and the left is absent
  • 37.
    • During embryologic development, the duct that passes through the umbilicus is the:
    • Mullerian
    • Wolffian
    • Gartner’s
    • Luschka
    • Omphalomesenteric (vitelline)