4. Congenital Nephropathies and Uropathies - Cox

• Bilateral agenesis due to large adrenal gland

  • leads to POTTER (oligohydramnios) sequence
• kidney hypoplasia - failure of kidney to develop to normal size without scarring

  • contrast with renal atrophy = renal develop initially normal
• renal tubular dysgenesis - short and poorly developed PCT

  • oligohydramnios - Potter sequence
• What can be seen in incomplete fusion of developing renal lobules?

  persistent fetal lobulation
• duplication of ureter is due to incomplete fusion of upper and lower poles
• What is the most common cause of neonatal ab masses?

  duplicated ureters - present as flank mass or pyelonephritis
• Duplication of ureter can cause complications by

  • vesicoureteral reflux due to improper ureter insertion causing bladder infection
  • obstruction - hydronephrosis
  • ureterocele - dilation of distal ureter
• Horseshoe kidney

  • fused at lower poles
• ectopic kidney - abnormal location of one or both kidney

  • due to arrested migration
• ureter obstruction - 3 points

  • Pelviureteric junction
  • Crossing iliac vessels
  • ureterovesical junction
• Pelviureteric junction (PUJ) obstruction cause antenatal hydronephrosis
• Congenital bladder diverticula can cause

  • bladder outpouching
  • urinary stasis leading to stones or infection
• Bladder Extrophy - Developmental failure anterior abdominal wall