2 congenital anomalies undergraduate

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Post on 12-Nov-2014



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  • 1. Ahmad HannoProfessor of UrologyHead of Pediatric Urology
  • 2. 1. Number2. Size and structure3. Rotation4. Location5. Fusion
  • 3. 1. Unilateral Renal agensis2. Bilateral renal agnesis
  • 4. Incompatible with life Severe oligohydraminos Potters syndrome (flattened facies, wide nasal bridge,hypoplastic lungs, club foot)
  • 5. Incidence 1 in 1000 Absent ureteric bud Asymptomatic Compensatory hypertrophy of contralateral kidney
  • 6. 1. Hypoplastic kidney2. Polycystic kidney3. Multicystic dysplastic kidneys
  • 7. Small kidney, less no of nephrons Renal vessels and ureter. Somewhat underdeveolped Treatment.. Depends Nephrectomy only if complicated Renovascular hypertension
  • 8. Adult: autosomal dominant Infantile: autosomal recessive
  • 9. Polycystic: non-communicating cysts, interveningparenchyma Multicystic dysplastic: non-communicating, variablesized, no intervening parenchyma, function nearlyzero
  • 10. 1. Malrotated kidneys
  • 11. Pelvis directed anteriorly (instead of medially) Calyces directed posteriorly ( laterally) Asymptomatic
  • 12. RENAL ECTOPIA1. Simple renal ectopia2. Crossed renal ectopia
  • 13. Failure of normal ascent of the kidney to its normallumbar location Commonest: Pelvic kidney
  • 14. Asymptomatic ?? Palpable ( superficial location) in iliac fossa Malrotated, short ureter Blood supply : iliac vessels, other surrounding vessels
  • 15. DD Nephroptosis Rx.. Nephropexy
  • 16. Kidney crosses midline, to the other side Fused or non fused Ureter crosses to normal location at the bladder
  • 17. 1. Horse shoe kidney2. Crossed fused renal ectopia
  • 18. Lower poles are connected by an isthmus Malrotated kidneys Lower level than normal (inferior mesenteric artery) Longitudinal axis downwards and medially Lower calyces . Directed medially
  • 19. Asymptomatic No Rx More liable for stones, PUJO
  • 20. PUJO Ureteral duplication Ureterocele VUR
  • 21. Causes of PUJ ObstructionIntrinsic StenosisUreteric Folds
  • 22. Lower pole vesselsHorseshoeKidney
  • 23. Complete1. Upper.. Lower & medial location2. Lower.. Upper & lateral location Incomplete
  • 24. A ureterocoele is a cystic dilatation of the lower end ofthe ureter. Herniation of urotheliun of the submucosal ureter. Occurs with a single system or double system. May be located in the bladder, or it may open in anectopic location below the bladder neck
  • 25. Rx Incision septic Excision Upper partial nephrectomy