Ahmad HannoProfessor of UrologyHead of Pediatric Urology
1. Number2. Size and structure3. Rotation4. Location5. Fusion
1. Unilateral Renal agensis2. Bilateral renal agnesis
 Incompatible with life Severe oligohydraminos Potter’s syndrome (flattened facies, wide nasal bridge,hypoplastic lungs...
 Incidence 1 in 1000 Absent ureteric bud Asymptomatic Compensatory hypertrophy of contralateral kidney
1. Hypoplastic kidney2. Polycystic kidney3. Multicystic dysplastic kidneys
 Small kidney, less no of nephrons Renal vessels and ureter…. Somewhat underdeveolped Treatment….. Depends Nephrectomy...
 Adult: autosomal dominant Infantile: autosomal recessive
 Polycystic: non-communicating cysts, interveningparenchyma Multicystic dysplastic: non-communicating, variablesized, no...
1. Malrotated kidneys
 Pelvis directed anteriorly (instead of medially) Calyces directed posteriorly ( laterally) Asymptomatic
 RENAL ECTOPIA1. Simple renal ectopia2. Crossed renal ectopia
 Failure of normal ascent of the kidney to its normallumbar location Commonest: Pelvic kidney
 Asymptomatic ?? Palpable ( superficial location) in iliac fossa Malrotated, short ureter Blood supply : iliac vessels...
 DD Nephroptosis Rx….. Nephropexy
 Kidney crosses midline, to the other side Fused or non fused Ureter crosses to normal location at the bladder
1. Horse shoe kidney2. Crossed fused renal ectopia
 Lower poles are connected by an isthmus Malrotated kidneys Lower level than normal (inferior mesenteric artery) Longi...
 Asymptomatic No Rx More liable for stones, PUJO
 PUJO Ureteral duplication Ureterocele VUR
Causes of PUJ ObstructionIntrinsic StenosisUreteric Folds
Lower pole vesselsHorseshoeKidney
 Complete1. Upper….. Lower & medial location2. Lower….. Upper & lateral location Incomplete
 A ureterocoele is a cystic dilatation of the lower end ofthe ureter. Herniation of urotheliun of the submucosal ureter....
 Rx Incision…… septic Excision Upper partial nephrectomy
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
2 congenital anomalies  undergraduate
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2 congenital anomalies undergraduate

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2 congenital anomalies undergraduate

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