Post on 12-Nov-2014
description
Ahmad Hanno
Professor of Urology
Head of Pediatric Urology
Congenital Anomalies Of the Upper Urinary Tract
1. Number
2. Size and structure
3. Rotation
4. Location
5. Fusion
Anomalies of the kidney
1. Unilateral Renal agensis
2. Bilateral renal agnesis
Anomalies of the number
Incompatible with life
Severe oligohydraminos
Potter’s syndrome (flattened facies, wide nasal bridge, hypoplastic lungs, club foot)
Bilateral renal agenesis
Potter’s syndrome
Incidence 1 in 1000
Absent ureteric bud
Asymptomatic
Compensatory hypertrophy of contralateral kidney
Unilateral agenesis
1. Hypoplastic kidney
2. Polycystic kidney
3. Multicystic dysplastic kidneys
Anomalies of size
Small kidney, less no of nephronsRenal vessels and ureter…. Somewhat
underdeveolped
Treatment….. Depends
Nephrectomy only if complicated
Renovascular hypertension
Hypoplastic kidney
Hypoplastic kidney
Polycystic kidneys
Polycystic kidneys
Adult: autosomal dominant
Infantile: autosomal recessive
Polycystic kidneys
Polycystic: non-communicating cysts, intervening parenchyma
Multicystic dysplastic: non-communicating, variable sized, no intervening parenchyma, function nearly zero
Cystic kidneys
1. Malrotated kidneys
Anomalies of rotation
Pelvis directed anteriorly (instead of medially)
Calyces directed posteriorly ( laterally)
Asymptomatic
Malrotated kidneys
RENAL ECTOPIA
1. Simple renal ectopia
2. Crossed renal ectopia
Anomalies of location
Failure of normal ascent of the kidney to its normal lumbar location
Commonest: Pelvic kidney
Simple renal ectopia
Asymptomatic
?? Palpable ( superficial location) in iliac fossa
Malrotated, short ureter
Blood supply : iliac vessels, other surrounding vessels
Pelvic kidney
DD
Nephroptosis
Rx….. Nephropexy
Pelvic kidney
Kidney crosses midline, to the other side
Fused or non fused
Ureter crosses to normal location at the bladder
Crossed renal ectopia
Crossed renal ectopia
1. Horse shoe kidney
2. Crossed fused renal ectopia
Anomalies of fusion
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
Horse shoe kidney
Horse shoe kidney
Asymptomatic
No Rx
More liable for stones, PUJO
Horse shoe kidney
PUJO
Ureteral duplication
Ureterocele
VUR
Anomalies of renal pelvis & ureter
PUJO
Causes of PUJ Obstruction
Intrinsic Stenosis
Ureteric Folds
Lower pole vessels
Horseshoe Kidney
Balloon on string
Pyeloplasty
Ureteral duplication
Duplicated ureter
Complete1. Upper….. Lower & medial location2. Lower….. Upper & lateral location
Incomplete
Duplicated ureter
Ureterocele
A ureterocoele is a cystic dilatation of the lower end of the 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 an ectopic location below the bladder neck
Ureterocele
Rx
Incision…… septic
Excision
Upper partial nephrectomy
Ureterocele