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