Common Urological Abnormalities in Children

Dr Wan Jazilah Wan Ismail Hospital Selayang

Bilateral Renal Agenesis

Potter syndrome Pulmonary hypoplasia Absence of amniotic fluid Facial compression: flattened nose Complete absence of kidneys

Unilateral Renal Agenesis

Complete absence of one kidney 1 in 2900 births in USA Commoner in infants of diabetic mothers Compensatory hypertrophy of contralateral kidney Other associated abnormalities: Mild to moderate sensory deafness on same side Uterine abnormalities in females

Unilateral Renal Agenesis

Symptomatic Incidental finding on ultrasound Care must be made to exclude ectopic kidney May confirm with DMSA scan

Primary renal hypoplasia

One single nephron: Glomerulus Proximal tubule Loop of henle Distal convoluted tubule Collecting duct

Primary Renal hypoplasia

At 37 completed weeks of gestation the nephron number is completed Range from 300 000 to 1000 000 nephrons per kidney Hypoplasia : insufficient nephron number If there is bilateral renal hypoplasia, Children will develop chronic renal failure

Renal Hypoplasia
In rats, defeciency of vit A in pregnant rats causes renal hypoplasia. ? True for human beings

Renal Dysplasia
Disturbances of nephrogenesis leads to renal dysplasia Dysplasia: abnormal renal tissues Dysplastic kidneys contain: Cysts Fibrosis Islands of cartilage

Hypoplasia/dysplastic kidneys
Risk of progressive renal failure Likelihood of developing renal failure is 20 70 %

Multicystic/dysplastic kidneys
Multiloculated abdominal massess with thin walled cysts Very disorganized renal architecture Very little normal looking tissues Incidence of unilateral multicystic kidneys; 1 in 4000 live births 25% have VUReflux on the other kidney

Multicystic/ Dysplastic kidneys (MCDK)

Unilateral MCDK may be associated with hypertension May undergo partial or complete involution 25% may involute completely by 2 years old If does not involute may transform into Wilms tumour or renal cell carcinoma

Abnormalities of kidney position and patterning

Horseshoe kidneys Incidence 1: 400-800 live births 30 % associated with Turners syndrome Slight increased risk of malignant change into Wilms tumour

Abnormalities in position
Ectopic kidneys Located in pelvis. Fail to ascend out of the pelvic cavity Rarely in thorax

Approach to the child with a Urinary Tract Abnormality

Family history of renal abnormalities Oligo hydramnios Abnormal genitalia Aniridia Ear anbormalities eg preauricular pits, ear tags, abnormal pinna Imperforated anus

MCU

Vesico- ureteric reflux

VUR Grade III

Vesico ureteric reflux

Backup of urine from the bladder into the ureters Flap valve mechanism at the ureterovesical junction that prevents reflux At risk for pyelonephritis

Vesico-ureteric reflux
Primary VUR is congenital
Secondary VUR is as a result of abnormal bladder dynamics eg Neuromuscular disease or abnormal voiding patterns

International Classification of Reflux

Grade 1
Grade II Grade 111 Grade IV Grade V

Fills the ureters only Fills the ureter and collecting system Mild blunting of calyces More than 50 % calyceal blunting Ureters are very dilated

Grades of Reflux Grades I,II : Mild Grades III,IV,V : Severe

Scarring

Small shrunken kidney Areas of scarring

VUR Grade IV

Vesico- ureteric reflux

A child usually presents with UTI Diagnosis is made : Prompt evaluation of UTI. MCU is usually done in children less than 2 years presenting as UTI Ultrasound has hydronephrosis. We will also do MCU Antenatal hydronephrosis

Pyelonephritis and renal scarring

Infection + VUR = pyelonephritis If inadequately treated or not treated,pyelonephritis leads to scarring Scarring: potential hypertension either during childhood or adulthood Renal failure End stage renal disease

Treatment
Prophylactic antibiotics until 5 years old Single dose before bedtime Avoid constipation Pass urine regularly and completely If all measures fail, for surgical intervention

Pelvic ureteric junction (PUJ)obstruction

An intrinsic or extrinsic narrowing at the pelvic-ureteric junction(PUJ) OR Blockage at the PUJ
Result in progressive dilatation of the pelvis and potential for nephron loss

Pelvic ureteric junction (PUJ)obstruction

Presentation:
In utero: Antenatal hydronephrosis At 5 to 15 years: UTI flank pain Abdominal mass Haemturia

Ultrasound of dilated kidney

Pelvic ureteric junction (PUJ)obstruction

Treatment Decision to do pyeloplasty: if there is moderate to gross hydronephrosis Kidney function >45 % of total GFR Symptomatic

Posterior Urethral Valves

Obstructing urethral folds within the lumen of the posterior urethra Only in male infants Common cause of bladder outlet obstruction Incidence 1 : 5000 to 8000 births

Normal male urethra

Bladder Neck External sphincter

Posterior urethral valves

MCU:

Posterior Urethral Valves

Presentation Acute illness in the neonatal period Ill,sepsis,dehydration and electrolyte imbalances( lose Na and HCO3) May have renal failure Poor urine stream

Posterior Urethral Valves

Treatment: By pass obstruction Catheterize patient with a 5 French feeding tube Arrange for vesiscostomy Fulgration of valves when baby is 1 2 years old or weight 10 kg

Posterior Urethral Valves Treatment

RP LFT Ca PHO4 Urine FEME Urine C&S IV Antibiotics IV Drip IV Na Hco3 Feeding: make sure there is weight gain Mother taught to recognize UTI Vesiscostomy care

Posterior Urethral Valves prognosis

Chronic renal failure leading to ESRD Frequent UTI Poor growth Incontinent Proteinuria hypertension

Neurogenic Bladder
Causes of dysfunctional voiding in children Myelodysplasia Lipomyelomeningocoele Cerebral palsy Traumatic injuries to the spine

Neurogenic Bladder
Causes of dysfunctional voiding in children Nonneurogenic neurogenic bladder dysfunction
Unstable bladder Infrequent voiding syndrome

Cerebral palsy

Myelomeningocoele

Antenatal hydronephrosis
Diagnosis of dilatation in the kidneys in utero AP diameter 7-8mm in the third trimester Postnatal ultrasound is mandatory after 48 hours.