Congenital Anomalies of the Kidney & Urinary Tract (CAKUT) Flashcards
Introduction to CAKUT
•These are the commonest causes of ________ in children.
•Presumably more so in developing countries because of _____________.
ESRD
late diagnosis.
Types of CAKUT
Malformation of the renal parenchyma
• Renal __________
• Renal ___________/________
• __________ __________ kidney
• __________ kidney disease
Renal agenesis
• Renal hypoplasia/dysplasia
• Multicystic dysplastic kidney
• Polycystic kidney disease
Types of CAKUT
Abnormalities of migration of embryonic kidney
• ________ kidney
• ________ kidney
• ________ ________ ectopia
Pelvic kidney
• Horse shoe kidney
• Crossed fused ectopia
Types of CAKUT
Abnormalities of the developing urinary collecting system
• __________________ obstruction
• __________________ obstruction
• __________________
• __________________ collecting system
• Pelvi-ureteric junction obstruction
• Vesico-ureteric junction obstruction
• Posterior urethral valve
• Duplicating collecting system
Posterior Urethral Valve
• Obstructing _________________ in the ______ of the ____________
• Occurs in 1 in 3000 live births
membranous folds ; lumen
posterior urethra.
Commonest cause of CKD in male children is????
Posterior urethral valve
Pathophysiology of PUV
• _____________ to urine outflow leads to __________ of the __________, __________ and __________
• With time the bladder wall becomes __________ & __________ and __________ occurs.
• There may be a secondary ___________________ (unilateral or bilateral)
Obstruction ; outflow dilatation
posterior urethra, bladder and upper tract
thickened ; trabeculated ; diverticulitis.
vesico-ureteric reflux
Pathophysiology of PUV (2)
The kidneys may be damaged by _________________ , recurrent _______
or concomitant renal _______/__________
• The bladder is usually an ————- , _______ volume, ______ compliance, _______
pressure bladder
high bladder pressure
UTI ; dysplasia/hypoplasia
overactive ; low ; low ; high
Clinical Manifestations of PUV
Prenatally:
•Most cases are picked up _________ in developed countries using ______
•Features include _______hydramnois, bilateral _________, _________ bladder
wall, _________ bladder and posterior urethra. Dilated calyces may rupture leading to urinary ascites.
antenatally ; USS
oligohydramnois ; hydronephrosis
thickened ; dilated
Clinical manifestations of PUV
Post Natal:
•Neonate: Respiratory distress, __________ __________, _________ urinary stream
•Infant: failure to thrive, __________, poor urinary stream, __________ or __________ when passing urine
•Older boys: __________ urinary stream, daytime __________, _______ & if not resolved ___________
abdominal distension; poor
urosepsis ; straining ; grunting
poor ; incontinence ; UTI
ESKD
Diagnosis of PUV
•_______ scan: Unilateral or bilateral _______________ ; _______________ bladder wall, __________ posterior urethral; significant postvoid bladder urine volume
•_______________ _______________ (diagnostic): Dilated posterior urethra with sudden transition to normal anterior urethra. The supportive features include trabeculated bladder; a unilateral or bilateral VUR
•_______________: visualizes the membranes and also for ______
•_________ scan: detects the presence of dysplasia/hypoplasia
•Urodynamic studies: to define bladder filling and emptying characteristics
•E/U/Cr
•Urinalysis/urine M/C/S
KUB ; hydroureteronephrosis; thickened
dilated ; Cystoscopy ; ablation
DMSA ; Urodynamic studies
Differential Diagnosis of PUV
________ agenesis/ ________/ ________ of the urethra; neuropathic bladder; bladder ________
Urethral agenesis
stricture
stenosis of the urethra
bladder stones
Management of PUV
•___________ the bladder by passing a ___________
•Pay close attention to fluid & electrolyte balance after decompressing the bladder
•May require ___________ ___________
•___________ the valves as soon as possible
•Long term follow up of bladder & kidney function
•Up to 30% may require kidney ___________
Decompress ; urethral catheter
antibiotics prophylaxis ; Ablate
kidney transplantation
Pelvi-ureteric junction Obstruction
•Occurs in 1 in 2000 births
•Congenital ___________ , usually a malformation at the ___________ of the ___________ and (proximal or distal?) aspect of the ___________.
•Occurs more commonly on the (left or right?) (2:1) and in (males or females?) (2:1)
•May be bilateral in 10-40 % of cases
narrowing ; pelvis
proximal ; ureter.
left ; males
Commonest CAKUT is ????
approximately 20% of all CAKUT
Pelvi-ureteric junction obstruction
Clinical presentations of PUJO
•Picked up incidentally as __________ prenatally. Commonly picked up this way in developed countries
• Postnatal Period: (gradual or sudden?) flank pain, nausea, vomiting and __________
•_________ is unusual and should warrant investigation for __________
•Renal impairment only occurs when it is bilateral or when present in a solitary kidney
•However, there is a great probability of spontaneous resolution
hydronephrosis prenatally.
sudden ; dehydration
UTI ; vesico-ureteric reflux
Diagnosis & Management of PUJO
•_________ scan: varying degree of hydronephrosis, dilated pelvis with normal ureter
•_____________ scan: delayed excretory phase despite administration of lasix •Intravenous _________ : similar to MAG-3 scan, provides both functional & anatomical imaging of the kidneys. However it involves considerable irradiation.
KUB
MAG- 3 diuretic scan
Intravenous Urography
Treatment of PUJO
•Most cases are followed up by _____________ and with resolution nothing is done
•Severe cases requires ___________ intervention in the form of ______________
serial imaging
surgical
dismembered pyeloplasty
