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Diagnostic imaging in pediatric nephrology
IPNA-ESPN Master Glasgow September 2017
Melanie Hiorns
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The basic tools
• Plain film Xrays
• Ultrasound
• Micturating cystogram
• IVU
• Nuclear medicine – MAG3 and DMSA
• CT
• MRI
• Angiography
Melanie Hiorns, UK
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Plain X-ray films
• Probably only indicated in stone disease
• Ultrasound more sensitive but X-rays can be useful for ureteric stones
• Augmented bladders
• Unexpected calculi
• Associated skeletal abnormalities – VATER, bladder extrophy
Melanie Hiorns, UK
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Melanie Hiorns, UK
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Melanie Hiorns, UK
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Main applications of ultrasound:
• Key first line investigation in children
• Superb tool for looking at structure
• Useful screening tool to exclude pathologies
• Less good for functional assessment
• No cellular information
Melanie Hiorns, UK
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• Congenital abnormalities
• Kidney architecture/echogenicity/size
• Dilatation
• Calcification
• Blood flow
• Bladder
Melanie Hiorns, UK
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Congenital abnormalities
• How many kidneys?
• What shape are they?
• What size are they?
• Where are they?
• Are there associated abnormalities?
• Can I bring this together as a diagnosis?
Melanie Hiorns, UK
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Crossed fused ectopic kidney
Melanie Hiorns, UK
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Pelvic kidney
Melanie Hiorns, UK
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Posterior urethral valves
Melanie Hiorns, UK
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Kidney architecture/echogenicity/size
• Small /large /normal size?
• Echobright?
• Duplex?
3 Melanie Hiorns, UK
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Multicystic dysplastic kidney Cystic dysplastic kidney
Melanie Hiorns, UK
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ADPKD –new born
Melanie Hiorns, UK
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Duplex kidney
Melanie Hiorns, UK
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Dilatation
• Collecting system
• Ureters
• Obstruction?
3 Melanie Hiorns, UK
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Supine versus prone
Melanie Hiorns, UK
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Prone
Post op nephropexy
Melanie Hiorns, UK
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PUJ obstruction
Melanie Hiorns, UK
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PUJ obstruction
Melanie Hiorns, UK
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Renal transplant – ureteric stricture
Melanie Hiorns, UK
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Dilated ureters - stones
Melanie Hiorns, UK
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Calcification
• In the parenchyma?
• In the collecting system?
• In the ureter?
• In the bladder
• Anywhere else?
5 Melanie Hiorns, UK
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In the parenchyma
Nephrocalcinosis
Melanie Hiorns, UK
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Nephrocalcinosis AND calculi Hyperoxaluria and
stones in ureter
Melanie Hiorns, UK
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In the collecting system
Immmobility stones – and 4
months later
Melanie Hiorns, UK
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XPN with collection
Melanie Hiorns, UK
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In the ureter
With or without ureteric dilatation
Melanie Hiorns, UK
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In the bladder
Reconstructed bladder
Cysteinuria
Melanie Hiorns, UK
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Other places
Stoma stonesMelanie Hiorns, UK
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Melanie Hiorns, UK
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Blood flow
Melanie Hiorns, UK
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Pitfalls
• In tiny infants avoid interpreting the contralateral kidney and the ‘second’ kidney when only one kidney present – ‘look through’
• Misinterpreting a hydrocolpos for bladder
• Misinterpreting slightly echogenic kidneys as abnormal in the new born
• Misinterpreting gross hydronephrosis for bladder or cystic kidneys including MCDK
• Distinguish PUJ from duplex
Melanie Hiorns, UK
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‘Look through’ in neonates
Don’t misinterpret
as one kidney –
you could be
looking right
through
Melanie Hiorns, UK
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Hydrocolpos
Looking for the ureteric jets can help
Or fill the bladder by the catheter and see
which cavity increases in size
Check if the ‘bladder’ is directly anterior to the
sacral spine – it shouldn’t be!Melanie Hiorns, UK
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MCDK versus hydronephrosis
Melanie Hiorns, UK
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MCDK ….
… not MCDK - hydronephrosis
Melanie Hiorns, UK
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PUJ not duplex
Melanie Hiorns, UK
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Bilateral ureteroceles
Bilateral ureteroceles
and bilateral dilated
ureters. Make sure
dilated ureters are not
mistaken for
ureteroceles and vice
versa
Melanie Hiorns, UK
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Renal pelvis dilatation
• Usually diagnosed antenatally• General agreement that it should be followed up
postnatally• AP pelvis diameter measurement is crucial• If extra renal dilatation we state the ‘true’ renal
pelvis measurement and the ‘extra renal pelvis’ measurement
• We must comment on the dilation (or not) of the calyces, renal pelvis and the ureters, length of kidney, thickness of renal cortex, and echogenicity
Melanie Hiorns, UK
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PUJ obstruction
• Male, and left sided, predominance
• Most commonly at the exact point the renal pelvis joins the ureter
• Possibly an abnormal deposition of muscle and collagen resulting in an aperistaltic segment
• Degree of distension cannot be used as an indicator of the degree of obstruction or of residual renal function
Melanie Hiorns, UK
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Neonatal PJ obstruction
Melanie Hiorns, UK
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Bilateral PUJ obstruction
Melanie Hiorns, UK
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Duplex kidneys
• Duplex: unilateral/bilateral,complete/partial, associated ureterocele or not
• Ureterocele is a cystic dilatation of the submucosal portion of the upper moiety of the duplex (but can also occur in a simplex kidney) and is associated with an ectopic insertion
• Caecoureterocele is a variation where the ureterocele involves the upper part of the urethra –and can cause obstruction on voiding
Melanie Hiorns, UK
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6 week old – infected duplex
Melanie Hiorns, UK
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Specific pitfalls with duplexes
Bubble not ureterocele!
Melanie Hiorns, UK
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Missed duplex
and PUV!
Melanie Hiorns, UK
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Missed duplexes
Melanie Hiorns, UK
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Collapsing ureterocele
Melanie Hiorns, UK
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Ectopic ureters
• Often associated with a duplex and ectopic (and low) insertion of the upper moiety ureter
• In girls it can insert into the lower bladder, the urethra, vestibule or vagina
• If infrasphincteric this will mean the girl is always dribbling and never achieve continence
• Always above the sphincter in boys so don’t have this problem, but may present with epididymo-orchitis
• MRU very useful for examining ectopic ureters
Melanie Hiorns, UK
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Ectopic insertion in a duplex
Melanie Hiorns, UK
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Renal cystic disease in neonates
• MCDK – must distinguish from hydronephrosis
• Cystic dysplasia
• Autosomal recessive polycystic kidney disease –PKHD1 gene mutation or deletion – affects cilia of the biliary ducts and collecting tubules of the kidneys – fusiform dilatation and ectasia of the collecting tubules – symmetrically enlarged echogenic kidneys with random small cysts
• Cystic diseases associated with syndromes
Melanie Hiorns, UK
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MCDK
Melanie Hiorns, UK
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Autosomal recessive polycystic kidney disease - ARPKD
Melanie Hiorns, UK
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NICE guidelines for UTI
Melanie Hiorns, UK
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Contraindications and antibiotics
Antibiotics are to be given for 3 days, starting one day before procedure.
Trimethoprim (oral dose)< 1 month 2mg / kg bd> 1 month 4mg / kg bdIn renal failure 15-30 mL/min/1.73 m²Normal dose for 3 days only
<15 mL/min/1.73 m² Use half above doses
• If child is clinically suspected or proven to have a current UTI• Essential if have prosthetic valve, valvular lesion, septal defect or PDA
that get antibiotics in advance• If allergic to contrast medium
Melanie Hiorns, UK
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Dose reduction
•Low frequency pulsed fluoroscopy – 3p/s
•Low magnification
•Image grab
•No grid
Melanie Hiorns, UK
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Melanie Hiorns, UK
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Posterior urethral valves
• Congenital folds of Wolffian duct origin located at the junction of the posterior and the penile urethra, and obstruct the male urethra
• Present with poor urinary stream, sepsis, shock, hyperkalaemia, maternal oligohydramnios or antenatal bilateral hydronephrosis
• Any male infant with bilateral hydronephrosis must have PUV excluded
Melanie Hiorns, UK
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Ultrasound findings in PUV
• Thick walled bladder
• Bilateral hydronephrosis
• Urinary ascites
• Perirenal urinomas
• Renal dysplasia
• Renal cysts
Melanie Hiorns, UK
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‘classic’ PUV
Melanie Hiorns, UK
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Missed valve picked up at age 7
Presented at birth with bilateral hydro, MCUG
reported as normal, re-presented age 7 with left
pyelonephritis and obstruction. Melanie Hiorns, UK
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Primary grade 5 reflux not valves
Melanie Hiorns, UK
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IVU
• Effectively obsolete
• Very occasionally useful in the specialist pre lithotripsy/PCNL planning
• Superseded by MR
Melanie Hiorns, UK
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Nuclear Medicine
• MAG3
• DMSA
Melanie Hiorns, UK
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Mid polar ‘cyst’
Melanie Hiorns, UK
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Use of CT
• Ultrasound remains the first choice investigation for almost every clinical scenario
• CT can be used for problem solving (after the US)
• CT can be used when you don't have access to MRI
3 Melanie Hiorns, UK
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CT
• Trauma if questions not completely answered by ultrasound
• Stone disease in difficult patients
• Occasionally for vascular anatomy – complex horseshoe kidney, complicated tumour vascular anatomy
Melanie Hiorns, UK
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CT and problem solving
• What is the clinical dilemma?
• Spatial resolution versus contrast resolution?
• Contrast or no contrast?
• Slice thickness
• How many series?
5 Melanie Hiorns, UK
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CT 4 hours post angioplasty
Melanie Hiorns, UK
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Chyluria
Melanie Hiorns, UK
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CT and calcification
• No doubt CT is very sensitive and specific for calcification
• How sensitive do you have to be?
• If the ultrasound has not shown obstruction is there an argument for ‘sitting it out’?
• Is it OK to use the radiation does in a curable disease that may need serial imaging?
4 Melanie Hiorns, UK
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Tricky stones
Melanie Hiorns, UK
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Nephrocalcinosis v. calculi
Melanie Hiorns, UK
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Melanie Hiorns, UK
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Infundibular stenosis v. cyst?
Melanie Hiorns, UK
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Abscess with extension
Melanie Hiorns, UK
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CT in complex vascular anatomy – functioning tissue across the midline
Melanie Hiorns, UK
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Surgical planning
Horseshoe kidney – 5 arteries
Melanie Hiorns, UK
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Bilateral Wilms
Melanie Hiorns, UK
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Tumour thrombus in Wilms
Melanie Hiorns, UK
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Nephroblastomatosis
Melanie Hiorns, UK
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Summary for CT
• Ultrasound remains the first choice investigation for almost every clinical scenario
• CT can be used for problem solving
• CT can be used when you don't have access to MRI
3 Melanie Hiorns, UK
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MR
• Tumours
• Problem solving
Melanie Hiorns, UK
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MRI Tumour protocolBuscopanSTIRs, axial coronal, sagittalNATIVE arterial axial (bilateral tumours only)T2-spaceADCT1 WE SE axial3D VIBECare bolus, coronal3D VIBE axialT1 WE SE
Pre transplant angiographyNATIVE arterial (axial oblique, and then MIPs)NATIVE venous (axial oblique, and then MIPs)
Melanie Hiorns, UK
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Nephroblastomatosis (STIR, ADC, fusion)
Melanie Hiorns, UK
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Wilms pre and post chemo – the value of ADC monitoring
Melanie Hiorns, UK
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T2 MR sequence showing the large Wilms’ tumor in the left kidney and nodules of nephroblastomatosis in the periphery of the right kidney. Coronal MR (BFFE sequence) showing the difference in signal between the Wilms’ tumor in the lower pole of the right kidney and the nodule of nephroblastomatosis on the lateral margin of the right kidney.
Melanie Hiorns, UK
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‘Crossing vessels’ causing PUJ obstruction
13 year old with intermittent right loin pain
14 year old with left sided loin pain
Melanie Hiorns, UK
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ARPKD
Melanie Hiorns, UK
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MRU
True FISP, coronal, sagittal, axialBuscopan3d T2 TSE sagittal3D VIBEFrusemideGadolinium3D VIBE – arterial and then repeat every 5 to 10 minutesT2 space
Melanie Hiorns, UK
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MRI: an unexpected right sided duplex kidney in a 7 month old girl whose anatomy couldnot be delineated by ultrasound, with a tiny lower moiety which is almost hidden by thedilated upper moiety (arrow)
MRI (T2 weighted sequence) demonstrating the extent of bilateral pelviureteric junctionobstruction and on-dilation o of the ureters confirming the obstruction to be at the PUJ
Melanie Hiorns, UK
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Angiography
• Renovascular disease
• Unexplained hypertension
• As part of more complex procedures
Melanie Hiorns, UK
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7 year old with neurofibromatosis type 1 and middle aortic syndrome
Melanie Hiorns, UK
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9 year old girl with left renal artery stenosis
Melanie Hiorns, UK
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Right renal artery stenosis
4% to 41%
Melanie Hiorns, UK
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AV fistula from biopsy in a transplant kidney
Melanie Hiorns, UK
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Rotational angio renal tranplant - stenosisMelanie Hiorns, UK
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Thank you