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Pediatric surgery > RENAL MALFORMATIONS > Flashcards

RENAL MALFORMATIONS Flashcards

1
Q

RENAL MALFORMATIONS

Embryology

A 
3 phases of development
1.Pronehros
✓ D22
✓ Nephrotoms and pronephric
duct

2.Mesonephros
✓ Mesonephric duct cloaca

3.Metanephros

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2
Q

Renal agenesis

A 
✓ The kidney does not develop
↑
• Abnormal formation of the mesonephric
duct
• No ureteric bud
• Abnormal metanephric blastema formation
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3
Q

Renal dysgenesia

A

✓ Abnormal kidney development (size, structure)

✓ Pathological entities:

  • Hypoplasia – small number of nephrons
  • Dysplasia - primitive duct persistency
  • Cystic dysplasia – multicystic dysplasic kidney (cystic kidney disease)
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4
Q

Cystic kidney disease

A

❑ Genetic
✓ Polycystic renal disease (autosomal dominant/ recessive)
✓ Congenital nephrosis
✓ Different syndroms (ex. Von Hippel Lindau, tuberous sclerosis)

❑ Non-genetic
✓ Multicystic kydney
✓ Acquired cystic kidney disease
✓ Pyelogenic cyst/ Caliceal diverticulae

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5
Q

Kidney position anomalies

A

❑ Renal ectopy
▪ Lower position of the kidney
Normally: pelvis lombar area

❑ Horseshoe kidney
❑ Sigmoid kidney
+/- simptoms (litiasis, UTI, VUR, HN)

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6
Q

MALFORMATIONS OF THE URINARY TRACT

PRENATAL DIAGNOSIS

A 
n = 3800/10 years
Ureteropelvic junction (UPJ) obstruction - 50%
Vesico-ureteral reflux - 15%
Multicystic kidney - 10%
Duplications /Ureterocele - 10%
Megaureter - 9%
Posterior urethral valve (PUV) - 4%
Other malformations - 2%
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7
Q

UPJ (ureteropelvic junction) obstruction and congenital HN

A

✓ intrinsic +/- extrinsic (abnormal blood vessels, adhesions) UPJ obstruction
↓
✓ Reduction of the urinary flow
✓ Pelvic and caliceal dilatation
✓ Less renal functional parenchyma ➔ RI (potential renal insufficiency)

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8
Q

ANTENATAL DIAGNOSIS

ULTRASOUND

A

Weeks: 12, 20, 32-34

  • evaluate the volume of the amniotic fluid
  • kidney position, renal parenchyma evaluation
  • +/- type of dilatation/ degree
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9
Q

Malformations of the UT
ANTENATAL DIAGNOSIS
- FETAL MRI
- BIOCHEMISTRY

A
  • fetal urinanalysis
  • amniotic fluid- for associated anomalies
  • fetal blood tests (if needed)
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10
Q

ANTENATAL DIAGNOSIS - Hydronephrosis
The Society for Fetal Urology
Diagnosis criteria

A

Grade,Central Renalcomplex,RenalParenchymalThickness
0 Intact Normal
1 Urine in pelvis barely splits sinus, Normal
2 Evident splitting of pelvis & major calyces,Normal
3 Wide splitting of pelvis,major&minor calyces,Normal
4 Further splitting of pelvis,major & minor calyces,Reduced

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11
Q

Malformations of the UT

PRENATAL ULTRASOUND

A

IDENTIFYING
AT-RISK PATIENTS
! Correlate with post-partum evaluation

First trimester ultrasound @ 20 weeks
< 5 mm pelvic AP diameter - normal
> 5 mm pelvic AP diameter - abnormal
? Ureteral dilatation
? Any abnormalities of the bladder

• Second trimester ultrasound @ 32-34 weeks
< 10 mm pelvic AP diameter - normal
> 10 mm pelvic AP diameter - abnormal

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12
Q

Malformations of the UT

Pathophysiological mechanism

A 
Renal obstruction
↓
Oligohydramnios
↓
Pulmonary hypoplasia
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13
Q

Malformations of the UT

Disease history

A

 +/- antenatal diagnosis of a pelvic dilatation
 ? Any UTI episodes so fac
 specific data regarding birth and perinatal period

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14
Q

Malformations of the UT

Clinical examination

A

 can be normal
 evaluate hydration status, examine external genital organ, identify potential malformations
 abdominal examination- any lombar mass?
 UTI: lower (cystitis) or renal (pielonephritis) +/- febrile
 intermitent lumbar pains

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15
Q

Malformations of UT

Lab investigations

A

 TBC
 clotting tests
 urea, creatinine
 urinanalysis, culture

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16
Q

First postnatal ultrasound - when to do it?

A

 Normally after 48 ore
 BUT earlier if:
✓ bilateral anomalies identified antepartum
✓ only one kidney
✓ oligohydramnios
✓ unilateral severe anomalies identified antepartum
 Ultrasound and creatinine level normal => next ultrasound at 1 month of age
 Important criteria: particularities of the patient, medical team experience
 Ultrasound before any surgery

17
Q

Postnatal ultrasound - what do we evaluate ?

A 
 AP pelvic diameter
 potential caliceal dilatations
 renal parenchyma index
 potential ureteral dilatations 
 potential bladder anomalies- examination pre- and 
post evacuation
18
Q

Prognostic factors - morphology

A 
✓Renal parenchyma - index
- ecogenicity
✓ Caliceal dilatations - central
- peripheric
✓ AP pelvic diameter
✓ Extrarenal pelvic surface
✓ Correlation of pre/postnatal examinations!!!
19
Q

Postnatal ultrasound

A

< 15 mm Normal
15-20 mm Moderate dilatation
> 20 mm Severe dilatation

20
Q

The risk of needing a surgical interventions

correlation with the AP diameter of the pelvis

A 
< 20 mm    11 %
20-30 mm 40 %
30-40 mm 90 %
40-50 mm 100 %
> 50 mm    100 %
21
Q

Caliceal aspect

A

0-5 mm Normal
6-10 mm Moderate dilatation
> 10 mm Severe dilatation

22
Q

Hydronephrosis index

A

Defined in 2008 (Shapiro)
◦ HI = % 100 x (Total area of the kidney - area
of dilated pelvis and calices)/(Total area)
◦ Quantified method to provide a reproducible
measure of HN that can be used for all conditions
◦ Higher sensitivity for third and fourth degree anomalies
◦ Great tool for monitoring evolution
◦ Standardized and reprocible

23
Q

Prognostic factors - function

A

Renal scintigraphy
 Useful for evaluating glomerular filtration and renal excretion
 BUT it can also identify morphological anomalies unidentified by the ultrasound
➢ Normal values for the newborn 45-55%
➢ Normally variations< 4% between subsequent evaluations
 OPTIONS: Dynamic scintigraphy DMSA; DTPA; MAG3 + furosemide

24
Q

Renal scintigraphy

A

✓Glomerular filtration (Technetium 99m- DTPA)
✓Tubular excretion (Technetium 99m- MAG3)
✓General principle – the radionuclide molecule will attach to the renal proteins (i.e.
Technetium 99m- DMSA)
✓ Results :
< 15% Severe renal dysfunction
15-40% Moderate renal dysfunction
> 40% Normal renal function

25
Q

1.Significant morphological
abnormalities →
2.Major morphological abnormalities →

A
  1. Zero function

2. Pseudonormal function…

26
Q

The role of urography + MRI

A

Anatomical and functional evaluation

100% sensitivity and specificity for the diagnosis of polar vessel obstruction

27
Q

Therapeutic options

PRENATALLY -How we decide what needs to be done?

A

Second trimester ultrasound @ 32-34 weeks
< 10 mm pelvic AP diameter
No caliceal dilatation
Normal echogenicity of the parenchyma
No associated abnormalities of the lower urinary
tract
NO antibiotic prophylaxia, micturating cystourethrogram (MCUG) or scintigraphy
YES ultrasound at 3 months ans 12 months of age

Second trimester ultrasound @ 32-34 weeks
> 10 mm pelvic AP diameter
? PUJ obstruction
- Caliceal dilatations
- Normal ureters
- Normal urinary bladder
Ultrasound+ MAG3 Renal scintigraphy
4-6 weeks post-partum
  • Bilateral HN
  • Ureteral dilatation
  • Abnormal bladder
    (>3mm bladder wall, abnormal evacuation)

Antibiotic prohylaxia
Additional investigations

28
Q

slide 40,41,42,50

A

print

29
Q

PUJ Obstruction - spotaneous evolution

A

35 % SURGERY
35 % SPONTANEOUS
FAVOURABLE EVOLUTION (1 - 3 years)
30 % SAME ASPECT IN TIME

30
Q

Indications for surgical treatment

A

Renal function < 40% or a decrese in function of
more than 10% from one examination to another
 Pelvic AP Ø > 30 mm

31
Q

Pieloplasty - surgical approaches

A

✓ Laparotomy
✓ Laparoscopy
✓RALP- Robotic Assisted Laparoscopic Pyeloplasty

32
Q

Open surgery

Laparoscopic surgery

A

Hynes Anderson procedure

33
Q

HN abnormal blood vessel - Vascular Hitch

A
  1. Ureter above the constricted area
  2. Abnormal blood vessel
  3. Ureter below the constricted area
34
Q

Fetal surgery

Prenatal retrieval of obstruction +/-?

A 
 Subvesical obstacles
 Oligohydroamnios
 Normal cariotype
 Complication rate 45%
 Still ongoing debate regarding the benefits of such intervention on  the renal function!
35
Q

Potential post-operative complications

A

 Problems with the sutures
 Urinoma formation
 Later stenosis

36
Q

IMMEDIATE POSTOP MONITORING

A 
 temperature
 diuresis
 +/- drainage (if one inserted in the lumbar area at the end 
of the procedure)
 presence of bowel movements
37
Q

Criteria for discharge

A

 normal temperature
 normal diuresis
 good aspect on the ultrasound (smaller dilatation)
 bowel movements present
 no drainage
 good healing of wounds
 ANTIBIOTIC PROPHYLAXIS if internal urinary drainage

38
Q

Later monitoring

A 
 at 4-6 weeks after the surgery
- urinalysis, culture
- ultrasound
- removal of internal urinary drainage
 Further evaluations at 3, 6, 12 months after the surgery

Pediatric surgery (11 decks)

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