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Congenital (UPJ) Ureteropelvic
Junction Obstruction

Mohammed Nabil J AlAli

5th Year Medical Student
At
Powerpoint Templates King Faisal University
Page
Group B (210006209) 1
Outlines:
-OVERVIEW
-PATHOPHYSIOLOGY
- ETIOLOGY
- CLINICAL PRESENTATION
- DIAGNOSIS
- DIFFERENTIAL DIAGNOSIS
- FOLLOW-UP
- MANAGEMENT
Powerpoint Templates
Page 2
OVERVIEW
• It is a partial or total blockage of
the flow of urine that occurs
where the ureter enters the
kidney.
• It is the most common pathologic
cause of antenatally detected
hydronephrosis
Powerpoint Templates

Page 3
EPIDEMIOLOGY
• Most common site of urinary tract
obstruction in children
• Majority are discovered antenatally
– 1:1500 secrend by ultrasound
– It is the most common anatomical cause of
antenatal hydronephrosis
– Boys > girls
– Most cases on the left
– 10-40% bilateral
Powerpoint Templates

Page 4
PATHOPHYSIOLOGY
• It is caused by anatomic lesions or
functional disturbances that restrict
urinary flow, resulting in hydronephrosis.
• Most cases are thought to be due to
partial obstruction, because complete
obstruction results in rapid destruction of
the kidney.
•In some cases, partial obstruction may
also lead to progressive deterioration of
renal function. Templates
Powerpoint
Page 5
Development of the
equilibrium state resulting
in stable renal function
depends on:
• Urinary rate and output
• Anatomy and degree of UPJ
obstruction
• Compliance of the renal pelvis
Powerpoint Templates

Page 6
ETIOLOGY
• It is both congenital and acquired
conditions.
• Usually caused by intrinsic stenosis
of the proximal ureter, and less
commonly by extrinsic compression
of the UPJ.
Powerpoint Templates

Page 7
Intrinsic narrowing
• In most cases of UPJ obstruction, the
upper segment of the ureter is narrowed
or kinked, resulting in obstruction of
urinary flow.
• Although the underlying mechanism is
not proven, it is thought that there is an
embryologic disruption of the proximal
ureter that alters circular musculature
development and/or collagen fibers, and
composition between and around the
Powerpoint Templates
muscular cells
Page 8
Extrinsic narrowing
In about 10 % of pediatric UPJ
obstruction, an aberrant or accessory
renal artery or arterial branch may
cross the lower pole of the kidney,
resulting in compression of the UPJ
and blockage of urinary flow

Powerpoint Templates

Page 9
CLINICAL PRESENTATION
• Historically presented as a
palpable mass
– Newborn
• Antenatal hydronephrosis 80%
• UTI, hematuria, failure to thrive, feeding
difficulties, sepsis, azotemia

– Later in life
• 30% diagnosed after UTI
• 25% diagnosed after hematuria
• Episodic abdominal pain and vomiting
due to intermittent obstruction
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Page 10
Associated Anomalies
• Another urologic abnormality-50%
– Contralateral UPJ 10-40%
– Renal dysplasia, aplasia, MCKD
– VUR up to 40%

• Found in 21% of VATER patients

Powerpoint Templates

Page 11
DIAGNOSIS
• It is generally suspected when
imaging studies, usually
ultrasonography, demonstrate
hydronephrosis.
• The diagnosis is confirmed by
diuretic renography.

Powerpoint Templates

Page 12
Ultrasonography (US)
Most cases of UPJ obstruction present as
a result of detecting hydronephrosis by
prenatal ultrasonographic screening

Abnormal
calyces

Normal
kidney
Powerpoint Templates

Page 13
Diuretic renography
• It (renal scan and the administration of a
diuretic) is used to diagnose urinary tract
obstruction.
• It measures the drainage time from the
renal pelvis (referred to as washout) and
assesses total and each individual kidney's
renal function.
•The washout measurement correlates
with the degree of obstruction.
In general, a half-life greater than 20
minutes to clear the isotope from the
kidney is considered indicative of
Powerpoint Templates
Page 14
obstruction.
Computed tomographic scan (CT)
- It is an alternative to ultrasonography in the
symptomatic child.
-It is not the preferred modality due to its radiation
exposure.
- In UPJ obstruction, the CT scan typically shows
hydronephrosis without a dilated ureter.

Powerpoint Templates

Page 15
Magnetic resonance imaging (MRI)
- It can be used to diagnose UPJ type
hydronephrosis.
-The advantage of MRI is the ability to
discern accurate anatomy defining the point
of obstruction.
-Also determine the split function of the
kidney and simulate the diuretic renogram
by providing washout data.
-The disadvantage of MRI is the cost and
the need for general
anesthesia and/or sedation
Powerpoint Templates

Page 16
Voiding cystourethrogram (VCUG)
-It is performed in patients with hydronephrosis
to confirm the presence or absence of VUR of
both the affected and contralateral kidneys.
-Ten percent of patients with UPJ obstruction
have contralateral low-grade vesicoureteral
reflux.
-Identification of VUR is important because
children with concurrent VUR and UPJ
obstruction may be at higher risk for severe
infection. Powerpoint Templates

Page 17
DIFFERENTIAL DIAGNOSIS
• It includes other causes of
hydronephrosis.
• Imaging studies differentiate UPJ
obstruction from the following conditions:
- Vesicoureteral reflux (VUR)
- Transient hydronephrosis
- Functional hydronephrosis
- Other urological anomalies including
posterior urethral valves, congenital
megaureter, ureterocele, and multicystic
dysplastic kidney
Powerpoint Templates

Page 18
FOLLOW-UP
• U/S on day 2 - 3 of life Persistent
hydronephrosis .
• VCUG to evaluate PUV or VUR
• Prophylactic antibiotics if VUR present
• No PUV or VUR - repeat U/S and diuretic renal
scan at 1 month
• Continued hydro - surgery vs. observation
• observation - U/S and/or renal scan every 3-4
months for 1 year and then every 4-6 months
• surgery - open/endopyelotomy/laparoscopy

Powerpoint Templates

Page 19
MANAGEMENT

Powerpoint Templates

Page 20
Conservative
• Principles:
– 50% of antenatal hydro resolved postpartum
– unable to accurately diagnose true
obstruction
– observations that asymptomatic
hydronephrosis can resolve spontaneously

• Studies with infants with renal
function >35-40% in the affected
kidney and variable washout
patterns
– “Rule of 1/3” - 1/3 stay the same, 1/3
improve, 1/3 worsen
Powerpoint Templates

Page 21
Indications
for Surgical Intervention
• Presence of symptoms associated
with the obstruction
• Impairment of overall renal function
• Progressive impairment of ipsilateral
function
• Development of stones or infection
• Hypertension
Powerpoint Templates

Page 22
Surgical
• Open Pyeloplasty
– Gold Standard
– Dismembered pyeloplasty is the most
common

Powerpoint Templates

Page 23
• Foley V-Y-Plasty
– Good for 1-2 cm obstruction
– Best for high inserting ureter
– Best with relatively small pelvis

Powerpoint Templates

Page 24
• Spiral flap
– Good for long obstructions (better in
adults)
– Length of flap limited only by size of
pelvis
• (keep length: width at 3:1)
• good when UPJ angle > 90

Powerpoint Templates

Page 25
• Endopyelotomy
– Antegrade or retrograde
– Cold knife or electric current
– Acucise is very popular
• dilation balloon with hot wire

– 86% success in adults
– Slightly less effective in children
– Direct vision antegrade approach is
most common
Powerpoint Templates

Page 26
• Laparoscopic pyeloplasty
– Same indications as open or
endourologic procedures
– Dismembered pyeloplasty is most
common procedure performed
• Without crossing vessels, may do any
number of flap procedures
• Up to 94% success rate, similar to open
pyeloplasty

Powerpoint Templates

Page 27
Any Question ?
Powerpoint Templates

Page 28
REFERENCES
- UpToDate

(press on the title )

- Department of Urology Section of Pediatric Urology
(University of Oklahoma ) (press on the title )

Powerpoint Templates

Page 29
Thank you

Powerpoint Templates

Page 30

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Congenital ureteropelvic (upj) obstruction

  • 1. Congenital (UPJ) Ureteropelvic Junction Obstruction Mohammed Nabil J AlAli 5th Year Medical Student At Powerpoint Templates King Faisal University Page Group B (210006209) 1
  • 2. Outlines: -OVERVIEW -PATHOPHYSIOLOGY - ETIOLOGY - CLINICAL PRESENTATION - DIAGNOSIS - DIFFERENTIAL DIAGNOSIS - FOLLOW-UP - MANAGEMENT Powerpoint Templates Page 2
  • 3. OVERVIEW • It is a partial or total blockage of the flow of urine that occurs where the ureter enters the kidney. • It is the most common pathologic cause of antenatally detected hydronephrosis Powerpoint Templates Page 3
  • 4. EPIDEMIOLOGY • Most common site of urinary tract obstruction in children • Majority are discovered antenatally – 1:1500 secrend by ultrasound – It is the most common anatomical cause of antenatal hydronephrosis – Boys > girls – Most cases on the left – 10-40% bilateral Powerpoint Templates Page 4
  • 5. PATHOPHYSIOLOGY • It is caused by anatomic lesions or functional disturbances that restrict urinary flow, resulting in hydronephrosis. • Most cases are thought to be due to partial obstruction, because complete obstruction results in rapid destruction of the kidney. •In some cases, partial obstruction may also lead to progressive deterioration of renal function. Templates Powerpoint Page 5
  • 6. Development of the equilibrium state resulting in stable renal function depends on: • Urinary rate and output • Anatomy and degree of UPJ obstruction • Compliance of the renal pelvis Powerpoint Templates Page 6
  • 7. ETIOLOGY • It is both congenital and acquired conditions. • Usually caused by intrinsic stenosis of the proximal ureter, and less commonly by extrinsic compression of the UPJ. Powerpoint Templates Page 7
  • 8. Intrinsic narrowing • In most cases of UPJ obstruction, the upper segment of the ureter is narrowed or kinked, resulting in obstruction of urinary flow. • Although the underlying mechanism is not proven, it is thought that there is an embryologic disruption of the proximal ureter that alters circular musculature development and/or collagen fibers, and composition between and around the Powerpoint Templates muscular cells Page 8
  • 9. Extrinsic narrowing In about 10 % of pediatric UPJ obstruction, an aberrant or accessory renal artery or arterial branch may cross the lower pole of the kidney, resulting in compression of the UPJ and blockage of urinary flow Powerpoint Templates Page 9
  • 10. CLINICAL PRESENTATION • Historically presented as a palpable mass – Newborn • Antenatal hydronephrosis 80% • UTI, hematuria, failure to thrive, feeding difficulties, sepsis, azotemia – Later in life • 30% diagnosed after UTI • 25% diagnosed after hematuria • Episodic abdominal pain and vomiting due to intermittent obstruction Powerpoint Templates Page 10
  • 11. Associated Anomalies • Another urologic abnormality-50% – Contralateral UPJ 10-40% – Renal dysplasia, aplasia, MCKD – VUR up to 40% • Found in 21% of VATER patients Powerpoint Templates Page 11
  • 12. DIAGNOSIS • It is generally suspected when imaging studies, usually ultrasonography, demonstrate hydronephrosis. • The diagnosis is confirmed by diuretic renography. Powerpoint Templates Page 12
  • 13. Ultrasonography (US) Most cases of UPJ obstruction present as a result of detecting hydronephrosis by prenatal ultrasonographic screening Abnormal calyces Normal kidney Powerpoint Templates Page 13
  • 14. Diuretic renography • It (renal scan and the administration of a diuretic) is used to diagnose urinary tract obstruction. • It measures the drainage time from the renal pelvis (referred to as washout) and assesses total and each individual kidney's renal function. •The washout measurement correlates with the degree of obstruction. In general, a half-life greater than 20 minutes to clear the isotope from the kidney is considered indicative of Powerpoint Templates Page 14 obstruction.
  • 15. Computed tomographic scan (CT) - It is an alternative to ultrasonography in the symptomatic child. -It is not the preferred modality due to its radiation exposure. - In UPJ obstruction, the CT scan typically shows hydronephrosis without a dilated ureter. Powerpoint Templates Page 15
  • 16. Magnetic resonance imaging (MRI) - It can be used to diagnose UPJ type hydronephrosis. -The advantage of MRI is the ability to discern accurate anatomy defining the point of obstruction. -Also determine the split function of the kidney and simulate the diuretic renogram by providing washout data. -The disadvantage of MRI is the cost and the need for general anesthesia and/or sedation Powerpoint Templates Page 16
  • 17. Voiding cystourethrogram (VCUG) -It is performed in patients with hydronephrosis to confirm the presence or absence of VUR of both the affected and contralateral kidneys. -Ten percent of patients with UPJ obstruction have contralateral low-grade vesicoureteral reflux. -Identification of VUR is important because children with concurrent VUR and UPJ obstruction may be at higher risk for severe infection. Powerpoint Templates Page 17
  • 18. DIFFERENTIAL DIAGNOSIS • It includes other causes of hydronephrosis. • Imaging studies differentiate UPJ obstruction from the following conditions: - Vesicoureteral reflux (VUR) - Transient hydronephrosis - Functional hydronephrosis - Other urological anomalies including posterior urethral valves, congenital megaureter, ureterocele, and multicystic dysplastic kidney Powerpoint Templates Page 18
  • 19. FOLLOW-UP • U/S on day 2 - 3 of life Persistent hydronephrosis . • VCUG to evaluate PUV or VUR • Prophylactic antibiotics if VUR present • No PUV or VUR - repeat U/S and diuretic renal scan at 1 month • Continued hydro - surgery vs. observation • observation - U/S and/or renal scan every 3-4 months for 1 year and then every 4-6 months • surgery - open/endopyelotomy/laparoscopy Powerpoint Templates Page 19
  • 21. Conservative • Principles: – 50% of antenatal hydro resolved postpartum – unable to accurately diagnose true obstruction – observations that asymptomatic hydronephrosis can resolve spontaneously • Studies with infants with renal function >35-40% in the affected kidney and variable washout patterns – “Rule of 1/3” - 1/3 stay the same, 1/3 improve, 1/3 worsen Powerpoint Templates Page 21
  • 22. Indications for Surgical Intervention • Presence of symptoms associated with the obstruction • Impairment of overall renal function • Progressive impairment of ipsilateral function • Development of stones or infection • Hypertension Powerpoint Templates Page 22
  • 23. Surgical • Open Pyeloplasty – Gold Standard – Dismembered pyeloplasty is the most common Powerpoint Templates Page 23
  • 24. • Foley V-Y-Plasty – Good for 1-2 cm obstruction – Best for high inserting ureter – Best with relatively small pelvis Powerpoint Templates Page 24
  • 25. • Spiral flap – Good for long obstructions (better in adults) – Length of flap limited only by size of pelvis • (keep length: width at 3:1) • good when UPJ angle > 90 Powerpoint Templates Page 25
  • 26. • Endopyelotomy – Antegrade or retrograde – Cold knife or electric current – Acucise is very popular • dilation balloon with hot wire – 86% success in adults – Slightly less effective in children – Direct vision antegrade approach is most common Powerpoint Templates Page 26
  • 27. • Laparoscopic pyeloplasty – Same indications as open or endourologic procedures – Dismembered pyeloplasty is most common procedure performed • Without crossing vessels, may do any number of flap procedures • Up to 94% success rate, similar to open pyeloplasty Powerpoint Templates Page 27
  • 28. Any Question ? Powerpoint Templates Page 28
  • 29. REFERENCES - UpToDate (press on the title ) - Department of Urology Section of Pediatric Urology (University of Oklahoma ) (press on the title ) Powerpoint Templates Page 29