Congenital UPJ Obstruction

Congenital (UPJ) Ureteropelvic
Junction Obstruction
Mohammed Nabil J AlAli

5th Year Medical Student
At King Faisal University
Powerpoint Templates
Page 1
Group B (210006209)
Outlines:
-OVERVIEW
-PATHOPHYSIOLOGY
- ETIOLOGY
- CLINICAL PRESENTATION
- DIAGNOSIS
- DIFFERENTIAL DIAGNOSIS
- FOLLOW-UP
- MANAGEMENT
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
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
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.
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
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.
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
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
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
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
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DIAGNOSIS
It is generally suspected when

imaging studies, usually
ultrasonography, demonstrate
hydronephrosis.
The diagnosis is confirmed by

diuretic renography.
Page 12
Ultrasonography (US)
Most cases of UPJ obstruction present as
a result of detecting hydronephrosis by
prenatal ultrasonographic screening
Abnormal
calyces
Normal
kidney
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
obstruction . Page 14
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.
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
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
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
Page 19
MANAGEMENT
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
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
Page 22
Surgical
Open Pyeloplasty
Gold Standard
Dismembered pyeloplasty is the most
common
Page 23
Foley V-Y-Plasty
Good for 1-2 cm obstruction
Best for high inserting ureter
Best with relatively small pelvis
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
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
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
Page 27
Any Question ?
Page 28
REFERENCES
- UpToDate (press on the title )
- Department of Urology Section of Pediatric Urology

(University of Oklahoma ) (press on the title )
Page 29
Thank you
Page 30

Congenital UPJ Obstruction

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Congenital UPJ Obstruction

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Congenital (UPJ) Ureteropelvic

Mohammed Nabil J AlAli

It is a partial or total blockage of

It is the most common pathologic

Most common site of urinary tract

Majority are discovered antenatally

Most cases are thought to be due to

It is both congenital and acquired

Usually caused by intrinsic stenosis

Although the underlying mechanism is

Another urologic abnormality-50%

It is generally suspected when

The diagnosis is confirmed by

- It can be used to diagnose UPJ type

-Ten percent of patients with UPJ obstruction

-Identification of VUR is important because

- Department of Urology Section of Pediatric Urology

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