Transcript Ureteropelvic junction obstruction : Intern 黃暉程

Ureteropelvic
junction
obstruction
報告者: Intern 黃暉程
Supervisor: 主治醫師: 邱元佑
Identification
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Name: 黃小弟
Birth date: 05/31/03 → 19 d/o
G2P2, NSD, Apgar score: 9’→10’
GA 41weeks, BW: 3000g(10~25%)
BL: 52.5cm(10~25%), HC: 35cm(10~25%)
DOIC(-), PROM(-)
Chief complaint:
left abdominal mass
for 2 days
Present Illness
GA 28-30wks
Prenatal exam at
姚博琳’s clinic: Left
hydronephrosis;
Oligohydromino(-);
Other abnormality(-)
92/05/31
One mass
over left
abdomen
noted by his
mother
92/06/18
92/06/16
GA 41wks, NSD:
Renal echo at 姚博琳’s
clinic: Hydronephrosis
is not identified
Brought to Dr. 邱:
A mass over LUQ
palpable
One 11x7cm soft
mass over left
abdomen;
percussion:
spongy-filling
Abdominal mass ~ approach
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Inspection, Percussion, Palpation
Abdominal mass by age
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Age-group
1 m~1 yr
Newborns
After 1 yr
Differential diagnosis
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Non-urologic
Abdominal distention, pyloric stenosis,
hepatosplenomegaly, intestinal obstruction,
malignany, feces
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Urologic
Hydronephrosis, cystic disease, Wilms’
tumor, neuroblastoma, distended bladder
Renal echo (Jun 18)
Left severe hydronephrosis
Cortex thickness: about 0.2cm
AP diameter: 4.48cm (>1.5cm)
Right moderate hydronephrosis
No parenchyma involvement
AP diameter: 1.2cm (>1cm)
Imp: suspect left ureteropelvic junction obstruction
Present Illness
(1) VCUG
(2) Antegrade
pyelography
92/06/18
Left PCN
92/06/27
92/7/2: discharge
92/06/19 92/06/23
Admission
PE
LAB:CBC/DC,
Biochemistry,
U/A
(1) Left dismembered
pyeloplasty
(2) Pathologic Dx:
Muscular hyperplasia and
fibrosis, compatible with
stenosis
Indication of PCN
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Obstruction with infection
Obstruction without infection
Stone disease
Prelude to endoscopic/ interventional
procedures
Delivery of medications/ chemotherapy
Urinary leaks
Urinary diversion for hemorrhagic cystitis
VCUG (Jun 23)
Imp:
No evidence
of vesicoureteral reflux
Antegrade
pyelography:
Left UPJ stenosis is
considered
Present Illness
Discharge!
(1) VCUG
(2) Antegrade
pyelography
Left PCN
92/06/19
92/06/27
92/06/23
92/7/2
(1) Left dismembered pyeloplasty :
UPJ obstruction, high insertion
(2) Pathologic Dx:
Muscular hyperplasia and fibrosis,
compatible with stenosis
Whitaker test during operation
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Measure the pressure gradient between the
pelvis & the bladder under fixed infusion rate
Less than 12 mmHg: no obstruction
Above 20 mmHg: obstruction
Pressure gradient was 14~15 mmHg
→ 1. intermediate
2. good compliance of pelvis and ureter
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Diagnosis
Left UPJ stenosis
Discussion
UPJ obstruction
UPJ obstruction
generally a congenital condition
 male, left-sided lesions predominating
 most frequently diagnosed cause of
urinary obstruction in children
 causes hydronephrosis which may
damage the kidney
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Pathology
Various interpretations Preponderance of longitudinal muscle fibers
 Excessive collagen fibers in & around muscle
bundles
 Compromised or attenuated muscle bundles
Our case: moderately lymphocytic infiltration &
focal suppurative inflammation
Symptoms & signs
Back or flank pain
 UTI with fever
old children
 Hematuria
 Abdominal mass → infants
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}
Diagnosis & tests
Prenatal
Maternal pregnancy ultrasound:
hydronephrosis
 Postnatal
Ccr, BUN, electrolytes, AP, DTPA, MAG3,
VCUG
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Etiology
Intrinsic:
Narrowed, dysfunctional or adynamic
segments
 Extrinsic:
Upper ureter is angulated, kinked or
compressed by bands or adhesions
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Intrinsic obstruction
mechanical: narrowed → incomplete
embryological ureteric bud recanalization;
muscular invaginations overdevelop as
flaps or valves
 functional: adynamic or dysfunctional
segment → inability to initiate, form or
conduct peristaltic waves across the UPJ
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Extrinsic obstruction
vessel or fibrous band may pass anterior
to the pelvis & ureter: most common
 may secondary to intrinsic disturbance
which produces pelvic overdistension &
rotation
 high insertion of the ureter into the pelvis
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Extrinsic ~ High insertion
Whitaker test: flow across UPJ
obstructions
Pressure dependent
Volume dependent
Intrinsic obstruction Extrinsic obstruction
Treatment
influenced by renal function, infection
 surgical correction of the obstruction
 infants: dismembered pyeloplasty
 adults: percutaneous or endoscopic
technique
 a nephrostomy stent is placed to drain
urine until the patients heals
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Surgical indication
Bilateral UPJO
 Palpable mass
 Unilateral UPJO with hydronephrosis
Grade 4 (Massive pelvic & calyceal
dilatation with thinned parenchyma); DTPA
< 30% or worsen > 10% in f/u
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Prognosis ~ pyeloplasty
Author and Year
Patients/Kidneys
Success (%)
Poulsen et al, 1987
35
100
O’Reilly, 1989
30
83–93
MacNeily et al, 1993
75
85
32/33 (<2 mo old)
97
30/33 (>2 mo old)
93
100
98
79
90
Austin et al, 2000
135/137
91
Houben et al, 2000
186/203
93
Shaul et al, 1994
Salem et al, 1995
McAleer and Kaplan, 1999
Prognosis ~ pyeloplasty
Expectantions
Rapid decompression of the kidney
immediately following birth can
substantially improve kidney function in an
infant with UPJ obstruction diagnosed
before the child is born.
 Most patients do well with no long-term
consequences
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Complications
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Permanent loss of kidney function-renal
failure
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require dialysis at some point in their lives
as a result of this problem
Thanks for your
attention!