Presented by Dr.Talal Alanzi Urology board yr 2 Surgical
rotation( Adan hospital) Supervised by Dr.Adel Allam Consultant :
Farwaniya Hospital
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OBJECTIVE: PUJ obstruction 1- etiology 2-pathophysiology
3-Investigation 4-Management Literature review 1.Outcome of
different surgical intervention 2.Role of open surgery 3.Antegrade
V.S retrograde pyeloplasty 4.Early and delayed pyeloplasty in
pediatric 5.Laparoscopic role in pediatric
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Definition significant impairment of urinary transport from the
renal pelvis to the ureter.
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General information 5 per 100,000 per yr. Commonest form
urinary tract obstruction in children. Male : female 5:2. Left :
right side 5:2. B/L obstruction 10-15%. Some genetic predisposing
factor.
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Majority are diagnosed antenatally.
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Embryological The UPJ forms during the fifth week.
Ureteropelvic and Ureterovesical portions of the ureter are the
last to canalize.
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Etiology 1- Idiopathic. Theory: premature arrest of ureteral
wall musculature development. growth factor(transforming growth
factor (TGF). improper innervation. Folding of the proximal ureter.
muscular discontinuity. 2-Intrinsic lesion: Aperstaltic segment.
stone disease,postoperative or inflammatory stricture, or
urothelial neoplasm. Less common, valvular mucosal folds,upper
ureteral polyps.
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Etiology Extrinsic: fibrous bands, kinks, and aberrant crossing
vessels. -Aberrant vessel count 25%. -If the PUJ is due to
extrinsic factor,Present in late childhood.
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Etiology Secondary causes: -severe VUR or lower urinary tract
obstruction. -permanent kink at PUJ (tortuosity) -high inserting
ureter.
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pathophysiology Overdistention of the pelvis leads to
hypertophy and reduce GFR. Parenchymal distortion and impaired its
function.(depending on degree). Loss of normal smooth muscle,
hypertrophy then fibrosis.
Presentation-new born UTI Hematuria Failure to thrive Feeding
difficulties Sepsis Azotemia. Palpable mass.
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Presentation- later life 30% after UTI. 25% after Hematuria.
Abd pain(periodically), nausea and vomiting. Palpable mass.
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Diagnosis Most of the cases are diagnosed antenatally. Routine
prenatal assessment typically occurs at 16-20 weeks' gestation.
Gestation age of 33 wk (expected AP diameter renal pelvis 4-7
mm).
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Criteria for fetal hydronephrosis Society of Fetal Urology
(SFU) consensus guidelines: Grade 0 Normal kidney Grade 1 Minimal
pelvic dilation Grade 2 Greater pelvic dilation without caliectasis
Grade 3 Pelviectasis and caliectasis without cortical thinning
Grade 4 Hydronephrosis with cortical thinning
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Criteria for fetal hydronephrosis US should be repeated 48 hr,
or 4 wks from delivery. Grade 1-2 F/U (6 month) for 1 yr.
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Criteria for fetal hydronephrosis Grade 3-4 need f/u (3-4
months) for 1yr. Followed up by 1-diuretic nuclear renogram(age of
1 month) 2-cystourethrography is performed for all patients (VUR
13-43%).
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INVESTIGATION (1) Ultrasound -AP diameter of the renal pelvis
(4-7 mm). -Effective screening and monitoring HN, but its results
cannot confirm the diagnosis of PUJ obstruction. -Dehydration may
also lead to false-negative.
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INVESTIGATION (2)Computed Tomography: -Assessing the causes of
acquired PUJ and ureteral obstruction. -Cortical thinning in HN.
-CT urography, further evaluation of anatomic and physiology of
kidney. False negative: massively dilated collecting system in the
absence of true functional obstruction.
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INVESTIGATION (3) IVP+ retrograde pyelogram: -Traditionally has
been the primary study for evaluating HN. -In pediatric replace by:
US +Renogram. -provides functional and anatomic detail.
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INVESTIGATION Retrograde pyelography provide good details if
IVP was unhelpful. Is the most invasive study. reveal the site of
obstruction. false-positive : If stone, external pressure.
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INVESTIGATION. (4) Nuclear medicine: -primary study for
defining ureteropelvic junction (PUJ) obstruction. -Assessing renal
function. -MAG3 has replaced DTPA (immature-chronic insufficient
kidney. -clearance rate of a radioisotope(washout half-life),
normal 10 min. -False-positive : full bladder- poor function
kidney.
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investigation (5) Angiography: -Performed before
surgery(aberrant vessel). -It provides no information as to whether
these arteries are causing mechanical obstruction.
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investigation (6) MCUG: -Its traditionally an unreliable test
for diagnosing PUJ obstruction itself. -Has no role in detecting
PUJ obstruction. -It detect the 10% of VUR associated with PUJ
obstruction.
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(7) Whitaker test: -It measures resistance to flow.
-Percutaneous pressure-flow study that allows the measurement of
renal pelvic pressures. -now rarely performed ( Invasive).
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Investigation (8) Magnetic Resonance Imaging: -Excellent but,
it does not offer significant benefit over others. -Not used in the
workup of PUJ obstruction. Disadvantage: Nephrogenic systemic
fibrosis (NSF).
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Management 1 conservative or 2 surgical intervention.
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management Conservative txt: -40% of antenatal HN resolved
postpartum. -Infant with renal function 35-40 % with variable wash
out would benefit mostly. -Role 1/3.(improve-same-worsen).
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Indication for surgical intervention Pain with obstruction.
Impairment of overall function. Progressive impairment of
ipsilateral function. Stone or infection. Hypertension.
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Aim of surgery Tension-free Water-tight repair Funnel-shaped
drainage to preserve renal function.
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Surgical intervention Less invasive procedure:
(1)Endopyelotomy: A. antegrade (cold knife-electric current) B.
retrograde (cold knife-electric current-Holmium laser) (2) Acucise
Endopyelotomy.
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Endopyelotomy Success rate 67-73%.
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Percutaneous Antegrade Endopyelotomy Ramsay and colleagues in
1984 Indication: PUJ obstruction+stones Stenosis 2cm Infection
Untreated coagulopathy
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Aberrant vessel can reduce the success rate. The incision
should generally be made posterior & laterally. because this is
the location devoid of crossing vessels
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Retrograde Ureteroscopic Endopyelotomy 1985 ( Bagley and
colleagues). Rigid or Flexible ureteroscopes. nephrostomy tube kept
for 48 hr. Balloon dilation up to 24-Fr.
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It allows direct visualization of the UPJ and assurance of a
properly situated, full-thickness endopyelotomy incision without
the need for percutaneous access.
retrograde balloon dilation Pearle et al, 1994. Retrograde
balloon dilation alone has been reported for treatment of PUJ
obstruction. Success rate of 42%.
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Acucise retrograde endopyelotomy Described Wickham and Kellet
1983. Suitable for segment less than 2 cm. Not fit for pt aberrant
vessel kidney stone infection
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Emergency In case of B/l obstructed uropathy, azotemia,
obstructed solitary kidney, infection. Drainage of the kidney by
Nephrostomy tube DJ stent Prophylaxis antiobiotic.
Dismembered Pyeloplasty Andersen-Hynes pyeloplasty Preferred by
most urologists. Gold standard. well suited to PUJ obstruction. Not
advisable with lengthy or multiple proximal ureteral
strictures-inaccessible intrarenal pelvis. Success rate of
91-95%.
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Foley Y-V-Plasty Indicated high ureteral insertion. Stone
+PUJO.
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Ureterocalycostomy Indicated for relatively small intrarenal
pelvis. Uureterocalycostomy is a well-accepted salvage technique
for the failed pyeloplasty.
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Laparoscopic Pyeloplasty Introduced in 1993 by Schuessler and
colleagues. Associated with greater technical complexity and a
steeper learning curve.
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Advantage of Lap. Provide lower patient morbidity Shorter
hospitalization, and faster convalescence, with the reported
success rates matching those of open pyeloplasty 90%.
British journal of urology. 1984-1995. Carried out 47 pt. Alder
Hey children hospital (Liverpool). Purpose: compare early and late
intervention with PUJO.
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Renogram after Reno gram before Initial renogram No ptOpen
pyeloplast 32.7%28.1%26Early intervention 37.5%30.5%44.8%21Late
intervention
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The Journal of urology. 2005. Carried out 1997-2005. University
medical center Mainz (Germany). 46 pt.
Take home message The importance of antenatal U/S. Diagnostic
test cant differentiate between who needs surgical intervention,
and those who improve spontaneously. Half of antenatal cases
resolve spontaneously
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Solitary kidney, bilateral UPJ, or poorly functioning kidneys
should be considered for earlier surgery. Robotic assisted
laparoscopic pyeloplasty is a promising technique. Criteria of
success after surgery Pain, Radiology Nuclear medicine. The optimal
length of follow-up after pyeloplasty is still unclear.