Management of PUJOAdult & Pediatric Presented by Dr.TalalAlanzi Urology board yr 2 Surgical rotation( Adan hospital) Supervised by Dr.AdelAllam Consultant : Farwaniya Hospital
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
Definition significant impairment of urinary transport from the renal pelvis to the ureter.
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.
Embryological • The UPJ forms during the fifth week. • Ureteropelvic and Ureterovesical portions of the ureter are the last to canalize.
Etiology • 1- Idiopathic. Theory: • premature arrest of ureteral wall musculature development. • growth factor(transforming growth factor β (TGFβ). • improper innervation . • Folding of the proximal ureter. • muscular discontinuity. • 2-Intrinsic lesion: • Aperstaltic segment. • stone disease,postoperativeor inflammatory stricture, or urothelialneoplasm. • Less common, valvular mucosal folds,upper ureteral polyps.
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.
Etiology • Secondary causes: -severe VUR or lower urinary tract obstruction. -permanent kink at PUJ (tortuosity) -high inserting ureter.
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.
Concept of volume-dependent flow
Associated anomalies • Contralateral PUJ. 10-40% • RENAL DYSPLASIA,APLASIA, MCKD. • VUR 10-40%.
Presentation-new born • UTI • Hematuria • Failure to thrive • Feeding difficulties • Sepsis • Azotemia. • Palpable mass.
Presentation- later life • 30% after UTI. • 25% after Hematuria. • Abd pain(periodically), nausea and vomiting. • Palpable mass.
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).
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
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.
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%).
INVESTIGATION (1) Ultrasound -AP diameter of the renal pelvis (4-7 mm). -Effective screening and monitoring HN, butits results cannot confirm the diagnosis of PUJ obstruction. -Dehydration may also lead to false-negative .
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.
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 .
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.
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.
investigation (5) Angiography: -Performed before surgery(aberrant vessel). -It provides no information as to whether these arteries are causing mechanical obstruction.
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.
(7) Whitaker test: -It measures resistance to flow. -Percutaneous pressure-flow study that allows the measurement of renal pelvic pressures. -now rarely performed ( Invasive).
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).
Management 1 conservative or 2 surgical intervention.
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).
Indication for surgical intervention • Pain with obstruction. • Impairment of overall function. • Progressive impairment of ipsilateral function. • Stone or infection. • Hypertension.
Aim of surgery • Tension-free • Water-tight repair • Funnel-shaped drainage to preserve renal function.
Surgical intervention • Less invasive procedure: (1)Endopyelotomy: • antegrade (cold knife-electric current) • retrograde (cold knife-electric current-Holmium laser) (2) AcuciseEndopyelotomy.
Endopyelotomy • Success rate 67-73%.
Percutaneous AntegradeEndopyelotomy • Ramsay and colleagues in 1984 • Indication: PUJ obstruction+stones Stenosis <2 cm • Contraindication Stenosis > 2cm Infection Untreated coagulopathy
Aberrant vessel can reduce the success rate. The incision should generally be made posterior & laterally. because this is the location devoid of crossing vessels
Retrograde UreteroscopicEndopyelotomy • 1985 ( Bagley and colleagues). • Rigid or Flexible ureteroscopes. • nephrostomy tube kept for 48 hr. • Balloon dilation up to 24-Fr.
It allows direct visualization of the UPJ and assurance of a properly situated, full-thickness endopyelotomy incision without the need for percutaneous access.
Retrograde UreteroscopicEndopyelotomy • Indication: functionally significant obstruction • Contraindication: Long segment(2 cm)-upper tract stones
retrograde balloon dilation • Pearle et al, 1994. • Retrograde balloon dilation alone has been reported for treatment of PUJ obstruction. • Success rate of 42%.
Acucise retrograde endopyelotomy • Described Wickham and Kellet 1983. • Suitable for segment less than 2 cm. • Not fit for pt aberrant vessel kidney stone infection