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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.

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Concept of volume-dependent flow

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Associated anomalies Contralateral PUJ. 10-40% RENAL DYSPLASIA,APLASIA, MCKD. VUR 10-40%.

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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.

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Retrograde Ureteroscopic Endopyelotomy Indication: functionally significant obstruction Contraindication: Long segment(2 cm)-upper tract stones

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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.

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Open pyeloplasty Approaches: 1. Anterior extraperitoneal (less mobilization). 2. posterior lumbotomy (thin-no previous surgery- pediatric). 3. extraperitoneal flank.(Subcostal)

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Types 1.Dismembered Pyeloplasty 2. Foley Y-V-Plasty 3.Culp-DeWeerd Spiral Flap 4.Scardino-Prince Vertical Flap 5.Ureterocalycostomy

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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%.

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Technique: 1. Standard transperitoneal approach, 2. Retroperitoneal approach, 3. Anterior extraperitoneal approach.

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Lap.pyeloplasty Preferred approach : 1. Andersen-Hynes pyeloplasty. 2. Y-V plasty and flap pyeloplasty. Transmesenteric approach to laparoscopic pyeloplasty.

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Robotic-Assisted Laparoscopic Approach First reported by Sung and colleagues (1999). Da Vinci Robot. Transperitoneal manner. Advantages: 1. Enhanced three-dimensional vision 2. Motion scaling, 3. Tremor reduction, 4. Increased range of motion.

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British jornal of urology. Published in 1997. Guys hospital London. Purpose of the study: comparing the major four surgical technique.

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Success rateFollow upNo. patientmethod 92%21 months202dismemberedOpen pyeloplasty 80.5%21 months542Flap technique 97.1%8 months31dismemberedLap.pyeloplast y 67-78%6 months80Ballon dilatation 67%6 months366Endoscopic endopyelotom y 89%6 months212ballon 80%19 months57Acucise endopyelotom y

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British journal of urology. Published in 1997. Christian medical school. (India) The purpose of the study, evaluation on the outcome open surgery.

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Foly YVdismemberedprocedure Open pyeloplasty same3-60 monthsFollow up 957No. pt resident Performed 8 days7.5 daysMain hospital stay

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treatmentNo. ptcomplication 3 ballon diatation 1 nepherectomy 4Persistent puj obstruction J-J STENT2VUJ OBS 1 J-J 1 PYELOPLASTY 2Urinary leakage meatotomy1Uretheral meatous stenosis 98%43 (63) - 92%Success rate

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British journal of urology. Carried between 1994-2004. Careggi hospital (Italy). Purpose of study to compare antegrade V.S. retrograde pyeolplasty.

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Success rateretrogradeAntegradeType of procedure 4919No pt 53%1819diathermy 80%30-Holmium laser 12.5%42%complication 1.5 day7 daysHospital stay same31 monthsF/U

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retrogradeantegradecomplication 01Bleeding required transfusion 11Bleeding required transfusion+ embolization 03Persistent pyrexia 31UTI 10Hematuria + clots 10sepsis

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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.

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(7-18 yr)(1-7 yr) (1-12 month) 171514No pt 44/46 (96%) Success rate 173 min169 min171 mintime 29 m F/U 110conversioncomplication 120PUJ leakage 001Nepherostomy 200redo

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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.