Management of PUJO Adult & Pediatric Presented by Dr.Talal Alanzi Urology board yr 2 Surgical rotation( Adan hospital) Supervised by Dr.Adel Allam 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.

Majority are diagnosed antenatally.

Embryological The UPJ forms during the fifth week. Ureteropelvic and Ureterovesical portions of the ureter are the last to canalize.

Etiology 1- Idiopathic. Theory: 2-Intrinsic lesion: 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,postoperative or inflammatory stricture, or urothelial neoplasm. 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, but its 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 (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).

1 conservative or 2 surgical intervention. 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) Acucise Endopyelotomy.

Endopyelotomy Success rate 67-73%.

Percutaneous Antegrade Endopyelotomy 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 Ureteroscopic Endopyelotomy 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 Ureteroscopic Endopyelotomy 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

Emergency In case of B/l obstructed uropathy, azotemia, obstructed solitary kidney, infection. Drainage of the kidney by Nephrostomy tube DJ stent Prophylaxis antiobiotic.

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

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

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%.

Foley Y-V-Plasty Indicated high ureteral insertion. Stone +PUJO.

Ureterocalycostomy Indicated for relatively small intrarenal pelvis. Uureterocalycostomy is a well-accepted salvage technique for the failed pyeloplasty.

Laparoscopic Pyeloplasty Introduced in 1993 by Schuessler and colleagues. Associated with greater technical complexity and a steeper learning curve.

Advantage of Lap. Provide lower patient morbidity Shorter hospitalization, and faster convalescence, with the reported success rates matching those of open pyeloplasty 90%.

Technique: Standard transperitoneal approach, Retroperitoneal approach, Anterior extraperitoneal approach.

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

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

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

Success rate Follow up No. patient method 92% 21 months 202 dismembered Open pyeloplasty 80.5% 542 Flap technique 97.1% 8 months 31 Lap.pyeloplasty 67-78% 6 months 80 Ballon dilatation 67% 366 Endoscopic endopyelotomy 89% 212 ballon 80% 19 months 57 Acucise endopyelotomy

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

Foly YV dismembered procedure Open pyeloplasty same 3-60 months Follow up 9 57 No. pt resident Performed 8 days 7.5 days Main hospital stay

treatment No. pt complication 3 ballon diatation 1 nepherectomy 4 Persistent puj obstruction J-J STENT 2 VUJ OBS 1 J-J 1 PYELOPLASTY Urinary leakage meatotomy 1 Uretheral meatous stenosis 98% 43 (63) - 92% Success rate

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

Success rate retrograde Antegrade Type of procedure 49 19 No pt 53% 18 diathermy 80% 30 - Holmium laser 12.5% 42% complication 1.5 day 7 days Hospital stay same 31 months F/U

retrograde antegrade complication 1 Bleeding required transfusion Bleeding required transfusion+ embolization 3 Persistent pyrexia UTI Hematuria + clots sepsis

British journal of urology. 1984-1995. Carried out 47 pt. Alder Hey children hospital (Liverpool). Purpose: compare early and late intervention with PUJO.

Renogram after Reno gram before Initial renogram No pt Open pyeloplast 32.7% 28.1% 26 Early intervention 37.5% 30.5% 44.8% 21 Late intervention

The Journal of urology. 2005. Carried out 1997-2005. University medical center Mainz (Germany). 46 pt.

44/46 (96%) (7-18 yr) (1-7 yr) (1-12 month) 17 15 14 No pt Success rate 173 min 169 min 171 min time 29 m F/U 1 conversion complication 2 PUJ leakage Nepherostomy redo

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

Robotic assisted laparoscopic pyeloplasty is a promising technique. 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.