Editing Ureteropelvic Junction Obstruction

*'''See [https://test.urologyschool.com/index.php/Pediatrics:_Surgery_of_the_Ureter#Ureteropelvic_Junction_Obstruction_(UJPO) Ureteropelvic Junction Obstruction Section] in [[Pediatrics: Surgery of the Ureter|Pediatrics Surgery of the Ureter Chapter Notes]]'''
==Etiology==
*'''<span style="color:#ff0000">Classified: congenital vs. acquired</span> or intrinsic vs. extrinsic'''
*'''Most cases are congenital,''' but may only become clinically apparent much later in life
**'''Congenital ureteropelvic junction obstruction (UPJO) usually results from intrinsic disease'''
***'''UPJO in neonates is most frequently found as a result of maternal-fetal ultrasound'''
****Many newborns diagnosed with hydronephrosis on are subsequently found to have UPJO
*'''<span style="color:#ff0000">Congenital causes of UPJO (5):</span><span style="color:#0000ff"> SHAVA</span>'''
*#'''<span style="color:#ff0000">True ureteral </span><span style="color:#0000ff">S</span><span style="color:#ff0000">tricture</span>'''
*#'''<span style="color:#0000ff">H</span><span style="color:#ff0000">igh insertion</span> found more frequently in the presence of renal ectopia or fusion anomalies'''
*#'''<span style="color:#0000ff">A</span><span style="color:#ff0000">berrant vessels</span> (controversy persists). Regardless, the presence of crossing vessels has a detrimental effect on the success rates of endopyelotomy'''
*#'''<span style="color:#ff0000">Kinks or </span><span style="color:#0000ff">V</span><span style="color:#ff0000">alves</span>''' produced by infoldings of the ureteral mucosa and muscularis
*#'''<span style="color:#0000ff">A</span><span style="color:#ff0000">peristaltic segment</span>'''
*'''<span style="color:#ff0000">Acquired causes of UJPO (5):</span><span style="color:#0000ff"> CRIIBS</span>'''
*#'''<span style="color:#0000ff">C</span><span style="color:#ff0000">ancer</span>'''
*#'''<span style="color:#ff0000">Vesicoureteral </span><span style="color:#0000ff">R</span><span style="color:#ff0000">eflux</span><span style="color:#0000ff">;</span>''' can lead to upper tract dilation with subsequent elongation, tortuosity, and kinking of the ureter
*#'''<span style="color:#ff0000">Post-</span><span style="color:#0000ff">I</span><span style="color:#ff0000">nflammatory or postoperative scarring or <span style="color:#0000ff">I</span><span style="color:#ff0000">schemia</span>'''
*#'''<span style="color:#0000ff">B</span><span style="color:#ff0000">enign lesion (e.g. fibroepithelial polyps)</span>'''
*#'''<span style="color:#0000ff">S</span><span style="color:#ff0000">tones</span>'''
==Diagnosis and Evaluation==
==='''History and Physical Exam'''===
*'''History'''
**'''<span style="color:#ff0000">Signs and Symptoms</span>'''[https://www.ncbi.nlm.nih.gov/books/NBK560740/ §]
**#'''<span style="color:#ff0000">Periodic abdominal pain (loin pain), usually after diuresis'''
**#'''<span style="color:#ff0000">Vomiting'''
**#'''<span style="color:#ff0000">Recurrent pyelonephritis'''
**#'''<span style="color:#ff0000">Fever'''
**#'''<span style="color:#ff0000">Uncommonly, abdominal mass, or hematuria secondary to infection'''
==='''Imaging'''===
*Performed to determine the anatomic site and functional significance of an apparent obstruction
*'''Diuretic renography'''
**'''Commonly used''' for diagnosing both UPJ and ureteral obstruction
**'''<span style="color:#ff0000">Most commonly used agent in renogram studies is technetium 99m mercaptoacetyltriglycine (99m Tc-MAG3), especially in the pediatric population[https://www.ncbi.nlm.nih.gov/books/NBK560740/ §]'''
***In the adult population, other agents can be used, such as diethylenetriamine pentaacetate (DTPA)
**'''<span style="color:#ff0000">Provides quantitative data regarding differential renal function and obstruction</span>''', even in hydronephrotic renal units '''(see Pathophysiology of UUT Obstruction Chapter Notes for nuclear imaging details)'''
***'''<span style="color:#ff0000">In general, kidneys with < 15% differential function are nonsalvageable in adults</span>''' (Chapter 48 suggests <10%)'''.'''
****'''If the potential for salvageability of function is still unclear, an internal stent or percutaneous nephrostomy may be placed for temporary relief of obstruction and renal function studies subsequently repeated'''
==Management==

=== '''<span style="color:#ff0000">Indications for interventions</span>''' ===

* '''<span style="color:#0000ff">Similar to diversion for ureteric stone + HTN (5):</span>'''
#'''<span style="color:#ff0000">Symptoms </span>associated with the obstruction'''
#'''<span style="color:#ff0000">Stones</span>'''
#'''<span style="color:#ff0000">Infection</span>'''
#'''<span style="color:#ff0000">Renal dysfunction</span>'''
#'''<span style="color:#ff0000">Causal hypertension</span>'''

=== '''<span style="color:#ff0000">Options</span>''' ===
*'''<span style="color:#ff0000">Observation</span>'''
**'''If patient is asymptomatic and the physiologic significance of the obstruction seems indeterminate, careful observation with serial follow-up renal scans is appropriate'''
*'''<span style="color:#ff0000">Intervention (4):</span>'''
*#'''<span style="color:#ff0000">Decompression (stent or nephrostomy tube)</span>'''
*#'''<span style="color:#ff0000">Endourological procedures (balloon dilation or endopyelotomy)</span>'''
*#'''<span style="color:#ff0000">Pyeloplasty</span>'''
*#'''<span style="color:#ff0000">Ureterocalycostomy</span>'''
====Endourologic procedures====
*'''<span style="color:#ff0000">Options (2):</span>'''
*#'''<span style="color:#ff0000">Balloon dilation</span>'''
*#'''<span style="color:#ff0000">Endopyelotomy</span>'''
*'''<span style="color:#ff0000">Advantages:</span>'''
*#'''<span style="color:#ff0000">Less invasive</span>'''
*#'''<span style="color:#ff0000">Reduced hospital stays and postoperative recovery</span>'''
*'''<span style="color:#ff0000">Disadvantages (2):</span>'''
*#'''<span style="color:#ff0000">Higher risk of failure than pyeloplasty</span>'''.
*#*'''Patients should be counseled that the success rate of any endourologic approach may be less than that of formal reconstruction.'''
*#**Success rates approach 85-90% for percutaneous endopyelotomy.
*#'''Requires taking into account the degree of hydronephrosis, ipsilateral renal function, concomitant calculi, and possibly the presence of crossing vessels,''' whereas pyeloplasty (open, laparoscopic, or robotic) can be applied to almost any anatomic variation of UPJO
*#*'''Moderate to severe hydronephrosis is most predictive of failure after percutaneous endopyelotomy'''
*'''<span style="color:#ff0000">Transplantation complications are particularly suited to endoscopic (antegrade or retrograde) management</span>'''
*'''<span style="color:#ff0000">Approaches:</span>'''
**'''<span style="color:#ff0000">Retrograde balloon dilation alone</span>'''
***'''Long-term follow-up studies have shown a diminishing success rate over time'''
**'''<span style="color:#ff0000">Endopyelotomy</span>'''
***'''<span style="color:#ff0000">Contraindications (3):</span>'''
***#'''<span style="color:#ff0000">>2 cm of obstruction</span>'''
***#'''<span style="color:#ff0000">Untreated UTI</span>'''
***#'''<span style="color:#ff0000">Untreated coagulopathy</span>'''
***'''Technique'''
****'''Approaches: percutaneous antegrade or retrograde ureteroscopic'''
*****The main advantage of retrograde ureteroscopic endopyelotomy is that it allows direct visualization of the UPJ and assurance of a properly situated, full-thickness endopyelotomy incision without the need for percutaneous access
*****'''<span style="color:#ff0000">Percutaneous endopyelotomy remains appropriate for patients with UPJO and concomitant pyelocalyceal stones,</span> which can be managed simultaneously'''.
****'''Methods: can be done with an endopyelotome, holmium laser or cutting balloon catheter'''
*****Cutting balloon catheters have the potential to better dilate ischemic and fibrotic lesions resistant to conventional balloon catheter dilation[https://pubmed.ncbi.nlm.nih.gov/19959311/]
*****'''Little evidence for significant differences in success among endopyelotomy techniques.'''
******Differences lie in technical considerations and complications.
*****'''<span style="color:#ff0000">If using an endopyelotome or laser, a full-thickness lateral incision</span> is made through the obstructing proximal ureter, from the ureteral lumen out to the peripelvic and periureteral fat'''
******'''Incision should usually be made laterally because this is the location devoid of crossing vessels'''
****'''A stent is placed across the incision and is left to heal.'''
*****No consensus as to the optimal stent size or duration after endopyelotomy
****'''Postoperative care'''
*****Avoidance of strenuous activity for 8-10 days after the procedure
*****'''Once the stent is removed, that patient returns 1 month later for history and physical exam, urinalysis, and diuretic renography'''
*****'''For most adults, 2-3 year follow-up is justified'''
******Studies indicate that even at 36 months some late failures are identified, but relatively few are identified at 60 months
****'''Complications'''
*****'''Early'''
*****#'''Bleeding requiring transfusion'''
*****#'''Urinary leak'''
*****#'''Drainage-related complications'''
*****#'''Hydropneumothorax'''
*****#*'''Risk is increased if upper pole access is used'''
*****'''Late'''
*****#'''Recurrent obstruction'''
*****#*Options if percutaneous endopyelotomy fails:
*****#*#Retrograde endopyelotomy
*****#*#Repeat percutaneous endopyelotomy
*****#*#Laparoscopic, robotic, or open operative intervention
====Pyeloplasty====
*'''<span style="color:#ff0000">Principles of UPJ anastomosis (5):</span>'''
*#'''<span style="color:#ff0000">Widely patent</span>'''
*#'''<span style="color:#ff0000">Watertight</span>'''
*#'''<span style="color:#ff0000">Without tension</span>'''
*#'''<span style="color:#ff0000">Heal over a stent</span>'''
*#'''Reconstructed UPJ should allow a <span style="color:#ff0000">funnel-shaped transition</span> between the pelvis and the ureter that is <span style="color:#ff0000">in a position of dependent drainage</span>'''
*'''Absolute contraindications (3):'''
*#'''Untreated UTI'''
*#'''Uncorrected coagulopathy'''
*#'''Cardiopulmonary compromise unsuitable for surgery'''
*'''Before definitive surgical management, drainage of a kidney with UPJO is recommended only for infection associated with the obstruction or renal dysfunction resulting from obstruction in a solitary kidney or bilateral disease [suggesting no stent in renal dysfunction from single side obstruction if contralateral kidney fine]'''
*'''Technique'''
**'''Approaches: transperitoneal, retroperitoneal, or anterior extraperitoneal'''
***'''Transperitoneal laparoscopic approach is the most widely used method''' due to its associated large working space and familiar anatomy.
***Retroperitoneal laparoscopic approach and anterior extraperitoneal approach rely on creation of a working space using manual or balloon dilation.
**'''<span style="color:#ff0000">Methods: dismembered vs. non-dismembered</span>'''
***'''Dismembered pyeloplasty'''
****'''Example: Anderson-Hynes pyeloplasty'''
****'''Preferred by most urologists because this procedure is almost universally applicable to different clinical scenarios'''
*****Can be used regardless of whether the ureteral insertion is high on the pelvis or already dependent.
*****Permits reduction of a redundant pelvis or straightening of a tortuous proximal ureter
*****'''Anterior or posterior transposition of the UPJ can be achieved when the obstruction is the result of accessory or aberrant lower pole vessels'''
******In the presence of crossing aberrant or accessory lower pole renal vessels associated with UPJ obstruction, a dismembered pyeloplasty is the only method to allow transposition of the UPJ in relation to these vessels.
******'''If dismembered pyeloplasty is performed for the presence of crossing vessels, the renal pelvis is first transected circumferentially above the UPJ and the lateral aspect of the proximal ureter is spatulated.''' The renal pelvis and proximal ureter are then transposed to the opposite side of the crossing vessel and the ureteropelvic anastomosis is then completed with intracorporeal suturing techniques
*****'''Unlike the flap techniques, only a dismembered pyeloplasty allows complete excision of the anatomically or functionally abnormal UPJ itself'''
****'''Dismembered pyeloplasty is not well suited to UPJO associated with (2):'''
****#'''Lengthy or multiple proximal ureteral strictures'''
****#*'''<span style="color:#ff0000">The spiral flap may be of significant value when both UPJO and a relatively long segment of proximal ureteral narrowing or stricture occur in the same setting</span>'''
****#'''Small, relatively inaccessible intrarenal pelvis'''
***'''Non-dismembered pyeloplasty'''
****'''Examples: Y-V plasty and flap pyeloplasty (Culp)'''
*****'''The Foley Y-V-plasty is designed for repair of a UPJ obstruction secondary to a high ureteral insertion.'''
*****Flap procedures are not appropriate in the setting of crossing vessels and when reduction of redundant renal pelvis is desired
**'''<span style="color:#ff0000">The provision of external drainage from the site of surgical repair is absolutely necessary</span>'''
***'''Helps reduce risk of urinoma formation leading to possible disruption of the suture line, scarring, or sepsis'''
**'''<span style="color:#ff0000">Post-operative care</span>'''
***'''<span style="color:#ff0000">The Foley catheter is usually removed 24 to 36 hours postoperatively, and the surgical drain is removed before hospital discharge if the drain output remains negligible.</span>'''
****'''<span style="color:#ff0000">If the drain output increases after the Foley catheter removal, the Foley catheter should be replaced for 7 days to eliminate urinary reflux along the stent in the treated ureter and decrease urinary extravasation at the ureteropelvic anastomosis.</span>'''
***'''<span style="color:#ff0000">Ureteral stent is typically removed 4-6 weeks later in an outpatient setting</span>'''
***'''<span style="color:#ff0000">Follow-up including the use of imaging studies such as diuretic renal scan is performed</span>'''
**'''Complications'''
***'''Late'''
****'''Persistent urinary drainage'''
*****'''Common after an unstented pyeloplasty is common, and will often require intervention.'''
*****'''When associated with a large blood clot,''' and likely edema at the anastomosis, '''the kidney will need early drainage until the bleeding resolves and edema improves.'''
******'''In children, this is best managed with a nephrostomy tube, as stent placement in a young infant would likely result in stent occlusion from the renal pelvic blood clot.'''
*****'''In the majority of cases not associated with an occlusive blood clot, the leak will resolve spontaneously, so observation is the best approach in the early postoperative period in these patients.'''
******'''If the leak is persistent and not associated with a consolidated clot, it would most likely resolve with retrograde stent placement.'''
****'''Recurrent obstruction'''
*****'''Most failures from laparoscopic pyeloplasty occur in the first 2 years.'''
*****'''<span style="color:#ff0000">Management</span>'''
******'''<span style="color:#ff0000">Options (2)</span>'''
*******'''<span style="color:#ff0000">Repeat pyeloplasty (open surgery has been used as a salvage procedure after failed laparoscopic pyelopasty)</span>'''
*******'''<span style="color:#ff0000">Endoscopic intervention (most patients can be managed</span>'''
====Ureterocalicostomy====
*'''<span style="color:#ff0000">Indications (3):</span>'''
*#'''UPJO or proximal ureteral stricture is associated with a relatively <span style="color:#ff0000">small intrarenal pelvis</span>'''
*#'''To provide completely dependent drainage when the UPJ is associated with <span style="color:#ff0000">rotational anomalies</span> such as horseshoe kidney'''
*#'''Salvage technique for <span style="color:#ff0000">failed pyeloplasty</span>'''
====Recurrent obstruction after treatment====
*'''<span style="color:#ff0000">An open or laparoscopic approach can be recommended to any patient in whom primary endourologic management has failed, and an endourologic approach in whom an open or laparoscopic repair has failed</span>'''

== Questions ==

== Answers ==

== References ==

* Wein AJ, Kavoussi LR, Partin AW, Peters CA (eds): CAMPBELL-WALSH UROLOGY, ed 11. Philadelphia, Elsevier, 2015, chap 49