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Mohammed Nabil J AlAliMohammed Nabil J AlAli5th Year Medical StudentAt King Faisal University

Group B (210006209)

Congenital (UPJ) Ureteropelvic Congenital (UPJ) Ureteropelvic Junction ObstructionJunction Obstruction

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-OVERVIEW -PATHOPHYSIOLOGY  - ETIOLOGY- CLINICAL PRESENTATION- DIAGNOSIS- DIFFERENTIAL DIAGNOSIS- FOLLOW-UP- MANAGEMENT

Outlines:

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OVERVIEW

• It is a partial or total blockage of the flow of urine that occurs where the ureter enters the kidney.

• It is the most common pathologic cause of antenatally detected hydronephrosis

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EPIDEMIOLOGY

• Most common site of urinary tract obstruction in children

• Majority are discovered antenatally– 1:1500 secrend by ultrasound– It is the most common anatomical cause of

antenatal hydronephrosis – Boys > girls– Most cases on the left– 10-40% bilateral

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PATHOPHYSIOLOGY  • It is caused by anatomic lesions or functional disturbances that restrict urinary flow, resulting in hydronephrosis.

• Most cases are thought to be due to partial obstruction, because complete obstruction results in rapid destruction of the kidney. •In some cases, partial obstruction may also lead to progressive deterioration of renal function.

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Development of the equilibrium state resulting in stable renal function depends on:

• Urinary rate and output• Anatomy and degree of UPJ obstruction• Compliance of the renal pelvis

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ETIOLOGY

• It is both congenital and acquired conditions.

• Usually caused by intrinsic stenosis of the proximal ureter, and less commonly by extrinsic compression of the UPJ.

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Intrinsic narrowing 

• In most cases of UPJ obstruction, the upper segment of the ureter is narrowed or kinked, resulting in obstruction of urinary flow.

• Although the underlying mechanism is not proven, it is thought that there is an embryologic disruption of the proximal ureter that alters circular musculature development and/or collagen fibers, and composition between and around the muscular cells

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Extrinsic narrowing 

In about 10 % of pediatric UPJ obstruction, an aberrant or accessory renal artery or arterial branch may cross the lower pole of the kidney, resulting in compression of the UPJ and blockage of urinary flow

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CLINICAL PRESENTATION• Historically presented as a

palpable mass– Newborn

• Antenatal hydronephrosis 80%• UTI, hematuria, failure to thrive, feeding

difficulties, sepsis, azotemia

– Later in life• 30% diagnosed after UTI• 25% diagnosed after hematuria• Episodic abdominal pain and vomiting

due to intermittent obstruction

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Associated Anomalies

• Another urologic abnormality-50%– Contralateral UPJ 10-40%– Renal dysplasia, aplasia, MCKD– VUR up to 40%

• Found in 21% of VATER patients

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DIAGNOSIS

• It is generally suspected when imaging studies, usually ultrasonography, demonstrate hydronephrosis.

• The diagnosis is confirmed by diuretic renography.

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Ultrasonography (US)  Most cases of UPJ obstruction present as a result of detecting hydronephrosis by prenatal ultrasonographic screening

Normalkidney

Abnormal calyces

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Diuretic renography • It (renal scan and the administration of a diuretic) is used to diagnose urinary tract obstruction.• It measures the drainage time from the renal pelvis (referred to as washout) and assesses total and each individual kidney's renal function. •The washout measurement correlates with the degree of obstruction.

In general, a half-life greater than 20 minutes to clear the isotope from the kidney is considered indicative of obstruction.

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Computed tomographic scan (CT) - It is an alternative to ultrasonography in the symptomatic child. -It is not the preferred modality due to its radiation exposure. - In UPJ obstruction, the CT scan typically shows hydronephrosis without a dilated ureter.

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Magnetic resonance imaging (MRI) - It can be used to diagnose UPJ type hydronephrosis. -The advantage of MRI is the ability to discern accurate anatomy defining the point of obstruction. -Also determine the split function of the kidney and simulate the diuretic renogram by providing washout data. -The disadvantage of MRI is the cost and the need for general anesthesia and/or sedation

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Voiding cystourethrogram (VCUG)

-It is performed in patients with hydronephrosis to confirm the presence or absence of VUR of both the affected and contralateral kidneys.

-Ten percent of patients with UPJ obstruction have contralateral low-grade vesicoureteral reflux.

-Identification of VUR is important because children with concurrent VUR and UPJ obstruction may be at higher risk for severe infection.

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DIFFERENTIAL DIAGNOSIS

• It includes other causes of hydronephrosis.

• Imaging studies differentiate UPJ obstruction from the following conditions:

- Vesicoureteral reflux (VUR)

- Transient hydronephrosis

- Functional hydronephrosis

- Other urological anomalies including posterior urethral valves, congenital megaureter, ureterocele, and multicystic dysplastic kidney

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FOLLOW-UP

• U/S on day 2 - 3 of life Persistent hydronephrosis .

• VCUG to evaluate PUV or VUR• Prophylactic antibiotics if VUR present • No PUV or VUR - repeat U/S and diuretic renal

scan at 1 month• Continued hydro - surgery vs. observation• observation - U/S and/or renal scan every 3-4

months for 1 year and then every 4-6 months• surgery - open/endopyelotomy/laparoscopy

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MANAGEMENT

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Conservative• Principles:

– 50% of antenatal hydro resolved postpartum – unable to accurately diagnose true

obstruction– observations that asymptomatic

hydronephrosis can resolve spontaneously

• Studies with infants with renal function >35-40% in the affected kidney and variable washout patterns– “Rule of 1/3” - 1/3 stay the same, 1/3

improve, 1/3 worsen

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Indications for Surgical Intervention

• Presence of symptoms associated with the obstruction

• Impairment of overall renal function

• Progressive impairment of ipsilateral function

• Development of stones or infection

• Hypertension

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Surgical• Open Pyeloplasty

– Gold Standard– Dismembered pyeloplasty is the most

common

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• Foley V-Y-Plasty – Good for 1-2 cm obstruction– Best for high inserting ureter– Best with relatively small pelvis

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• Spiral flap– Good for long obstructions (better in

adults)– Length of flap limited only by size of

pelvis• (keep length: width at 3:1)• good when UPJ angle > 90

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• Endopyelotomy

– Antegrade or retrograde– Cold knife or electric current– Acucise is very popular

• dilation balloon with hot wire

– 86% success in adults– Slightly less effective in children– Direct vision antegrade approach is

most common

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• Laparoscopic pyeloplasty– Same indications as open or

endourologic procedures– Dismembered pyeloplasty is most

common procedure performed• Without crossing vessels, may do any

number of flap procedures• Up to 94% success rate, similar to open

pyeloplasty

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Any Question ?Any Question ?

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REFERENCES

- UpToDate (press on the title )

- Department of Urology Section of Pediatric Urology (University of Oklahoma ) (press on the title )

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Thank you