What is the treatment for congenital Pelvic Ureteric Junction (PUJ) obstruction?

Treatment of Congenital Pelvic Ureteric Junction (PUJ) Obstruction

Most patients with congenital PUJ obstruction can be managed conservatively with observation and serial imaging, with surgical intervention reserved for those with deteriorating renal function, increasing hydronephrosis, or symptoms. 1

Diagnosis and Classification

• Congenital PUJ obstruction is one of the most common causes of urinary tract dilation (UTD) detected prenatally 1
• It falls under the category of obstructive uropathies in the classification of causes of urinary tract dilation 1
• Diagnosis is typically made through prenatal ultrasound and confirmed postnatally 1

Management Approach

Conservative Management

• The majority of patients with PUJ-like UTD will not need surgical correction and can be observed with serial imaging 1
• Conservative management includes:
  • Regular ultrasound monitoring to assess degree of hydronephrosis 1
  • Renal function assessment through nuclear medicine studies (diuretic renography) 2
  • Monitoring for symptoms such as pain, urinary tract infections, or hematuria 3

Indications for Surgical Intervention

Surgical intervention (pyeloplasty) is indicated in the following scenarios:

• Differential renal function <40% on nuclear medicine scan 2
• Renal pelvic diameter >35 mm 2
• Progressive deterioration in renal function on serial studies 2, 3
• Increasing hydronephrosis on serial ultrasound examinations 1
• Development of symptoms (pain, recurrent infections) 3
• An antenatal anterior-posterior renal pelvic diameter (APD) cutoff of 15 mm has been identified as predictive of the need for intervention 1

Surgical Options

1. Open Pyeloplasty:

   • Traditional approach with excellent success rates (>95%) 3, 4
   • Anderson-Hynes dismembered pyeloplasty is the most common technique 3
   • Heineke-Mirhulicz pyeloplasty is an alternative technique 3

2. Laparoscopic Pyeloplasty:

   • Increasingly used with success rates comparable to open surgery (93-97%) 5
   • Benefits include shorter hospital stay and improved cosmesis 5
   • Technically feasible even in infants, though operative time is longer than open surgery 5
   • Typically uses 3-port technique with JJ stent placement 5

3. Endoscopic Management:

   • Endopyelotomy may be an option for mild to moderate obstruction without complex anatomy 6
   • Less commonly used in primary congenital cases 6

Special Considerations

Antibiotic Prophylaxis

• Continuous antibiotic prophylaxis (CAP) may be considered for patients with high-risk features 1:
  • Female gender 1
  • Intact foreskin with moderate to severe UTD 1
  • Severe UTD (P3) 1
  • Distal ureteral dilation ≥7mm 1
  • Associated vesicoureteral reflux 1
  • However, data from the SFU Hydronephrosis Registry showed no benefit for CAP in isolated UPJ-like hydronephrosis 1

Bilateral PUJ Obstruction

• Requires more aggressive management due to higher risk of renal impairment 3
• May require preliminary drainage procedures (double J stents, nephrostomies) to recover renal function before definitive surgery 3
• Hemodialysis may be needed in cases with significant renal impairment 3

Duplex Systems with PUJ Obstruction

• Requires specialized surgical approach 7
• Options include pyelo-ureterostomy, lower pole pyeloplasty, or ipsilateral uretero-ureterostomy depending on the anatomy 7
• Laparoscopic reconstruction is feasible with good outcomes 7

Follow-up Protocol

• Regular ultrasound monitoring (every 3-6 months initially, then annually) 2
• Diuretic renography to assess drainage and differential renal function 2
• Clinical monitoring for symptoms 3
• Post-surgical follow-up should include ultrasound at 1-3 months and nuclear scan at 6 months 5

Pitfalls and Caveats

• Avoid dismissing mild hydronephrosis without proper follow-up as some cases may progress 1
• Be cautious with watchful waiting in cases with borderline renal function, as further deterioration may occur 2
• Ensure proper stent placement during surgery to prevent complications 5
• Monitor for post-operative complications including urinary tract infections (most common), anastomotic leakage, and stent-related issues 3, 5