What is the management approach for a patient with a corkscrew ureter?

Management of Corkscrew Ureter

For a patient with symptomatic corkscrew ureter causing obstruction and hydronephrosis, laparoscopic dismembered pyeloplasty is the definitive surgical treatment, while initial management of acute obstruction requires urgent urinary decompression via retrograde ureteral stenting or percutaneous nephrostomy. 1

Clinical Presentation and Diagnosis

Corkscrew ureter (spiral ureteral deformity) is a rare congenital anomaly that typically presents with:

• Flank pain and significant hydronephrosis when symptomatic 1
• Characteristic spiral configuration of the proximal ureter on retrograde ureterography 1
• Most cases are asymptomatic and discovered incidentally 1

Acute Management of Obstruction

When a corkscrew ureter presents with acute obstruction, immediate urinary decompression is essential:

First-Line Decompression Options:

• Retrograde ureteral stenting is the preferred initial approach if technically feasible 2
• Percutaneous nephrostomy (PCN) should be performed if retrograde stenting fails or is not possible due to the anatomic distortion 2
• If infection is present (obstructive pyelonephritis), immediate drainage is mandatory as antibiotics alone are insufficient and mortality reaches 40% without drainage 3

Antibiotic Coverage:

• Initiate third-generation cephalosporin (ceftazidime) as first-line empiric therapy if infection is suspected, as it demonstrates superior outcomes compared to fluoroquinolones 2, 3
• Adjust based on culture results at 48-72 hours 3

Definitive Surgical Management

Laparoscopic Dismembered Pyeloplasty:

This is the definitive treatment for symptomatic corkscrew ureter with obstruction 1

Technical approach:

• Laparoscopic transperitoneal approach allows excellent visualization 1
• Dismembered pyeloplasty straightens the spiral configuration and relieves the ureteropelvic junction obstruction 1
• This represents the first reported successful laparoscopic reconstruction for this specific anomaly 1

Alternative Surgical Options (if pyeloplasty not feasible):

• For longer segment involvement (2-3 cm), primary ureteroureterostomy may be considered 4
• Kidney mobilization techniques can bridge defects when direct anastomosis is not possible 4
• Ureteral stenting is necessary during any reconstruction to preserve renal function 5

When Conservative Management May Be Appropriate

Asymptomatic corkscrew ureters without obstruction or hydronephrosis typically lack clinical significance and require only observation 1

Monitor for:

• Development of flank pain 1
• Progressive hydronephrosis on follow-up imaging 2
• Recurrent urinary tract infections 5

Common Pitfalls to Avoid

• Do not attempt endoscopic management alone for symptomatic corkscrew ureter with significant anatomic distortion, as the spiral configuration requires surgical reconstruction 1
• Do not delay drainage if obstruction is present, even if planning definitive surgery later, as this risks irreversible renal damage 2
• Ensure a safety guidewire is placed during any endoscopic intervention to facilitate rapid re-access if complications occur 6
• If retrograde stenting fails completely, immediately proceed to PCN rather than repeated attempts that may cause further trauma 7

Follow-Up Requirements

After definitive surgical repair:

• Repeat imaging at 5-9 days post-procedure to confirm resolution of obstruction 3
• Monitor renal function at 1,3, and 6 months 3
• Long-term surveillance for stricture formation at the anastomosis site 4