What is Dietl's crisis?

Dietl's Crisis: Diagnosis and Management

Dietl's crisis is an intermittent ureteropelvic junction obstruction characterized by episodes of severe colicky abdominal pain associated with temporary hydronephrosis that resolves spontaneously after the episode. This condition is often underdiagnosed, leading to significant delays in proper treatment.

Clinical Presentation

• Recurrent episodes of severe colicky abdominal pain, often accompanied by nausea and vomiting 1, 2
• Pain may be present for many years intermittently 3
• Abdominal distension may occur during episodes 4
• Symptoms typically resolve spontaneously after the passage of large amounts of urine 4
• Often misdiagnosed initially, with patients suffering for a year or more before correct diagnosis 2
• More common in males than females 2

Pathophysiology

• Caused by intermittent obstruction at the ureteropelvic junction (UPJ) 5
• Most common causes include:
  • Aberrant renal vessels at the lower pole causing external compression 4, 6
  • High insertion of the ureter at the renal pelvis 4
• The obstruction leads to acute hydronephrosis with stretching of the renal pelvis, resulting in pain 4
• The intermittent nature of the obstruction makes diagnosis challenging 1

Diagnostic Approach

• Imaging during an acute episode is crucial for diagnosis 6
• Ultrasound during an episode typically shows hydronephrosis that resolves on follow-up imaging 1
• Magnetic Resonance Urography (MRU) can demonstrate:
  • Hydronephrosis
  • Delayed calyceal transit time
  • Delayed time-to-peak enhancement
  • Delayed excretion 1
• Diuretic renography may show various patterns:
  • Cortical retention (pathognomonic finding)
  • Obstructed scan (T1/2 ≥ 20 minutes)
  • Initially non-diagnostic scan that becomes diagnostic on subsequent imaging 5
• Intravenous urography during an episode can show obstruction at the ureteropelvic junction 6

Differential Diagnosis

• Non-specific abdominal pain 3
• Gastrointestinal disorders 6
• Biliary colic 6
• Other causes of intermittent abdominal pain 2

Management

• Surgical intervention with pyeloplasty is the definitive treatment 1, 2
• Pyeloplasty provides complete symptomatic resolution in most cases 1, 2
• Significant improvement in renal function can be observed post-surgery, even in kidneys with initially poor function 5, 4
• Patients with cortical retention on renography show the most significant recovery of function after pyeloplasty (mean differential renal function change of 13.59%) 5

Prognosis

• Excellent prognosis with appropriate surgical intervention 2
• Resolution of abdominal symptoms occurs following pyeloplasty 2
• Renal parenchyma is typically preserved despite the intermittent obstruction 2
• Without treatment, patients may suffer from recurrent episodes for years 6

Clinical Pearls

• Consider Dietl's crisis in patients with recurrent abdominal pain, particularly in males 2
• The key to diagnosis is imaging during an acute attack 6
• Children with periumbilical pain and vomiting would benefit from ultrasound imaging 2
• Renal function may appear significantly compromised during an acute episode but can improve dramatically after resolution 4
• Detailed pain history and timely cross-sectional abdominal imaging during an attack are crucial for diagnosis 6