General Abdomen: Retroperitoneal Fibrosis

Causes

• Primary/Idiopathic (Most Common)
• Secondary – Drugs, Malignancy, Infection, Radiation or Surgery
  • Most Common Drug: Methysergide (Sansert) – Ergot Alkaloid Used for Migraines

Presentation

• Pain (Most Common Symptom)
• Trapped Ureters – Deviated Medially
  • Lateral Deviation More Likely Related to Malignancy
• Renal Impairment/Hydronephrosis
• Lymph Obstruction
• Claudication or Mesenteric Ischemia

Diagnosis

• Dx: CT
• Require Bx if Atypical or Concern for Malignancy
• Rarely Used: IV Urography or Pyelography

Treatment

• Management of Fibrosis:
  • Idiopathic Cause: Steroids/Immunosuppression
  • Secondary Cause: Treat Underlying Etiology
• Management of Obstruction/Renal Impairment:
  • Primary Treatment: Stent vs. Surgery (Ureterolysis & Wrap Ureter in Omentum)
  • If Active Infection Present: Consider Percutaneous Nephrostomy with Delayed Definitive Surgery