<s>[INST] context: • Uncommon inflammatory condition characterized by the proliferation of fibrous tissue in the retroperitoneum
• The fibrosis is usually confined to the central and paravertebral spaces between the renal arteries and sacrum and tends to encase the aorta, IVC and ureters.
• The process usually begins at the level of the aortic bifurcation and spreads cephalad upto renal artery generally.
Etiology
• Around 70% cases are primary or idiopathic (Ormond’s disease)
Causes of Secondary (30%)Q Retroperitoneal Fibrosis
• Inflammatory conditions: CATH (Chronic pancreatitis, Actinomycosis, Tuberculosis, Histoplasmosis)
• Drugs: Methylsergide (Most important), methyldopa, hydralazine, entacapone, beta-blockers, bromocriptine, phenacetin, amphetamines (MAHE-BP)
• Malignancies: CA prostate, NHL, CA stomach, sarcoma and carcinoid tumor
• Autoimmune disorders: SLE, PAN and ankylosing spondylitis
• Radiation
Clinical Features
• More common in males of 40-60 years.
• Early symptoms are vague and non-specific (abdominal or flank pain, weight loss, malaise, and hypertension)
• Obstructive uropathy (dysuria, frequency, fever due to secondary infection of hydroneprotic kidney) is the earliest and MC specific symptom.
• Ureters are MC involved, MC site is lower third of ureter.
• Partial or complete obstruction occurs in 75% patients
Diagnosis: In absence of uremia, diagnosis is made by IVP.
• IVP or RGP:
−− Hydronephrosis with dilated tortuous upper ureter,
−− Medial pulling of ureters or pipestem ureters
−− Extrinsic ureteral compression
• CT scan is IOC for retroperitoneal fibrosis.
• MRI is IOC in cases of compromised renal function, because contrast cannot be given.
Treatment
• Primary, idiopathic retroperitoneal fibrosis: Ureteral stenting and immunosuppression (TAPS: Tamoxifen, Azathioprine, Penicillamine, Steroids)
• Secondary retroperitoneal fibrosis: Midline transperitoneal ureterolysis with wrapping the ureter with omental flap or lateral retroperitoneal ureteral transposition.
TAPS: Tamoxifen, Azathioprine, Penicillamine, Steroids are used in primary retroperitoneal fibrosis. [/INST] question: Ormond's disease is - | options: A: Idiopathic lymphadenopathy B: Retractile testis C: Idiopathic retroperitonial fibrosis D: Idiopathic mediastinitis | answer: C </s>