Ormond's disease is -

• 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.