Ormond’s disease is –
Correct Answer: Idiopathic retroperitonial fibrosis
Description: • 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.
Category:
Surgery
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now