Thursday , 5 December 2024

Review on Retroperitoneal Fibrosis: Ormond’s Disease

V. Sai Chitra Prathyusha*, Ramesh Dhani
Ratnam Institute of Pharmacy, Pidathapolur, Muthukur, SPSR Nellore, Andhra Pradesh, India.

A B S T R A C T
Ormond’s disease is also known as retroperitoneal fibrosis & it is a rare disease of unknown etiology, characterized by inflammation and fibrosis of the retro peritoneum resulting in compression and encasement of the ureter, and/ or the retro peritoneal blood vessels.  RPF may be Primary /idiopathic (most common) or secondary (eg., drug-reduced, inflammatory iatrogenic) patients often present with not-specific symptoms (eg., fever, malaise, weight loss, flank pain, etc) Bilateral apteral obstruction, with subsequent hydro nephritis and obstructive nephropathy, is common.  Diagnosis is often suspected in patents who present with bilateral hydro nephrosis of unknown etiology. Contrast CT is the diagnostic test of choice and reveals a retriperitoneal mass encasing and obtstructing the ureters and/or the aorta and IVC diagnosis is confirmed on CT guided biopsy of the mass High dose glucocorticoids are the mainstay of treatment of primary RPF secondary  RPF is managed by treating the underlying cause (stopping the offending drug, treating the infection, etc) Symptomatic/severe obstruction of the retroperitoneal structures require treatment cureteric stenting, uteceric stenting, ureterolysis, arterial stenting etc) prognosis of non-malignancy – induced RPF is good, but recurrence rates are high (70%).
Keywords: Retroperitoneal fibrosis, bilateral hydro nephrosis, glucocorticoids, IVC diagnosis, flank pain.

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