Which of the following statements about idiopathic retroperitoneal fibrosis is correct?
|A||It occurs most frequently in males|
|B||The condition usually presents initially as an asymptomatic mass found during a routine physical examination|
|C||Duodenal obstruction is the most common complication of the process|
|D||Fine needle biopsy under fluoroscopic control is the best study to confirm the diagnosis|
a. Retroperitoneal fibrosis is an uncommon inflammatory condition characterized by the proliferation of fibrous tissue in the retroperitoneum.
b. Seventy percent of cases are idiopathic (termed Ormand disease), whereas 30% are associated with various drugs (most notably, ergot alkaloids or dopaminergic agonists), infections, trauma, retroperitoneal hemorrhage or retroperitoneal operations, radiation therapy, or primary or metastatic neoplasms.
c. Many of the idiopathic cases are associated with inflammatory abdominal aortic aneurysms or vasculitis syndromes.
d. The fibrosis is usually confined to the central and paravertebral spaces .
e. The process usually begins at the level of the aortic bifurcation and spreads cephalad.
f. Patients present with a vague constellation of symptoms, including abdominal or flank pain, weight loss, malaise, and hypertension.
g. Scrotal or leg edema caused by lymphatic obstruction may also be present.
h. Laboratory tests often provide evidence of renal insufficiency and anemia.
i. Other laboratory abnormalities include an elevated erythrocyte sedimentation rate and an elevated C-reactive protein level.
j. The diagnosis is based on the patient’s history and IV urography demonstrating hydronephrosis and hydroureter associated with delayed excretion and medial deviation of the ureters.
k. Most commonly, the disease is bilateral, although unilateral cases do occur.
l. CT without IV contrast & MRI are diagnostic.