Retroperitoneal fibrosis is a rare condition characterized by the development of fibrous plaques in the retroperitoneal space. The fibrous plaques characteristically arise distal to the bifurcation of the abdominal aorta and progress to encase the iliac vessels distally and are defined by the associated encasement of one or both ureters. Imaging plays an important role in not only establishing the diagnosis, but also in monitoring disease progression. Historically, the radiological diagnosis was made predominantly by intravenous urography and retrograde pyelography. More recently, advances in cross-sectional imaging with ultrasound and contrast-enhanced CT have allowed for a more precise diagnosis as well as helping to accurately define the extent of the disease. At our institution, we have found ultra-fast MRI to also play a useful role in establishing the diagnosis. In particular, magnetic resonance urography using HASTE (half Fourier-acquired single shot turbo spin-echo) sequences allow a safe alternative to intravenous urography, particularly in patients with poor renal function. The purpose of this article is to describe the role of the various imaging methods available to the radiologist and to emphasize the important role that the interventional radiologist now plays, not only in obtaining tissue for diagnosis, but also in providing treatment of the disease by percutaneous nephrostomy drainage and subsequent stent placement in select cases.
Pneumatosis Intestinalis in association with portal venous gas is a very rare finding in children and young adults. When present, it is typically associated with bowel infarction and carries a poor prognosis. We present an extremely unusual case where imaging revealed extensive pneumatosis intestinalis and portal venous gas in a patient with acute appendicitis.
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