|IMAGES IN NEPHROLOGY
|Year : 2007 | Volume
| Issue : 1 | Page : 39-40
A case of retroperitoneal fibrosis
A Lal, M Singhal
Department of Radiodiagnosis, PGIMER, Chandigarh, India
Department of Radiodiagnosis, PGIMER, Chandigarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Lal A, Singhal M. A case of retroperitoneal fibrosis. Indian J Nephrol 2007;17:39-40
A 45-year-old lady presented to the PGIMER outpatient department with complaints of vague discomfort in the abdominal and back of six months duration. Over the last four to six weeks, she had developed progressive effort intolerance, malaise and loss of appetite. Physical examination revealed elevated blood pressure (160/100 mmHg) and the systemic examination was normal. Laboratory investigations showed mild anemia (hemoglobin 10.5 g/dl), elevated ESR (66 mm/h) and serum creatinine (3.5 mg/dl). Abdominal ultrasound showed normal-sized kidneys and mild dilatation of pelvicalyceal systems and upper ureters on both sides. An ill-defined mass was noted in the retroperitoneal region anterior to the sacrum. A contrast enhanced CT scan of the abdomen was done. Axial images (at the level of renal hilum) showed a homogenous plaque-like rind of soft tissue (arrows) in the retroperitoneum anterior to the aorta and inferior vena cava. On both sides the ureters were encased by this soft tissue causing mild hydronephrosis (*). The soft tissue was not extending behind the aorta and no enlarged lymph nodes were seen. These CT findings are suggestive of retroperitoneal fibrosis (RPF).
Retroperitoneal fibrosis, also known as Ormond disease and chronic periaortitis, is an uncommon condition that can cause extensive fibrosis throughout the retroperitoneum. It is three times more common in men and most commonly occurs in age range of 40-60 years. No etiologic factor can be identified in about 70% patients. Therefore, the term idiopathic RPF is used. Recent evidence suggests that RPF is an autoimmune response to an insoluble lipid called ceroid that has leaked through a thinned arterial wall from atheromatous plaques. Other implicated causes include drugs, abdominal aortic aneurysm, ureteric renal injury, infection, retroperitoneal malignancy, radiation therapy and chemotherapy.
About 8-10% of all cases are due to a severe desmoplastic reaction to infiltrating retroperitoneal metastasis, especially from tumors of the GI tract, breast, lung, prostate, cervix and kidney. This is termed malignant retroperitoneal fibrosis. Retroperitoneal fibrosis is classically centered on the distal aorta and does not extend to the pelvic brim. The ureters are encasesd and displaced medially.
Signs and symptoms of the disease are vague and nonspecific and the diagnosis relies heavily on imaging findings. The clinical manifestations are mainly due to entrapment and obstruction of retroperitoneal structures, most commonly the ureters. The inferior vena cava and iliac vessels can also be affected. The classic form is centered on the distal aorta, does not go below the pelvic brim and encases the ureters and displaces them medially. In more extensive disease the appearance can mimic other entities, including lymphoma and metastatic disease and biopsy is frequently required to make a definitive diagnosis. On sonograms, RPF may appear as a relatively echo-free mass centered on the sacral promontory. Imaging the retroperitoneum may not be possible in obese patients or in those with excessive bowel gas.
The computerized tomography (CT) and magnetic resonance imaging (MRI) highlight the fibrosis in more detail and are the imaging modalities of choice for evaluating the extent of the process. The symmetric distribution and geometric shape are highly suggestive of RPF. The CT typically shows a rind of enhancing soft tissue surrounding but not displacing the aorta. On MRI, on T2-weighted images, RPF may appear hyperintense when active inflammation is present and hypointense in end-stage fibrosis. Inhomogeneous signal intensity on T2-weighted MRI is thought to be more suggestive of malignancy.
Extensive biopsy, with multiple samples taken from the lesion is generally required to distinguish malignant from nonmalignant forms. The prognosis for the nonmalignant form depends on the extent to which other systems are involved, but is generally excellent.
Treatment may be surgical or medical, with the best outcome observed in patients receiving both. Steroids can be used as an adjuvant to surgical ureterolysis. More recently, immunosuppressive drugs, such as azathioprine, cyclophosphamide and tamoxifen, have been used. Surgical approaches include ureterolysis with relocation or reimplantation of the ureters, sometimes combined with omental wrapping of the ureter.
| ~ References|| |
|1.||Jois RN, Gaffney K, Marshall T, Scott DG. Chronic periaortitis. Rheumatology Oxford 2004;43:1441-6. [PUBMED] [FULLTEXT]|
|2.||Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol Clin North Am 1996;34:1259-75. [PUBMED] |
|3.||Vivas I, Nicolas AI, Velazquez P, Elduayen B, Fernαndez-Villa T, Martνnez-Cuesta A. Retroperitoneal fibrosis: Typical and atypical manifestations. Br J Radiol 2000;73:214-22. |
[Figure - 1], [Figure - 2]