Urinary Ascites due to Retroperitoneal Fibrosis: A Case Report

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Acta Radiologica

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Urinary ascites due to retroperitoneal fibrosis: a case report

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Acta Radiologica SRAD-2006-0245.R1 Case Report

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Adults < Subject Matter, CT < Modalities/Techniques, Peritoneum < Structures, Ureters < Structures

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Abstract Urinary ascites in adults is usually secondary to iatrogenic or traumatic injuries. We describe the case of a 69 years old male with abdominal pain and decreased renal function. Ultrasounds showed bilateral hydronephrosis and ascites. CT revealed retroperitoneal fibrosis complicated by rupture of the right ureteropelvic junction and urine extravasation in the perirenal and intraperitoneal space. Delayed scans showed leaking of contrast media from the anterior perirenal space to the peritoneal cavity. In our patient urinary ascites resolved using a double-J stent. Introduction Urinary ascites is the commonest cause of ascites in the newborn (10), but it’s an unusual condition in adults where frequently results from iatrogenic or traumatic urinary tract injuries (6). However urine leaks in the peritoneum, though rarely, may develop as a consequence of obstructive uropathy (2, 5) with disruption of the collecting system. Leakage of urine from an urinoma to the peritoneal cavity can happen by transudation or by direct communication (3). We present a case of urinary ascites secondary to right pielo-ureteral rupture caused by retroperitoneal fibrosis (RPF) diagnosed with computed tomography (CT). Only few reports exist in the literature about ureteral rupture complicating RPF (11, 15), and even less with urinary ascites (2). Case report A 69 years old man presented in the emergency room because of sudden onset of malaise and a swollen abdomen. His past medical history was unremarkable with no history of trauma, previous surgery or malignancy. On clinical examination the patient had abdominal distension with tenderness and guarding. The patient was oliguric and laboratory tests showed elevated serum creatinine (8.0 mg/100 ml). Ultrasonography revealed abundant ascites and bilateral hydronephrosis. CT showed a thick, mildly enhancing, solid tissue encasing the abdominal aorta, the inferior vena cava and both the ureters (Fig. 1), extending from the level of the renal arteries to the origin of the common iliac arteries. Free fluid in the right perirenal space and in the peritoneum was seen. On delayed scans contrast agent leakage at the level of the right ureteropelvic junction was clearly evident. The iodinated material accumulated in the perirenal space and passed in the peritoneum trough a tear of the anterior renal fascia while the right distal ureter was not opacified (Fig. 2, Fig. 3). These finding were consistent with retroperitoneal fibrosis complicated by bilateral hydronephrosis and right ureteropelvic joint rupture with urinoma and urinary ascites. Retrograde JJ stent placement in the right ureter was performed. The urinoma and ascites spontaneously resolved without need of drainage and serum creatinine returned to normal values. The patient then started steroid therapy.

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Discussion Urinary ascites is the commonest form of ascites in the newborn (10) where is usually the result of obstructive uropathy. Obstruction leads to disruption of the urinary collecting system and urinoma formation close to the point of laceration. The urine leak can subsequently dissect into the contiguous portions of the retroperitoneum and even extend in the peritoneal cavity if the fascial planes are disrupted (6, 7) or by transudation across the peritoneal membrane (3). In the adult ureteral rupture is usually secondary to trauma or iatrogenic injuries (9, 12) and intraperitoneal passage of urine collections is less common because of the stronger renal fascia (1).

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Acta Radiologica

Obstructive uropathy, though rarely, can result in ureteral rupture in adults because of back pressure or ischemic changes (2, 5). The cause of obstruction can be either stones, ureteral tumors or extrinsic masses like retroperitoneal fibrosis (RPF) as in our patient. This disease is an uncommon (incidence of 1 case per 200,000 population) inflammatory process that can cause extensive fibrosis throughout the retroperitoneum. The etiopathogenesis is unclear and still poorly understood. Only in one third of the patients is possible to find a primary cause like drugs, inflammatory aneurism, metastatic malignancy, radiation therapy or infection, while the vast majority of the cases remain idiopathic. Some authors believe RPF could represent an autoimmune response to atheromasic plaque components. This hypothesis is supported by the common coexistence of RPF with diseases like rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis etc. The typical radiologic appearance of RPF is represented by a fibrous mass surrounding the abdominal aorta, usually between the renal hilar region and the sacral promontory. The inferior vena cava and iliac vessels can also be involved. The fibrosis frequently extends laterally to encase the ureters, resulting in hydronephrosis with medial deviation and smooth extrinsic narrowing of one or both ureters on excretory urography. On ultrasonography RPF may appear as a relatively echo-free mass, though imaging the retroperitoneum is not always possible because of obesity or bowel gas. The fibrosis, its extent and ureters involvement can be shown in more detail with CT. The attenuation value of the mass is similar to that of muscle and shows mild to moderate enhancement depending on the stage of the disease.

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Ureteral rupture rarely complicates RPF because of the gradual onset of obstruction (2), though longstanding hydroureteronephrosis can ultimately lead to pielo-ureteral joint disruption resulting in urinoma (11) and urinary ascites if tears in the fascial planes are present (6). CT is the study of choice in the diagnosis of renal urine leaks and urinomas. Delayed scans (obtained 5–20 minutes after contrast media injection) represent the key to demonstrate enhancement of the free fluid (6, 13). Treatment consists of medical therapy (mainly steroids) to arrest the progression of the disease and endoscopic stent placement or surgical ureterolysis to relieve ureteral obstruction. A simple JJ stent can resolve both hydroureteronephrosis and ureteral leaks and can be preferred to more complex surgical procedures (8) because urine leaks may represent in the akinetic intraperitonealized ureter (15). MRI and PET can be used in the follow-up to differentiate active disease from stabilized residual fibrosis (4, 14). In conclusion, pielo-ureteral rupture and urinary ascites represent uncommon complications of retroperitoneal fibrosis. In the case we described contrast enhanced CT with delayed imaging defined the cause of hydronephrosis and ascites. References

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1. Adzick NS, Harrison MR, Flake AW, de Lorimer AA. Urinary extravasation in the fetus with obstructive uropathy. J Pediatr Surg 1985;20:608–615. 2. Arslan D, Degirmenci B, Aslan G, Ozdogan O, Esen A, Durak H. A False-Negative Diuretic Radionuclide Renography in a Patient with Urinary Ascites due to Retroperitoneal Fibrosis. Urol Int 2003;71:233–234. 3. Beetz R, Stein R, Rohatsch P, Brzezinska R, Thuroff JW. Acute perirenal extravasation of urine in an infant with non-refluxing megaureter Pediatr Nephrol 2004;19:357–360.

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4. Burn PR, Singh S, Barbar S, Boustead G, King CM. Role of gadolinium-enhanced magnetic resonance imaging in retroperitoneal fibrosis. Can Assoc Rodiol J 2002;53:168-70.

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5. Fernandes M, Seebode JJ, Rilli CF. Obstructed solitary pelvic kidney and urinary ascites. J Urol 1993;150:1216–1217. 6. Gayer G, Zissin R, Apter S, Garniek A, Ramon J, Kots E, et al. Urinomas caused by ureteral injuries: CT appearance. Abdom Imaging 2002;27:88–92.

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7. Hirsch M. Enhanced ascites: CT sign of ureteral fistula. J Comput Assist Tomogr 1985;9:825–826.

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8. Ilie CP, Pemberton RJ, Tolley DA. Idiopathic retroperitoneal fibrosis: the case for nonsurgical treatment. BJU Int 2006;98:137-140. 9. Kawashima A, Sandler CM, Comere JN Jr, Rodgers BM, Goldman SM. Ureteropelvic Junction Injuries Secondary to Blunt Abdominal Trauma. Radiology 1997;205:487-492. 10. Kay R, Brereton RJ, Johnston JH. Urinary ascites in the newborn. Br J Urol 1980;52:451–454. 11. Sakai Y. A case of idiopathic retroperitoneal fibrosis with renal subcapsular urinoma resolved by steroid therapy. Hinyokika Kiyo 1999;45:249-51. 12. Selzman AA, Spirnak JP. Iatrogenic ureteral injuries: a 20-year experience in treating 165 injuries. J Urol 1996;155:878-81. 13. Titton RL, Gervais DA, Hahn PF, Harisinghani MG, Arellano RS, Mueller PR. Urine Leaks and Urinomas: Diagnosis and Imaging-guided Intervention. RadioGraphics 2003;23:1133–1147. 14. Vaglio A, Greco P, Versari A, Filice A, Cobelli R, Manenti L, et al. Post-treatment residual tissue in idiopathic retroperitoneal fibrosis: active residual disease or silent "scar" ? A study using 18Ffluorodeoxyglucose positron emission tomography. Clin Exp Rheumatol 2005;23:231-234. 15. Valero Puerta JA, Medina Perez M, Valpuesta Fernandez I, Sanchez Gonzalez M. Rupture of kidney pelvis in retroperitoneal fibrosis. Arch Esp Urol. 1999;52:269-271.

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Figure Legends Fig. 1. Contrast enhanced CT scan, portal phase. Mildly enhancing periaortic tissue is seen. Fluid is present in the perirenal space and in the peritoneum. Fig. 2. Contrast enhanced CT scan, delayed phase (20 minutes). An urinoma is seen at the level of the right pielo-ureteral joint. The extravasated contrast media is seen passing directly from the perirenal space to the peritoneal cavity trough a defect of the anterior renal fascia. Fig. 3. Contrast enhanced CT scan, delayed phase (20 minutes). Scan at a lower level than Fig.2. The not opacified distal ureter is seen surrounded by contrast material. The thick fibrous plaque encasing the aorta is also noted.

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Fig. 1. Contrast enhanced CT scan, portal phase. Mildly enhancing periaortic tissue is seen. Fluid is present in the perirenal space and in the peritoneum.

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Fig. 2. Contrast enhanced CT scan, delayed phase (20 minutes). An urinoma is seen at the level of the right pielo-ureteral joint. The extravasated contrast media is seen passing directly from the perirenal space to the peritoneal cavity trough a defect of the anterior renal fascia.

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Fig. 3. Contrast enhanced CT scan, delayed phase (20 minutes). Scan at a lower level than Fig.2. The not opacified distal ureter is seen surrounded by contrast material. The thick fibrous plaque encasing the aorta is also noted.

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