Retroperitoneal fibrosis (Morbus Ormond)
Review literature: (Vaglio et al, 2006).
Definition of Retroperitoneal Fibrosis
Ormond's disease (Retroperitoneal fibrosis) is a disease with unknown etiology, which leads to increasing fibrosis of the retroperitoneum and compression of ureters and retroperitoneal vessels.
Epidemiology of Retroperitoneal Fibrosis
40–60 years, male:female = 2:1.
Causes (Etiology) of Retroperitoneal Fibrosis
In 70% of patients with retroperitoneal fibrosis, the etiology of Ormond's disease is unclear. One assumed mechanism of disease is an autoimmune process with periaortitis as the initial pathophysiological mechanism. Later, the inflammatory-fibrotic mass extends into the retroperitoneum and compresses the ureters.
Known Causes and Risk factors of Retroperitoneal Fibrosis
Ergot alkaloids, β-blocker, phenacetin, LSD, haloperidol, amphetamine, reserpine.
Lymphangitis, chronic inflammatory bowel disease, gonorrhea, syphilis, urogenital tuberculosis, chronic urinary tract infection, sarcoidosis.
Association with fibrotic diseases:
Sclerosing mediastinitis, sclerosing cholangitis, orbital pseudotumor, Riedel's thyroiditis. In children association with systemic lupus erythematosus or juvenile rheumatoid arthritis.
Irradiation, retroperitoneal bleeding, pelvic surgery, Purpura Hennoch.
Pathology of Retroperitoneal Fibrosis
Retroperitoneal fibrosis leads a strong mass of connective tissue, which surrounds the large retroperitoneal vessels and may also include the ureter into the mass. The retroperitoneal mass extends in craniocaudal direction from the renal hilum to the sacral promontory. At the edges of the active fibrosis, there are signs of inflammation in the connective tissue (lymphocytes, plasma cells, histiocytes...). In the center of the mass, inactive connective tissue is found without signs of inflammation.
Signs and Symptoms
- Flank pain: constant dull pain, starting in the flanks with radiating to the lower abdomen.
- Nonspecific symptoms such as weight loss, malaise, nausea and vomiting may be present at the beginning of the inflammatory process. Lymphadenopathy is not typical.
- Late in the course of disease, symptoms of uremia may develop.
Diagnostic Work-up of Retroperitoneal Fibrosis
Elevated erythrocyte sedimentation rate (ESR). Further important tests are creatinine, electrolytes and blood count.
Signs of hydronephrosis on both sides. Displacement of the proximal and mid ureter to the middle.
Retrograde pyelography is specially indicated in renal insufficiency or other contraindications for intravascular contrast medium. Radiological signs are similar as in intravenous urogram, an intrinsic ureteral obstruction can easily be excluded.
CT or MRI of the Abdomen:
Abdominal imaging reveals a flat mass which surrounds the great vessels and the ureters with bilateral hydronephrosis. Furthermore, renal function or damage can be estimated with the help of the renal parenchyma thickness.
Renal scintigraphy is indicated for the determination of the renal clearance and for the objectivation of obstructive uropathy. Renal scintigraphy should be done after diagnosis of retroperitoneal fibrosis and later in the course of the disease, if loss of renal function is suspected.
Biopsy of the retroperitoneal mass should be done to exclude a malignant etiology, especially with suspected retroperitoneal lymphoma (pathological blood count, lymphadenopathy) or cancer in the history. With typical clinical and radiological criteria of retroperitoneal fibrosis, it is controversial, whether a biopsy is necessary before medical treatment.
Differential Diagnosis of Retroperitoneal Fibrosis
The most common differential diagnosis is lymphoma. Rare differential diagnoses are carcinoid, multiple myeloma, pancreatic cancer, prostate cancer metastases, sarcoma, urogenital tuberculosis.
Treatment of Retroperitoneal Fibrosis
Acute Decompression of Obstructive Uropathy
Initial treatment of retroperitoneal fibrosis after retrograde pyelography is the decompression of hydronephrosis with insertion of DJ ureteral stents.
Medical Treatment of Retroperitoneal Fibrosis
Cornerstone of medical treatment is immunosuppression with steroid therapy, possibly also other immunosuppressants such as azathioprine and mycophenolate mofetil. In patients with signs of active inflammation (elevated erythrocyte sedimentation rate, leukocytosis), the steroid therapy is especially effective. Response to tamoxifen therapy has been reported.
Surgical Treatment of Retroperitoneal Fibrosis
The surgical treatment (ureterolysis and intraperitoneal transfer of the ureter) is indicated for failure of medical treatment or if diagnosis by biopsy is unclear. Nephrectomy is indicated for a nonfunctioning kidney.
Technique of the ureterolysis:
Preoperatively, new DJ stents are inserted. After a median laparotomy and incision of both paracolic gutters, the colon is mobilized medially on both sides to expose the retroperitoneum. Biopsies from the retroperitoneal fibrosis are sent for frozen section. The ureters are freed from the retroperitoneal mass, the dissection is best done from distal to proximal. After complete mobilization of the ureters, they are relocated to intraperitoneal cavity, and the peritoneum is closed again. The hiatus of the peritoneum for the ureters must be large enough to prevent iatrogenic ureteral compression. Omentum majus can be wrapped around the ureters for a safe separation to the retroperitoneal fibrosis. Alternatively, the ureters are left in the retroperitoneum lateral to the fibrotic mass.
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Vaglio u.a. 2006 VAGLIO, A. ; SALVARANI, C. ;
- Retroperitoneal fibrosis.
367 (2006), Nr. 9506, S. 241–51