Dr. med. Dirk Manski

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Retroperitoneal fibrosis (Morbus Ormond)

Review literature: (Vaglio et al, 2006).

Definition of Retroperitoneal Fibrosis

Ormond 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

Prevalence 1–2/100.000, age of onset 40–60 years, male to female ratio = 2:1.

Causes (Etiology) of Retroperitoneal Fibrosis

Unknown Etiology

In 70% of patients with retroperitoneal fibrosis, the etiology of Ormond 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. There is also evidence of a local manifestation of a systemic increased willingness to develop autoimmune diseases (see below association with fibrotic diseases).

Known Triggers

Drugs:

Ergot alkaloids, β-blocker, phenacetin, LSD, haloperidol, amphetamine, reserpine.

Infections:

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.

Other risk factors:

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

Diagnostic Work-up of Retroperitoneal Fibrosis

Laboratory tests:

Elevated erythrocyte sedimentation rate (ESR) and CRP. Further important tests are creatinine, electrolytes, full blood count, autoantibodies (ANA, ANCA), immunoelectrophoresis, rheumatoid factor, in men PSA, AFP, HCG, LDH.

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 thickness of the renal parenchyma.

Intravenous urogram:

Signs of hydronephrosis on both sides. Displacement of the proximal and mid ureter to the middle. IVP should only be performed if cross-sectional imaging is not available.

Retrograde pyelography:

Retrograde pyelography is indicated in renal insufficiency and before endoscopic therapy. Radiological signs are similar as in intravenous urogram, an intrinsic ureteral obstruction can easily be excluded.

Renal Scintigraphy:

To determine spit renal function and to objectify urinary obstruction at the onset and later in the course of the disease.

Percutaneous Biopsy:

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, less common are carcinoid, multiple myeloma, pancreatic cancer, prostate cancer metastases, sarcoma or urogenital tuberculosis.

Treatment of Retroperitoneal Fibrosis

Acute treatment of hydronephrosis

Retrograde pyelography and placement of DJ ureteral stents.

Medical Treatment of Retroperitoneal Fibrosis

Steroid therapy with prednisolone 1 mg/kg body weight per day for two days, then reduction of the daily dose to 40 mg, 20 mg, 10 mg every two weeks, maintenance dose 5 mg per day for one year (Brandt et al, 2016). Depending on the response and side effects, immunosuppressants such as azathioprine and mycophenolic acid are combined. Treatment success has also been reported with tamoxifen. Steroid therapy is particularly effective in patients with active signs of inflammation (elevated ESR, leukocytosis).

Secondary retroperitoneal fibrosis:

Diagnosis and therapy of possible causes, see etiology known triggers.

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 mass 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.

Ureter reconstruction:

Depending on the manifestation of the disease and involvement of the ureter, the ureter must be replaced segmentally or completely. The following techniques are used: Boari flap, intestinal interposition or autologous kidney transplantation, see section ureteral stricture.

Follow-Up

Retroperitoneal fibrosis is a chronic relapsing disease, lifelong follow-up care is necessary (ESR, CRP, sonography of the kidneys and possibly CT/MRI every 1–2 years after discontinuation of immunosuppression).





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References

Vaglio u.a. 2006 VAGLIO, A. ; SALVARANI, C. ; BUZIO, C.:
Retroperitoneal fibrosis.
In: Lancet
367 (2006), Nr. 9506, S. 241–51



  Deutsche Version: Morbus Ormond: retroperitoneale Fibrose