What Is Retroperitoneal Fibrosis?

Retroperitoneal fibrosis, also known as idiopathic retroperitoneal fibrosis, refers to chronic non-specific inflammation of the fascia and adipose tissue behind the peritoneum, which gradually evolves into fibroproliferative diseases, and the lesions can develop into the abdominal cavity, pelvic cavity and other tissues. Compression of the cavity organs behind the peritoneum causes obstruction.

Basic Information

English name

idiopathic retroperitoneal fibrosis

Visiting department

Basic surgery

Multiple groups

Middle-aged man

Common locations

Retroperitoneum

Common causes

Autoimmune response, ergot alkaloids as haptens cause allergies or autoimmune reactions, taking drugs such as pain medication

Common symptoms

Back pain, abdominal pain, and abdominal pain, anorexia, weight loss, and fatigue

Causes of Idiopathic Retroperitoneal Fibrosis

The etiology of this disease is unknown and may be related to the body's non-specific response to certain chronic infections and stimuli. It may also be related to autoimmune response, genetic factors, and so on.

Clinical manifestations of idiopathic retroperitoneal fibrosis

It can occur at any age and even newborns, but it is more common in middle-aged people. Men are twice as likely as women. Onset is usually insidious and has a long course. Early manifestations of blunt pain on both sides of the lower abdomen, mostly blunt pain and discomfort on the outside of the lower abdomen, lumbosacral or lower abdomen. Other symptoms include anorexia, weight loss, and fatigue. Swelling of one or both legs, swelling of the scrotum, or moderate fever may occasionally touch the abdomen or pelvic mass. Clinical manifestations in the advanced stage are often compression or involvement of adjacent organs. If ureteral stenosis can cause proximal infection or dilation, it can cause pain in the waist or rib spine, frequent urination and nocturia; bilateral ureteral compression is sudden. Anuria occurs; waist tenderness is common due to frequent hydronephrosis or kidney infections. Hypertension is common (one of the causes of headaches), and it is mostly caused by renal obstruction. Gastrointestinal symptoms can be related to uremia or direct damage to the gastrointestinal tract, such as stenosis. It has been reported that biliary and pancreatic duct stenosis, if involving the portal vein or splenic vein, can cause portal hypertension, esophageal and gastric fundus varices, and peritoneal effusion. The posterior peritoneal lymph, vein or arterioles are compressed or obstructed, and swelling of one or both legs, swelling of the penis, or scrotal edema may occur. There may even be filling or varicose veins in the abdominal wall, thrombosis of the lower limbs, weak pulses at the lower extremities, intermittent claudication . May be accompanied by fibrosis in other parts (such as the mediastinal bile ducts), and even sclerosing cholangitis, Peyronie disease (Peronie disease, scleroderma of the penis, fibrous painful penile erection, fibrous cavernitis), etc. .

Idiopathic retroperitoneal fibrosis

Laboratory inspection

Frequently, the erythrocyte sedimentation rate increases, anemia and leukocyte counts increase in varying degrees, occasionally eosinophils increase, and protein electrophoresis and globulin are added. Routine urine tests can be normal or have a small amount of white blood cells and red blood cells, and uremia may be present at a later stage. Therefore, patients with uremia should pay attention to whether it is caused by retroperitoneal fibrosis when the urine is normal.

2.X-ray inspection

X-ray urography shows the displacement of one or both sides of the ureter. The diagnostic manifestation is the tapering of the middle ureter with segmental stenosis, which is different from the narrowing caused by tumors or stones, which does not taper And only irregular narrow. Double-contrast X-ray imaging of the digestive tract may reveal that the affected intestine, such as the duodenum, has segmental stenosis, pelvic fibrosis can cause rectal stricture and straightening, and the bladder is elevated in teardrop shape. Venography can show inferior vena cava or iliac vein stenosis.

3.CT and MRI

Most can find fibrous plaques or abnormal soft tissue masses, and enhanced scans show thicker fibrous tissue signs. As fibrosis develops laterally, changing the angle between the aorta and the left psoas muscle, the inferior vena cava, and the right psoas muscle can also show proximal ureteral dilatation. Because magnetic resonance is not superior to CT in displaying fibrous plaques, but it can show changes in blood vessel flow velocity. Therefore, CT examination is preferred, and magnetic resonance is selected when further understanding of hemodynamic changes is needed.

4. Type B ultrasound

PRF masses are hypoechoic or non-echoic and have no characteristic manifestations. Can still observe the degree of urinary tract obstruction and hydronephrosis.

Idiopathic retroperitoneal fibrosis diagnosis

The diagnosis of this disease focuses on strengthening awareness and alertness. Those who regularly take painkillers, medrocrine, and other drugs with unexplained back pain, abdominal pain, dull pain in the lumbosacral or lower abdomen, and portal hypertension, ascites, and protein-losing bowel disease should consider this disease. B-mode ultrasound, CT, and X-ray examinations can help diagnosis.

Differential diagnosis of idiopathic retroperitoneal fibrosis

It should be distinguished from retroperitoneal lymphoma, proliferative lymphadenitis, metastases, primary tumors, peri-aortic hematomas, and amyloidosis. In addition to clinical manifestations, it mainly depends on imaging studies.

Idiopathic retroperitoneal fibrosis

Glucocorticoid

Early application of glucocorticoids can be effective within a few weeks, and can even significantly reduce or disappear the mass. Prednisone is more suitable for patients with mild to moderate urinary tract disease, old and infirm, or systemic disease. Sometimes it is also used for preoperative preparation or postoperative prevention of recurrence. The initial dose is 30 to 60 mg of prednisone or prednisone daily. After the condition is stable, the dose is gradually reduced to the minimum effective maintenance amount, at least 3 months. Some people have achieved better results with the combined use of hormones and azathioprine, and the effect of radiotherapy is not yet certain.

2. Surgery

Although fibrosis itself rarely requires surgical resection, once a large amount of fibrosis develops, hormonal therapy has little effect. When organs are affected by pressure, surgery is needed.

Taking a bilateral ureteral release procedure, the ureter can be wrapped with the omentum, and the ureter can be moved to the outside, which can achieve better sustained relief. Simple remission has a high recurrence rate.

In the late stage, for severe urinary tract obstruction, percutaneous nephrostomy drainage is feasible. This method is better than retrograde ureteral intubation or bracing, which not only can reduce symptoms in time, but also can monitor renal function through urine electrolyte measurement, so that most patients avoid Hemodialysis.

Idiopathic retroperitoneal fibrosis prognosis

Although the disease is progressive, it can sometimes resolve on its own and the prognosis is generally good. However, treatment of severe complications such as renal insufficiency in the later stages is more difficult.