Retroperitoneal fibrosis( Ormond's disease) - Causes, symptoms and treatment. MF.

Retroperitoneal fibrosis( retroperitoneal fibrosis, Ormond's disease) is a chronic inflammation of the adipose tissue of the retroperitoneal space, which gradually leads to compression and disruption of the tubular structures of this anatomical site. Most often, these umbilical structures are the ureters.

Causes of retroperitoneal fibrosis

There are two theories of the occurrence of Ormond's disease. The first is based on inflammatory, and the second - on immune reactions. Also in some medical sources, Ormond's disease belongs to the group of true collagenoses, that is, systemic diseases of connective tissue.

There are many factors that, according to experts, can lead to the appearance of retroperitoneal fibrosis. These include malignant neoplasms, chronic pancreatitis, chronic hepatitis, tuberculosis of the spine, radioactive irradiation, large hematomas of the lumbar region, exposure to toxic drugs and chemicals. Despite such a large list of risk factors, many patients with Ormond's disease can not detect any. Then we have to talk about idiopathic retroperitoneal fibrosis.

The mechanism of development of pathology is due to the fact that under the influence of these factors there is sclerosis of adipose tissue of the retroperitoneal space, as a result of which the vessels that are there are mechanically compressed and the ureter. This leads to the emergence of hydronephrosis or pyelonephritis, and ultimately, to the wrinkling of the kidney. In addition, there may be a clinical picture of intestinal obstruction, due to the compression of fatty tissue on the large intestine.

Symptoms of Ormond's disease

Symptoms of retroperitoneal fibrosis depend on the stage and degree of activity of the pathological process. Academically, the course of the disease is divided into three stages: the debut of the disease, the period of activity and the period of contraction of the fibrous mass.

In the first stage, patients complain of constant dull pain in the lumbar region, which begin with one side and gradually spread to the second. In this case, pain can be given to the back, abdomen and external genitalia. At this stage, there may also be a slight increase in blood pressure and symptoms of general asthenia: fatigue, headaches, sweating. Approximately aunt patients with Ormond pathology at this stage note the presence of a tumor-like formation in the pelvis or abdomen.

In the second and third stages of the disease, the symptoms progress slowly. Together with increased pain in patients, a decrease in the amount of urine, down to complete anuria( lack of urine) is noted. If the disease has a one-sided nature, then the second kidney compensates for the insufficiency of the patient. With Ormond's two-sided disease, protein-exchange products that are not excreted in the urine get into the blood. This leads to the onset of a septic condition called uremia. Such patients need immediate connection of an artificial kidney apparatus. Otherwise, the disease ends lethal.

Along with the symptoms of the kidneys, hypertension is progressing, which is developing due to pressure on the renal artery. Patients complain of a constant increase in the level of blood pressure, which manifests itself as headaches, especially in the nape of the neck.

A typical symptom of retroperitoneal fibrosis is venous hypertension, which arises from the compression of the inferior vena cava. Symptoms of this pathological condition is varicose veins of the lower extremities and veins of testicles in men. As a complication of blood stagnation in the lower half of the body, thrombosis of the inferior vena cava can occur, which, incidentally, is quite rare.

If you have at least one of the above symptoms, you need immediate advice from a urologist, since the Ormond disease has a rule that the sooner you start treatment, the better the results will be.

Diagnosis of Ormond's disease

In the early stages of the disease, markers of retroperitoneal fibrosis can only be laboratory blood counts. The rate of erythrocyte sedimentation is determined, which, as a rule, is increased and such markers of inflammation as C-reactive protein and alpha-globulins.

In the second stage of the disease, excretory urography is used to verify the diagnosis. In the picture, the enlargement of the renal pelvis and the change in the ureteral contour are determined. Moreover, a detailed examination of the picture shows that the deformation of the ureter occurs due to external compression.

Excretory urography - narrowing of the ureter

Among other methods of research, it is worthwhile to identify computed tomography and ultrasound. As in the first, and in the second case it is possible to reveal consolidation of retroperitoneal tissue.

The only reliable method of verifying the diagnosis of Ormond's disease is biopsy of the lumbar region. On histological sections it is possible to reveal the increased formation of fibrin fibers in adipose tissue.

Also with bright clinical symptoms from the cardiovascular or digestive system, irrigography( contrast study of the large intestine) and contrast aortography are carried out. The latter makes it possible to determine the functional and anatomical state of the renal arteries.

Treatment of retroperitoneal fibrosis

Conservative treatment of Ormond's disease is applied only when the patient has no symptoms of compression of the ureter, intestines and vessels. The purpose of its use is to prevent the progress of the process. For conservative treatment of retroperitoneal fibrosis, as a rule, hormonal drugs and non-steroidal anti-inflammatory drugs are used. A scheme of combined use of prednisolone and celecoxib has proved to be very useful. These drugs can be bought without a prescription, but this can not be done, given the large number of their side effects.

In the absence of the effect of conservative treatment or if the patient has symptoms of compression of the tubular structures, the patient is recommended surgical treatment. The essence of the operation is to cut out the fibro-modified fatty tissue and restore the lumen, both the ureter and the vessels. Quite often it happens that to restore the normal passage of urine or blood flow it is necessary to do the plasty of the ureter or vessels with the tissues of the body.

In the advanced stages, when the lumen of both ureters completely overlaps and there is no possibility of plastic surgery of the latter, an operation is performed to apply a nephrostomy, the essence of which is the removal of a tube into the anterior abdominal wall, through which the urine leaves in a special reservoir.

Laparoscopic surgery for Ormond's disease

Treatment with folk remedies

Treatment of retroperitoneal fibrosis with folk remedies usually involves the use of compresses. Used lotions from the decoctions of many herbs and plants. It is worth noting that in some cases, the use of such treatment can give temporary results and slightly delay the development of fibrosis. On the one hand, this may seem a good practice, although representatives of traditional medicine do not recommend them to get involved. The fact is that after receiving even minimal relief, patients begin to abuse folk methods of treatment, forgetting about traditional ones. In the end, this leads to the inevitable progress of the disease and its transition into a severe form, the treatment of which is possible only in an operative way. Urologists recommend using compresses in combination with drug treatment methods, which gives more confident results, although it does not completely cure the disease.

Features of diet and lifestyle with Ormond's disease

Patients with retroperitoneal fibrosis are recommended to adhere to a strict diet with the exclusion of fried, salty, acidic and smoked foods. This is especially important in the early stages of the disease, when the first priority is to prevent the progression of the pathological process. If the patient already has symptoms of narrowing of the ureter, then he needs to recommend the use of a large amount of fluid, so that the constant circulation of urine prevents the disease from progressing.

Peculiarities of the patient's lifestyle can only be changed if he has a nephrostomy after the operation. Such patients need to be taught how to use the urine collection properly, so that maceration( mocculation) of the skin around the nephrostomy tube does not occur.

Complications of retroperitoneal fibrosis

The first and most formidable complication of Ormond's disease is anuria, followed by the development of chronic renal failure. It arises from the constant stagnation of urine in the tubular system of the kidney and the destruction of its cells.

Another serious complication of retroperitoneal fibrosis is arterial hypertension, which arises from narrowing of the renal artery and compensatory isolation of large amounts of renin, which is a potent hypertensive agent.

The narrowing of the lumen of the inferior vena cava can threaten the patient with the development of varicose veins of the lower extremities. It is manifested by an increase in the lumen of the veins of the lower limbs and pain in their area. With a strong expansion, even trophic ulcers can develop, which have a very poor propensity to heal.

The dilatation of the lumen of the large intestine can contribute to the development of chronic intestinal obstruction, which is manifested by prolonged constipation, a violation of the escape of gases and swelling of the intestine. With neglected forms, this can lead to the development of intestinal intoxication.

Prevention of retroperitoneal fibrosis

Prophylaxis of Ormond's disease, first of all, is to eliminate those factors that can contribute to its development. If a patient has chronic pancreatitis, hepatitis, tuberculosis or systemic vascular disease, then first of all, it is necessary to deal with their treatment.

Then you need to carefully monitor your health, so as not to miss the development of the first clinical symptoms. It should be remembered that if early detection of Ormond's disease, then one hormone therapy can avoid surgical intervention.

Rev.doctor urologist Astashin Е.Е.