Multi-cystic dysplasia of the kidney - intrauterine lesion

Renal dysplasia is a violation of the development of embryogenesis, associated with delayed evolution and abnormal development of embryonic structures. The formation of cartilaginous and connective tissue is a characteristic sign accompanying the beginning of the development of this type of dysplasia in the human body. The whole kidney, or part of it, can be affected, and this process can be either unilateral or bilateral. To renal cystic dysplasia include: multicystic hypoplastic kidney dysplasia, cortical multicystic kidney damage, multilocular renal cyst and multicystic kidney.

Hypoplastic cystic dysplasia is 22% of all cystic dysplasia. With this kind of pathology, it is most often recorded that the kidneys are reduced in size and volume. Glomerular cysts are located in the subcapsular zone and reach up to 3-4 mm. Tuberculosis cysts occur both in the cortex, there and in the brain substance. Hypoplastic dysplasia occurs quite often in combination with lesions of the genitourinary, digestive and cardiovascular system.

The clinical picture of this disease is caused by the development of pyelonephritis and chronic renal failure, the development of which depends on the degree of organ damage and the amount of parenchyma preserved.

Cortical multicystic dysplasia is a congenital nephrotic lesion of the "Finnish" type, in which the size of the kidney and its lobate structure does not change. Small glomerular and tubular cysts are found. The flow of this type is hormone-dependent, with an unfavorable prognosis.

Multilocular dysplasia is characterized by focal formation of the cyst, which is localized in one of the poles of the kidney and has a multi-chamber structure. Such a cyst is divided from the inside by partitions, and from the healthy tissue it is separated by a capsule, in which the cyst itself is located.

The clinical picture of this pathology is accompanied by the appearance of a pain syndrome in the abdomen and lower back, as well as a violation of the outflow of urine, which is mainly due to compression of the cyst of the pelvis and ureter.

Multi-cystic renal dysplasia is a structural congenital anomaly of development characterized by an organ enlargement due to the presence of a large size of cysts. The kidney, in which the development of multicystic dysplasia was diagnosed, unfortunately, is already completely unable to perform all its functions.

Causes of development of

Development of renal dysplasia is explained by embryopathy, which can be both familial and hereditary.

This kind of pathology is formed due to the complete absence of the primordial buds of the metonephrenic and meso-neural blastema. Metonephrotene tissue contributes to the production of urine, with insufficient development of urine accumulates in the tubules and this contributes to their stretching, forming cysts.

Multi-cystic dysplasia of the kidneys in the fetus is formed by the end of the intrauterine life. It can be said that at the time of birth, there is a complete replacement of metronfragennaya blastema with numerous cysts, heterogeneous in form and size.

This pathology is a cluster of different sizes and forms of cysts with no signs of renal tissue. Lohanka with such a disease is absent, and the ureter has the appearance of a strand, most often underdeveloped or abruptly terminating. The maximum number of cysts in one kidney reaches 12-15 and rarely exceeds this number, basically it depends on the initial size of the organ and the overall development of the body.

The contents of the cysts are a transparent exudate with a yellowish tinge or a thick, uniform mass. Microscopically, the contents of the cysts show a drop of cholesterol, hyaline and urea. Quite often there are cysts with calcified walls, this is well reflected in the review radiograph of the kidneys. The renal leg in such cases is a few thin vessels.

The histologically superficial layer of the cyst wall consists of fibrinous tissue, and the inner layer of the cubic epithelium. In the structure of the walls of some cysts, the remains of the underdeveloped kidney tissues are found.

When only one kidney is affected, no clinical manifestations are observed. Most often, this pathology is detected by accident, during the procedure of palpation. The lack of functional ability of the kidney is confirmed with the help of urography or radioisotope rheogram.

Diagnostic Features

When scanning, the kidney does not absorb radioisotopes. In cystoscopy, the ureteral opening in the image may either be absent or very small in size. During the procedure of ureterography, one can observe a sharp termination of the ureter, not reaching the place of its connection with the pathological kidney, there are also cases when only the lower part of the ureter can be filled with contrast. On X-ray images, one can see round foci of calcification of the cyst walls. During arteriography, there is a decrease in the artery and its hypoplasia. Retrograde pyelography is not performed due to segmental obliteration of the ureter.

This is important! To date, it is very important to correctly and timely diagnose multicystic kidney dysplasia and polycystosis. Multicystosis is a benign, non-progressive kidney damage that carries a relatively favorable outcome if the contralateral kidney is healthy, and polycystic is a severe organ damage leading to death. Unilateral multicystotic kidney damage today is not a serious illness.

Clinical picture

Clinical course of multicystic renal dysplasia depends on the severity and localization of the pathological process. Sometimes patients complain of dull pain in the affected kidney and a violation of the outflow of urine due to compression of the cyst of the pelvis or ureter. In addition, due to pressure on the digestive organs, symptoms of their defeat appear. The analysis reveals transient hematuria and leukocyturia, high protein content and low specific gravity.

The disease is often detected with urinary tract infection, urography, angiography, renography and kidney scanning.

Treatment of the disease

Treatment of multicose renal dysplasia is the elimination of symptoms and a possible cause of the development of pathology. When the diagnosis of chronic renal failure is established, hemodialysis is carried out, in the future, with a deterioration in the general condition of the patient, a transplant is prescribed.

With a timely begun treatment, the prognosis is favorable, it worsens at the stage of adherence to the disease of hypertension.

Equally often occurs as multicystic dysplasia of the left kidney of the fetus, as well as multifocal dysplasia of the right kidney.

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