Anomalies of the kidneys are intrauterine disorders in the development of organs in the fetus, which can threaten life and he alth or do not interfere with full-fledged life. Pathologies of the kidneys of a congenital nature consist in a violation of the structure of the organ, its vessels, localization or performance. The main causes of kidney anomalies are genetic abnormalities during the prenatal formation of internal organs and systems (first trimester of pregnancy). Harmful factors affecting a pregnant woman can also negatively affect the excretory system of the unborn child.
Causes of kidney abnormalities
Defects of the excretory system are diagnosed more often than other congenital disorders. The thing is that an anomaly in the development of the kidneys (according to the ICD-10 code Q63) is not only accompanied by pronounced symptoms, but also has a negative impact on the functioning of other systems in the body. Together with the kidneys, the liver most often develops abnormally in the fetus.
In the process of laying and differentiation of tissues at the stage of maturation of embryoniccells there is a certain failure, which causes the formation of internal organs with deviations. Pathology may occur due to the influence of various endogenous and exogenous factors on the fetus:
- strong drugs (antibiotics, corticosteroids, ACE inhibitors, etc.);
- radioactive study;
- pathogenic microorganisms (viruses, bacteria, fungi, protozoa).
The cause of congenital anomalies of the kidneys in children may be a genetic failure. In this case, the deviation in the development of the excretory system is combined with disorders of another localization, which leads to the formation of various syndromes. Depending on which of the processes a violation occurred, a certain defect occurs (dysplasia, dystopia and other kidney pathologies).
All deviations of intrauterine kidney formation can be divided into:
- anomalies in quantity (unilateral and bilateral agenesis and aplasia of the kidney, doubling and the presence of a third additional kidney);
- structural disorders (dysplasia, kidney cyst - abnormal structure of the kidney tissue);
- position pathology (kidney dystopia, that is, the omission of an organ or its location in an atypical place, for example, below the waist).
Most congenital abnormalities are combined with other anomalies of the genitourinary system. Some of the genetic kidney defects can be absolutely incompatible with life, while others carry a hidden he alth hazard, so any unnatural changes in the internal organs require constant monitoring.
Symptoms of congenital pathology
Abnormally developed or improperly located kidney, as a rule, does not have any specific manifestations. In most cases, there is no symptomatic picture, and the pathology is confirmed by chance, often during an ultrasound appointment for a completely different reason.
The situation is slightly different in the presence of bilateral defects: they are usually detected in a short period after childbirth due to partial or complete dysfunction of the organ. The most dangerous anomaly in the development of the kidneys in children is agenesis, and in some cases even a unilateral form can lead to the death of the fetus while still in the mother's womb. If this violation occurs, then, most likely, during the first months and years of life it will lead to severe renal failure. Such an anomaly can be suspected in the presence of malformations of the skeleton and other internal organs.
Speaking of such disorders as hypoplasia, accessory renal lobule or polycystic, it is worth noting that they are accompanied by signs of pyelonephritis resulting from a violation of the outflow of urine. Children usually complain of pain in the lower back, possibly fever and symptoms of intoxication. Anomalies in the relationship of the kidneys are accompanied by arterial hypertension. With an increase in pressure at an early age, the first thing to rule out is disturbances in the work of the renin-angiotensin system.
Additional lobule of the kidney - an anomaly in the development of the body, which is characterized by urinary incontinence andfrequent colic. The most common reason for contacting specialists in this case is hematuria, a change in the color and transparency of urine.
Diagnosis
The study of the internal organs and systems of the unborn baby is carried out in the period from 12 to 16 weeks. During this period, it is already possible to identify most of the possible anomalies of the kidneys in the fetus. Ultrasound screening will indicate the presence of a defect by incorrect laying at the location of paired organs or the absence of a bladder, which can also be considered a secondary sign of a deviation of the excretory system.
In young children, an indication for clinical diagnosis is the presence of relevant symptoms from the urinary system. For patients who turn to a pediatrician with complaints, the doctor should prescribe general laboratory tests of urine and blood, which allow assessing the functionality of organs and detecting signs of an inflammatory process. If a bacterial etiology of secondary pyelonephritis is suspected, a urine culture is performed.
Diagnosis of kidney anomalies in newborns and older children is carried out using instrumental procedures. First of all, an ultrasound is performed, which can reveal violations in the number, position of organs, and suspect dysplastic tissue disorders. If the object of study is the urinary function, excretory urography will become more informative, which will also help to assess the structure of the pelvicalyceal system and indicate hydronephrosis. In addition, if you suspectpolycystic disease and inconclusive results of previous studies, CT or MRI is performed.
Vascular disorders
In ICD-10, the code for an anomaly in the development of the kidneys, which was caused by poor blood supply to the organ, is code Q63.8 "Other specified congenital abnormalities." It is precisely because of problems with the vessels that the full outflow of urine from the organ is disturbed, as a result of which pyelonephritis often occurs, blood pressure rises, and kidney failure develops.
Diagnosing a congenital anomaly of the kidney vessels, first of all, it is necessary to differentiate it from tuberculosis, hematological and hepatological diseases. Vascular disorders are usually an accompanying phenomenon for the defect of the kidney itself. Conventionally, the pathology of the vessels of the kidneys is divided into anomalies of the veins and arteries. A defect in the formation of the main vessels of the excretory system can be:
- formation of multiple vessels;
- the appearance of an accessory artery, which is already the main one and is attached to the organ at the top or bottom;
- presence of an additional blood vessel of similar size;
- fibromuscular stenosis (narrowing of the lumen of blood vessels due to the intensive growth of cystic and muscle tissue);
- artery having a knee-shaped form;
- aneurysms.
Venous malformations are multiple and accessory veins, as well as a variant of the arrangement of vessels emerging from the front and back of the organ in an annular shape. Both extracaval confluence and retroaortral placement of the left renal vein, and the attachment of the right testicular vein tokidney vessel. To detect such deviations, dopplerography is prescribed. According to the results of the study, the doctor will be able to give an objective assessment of the condition of the renal vessels.
Agenesia
In the absence or deep underdevelopment of the kidneys, the fetus is not viable. The reason for such violations in the formation of the excretory system is a gene mutation or a powerful negative effect on the unborn baby in the mother's womb.
The most favorable prognosis is given to children with mild kidney anomalies. In the partial absence of one of the paired organs, the quality of life does not change. A he althy kidney performs a double amount of work, fully compensating for the dysfunction of the second. Pathology may not manifest itself throughout life.
With the complete absence of kidneys (bilateral agenesis), it is impossible to live. Such children are born dead or die in the first days after birth. Immediate transplantation can save them.
Aplasia
Unlike agenesis, this pathology is a partial absence of one of the organs. Even if outwardly the anomaly is invisible in the shape of the kidneys, this does not always mean that the organ is fully developed. For the incapacitated share, all functions are performed by the he althy part. If the kidney copes, and the excretory system does not fail, the patient may not be aware of the presence of a problem, since no symptoms appear with this form of aplasia.
Most often, pathology is discovered by accident, but notreatment is not given. The patient is recommended to undergo an annual ultrasound, take blood and urine tests and lead a he althy lifestyle. It is important to follow a sparing diet and give up bad habits. If a he althy organ cannot cope with the increased load, the patient will experience characteristic symptoms, which will indicate the need for urgent treatment.
Other organ count anomalies
Pathology lies in the congenital formation of an additional organ - the third kidney. Such an anomaly is rare in medical practice, especially if the accessory lobe is fully functional and properly developed. The structure of the third organ is no different from the main one, it is also endowed with a separate blood supply.
The third kidney is located on the right or left side under one of the paired organs. At the same time, its size may be slightly smaller, but this is not an anomaly of development. The additional lobule of the kidney does not interfere with the vital activity of the body. In isolated cases, the patient may begin to leak urine. The deviation is detected by chance during an ultrasound, the need for which arose with unsatisfactory blood and urine tests showing the presence of inflammation. In chronic pyelonephritis, an additional organ can be removed.
Special attention deserves hypoplasia - this is a pathological phenomenon in which one or both kidneys do not grow to normal size due to their underdevelopment. At the same time, the functionality and histological structure of the organs do not have any differences. The only thing inthe difference lies in the smaller lumen of the vessels. Basically, only the lobe of the kidney is affected. The need for treatment arises only in cases where glomerulonephritis develops.
Disturbances in the formation of the renal pelvis
Abnormal doubling and bifurcation of the kidneys is quite common. Deviation in most cases occurs at the genetic level. However, there are also acquired anomalies of bifurcation of the kidney, which develop before the birth, but after the organ laying has occurred. The violation consists in partial doubling of the pelvis and ureters, which are combined into one in front of the bladder, or complete bifurcation - in this case, two independent ureters lead to the bladder.
Pathology can develop from one or both sides at once. An anomaly can be diagnosed with the help of general and bacteriological urinalysis, ultrasound screening, urography, cystoscopy. This pathology is treated only surgically. The size of a bifurcated kidney is usually larger than normal, and the upper and lower sections are separated. Each fragment of the body is supplied with blood equally. The renal anomaly may be partial and often leads to complications such as pyelonephritis, connective tissue tuberculosis.
Dystopia organ
During the prenatal period, the fetal kidneys can move to the lumbar region or, conversely, rise into the chest cavity. A change in the position of the organs is called dystopia.
This deviation may affect only one or both shares. This defect is the most common. It is not life threatening and is not a deadly disease. However, the displacement can bring serious discomfort and threaten the full functioning of the excretory system, abdominal organs and small pelvis. Anomaly in the position of the kidneys can be of several types:
- Lumbar - the organ is palpated in the lower hypochondrium, the blood vessels run perpendicular.
- Iliac - the share is localized in the ileum, it is fed by many branched vessels, pain in the lower back is possible.
- Pelvic - with this anomaly, the location of the kidneys negatively affects the work of the rectum and gallbladder.
- Thoracic - the renal lobe rises into the chest cavity, most often on the left side.
- Crossed is a congenital defect that involves the displacement of one of the kidneys closer to the other, as a result of which both organs are localized on one side, their fusion is not excluded.
Horseshoe and biscuit shape
The causes of such pathologies, as described above, are congenital changes at the genetic level. Violations are laid during pregnancy. An anomaly of the horseshoe kidney is its fusion with two ends together, as a result of which the organ takes on the shape of a horseshoe.
Often, ultrasound examination allows you to determine that this organ is localized below normal, while its blood supply is not changed. With an anomaly of fusion of the kidneys, the junction can compress the vessels and nerves.roots in an uncomfortable position of the body. A characteristic symptom in this case is a sharp paroxysmal pain. With a horseshoe shape, inflammatory processes often occur in the structure of the organ, which quickly become chronic. Pathology is treated exclusively by surgery, without the use of medicines.
Crack-like kidneys are another kind of anomaly in the relationship of the kidneys that occurs during pregnancy. For such a defect, the fusion of both kidneys with each other is characteristic. The organs are connected by medial edges, while the pathology itself is localized in the midline of the spinal column in the pelvic region. The fusion site is easily palpable on palpation of the abdomen. You can also determine this anomaly of the kidneys when viewed on a gynecological chair. For diagnosis, the same diagnostic methods are used as for dystopia.
Violation in the structure of the kidney
The altered structure of an organ can also be a defect laid in utero. Congenital anomalies in the structure of the kidneys in children develop under the influence of teratogenic factors (potent drugs, narcotic substances, intoxication of the body, etc.). The consequence of these deviations in the structure of the body is a predisposition to inflammatory and infectious processes. Treatment involves surgery. Structural renal anomaly may be:
- cyst;
- dysplasia;
- congenital hydronephrosis;
- hypoplasia.
Single cystic formations
If there is only one benign tumor,it is called solitary. The cyst is formed in utero, but it can also be acquired, localized on the surface of the organ or inside it. As a rule, the neoplasm has a rounded shape.
Over time, the cyst increases in size, provokes atrophy and negatively affects the functioning of the organ. Anomalies are accompanied by dull lumbar pain and an increase in the size of the organ. Pathology can be seen on ultrasound or x-ray. This defect can be cured only by surgical excision of the neoplasm, but most often the cyst is left under many years of observation.
Multicystic and polycystic kidney disease: what's the difference
In the first case, we are talking about the complete replacement of the renal tissue with fibrous tissue, in the second - about abnormal compaction and numerous growths on the surface of the organ.
Multicistosis often affects only a fragment of one of the kidney lobes. Pathology occurs if during the period of intrauterine development the excretory apparatus is not laid in the fetus. Urine is not fully excreted, its remnants accumulate, which ultimately leads to the replacement of the parenchyma with connective tissue and cystic formations. With multicystosis, the renal pelvis is also modified, sometimes it is completely absent. This disorder is not hereditary and in most cases is detected by accident. With multicystosis, the kidney is palpable through the abdomen, especially with the parallel development of hypoplasia.
The symptoms of this disease include a dull aching pain in the lower back. It is possible to increase the temperature to subfebrile values,flatulence. As a rule, a general analysis of urine does not show deviations from normal values, but changes will be noticeable on ultrasound.
Polycystic disease, unlike multicystic disease, is most often transmitted genetically. This disease is very often accompanied by numerous cysts in the liver, pancreas or other organs. If polycystic kidney disease is suspected, an examination is recommended not only for the patient, but also for all his close relatives, especially if the patient is a pregnant woman.
In severe cases, polycystic disease affects both lobes of the kidneys. Symptoms of polycystic disease are not much different from multicystic. In addition to them, the patient may suffer from constant thirst, loss of strength, fatigue. Polycystic disease is almost always accompanied by pyelonephritis, which, if left untreated, can lead to atrophy and renal failure. To confirm the pathology, the following diagnostic methods are used:
- ultrasound;
- determination of the level of leukocytes in the urine;
- computed tomography;
- x-ray examination.
With polycystic kidney disease, symptomatic therapy is carried out. General recommendations include exclusion of excessive and prolonged exercise, prevention of chronic infections (caries, SARS, tonsillitis, sinusitis, etc.), adherence to a high-calorie, vitamin-rich diet with protein and s alt restriction.
Danger of dysplasia
A deadly pathology that rarelydoes without transplantation, is the underdevelopment of the kidneys as a result of dysplastic tissue damage. With dysplasia, the size of the organ is smaller than usual. Improper growth and pathological intrauterine formation of the kidneys adversely affect the structure of the parenchyma and the functionality of the organ in the future.
Dysplasia can be dwarf and rudimentary. In the first case, the small size of the organ and the absence of the ureter are implied. Rudimentary dysplasia involves stopping the development of the kidneys in the first trimester of pregnancy. Instead of a kidney, the fetus has a small amount of connective tissue in the form of a cap on the ureter. A newborn child with such a pathology is doomed.
Prognosis for anomalies in the development of the kidneys is favorable if the functions of the urinary system remain normal. The exceptions are gross malformations and bilateral anomalies that require urgent organ transplantation or permanent hemodialysis procedures. Prevention of deviations in the development of the excretory system is carried out during pregnancy.
