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Neuromuscular dysplasia of the ureter
Neuromuscular dysplasia of the ureter is one of the most common malformations of the urinary tract. In children, neuromuscular dysplasia of the ureter is often associated with other malformations of the genitourinary system (doubling the kidneys, polycystic kidneys, cryptorchidism). To date, no known specific mechanism of this anomaly. It is assumed the presence of a combination of urine outflow obstruction at or below the ureter and congenital disorders of muscular layer of the wall forming the ureter.
When neuromuscular dysplasia of the ureter is a gradual violation of the tone of the muscular layer of the wall mochetochnika.V normal muscle fibers of the ureter wall are reduced, creating an undulating movement, which promotes urine from the upper to the lower - from the kidneys to the bladder. If you violate the tone of ureteral urine flow slows down and there is a gradual enlargement and elongation of the ureters. The process may be unilateral but is more common in bilateral neuromuscular dysplasia of ureters. If extended only to the lower part of ureter - this is called achalasia ureter. In this case the upper sections of the ureter are functioning normally. When engaging in the process of all parts of the ureter expands and elongates. This condition is called megaureter. In this case, the reduction in the muscle layer ureter greatly weakened the movement of urine slows emptying of the ureter is violated.
There are three stages of development of neuromuscular dysplasia of the ureter:
Latent stage or the stage of compensation. At this stage, the anomaly does not appear, and the patient's complaint is not seen. There is a gradual increase in the lower ureter - urethra achalasia.
The emergence of the expansion in all parts of the ureter (megaureter). The patient may see fatigue on exertion, headache, general weakness. Pain in the lumbar region, in the abdomen.
Loss of function of the muscular layer of the ureter and the development of gidroureteronefroza. At this stage the patient's condition deteriorates. In the blood, increases the amount of urea, creatinine. Ureters are expanding rapidly. Their diameter may take up to 4 cm length of the ureter increases.
Gidroureteronefroticheskaya transformation always leads to the development of chronic renal failure. Characteristic signs of the disease have does not exist. Neuromuscular dysplasia of the ureter at the beginning goes unnoticed, especially in children. Therefore, the disease is usually diagnosed too late when the patient appears pyelonephritis or signs of kidney failure.
In the diagnosis of neuromuscular dysplasia of the ureter using ultrasound, which detects the expansion of the ureter, renal pelvis and calyces. When administered intravenously cystochromoscopy indigo stands out from the mouth of the anomalous ureter with a lag. At excretory urography detect the expansion of part or all of the ureter. The course of the disease progressing, gradually leading to chronic renal failure.
Surgical treatment of neuromuscular dysplasia of the ureter is carried out as soon as possible to preserve renal function. The aim of surgical interventions to restore or improve the flow of urine to the affected ureter. Techniques of surgical interventions are applied are different. Sometimes part of the ureter is removed, the remaining stitch, a substitute ureter section of the small intestine.
