Anomalies of ureters

Anomalies of the ureters - a relatively common disease of the genitourinary system. They account for 13.4% of kidney and vestibular malformations.

The classification of the anomaly of ureters is based on such signs as their number, position, shape and structure. To date, the classification accepted at the 2nd All-Union Congress of Urologists in 1978 is generally recognized:

• anomalies of the number of kidneys (aplasia, doubling, trebling, etc.);
• abnormalities of the kidneys position (retrocaval ureter, retroileal ureter, ectopic ureter opening);
• anomalies of the form of the kidneys (corkscrew, annular ureter);
• abnormalities of the kidney structure (kidney hypoplasia, neuromuscular dysplasia, including achalasia, megaureter, hydroureteronephrosis, diverticulum valves, ureterocele).

[1], [2], [3]

Forms

[4], [5], [6], [7]

Anomalies in the number of ureters

Aplasia (agenesis) of the ureter is an extremely rare anomaly. A bilateral anomaly is usually combined with bilateral renal agenesis, less often with a bilateral multicystic kidney, it is incompatible with life.

Duplication of the ureter is the most common congenital anomaly of the urinary tract. In this case, one ureter collects urine from the upper half of the kidney, and the other - from the lower one. It is characteristic that the upper half is smaller and consists on the average of only two or three cups. Duplication of the ureter may be complete (ureter duplex) or incomplete (ureter fissus). Incomplete doubling of the ureter occurs in the case of premature division of the mesonephrosis duct (before the fusion with the metanephrogenic blastema occurs) on the branch. This division can begin both in the most distal and in the most proximal parts of the ureter.

The formation of a complete doubling occurs due to the formation of two mesonephros channels on one side, rushing to the metanephrogenic blastema. According to the Meyer-Weigert law, the ureter from the upper half will enter the bladder below and the medial (ectopic ureter) relative to the ureter draining the lower half (orthotopic ureter). When doubling, both ureters usually pass in one fascial bed. In detail, this type of anomaly is described in the section on the anomalies of the number of kidneys. Extremely rare is the tripling of the ureters.

[8], [9], [10]

Anomalies of the ureter

The retractile ureter is a comparatively rare developmental malformation (0.21%), in which the right ureter (upper and partly, middle third) spirals up to the lower hollow vein at level L3-4. Retroiliac ureter is an extremely rare anomaly in which the ureter is located behind the common or external iliac vein. Excretory urography usually reveals a J-shaped bend of the upper third of the ureter due to obstruction in the backbone segment, and only retrograde ureterography permits the detection of an S-shaped bend. Cavography does not provide additional information, CT and MRI also help in detecting an escalating conflict. If necessary, CT may be an alternative to retrograde ureterography. Spiral CT allows visualization of the ureter. These anomalies are manifested by the development of ureterohydronephrosis. The treatment is aimed at the restoration of urodynamics with the exclusion of the zakapakvalnoy or posadieleakalnogo area of the ureter. It is produced both by the open method and laparoscopically.

Ectopia of the urethral opening is an anomaly of the location of one or two ureteral orifices in the bladder or extravesical.

M.E. Campbell (1970) identified the etopia of the mouth in 10 cases with the dissection of 19 046 corpses of children (0.053%). In 80% of cases, ectopy is associated with doubling of the ureter. The clinical picture depends on the place of confluence of the ectopic estuary and the condition of the VMP. The confluence of the ureteral orifice into the urethra, vagina, uterus, external female genitalia is accompanied by uncontrolled leakage of urine against the background of preserved urination. The presence of the ureter in the bladder is higher than the sphincter of the urethra, the seminal vesicle, the vas deferens, the gut is not accompanied by leakage of urine and may not have characteristic symptoms or be accompanied by long-term inflammatory diseases of the internal genital organs.

In most cases, ectopy is associated with ureterohydronephrosis. It should be noted that ultrasound and excretory urography can diagnose only ureterohydronephrosis. MSCT and MRI help to diagnose even with a sharp decrease in kidney function. Treatment operative. Ureterocystostomy with stricture of the terminal ureter. Gemi- or nephrectomy with terminal changes in the kidney and the VMP.

[11], [12]

Anomalies in the structure and shape of the ureters

A corpulent or annular ureter is an extremely rare anomaly. M.E. Campbell found her only twice at the opening of 12,080 children. It is manifested by a spiral rotation of the ureter around the kidney more than one turn, as well as hydronephrosis. It is combined with stricture LMS.

Hypoplasia of the ureter is usually combined with hypoplasia of the corresponding kidney or its half when doubled with multicystic kidney dysplasia. The ureter is a thin tube with a reduced diameter. It can be obliterated in some areas.

Stenosis of the ureter is not rare. Most often, the stenoses are localized in the tuberculosis-ureteric anastomosis, the vesicoureteral segment (PMS), less often - at the level of the intersection with the iliac vessels. Neither clinically nor histologically it is usually impossible to differentiate between congenital and acquired strictures. Treatment is prompt, in the case of preservation of renal function is aimed at the restoration of urodynamics. At terminal changes - a nephrectomy.

Valves of the ureter are a duplicate of the urothelial membrane. Sometimes, valvular folds consist of all layers of the ureter wall. They are usually localized in the proximal, pelvic and caulinian areas of the ureter. Anomaly is relatively rare, equally common in both sexes, as well as on the right and on the left. It is manifested by a violation of the outflow of urine from the kidney with the development of ureterohydronephrosis. Treatment is usually prompt.

The diverticulum of the ureter is a hollow formation that connects to the lumen of the ureter, almost always located in the lower third. It is rare. Can be of multiple character. The wall of the diverticul only remotely contains the same elephant as the ureter. Diagnosis of the ureteral diverticulum is based on the data of excretory urograms. On which a peloidal or saccular shadow is found in the pelvic ureter.

Ureterocele - cystic enlargement of the intravesical segment of the ureter. It is a defect in the development of the walls of the distal ureter in the form of an enlargement of the intravesical region, which protrudes to the cavity of the bladder and prevents the passage of urine. The wall of the ureterocele is covered with the mucosa of the urinary bladder and consists of all layers of the ureter wall. From the inside, the cystic formation is lined with the mucous membrane of the ureter.

Ureterocele is a frequent developmental disorder (1.6% among all kidney anomalies and VMP). Can be one- or two-sided. Very often there is a ureterocele of one of the doubled ureters. The causes of the development of ureterocele are the underdevelopment of the neuromuscular apparatus of the distal ureter in the narrowed mouth. In pediatric urology, 80% of all ureterocele is ectopic, adult orthotopic is more common in adult practice, as ectopic ureterocele more violates urine outflow from the kidney, leading to parenchyma death. Therefore, the number of such patients in children's urological hospitals prevails.

Orthotopic ureterocele can for a long time not violate urodynamics and, accordingly, do not require correction. Diagnosis of this defect to date is not difficult. The most commonly ureterocele is diagnosed with ultrasound, if necessary, excretory urography, retrograde cystography for PMR, CT, MSCT, MRI and magnetic resonance urography can be used. Further excretory urography with descending cystography is performed. On radiographs, a noticeably rounded form of contrast enhancement in the bladder at the site of the ureteral confluence. In MSCT and MRI, the rounded cavity is also clearly defined. Going into the lumen of the bladder.

There are two points of view on the problem of treatment of ureterocele. The first - for conducting open operations, the second - in support of endoscopic dissection. Plastic surgery can be as follows:

• one-stage ureterocystostomy with dissection of ureterocele even with a practically non-functioning kidney or half of it;
• pyelo- or uretero-ureterostomy with preserved kidney function with excision (dissection) or without ureterocele;
• Hemin nephrectomy with excision (dissection) or without ureterocele.

This tactic is shared by most children's urologists. The main argument in favor of "open" surgeries is a high probability of MTCT occurrence (30%) after the endoscopic dissection of the ureterocele. Especially at its ectopic location. Proponents of endoscopic dissection, first proposed I. Zielinski (1962), agree with the probability of the emergence of PMR. In the case of the appearance of PMR, they offer reconstructive surgery, the need for which occurs in a third of patients. In this case, endoscopic dissection serves as the first stage of treatment, which allows achieving a certain reduction in the dilatation of VMP. Facilitating the subsequent plastic surgery. Absence of violations of urodynamics and clinical manifestations of obstruction in 52.38% of patients allows dynamic monitoring without operative correction.

Anomalies of the kidneys and VMP are often a predisposing factor in the development of ureterohydro- and hydronephrosis, kidney tumors, urolithiasis, acute pyelonephritis. However, there are significant difficulties in diagnosing and treating such patients, since some vices may be mistaken for the disease, and vice versa. All this can lead to diagnostic and medical errors. In addition, most anomalies affect the tactics of treatment and make it difficult to conduct surgical interventions.

Today, due to the introduction of non-invasive and minimally invasive diagnostic methods, various variants of endoscopy, it became possible not only to determine the type of defect based on indirect signs, but also to study the pathological processes that have arisen in detail, and also to evaluate the condition of neighboring organs, their relationship with abnormally developed kidneys and VMP. Obviously, the notion of anomalies of the kidneys and the VMP are at a new stage in development. Modern therapeutic tactics differ from the generally accepted 5-10 years ago. The high incidence of kidney and vestibular abnormalities and the complexity of open surgical manuals against their background made it necessary to introduce new minimally invasive methods of treatment.

[13], [14],