Rarely occurring bladder malformations

Urologists classify the following pathologies as rare malformations of the urinary bladder: hypertrophy of the interureteral ligament, excess mucous membrane of the ureteral triangle, anomalies of the urinary duct, vesicoumbilical fistula, cyst of the urinary duct, incomplete umbilical fistula.

[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ]

Forms

Hypertrophy of the interureteral ligament

Hypertrophy of the interureteral ligament is extremely rare in newborns and infants. The diagnosis is established by cystoscopy: excessive development of the bundle of muscle fibers running along the upper border of the Lieto triangle between the two ureteral orifices is detected. The main clinical symptom is difficult and sometimes frequent urination.

[ 6 ], [ 7 ], [ 8 ]

Excess of the mucous membrane of the urinary bladder triangle

Cystoscopy reveals a valve that hangs over the bladder neck, causing a disturbance in urination. A cystogram reveals a filling defect at the bladder outlet.

In case of mild excess of the mucous membrane, bougienage of the urethra is performed against the background of antibacterial therapy; in case of severe excess, resection of excess tissue is performed.

Other extremely rare malformations of the urinary bladder include an hourglass bladder, partial or complete septa of the bladder located in the frontal or sagittal plane. bladder agenesis, congenital hypoplasia of the urinary bladder, etc. Agenesis of the urinary bladder is extremely rare when combined with other malformations. Therefore, this anomaly is incompatible with life. Stillborn babies are born or newborns die in the near future.

Urinary duct anomalies

Normally, the upper anterior part of the urinary bladder forms the apex (apex vesicae), which is clearly visible when the urinary bladder is full. The apex passes upward toward the navel into the middle umbilical ligament (ligamentum umbilicak medianum). connecting the urinary bladder with the navel. It is an obliterated urinary duct (urachus) and is located between the peritoneum and the transverse fascia of the abdomen. The size of the urinary duct varies (3-10 cm in length and 0.8-1 cm in diameter). It is represented by a muscular tube with three layers of tissue:

• epithelial canal represented by cuboidal or transitional epithelium;
• submucosal layer;
• superficial smooth muscle layer, similar in structure to the wall of the urinary bladder.

[ 9 ], [ 10 ]

Embryological data

The allantois is the extraembryonic cavity (which later forms the urinary bladder) inside the precursor of the allantoic stalk, located on the anterior surface of the cloaca. The immersion of the urinary bladder into the pelvis occurs in parallel with the elongation of the urethra, the tubular structure of which extends from the fibrous allantoic duct to the anterior wall of the urinary bladder. By the fifth month of pregnancy, the urethra gradually turns into an epithelial tube of small diameter, necessary for the removal of urine from the embryo into the amniotic fluid. After the completion of embryonic development of the fetus, the urachus gradually overgrows, and in cases where for one reason or another the process of overgrowth (obliteration) of the urethra is disrupted, various variants of its diseases develop.

[ 11 ], [ 12 ], [ 13 ], [ 14 ], [ 15 ], [ 16 ], [ 17 ], [ 18 ]

Vesico-umbilical fistula

Of all the variants of duct obliteration, the most common is a complete urinary fistula. The diagnosis of this disease is not difficult. Clinically, urine is observed to flow through the umbilical ring in a stream or drops. Sometimes parents complain of their child's periodically "weeping navel".

In order to confirm the diagnosis, as a rule, in adult patients with suppuration of the urethral cyst, ultrasound, fistulography, contrasting of the fistula with indigocarmine solution, micturition cystourethrography, CT and sometimes radioisotope examination can be performed. Differential diagnostics should be carried out with healing of the umbilical stump, omphalitis, granuloma and non-closure of the vitelline duct. Persistence of urinary and intestinal fistula in one patient is extremely rare, but this type of anomaly should still be remembered. In children of a younger age group, the urethra can often close on its own in the first months of life, so sometimes these children are shown only observation. However, a long-standing fistula in some cases provokes the development of cystitis and pyelonephritis.

[ 19 ], [ 20 ], [ 21 ], [ 22 ], [ 23 ], [ 24 ], [ 25 ]

Urethral cyst

A cyst of the urethra is formed when its obliteration occurs in the proximal ileal sections. Most often, it is located closer to the navel and less often to the bladder. The contents of the cyst are stagnant urine with embarrassed epithelium or pus. Clinically, cysts of the urethra are asymptomatic and are an accidental finding during an ultrasound examination of the patient, but sometimes manifestations of acute purulent infection occur. Serious complications include peritonitis, which can develop when the abscess breaks into the abdominal cavity.

Sometimes it is possible for the cyst to drain spontaneously through the navel or bladder, as well as for a sinus to form (intermittent variant).

The most common symptoms of cyst infection are abdominal pain, increased body temperature, and urination problems (painful, frequent, even if a general urine test does not reveal any pathological changes).

Sometimes it is possible to palpate a neoplasm in the anterior abdominal cavity.

Additional diagnostic methods include CT and radioisotope examination, which help to clarify the diagnosis. Treatment of a cyst of the urethra depends on the symptoms and age of the patient. In the "cold" period, the cyst can be removed using laparoscopic or open surgery. In the acute period, when a cyst of the urethra becomes purulent, the abscess is opened and drained. In small children, if it is asymptomatic, observation is possible; if inflammation occurs, the formation is excised and drained. The final treatment is carried out after the inflammatory process has subsided; it consists of complete excision of the cyst walls.

[ 26 ], [ 27 ], [ 28 ]

Incomplete umbilical fistula

An incomplete umbilical fistula is formed when the process of obliteration of the urinary duct in the umbilical segment is disrupted. Clinical manifestations are possible at any age. Most often, patients are bothered by purulent discharge in the umbilical ring area, accompanied by constant or intermittent weeping in this area, often with signs of omphalitis. In cases of disruption of the outflow of purulent contents, signs of intoxication are possible. Sometimes, in the umbilical ring area, there is an overgrowth of granulations protruding above the surface of the skin.

To clarify the diagnosis, ultrasound and fistulography are required (after stopping the inflammatory process in the umbilical ring area).

Treatment of incomplete umbilical fistula consists of daily sanitizing baths with potassium permanganate solution, treatment of the umbilicus with 1% brilliant green solution, cauterization of granulations with 2-10% silver nitrate solution. If conservative measures are ineffective, the urinary duct is radically excised.

Treatment bladder malformations

The optimal surgical treatment for urinary duct anomalies is the laparoscopic method.

Stages of performing laparoscopic excision of the urethra (for fistulas and cysts of the urethra)

• Open laparoscopy with the introduction of three small-diameter trocars (3 or 5.5 mm). Trocar No. 1 (for the laparoscope; 5 mm. 30°) is usually inserted along the midline, midway between the umbilical ring and the xiphoid process of the sternum. Trocars No. 2 and 3 (for working instruments) are most often inserted in the left and right abdominal regions.
• Laparoscopic revision using optics with an angular end cut (30° or 45°), visualization of the urinary duct along its entire length (from the umbilical ring to the bladder) or the area of its cystic expansion.
• Excision of the urinary duct (usually begins with its dissection in the area of the umbilical ring). The urinary duct in this place is isolated circularly, cut off after careful bipolar coagulation. At the same time, additional surgical treatment of the umbilical ring area is performed from the outside to completely remove the fistula.
• Isolation of the urethra to the point of its connection with the bladder by careful blunt dissection with monopolar or bipolar coagulation. Ligation of the base of the urethra is performed, most often using endoloops. The ligated urethra is cut off and removed through one of the trocars.
• Suturing the surgical wound (with intradermal sutures).

The duration of laparoscopic surgery usually does not exceed 20-30 minutes, patients can be discharged from the hospital 1-3 days after the operation.

Similar operations in children aged 1-17 years for fistulas and cysts of the urethra confirm the versatility, simplicity and convenience of using endosurgical technologies in the treatment of this anomaly.

In cases where laparoscopic excision of the urethra is impossible, an open operation is performed. Access depends on the level of obliteration. In younger children, the urethra is easily removed from a semilunar incision along the lower edge of the umbilical ring due to anatomical features and the high-standing apex of the bladder. In older children and adults, a lower midline laparotomy is performed and the urethra is completely excised along its entire length. In cases where the duct walls are intimately fused with the surrounding tissues due to a previously suffered inflammatory process, excision is performed within the healthy tissue.