Treatment of epispadias and bladder exstrophy in children

Immediately after the birth of the child with bladder exstrophy, questions about the methods of examination, the duration of preoperative preparation, the nature of the surgical intervention, the form of skeletal traction and the management of the postoperative period are discussed. Usually the operation of primary bladder plastic surgery is carried out within 48-96 hours after birth. If it is necessary to transport the child for a long distance, appropriate hydration is carried out.

Methods of treatment of bladder exstrophy

Treatment of bladder exstrophy is aimed at solving the following

• elimination of defects of the bladder and anterior abdominal wall;
• the creation of a penis that is acceptable in both cosmetic and sexual terms;
• preservation of kidney function and ensuring retention of urine.

All patients are candidates for the plastic (closure) of the bladder, and only in isolated cases have to go to the urine diversion. Even in children with a very small bladder (2-3 cm), it is remarkably fast growing after primary closure.

Stage treatment of epispadias and bladder exstrophy includes three stages:

• Correction of anomalies (the first stage) begins with the closure of the bladder immediately after birth, usually in combination with osteotomy of the iliac bones (in children older than 10-15 days or with a site size of 5 cm or more). After the closure of the bladder, a period of urinary incontinence is selected, during which the bubble gradually grows and its capacity increases.
• Operative correction of epispadias in boys (the second stage) is currently performed in this period of incontinence (usually 2-3 years). No attempts are made to maintain urine retention until 3.5 to 4 years.
• At 3,5-4-year-old children spend a plastic of a neck of a bladder (the third stage). Before this, assess the volume of the bladder. Reconstruction of the neck of the bladder is not carried out until it reaches an adequate volume (more than 60 ml) and until the child grows to such an extent that he himself will begin to realize the need for urine retention.

Primary plastic (closure) of the bladder

The goals of primary closure of the bladder are as follows:

• rotation of anonymous bones for approaching the symphysis of the symphysis;
• closure of the bladder and its displacement in the rear position, into the cavity of the small pelvis;
• formation of the neck of the bladder and ensuring the free discharge of urine through the urethra;
• if necessary, primary elongation of the penis (partial mobilization of cavernous bodies from the frontal bones);
• suturing the defect of the anterior abdominal wall.

Before the operation, antibiotics of a wide spectrum of action are prescribed to reduce the risk of wound infection and osteomyelitis.

Children with inguinal hernia, simultaneously with the plastic of the bladder, perform bilateral hernia repair. This tactic allows you to avoid emergency operations in the early postoperative period about the infringed inguinal hernia. In the presence of cryptorchidism, also perform orchopexy, but usually the testicles only seem highly located due to the displacement of the rectus muscle.

Operative interventions. Osteotomy

In the case of osteotomy, an intersection of pelvic bones at the back or in front (a posterior or anterior osteotomy) or a combination thereof can be performed.

Indications for osteotomy are:

• a large diastasis of the lateral bones (more than 4-5 cm) and the difficulty of reducing them during primary plasticization in newborns;
• the age of the child is more than 10-15 days.

Researchers believe that the bones of a newborn every day become more dense and elastic. Reduction of bones without osteotomy at the age of 2 honey is often accompanied by a divergence of the symphysis at a later date.

Earlier back osteotomy was used more often and achieved good results. To access the iliac bone, two vertical incisions were made lateral to the sacroiliac joint. After distinguishing the contents of the large sciatic foramen (gluteal nerves and vessels), crossed both iliac bone plates (surfaces) from the posterior crest of the ilium to the sciatic notch. Currently, most surgeons prefer the front iliac osteotomy of the pelvic bones (analogous to the operation of Chiari).

Front access has advantages and is more convenient, since both osteotomy and bladder plastic are carried out in one position of the child - it needs to be turned over during the intervention. For the stabilization of bone fragments, a coxitic gypsum dressing is used, or spokes or metal pins are passed through the osteotomy zones. The spokes are fixed with the help of an external device (metalosteosynthesis), which is installed after suturing the abdominal wall. Primary plastic (closure) of the bladder The operation begins with a cut that fringes the exstrophied mucosa from the navel to the seminal tubercle in boys, and in girls to the vaginal opening. Do not often touch the tuffle of the exstrophied mucosa during the intervention: this can lead to the appearance of erosive surfaces on it.

Carefully isolate the bases of the cavernous bodies from the bosom for 5-9 mm and bring them closer by separate absorbable sutures. This method contributes to the elongation of the visible part of the penis. Even greater elongation arises after the reduction and fixation of the bones. However, excessive selection of cavernous bodies on the lower arc of the lateral bone may contribute to the disruption of the blood supply of the cavernous bodies. In girls, the uterus opens outward freely, so the correction of any minimal anomalies of internal female genital organs can be postponed to a later date.

During primary plastic surgery of the bladder, attempts should not be made to correct epispadias. Additional rectification and lengthening of the penis should be performed after reaching the age of 6-12 months.

The navel can be left if it is not strongly biased downwards. Usually the umbilical cord is excised, continuing the incision upwards, simultaneously with the elimination of the hernia of the umbilical cord (if there is one). After removing the natural navel, a new navel is formed in a more "right" position - 2-3 cm above its original location.

Then below the navel penetrate into the retroperitoneal space and the bladder is widely separated from the straight muscles. The selection is continued downwards towards the bosom. Without damaging the periosteum, the tendon-muscle bundles are separated from the pubic bone from both sides. After that, the prostatic and membranous parts of the urethra are mobilized from the bone. When isolating the detrusor, care should be taken to preserve the unaffected vascular pedicle of the bladder on each side.

Removal of urine is performed with cystostomy and ureteral drainage. The mouth of the ureters is catheterized with small tubes (3-5 CH), which are fixed by flashing with a thin chrome catgut. The bladder and the proximal part of the urethra (the area of the neck of the bladder) are then closed in the longitudinal direction by layer-by-layer thin absorbable sutures. Through the bottom of the bladder, a cystostomic drainage of 8-10 CH is formed, leading it out through the newly formed navel. The bladder neck is sutured on the catheter 12-14 CH so that the opening is fairly wide and provides an effective outflow during the incontinence period, and on the other hand, it is sutured very tightly to prevent the bladder from falling out.

After the formation of the neck, a catheter is removed from the urethra. No urethral catheters or tubes leave, as they can promote necrosis and erosion in the urethra of seams applied to the bony bone.

After the bladder and urethra are closed, the assistant rotates (manually) large spits on both sides to bring the bones together, for which nylon seams 2/0 (non-absorbable thread) are used. The horizontal mattress suture is applied laterally, in the calcified part of the bone, by the node anteriorly to prevent the opening of the sutures in the urethra. With anterior osteotomy of the pelvic bones, the external fixation of the dissected pelvic bones prevents the postoperative divergence of the bosom. The skin is sutured with thin individual nylon sutures above the bladder and subcutaneous absorbable sutures on the perineum. Girls can try to pull together the clitoral bodies, but this can be done later.

If the newborn is operated, then it is advisable to carry out modified traction according to Blount. When applying a coke gypsum bandage, it is important to ensure easy bending of the knees in order to prevent blood circulation disorders with passive internal hip rotation.

The Blount tract is carried out for 3 weeks, external fixation for 6 weeks. For a week, antibiotics of a wide spectrum are prescribed for prophylactic purposes, and then they switch to antibiotics used inside, continuing to give them during the entire period of urinary incontinence. This helps prevent kidney damage before the time when bladder ureter reflux is eliminated during the reconstruction of the bladder neck.

Before the removal of the cystostomy tube, which is located above the bosom, the permeability of the urethra is determined. Begin to clamp the catheter for 6-8 hours by measuring the amount of residual urine in the bladder. If the neck of the bladder prevents the release of urine, then a careful dilatation of the urethra by bougies. The tube draining the bladder should not be removed until there is a firm belief in adequate emptying of the bladder.

Incontinence period

After the closure of the bladder, dynamic observation is necessary for 1-2 years. In the case of a successfully completed first stage of bladder exstrophy correction, its growth and increase in volume are noted, usually on an average of 50 ml in 1.5 years. The recommended intake of uroseptics and antibiotics during this period supports the sterility of urine. Frequent exacerbations of pyelonephritis are possible in the presence of vesicoureteral reflux (found in 86% of patients), urolithiasis (it is necessary to exclude bladder stone with ultrasound or cystoscopy). Stenosis of the urethra can also accompany urinary tract infections. Indirect sign of narrowing of the urethra - presence of residual urine after urination. In the future, it may require bougie, stone removal, endoscopic correction of vesicoureteral reflux, or reimplantation of the ureter to treat infection and establish an adequate outflow of urine. Bladder concusses often occur if there is a ligature in the lumen of the bladder. Detrusor stones are destroyed by forceps endoscopically intravesical, they are extracted with crushed.

Reconstruction of the neck of the bladder is recommended to perform in patients with a volume of the bladder of not less than 60 ml. The initial size of the vesicle pad in children with bladder exstrophy is very small, and it is not always possible to rapidly increase the volume of the bladder after the first operation. In such cases, it is possible to carry out the epispadia correction stage before the bladder neck plasty. Expansion of the cavernous bodies and the creation of a long urethra improve the retention of urine and significantly increase the volume of the bladder.

Treatment of epispadias

The penis member is shortened, but with bladder exstrophy it is especially pronounced. According to some data, the average length of the penis in adult men with bladder exstrophy after the correction is twice shorter than normal and corresponds to an average of 7-10 cm. Therefore, the main goal of epispadias correction is the elongation and elimination of deformation of the cavernous bodies in combination with the formation of the urethra channel, providing a normal urination. To prepare for the operation two weeks before the intervention, the treatment of the penis is prescribed with a cream containing testosterone. 2 times a day, which increases the length and improve the blood supply of the cavernous bodies and foreskin. There are many methods of operative treatment of epispadias.

Since during the primary plasty of the bladder the lengthening of the penis is performed, as an intervention for epispadia, modification of urethroplasty along the Yanggu or modification of the Cantwell-Rensley method may be used. Initially, the suture is placed on the head of the penis. Then cut the mucous membrane on the urethral area, edging the external opening of the urethra at the base of the penis, and continue the incisions to the top of the head, forming a longitudinal flap in the form of a strip 14-18 mm wide. At the top of the head, a longitudinal dissection of the tissues along the Heinek Mikulich is performed, followed by their cross-linking in such a way that the new opening of the urethra is in the ventral position.

The tissues of the urethral area are widely mobilized, being careful not to damage the paired neuromuscular bundles located along the dorsal-lateral surface. The cavernous bodies are again separated from the frontal bones, if they were not sufficiently separated during the primary intervention. Through a very careful and careful preparation completely separate the urethral area from the cavernous bodies along the entire length from the neck of the bladder slightly distal to the seminal tubercle to the head. To secure the head of the penis securely on its wings, two wedge-shaped flaps are excised. The urethra is formed by a thin 6/0 continuous suture of the PDS on a soft silicone catheter. The second row of seams on the surrounding tissues is superimposed with separate nodular seams of the PDS.

The tube is hemmed to the head of the penis. Cavernous bodies with epispadias have a pronounced dorsal deformation, which is well revealed by a breakdown with artificial erection after the administration of an isotonic sodium chloride solution. Excision of connective tissue scar is not enough for complete spreading. To eliminate deformation, a cross-section is made along the dorsal surface of both cavernous bodies. The white membrane is mobilized, transforming the transverse defect into a rhomboid one, then the cavernous bodies rotate medially and stitch them together. In this case, the created urethra is located on the floor with cavernous bodies and neuromuscular bundles in anatomically correct position. The second series of seams on the cavernous bodies and surrounding tissues are superimposed with separate nodular seams of the PDS.

The tube is hemmed to the head of the penis. The ventral part of the prepuce is dissected and turned dorsally to cover the newly formed urethra. If, after mobilization of the cavernous bodies, the length of the urethra is insufficient, then for its lengthening it is possible to use free skin preputial flaps, flaps of the mucosa of the bladder or transverse areas of the skin of the ventral part of the prepuce.

However, with a pronounced deformation of the penis for a real increase in its length and eliminating the curvature of dissection and rotation of the cavernous bodies may not be enough. The plasticization of cavernous bodies by the method of grafting makes it possible to achieve a better result.

Under grafting, an increase in the length of the dorsal (hypoplastic) surface of the penis is understood by dissecting the belly coat and sewing 2-3 loose patches of de-epithelial skin. This requires carefully and very carefully to separate the cavernous bodies from the urethral area and the neurovascular bundle. Damage a. Penialis, n. Penialis can lead to sclerosis of the glans penis and impotence. In each cavernous body, two H-shaped incisions are performed on the dorsal surface. Mobilize the belly coat, increasing the length of the dorsal surface of the penis, turning a linear incision into a square defect with a length of 5x5-10x10 mm. Then the arisen defect of the gallbladder shell is closed with a pre-prepared free flap of dezepitelized skin of the foreskin. This method allows you to eliminate the curvature of the penis, increase its visual dimensions and translate into a natural anatomically correct position.

The operation is completed by applying a circular dressing with glycerol (glycerin) for 5-7 days, similar to that used for hypospadias. The tube is removed on the 10th day after the operation. The most common complication after surgery is the urinary fistula of the urethra. Do not attempt to close it before 6 months, because it is necessary to complete the scar processes in the surrounding fistula tissues.

Typical localization for fistula formation in epispadias is the region of the coronal sulcus. In this place, the "neuretra" is the least covered with skin, and it is here after the operation that the greatest tension is noted. In most patients, repeated intervention is necessary to close the fistula. Reconstruction of the neck of the bladder

The main goal of plasty of the neck of the bladder is to ensure free urination with retention of urine without the risk of impaired renal function. This operation is possible only if the child has grown so much that he understands and fulfills the settings and recommendations of the doctor and parents. It is very difficult to teach a child to feel the sensations of the fullness of the bladder that are unknown to him. It is even more difficult to learn how to hold urine and effectively urinate when the bladder is full.

During this entire period, the child and his parents should be under constant supervision, usually frequent visits to the medical center and telephone consultations are required, sometimes periodic catheterization, cystoscopy and control of urine tests are sometimes necessary. According to some researchers, for a successful operation, the volume of the bladder should be at least 60 ml. Attempts to reconstruct the neck with less capacity usually fail. In addition, the child should not have a manifestation of urinary infection. Cystography under anesthesia before the operation allows you to determine the true volume of the bladder, exclude the presence of stones and assess the condition of the heart.

The presence of a large diastase (divergence) of the bones serves as an indication for osteotomy, sometimes even for repeated osteotomy. A sufficient approach of the bosom, allowing to place the urethra inside the pelvic ring, is an indispensable condition for ensuring free controlled urination. The "support" of the urethra by the striated muscle of the urogenital diaphragm and the "suspending" of the neck of the bladder contribute to better retention of urine. To date, the main problems are not related to the intersection and convergence of the pelvic bones, but to their retention in the created right position. The reason for this can be both the eruption of the binding ligatures, and the lag of the underdeveloped shortened bony bone. This view of pathology provides for the implementation of ostomyotomy, which creates optimal anatomical conditions for the full operation of the newly formed urine retention mechanism.

The operation begins with the dissection of the bladder by a very low transverse incision near the neck of the bladder with its prolongation in the vertical direction.

Find the mouth of the ureters and catheter them. The mouth of the ureters is too low, and to strengthen the neck they must be moved higher. It is possible to perform a crossover reimplantation of ureters according to Coznu. The next step is the modified intervention of the Nadbetter. They cut a strip of the mucous membrane of the bladder 30 mm long and 15 mm wide. Starting from the urethra and prolonging the incisions above the pancreatic triangle. After injection of epinephrine (epinephrine), the epithelium adjacent to the carved band is removed under the mucous membrane. The strip is stitched, forming a tube on the catheter 8 SN from it. Then de-impellers detrusor is sewn over the tube in such a way that three layers of tissues are formed. The edge of the detrusor is pre-inscribed with several incisions to lengthen the neck of the bladder, without decreasing its capacity.

The urethra and the bladder are separated from the womb to place the urethra as deep as possible within the pelvic ring. This technique allows you to apply seams to the neck, the purpose of which is to "lift" the neck. Intraoperative urethral pressure after suturing is usually higher than 60 cm of water column. If the visualization of the urethra is difficult, then to ensure good access it is possible to dissect the symphysis of the foramen and dilute it with retinas. U-shaped sutures cover the neck of the bladder muscles first right, and then left side on the principle of "double smelling". Layered (in two layers) by the type of "double smelling" suturing along the midline of the lower transverse incision further narrows and extends the neck of the bladder. The bladder is drained with cystostomic drainage for 3 weeks. Ureteral catheters are left for at least 10 days. In the urethra, no catheters are left.

With the urethra, no manipulations are performed for 3 weeks, then a catheter 8 CH is conducted along it. You may need careful bougieirovanie. Sometimes an accurate anatomy is helped by urethroscopy. Suprapubic (cystostomic) drainage can be removed only when the urethra is catheterized freely. In this case, cystostomic drainage is squeezed, and the child is allowed to urinate. If the child urinates without difficulty, then perform ultrasound of the kidney and ureter or intravenous urography to determine if there is ureterohydronephrosis. If there is no hydronephrosis or it is present, but does not progress as compared to preoperative data, then the cystostomy tube is removed.

Careful monitoring is carried out until the capacity of the bladder increases. In addition, it is mandatory to make regular urine tests so as not to miss the urinary infection. If episodes of acute urinary infection occur frequently, then ultrasound, x-ray examination or cystoscopy is performed to exclude stones or foreign body. Exstrophy of the bladder is a rare pathology in pediatric urology. Such complex patients are traditionally concentrated in large clinics, which have accumulated extensive experience in the treatment of epispadias and bladder exstrophy. Ensuring an adequate retention of urine in children with bladder exstrophy without compromising kidney function and forming genitals visually not different from the norm is the direction that characterizes the current stage of treatment of this severe pathology. Correction of bladder exstrophy requires timely surgical stages of treatment and long painstaking monitoring of the patient's condition during the period of bladder growth.

Patients with bladder exstrophy constantly need to solve everyday problems. This warning of exacerbations of pyelonephritis and correction of vesicoureteral reflux, prevention of bladder concretion and the search for minimally invasive methods for their removal, treatment of restrained inguinal hernias and correction of cryptorchidism. The second stage of treatment - correction of epispadias is difficult to call easy. Complete reliable removal of the deformation of the cavernous bodies and the creation of a long, tight, urethral canal in a child with a minimum penis size at an early age (1-3 years) also requires special training. Achieving gradual growth and increase in the volume of the urinary bladder to 100-150 ml in 3-4-year-old children, satisfactory retention of urine with dry intervals of 1-3 hours remains the most difficult task even for specialists. Good results in the treatment of exstrophy are the result of several severe urological and orthopedic surgeries. It is very important that each surgical intervention be performed on time according to the indications of surgeons who have sufficient experience of epispadias and bladder exstrophy.