Treatment of epispadias and bladder exstrophy in children

Immediately after the birth of a child with bladder exstrophy, questions about examination methods, duration of preoperative preparation, nature of surgical intervention, type of skeletal traction and postoperative management are discussed. Primary bladder plastic surgery is usually performed within 48-96 hours after birth. If the child needs to be transported over a long distance, appropriate hydration is provided.

Treatment methods for bladder exstrophy

Treatment of bladder exstrophy is aimed at solving the following

• elimination of defects of the urinary bladder and anterior abdominal wall;
• creation of a penis that is acceptable both cosmetically and sexually;
• maintaining kidney function and ensuring urine continence.

All patients are candidates for bladder plastic surgery (closure), and only in isolated cases is it necessary to resort to urine diversion. Even in children with a very small bladder (2-3 cm), surprisingly rapid growth is noted after primary closure.

The staged treatment of epispadias and bladder exstrophy includes three stages:

• Correction of the anomaly (the first stage) begins with closure of the urinary bladder immediately after birth, usually in combination with osteotomy of the iliac bones (in children older than 10-15 days or with a platform size of 5 cm or more). After closure of the bladder, a period of urinary incontinence is distinguished, during which the bladder gradually grows and its capacity increases.
• Surgical correction of epispadias in boys (second stage) is currently performed during this period of incontinence (usually at 2-3 years). No attempts to ensure urinary continence are made until 3.5-4 years.
• In 3.5-4 year old children, bladder neck plastic surgery is performed (stage three). Before this, the bladder volume is assessed. Bladder neck reconstruction is not performed until an adequate volume is achieved (more than 60 ml) and until the child grows so much that he or she begins to realize the need to hold urine.

Primary plastic surgery (closure) of the bladder

The goals of primary bladder closure are:

• rotation of the innominate bones to bring the pubic symphysis closer together;
• closure of the bladder and its displacement to the posterior position, into the pelvic cavity;
• formation of the neck of the urinary bladder and ensuring the free discharge of urine through the urethra;
• if necessary, primary lengthening of the penis (partial mobilization of the cavernous bodies from the pubic bones);
• suturing of the defect of the anterior abdominal wall.

Broad-spectrum antibiotics are prescribed prior to surgery to reduce the risk of wound infection and osteomyelitis.

In children with inguinal hernia, bilateral hernioplasty is performed simultaneously with bladder plastic surgery. This tactic allows avoiding emergency operations in the early postoperative period for strangulated inguinal hernia. In the presence of cryptorchidism, orchopexy is also performed, but usually the testicles only appear to be located high due to the displacement of the rectus muscle.

Surgical interventions. Osteotomy

If osteotomy is necessary, the pelvic bones can be cut from behind or in front (posterior or anterior osteotomy) or a combination of both.

Indications for osteotomy are:

• large diastasis of the pubic bones (more than 4-5 cm) and difficulties in bringing them together during primary plastic surgery in newborns;
• the child is older than 10-15 days.

Researchers believe that the bones of a newborn become more dense and elastic with each day of life. Reduction of the pubic bones without osteotomy at the age of 2 honey is often accompanied by divergence of the symphysis in the distant future.

Previously, posterior osteotomy was used more often and achieved good results. To access the ilium from behind, two vertical incisions were made lateral to the sacroiliac joint. After delimiting the contents of the greater sciatic foramen (gluteal nerves and vessels), both plates (surfaces) of the ilium were transected from the posterior iliac crest toward the sciatic notch. Currently, most surgeons prefer anterior iliac osteotomy of the pelvic bones (analogous to the Chiari operation).

The anterior approach has advantages and is more convenient, since both osteotomy and bladder plastic surgery are performed in the same position of the child - he must be turned during the procedure. To stabilize the bone fragments, a coxite plaster bandage is used, or pins or metal pins are passed through the osteotomy zones. The pins are fixed with an external device (metal osteosynthesis), which is installed after suturing the abdominal wall. Primary bladder plastic surgery (closure) The operation begins with an incision bordering the extrophic mucous membrane from the navel to the seminal tubercle in boys, and to the vaginal opening in girls. Do not frequently touch the extrophic mucous membrane with a swab during the procedure: this can lead to the formation of erosive surfaces on it.

The bases of the cavernous bodies are carefully separated from the pubis by 5-9 mm and brought together with separate absorbable sutures. This technique helps to lengthen the visible part of the penis. Even greater lengthening occurs after the pubic bones are brought together and fixed. However, excessive separation of the cavernous bodies on the lower arch of the pubic bone can contribute to disruption of the blood supply to the cavernous bodies. In girls, the uterus opens outward freely, so correction of any minimal anomalies of the internal female genital organs can be postponed until a later date.

During primary bladder plastic surgery, no attempt should be made to correct epispadias. Additional straightening and lengthening of the penis is advisable after reaching the age of 6-12 months.

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

The retroperitoneal space is then penetrated below the umbilicus and the bladder is widely separated from the rectus muscles. The separation is continued downwards towards the pubis. Without damaging the periosteum, the tendon-muscle bundles are separated from the pubic bone on both sides. The prostatic and membranous parts of the urethra are then mobilized from the bone. When separating the detrusor, care must be taken to preserve the vascular pedicle of the bladder on each side.

Urine diversion is performed using a cystostomy and ureteral drainage. The ureteral orifices are catheterized with small tubes (3-5 CH), which are fixed by suturing with thin chromic catgut. The urinary bladder and the proximal part of the urethra (bladder neck area) are then closed longitudinally in layers with thin absorbable sutures. A cystostomy drainage of 8-10 CH is formed through the bottom of the urinary bladder, leading it out through the newly formed navel. The neck of the urinary bladder is sutured on a 12-14 CH catheter so that the opening is quite wide and ensures effective outflow during the period of urinary incontinence, and on the other hand, it is sutured very tightly to prevent prolapse of the urinary bladder.

After the neck is formed, the catheter is removed from the urethra. No urethral catheters or tubes are left in place, as they may contribute to necrosis and erosion of the sutures placed on the pubic bones into the urethra.

After the bladder and urethra are closed, the assistant manually rotates the greater trochanters on both sides to approximate the pubic bones using 2/0 nylon (nonabsorbable) sutures. A horizontal mattress suture is placed laterally in the calcified portion of the bone with the knot anteriorly to prevent the sutures from cutting into the urethra. In anterior pelvic osteotomy, external fixation of the severed pelvic bones prevents postoperative pubic dehiscence. The skin is closed with fine interrupted nylon sutures over the bladder and subcutaneous absorbable sutures on the perineum. In girls, an attempt may be made to approximate the bodies of the clitoris, but this may be done later.

If a newborn is being operated on, it is advisable to perform a modified Blount traction. When applying a coxite plaster cast, it is important to ensure slight flexion of the knees to prevent disruption of the blood supply with passive internal rotation of the hips.

Blount traction is used for 3 weeks, external fixation for 6 weeks. Broad-spectrum antibiotics are given prophylactically for a week, and then oral antibiotics are given for the duration of urinary incontinence. This helps prevent kidney damage until the vesicoureteral reflux is eliminated by bladder neck reconstruction.

Before removing the cystostomy tube above the pubis, the patency of the urethra is determined. The catheter is clamped for 6-8 hours, measuring the amount of residual urine in the bladder. If the neck of the bladder prevents urine from flowing, then careful dilation of the urethra is performed with bougies. The tube draining the bladder should not be removed until there is firm confidence in adequate emptying of the bladder.

Period of urinary incontinence

After bladder closure, dynamic observation is required for 1-2 years. In case of successful first stage of bladder exstrophy correction, its growth and increase in volume are noted, usually on average up to 50 ml in 1.5 years. The recommended intake of uroseptics and antibiotics during this period maintains urine sterility. Frequent exacerbations of pyelonephritis are possible in the presence of vesicoureteral reflux (occurs in 86% of patients), urolithiasis (it is necessary to exclude a bladder stone using ultrasound or cystoscopy). Stenosis of the urethra can also accompany urinary tract infections. An indirect sign of narrowing of the urethra is the presence of residual urine after urination. In the future, its bougienage, removal of stones, endoscopic correction of vesicoureteral reflux or reimplantation of the ureters may be required to treat the infection and establish adequate urine outflow. Bladder stones are more likely to occur if there is a ligature in the bladder lumen. Detrusor stones are destroyed with forceps endoscopically intravesically, they are removed crushed.

Reconstruction of the bladder neck is recommended for patients with a bladder volume of at least 60 ml. The initial size of the bladder area in children with bladder exstrophy is very small, and it is not always possible to quickly increase the bladder volume after the first operation. In such cases, it is possible to perform the epispadias correction stage before bladder neck plastic surgery. Straightening the cavernous bodies and creating a long urethra improves urine retention and significantly increases the bladder volume.

Treatment of epispadias

The penis with epispadias is shortened, but with bladder exstrophy this is especially pronounced. According to some data, the average penis length in adult men with bladder exstrophy after correction is two times shorter than normal values and is on average 7-10 cm. That is why the main goal of epispadias correction is to lengthen and eliminate the deformation of the cavernous bodies in combination with the formation of the urethra, ensuring normal urination. To prepare for surgery, two weeks before the intervention, the penis is prescribed to be treated with a cream containing testosterone. 2 times a day, which helps to increase the length and improve the blood supply to the cavernous bodies and foreskin. There are many methods of surgical treatment of epispadias.

Since the penis is lengthened during primary bladder plastic surgery, a modification of the Young urethroplasty or a modification of the Cantwell-Wrensley method can be used as an intervention for epispadias. Initially, a stay suture is applied to the glans penis. Then, an incision is made in the mucous membrane on the urethral site, bordering the external opening of the urethra at the base of the penis, and the incisions are continued to the top of the glans, forming a longitudinal flap in the form of a strip 14-18 mm wide. At the top of the glans, a longitudinal dissection of the tissues is performed according to Heineke-Mikulich, followed by their suturing in the transverse direction so that the new opening of the urethra is in a ventral position.

The urethral site tissues are widely mobilized, taking care not to damage the paired neurovascular bundles located along the dorsal-lateral surface. The cavernous bodies are again separated from the pubic bones if they were insufficiently separated during the primary intervention. By very careful and gentle dissection, the urethral site is completely separated from the cavernous bodies along the entire length from the neck of the bladder slightly distal to the seminal tubercle to the glans. To reliably suture the glans penis, two wedge-shaped flaps are excised on its wings. The urethra is formed with a thin 6/0 continuous PDS suture on a soft silicone catheter. The second row of sutures on the surrounding tissues is applied with separate interrupted PDS sutures.

The tube is sutured to the head of the penis. The cavernous bodies in epispadias have a pronounced dorsal deformation, which is well revealed by a test with an artificial erection after the introduction of an isotonic sodium chloride solution. Excision of connective tissue scars is sometimes insufficient for complete straightening. To eliminate the deformation, a transverse incision is made along the dorsal surface of both cavernous bodies. The protein membrane is mobilized, turning the transverse defect into a diamond-shaped one, then the cavernous bodies are rotated medially and sutured together. In this case, the created urethra is located under the cavernous bodies and neuromuscular bundles in an anatomically correct position. The second row of sutures on the cavernous bodies and surrounding tissues is applied with separate interrupted PDS sutures.

The tube is sutured to the glans penis. The ventral foreskin is incised and rotated dorsally to close the newly formed urethra. If the urethra is insufficient in length after mobilization of the corpora cavernosa, free preputial skin flaps, bladder mucosa flaps, or transverse skin sections of the ventral foreskin can be used to lengthen it.

However, in case of severe deformation of the penis, dissection and rotation of the cavernous bodies may not be enough to actually increase its length and eliminate curvature. Cavernous body plastic surgery using the grafting method allows achieving a better result.

Grafting is understood as increasing the length of the dorsal (hypoplastic) surface of the penis by dissecting the tunica albuginea and suturing 2-3 free flaps of de-epithelialized skin. This requires careful and very gentle separation of the cavernous bodies from the urethral platform and the vascular-nerve bundle. Damage to a. penialis, n. penialis can lead to sclerosis of the glans penis and impotence. Two H-shaped incisions are made on the dorsal surface of each cavernous body. The tunica albuginea is mobilized, increasing the length of the dorsal surface of the penis, turning the linear incision into a square defect 5x5-10x10 mm long. Then the resulting defect of the tunica albuginea is covered with a pre-prepared free flap of de-epithelialized foreskin skin. This method allows you to eliminate the curvature of the penis, increase its visual size and transfer it to 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 the operation is a urinary fistula of the urethra. No attempt should be made to close it earlier than 6 months later, since it is necessary to complete the cicatricial processes in the tissues surrounding the fistula.

The typical location for the formation of a fistula in epispadias is the coronal sulcus. In this area, the "neourethra" is least covered by skin, and it is here that the greatest tension is observed after surgery. In most patients, repeated intervention is necessary to close the fistulas. Reconstruction of the neck of the bladder

The main goal of bladder neck plastic surgery is to ensure free urination with urine retention without the risk of kidney dysfunction. This operation is possible only if the child has grown so much that he understands and follows the instructions and recommendations of the doctor and parents. It is very difficult to teach a child to feel the unfamiliar sensations of a full bladder. It is even more difficult to learn to hold urine and urinate effectively when the bladder is full.

During this entire period, the child and his parents should be under constant supervision, usually requiring frequent visits to the medical center and telephone consultations, sometimes periodic catheterization, cystoscopy and urine analysis monitoring. According to some researchers, for a successful operation, the bladder volume should be at least 60 ml. Attempts to reconstruct the cervix with a smaller capacity usually fail. In addition, the child should not have manifestations of urinary infection. Cystography under anesthesia before surgery allows you to determine the true volume of the bladder, exclude the presence of stones and assess the condition of the womb.

The presence of a large diastasis (divergence) of the pubic bones is an indication for osteotomy, sometimes even for repeated osteotomy. Sufficient convergence of the pubis, allowing the urethra to be placed inside the pelvic ring, is a prerequisite for ensuring free, controlled urination. "Support" of the urethra by the striated muscles of the urogenital diaphragm and "suspension" of the neck of the bladder contribute to better urine retention. Today, the main problems are not associated with the intersection and convergence of the pelvic bones, but with their retention in the created correct position. This can be caused by both the eruption of connecting ligatures and the lag in the incorporation of underdeveloped shortened pubic bones. This view of the pathology involves performing staged osteotomies, which creates optimal anatomical conditions for the full functioning of the newly formed urine retention mechanism.

The operation begins with a very low transverse incision into the bladder near the neck of the bladder, extending it in a vertical direction.

The ureteral orifices are located and catheterized. The ureteral orifices are located too low and must be moved higher to strengthen the neck. Crossed ureteral reimplantation according to Kozn is possible. The next step is a modified Niadbetter procedure. A strip of bladder mucosa 30 mm long and 15 mm wide is excised, starting from the urethra and extending the incisions above the bladder triangle. After injecting epinephrine (adrenaline), the epithelium adjacent to the excised strip is removed under the mucosa. The strip is sutured, forming a tube on an 8 CH catheter. Then the deepithelialized detrusor is sutured over the tube so that three layers of tissue are formed. The edge of the detrusor is preliminarily incised with several incisions to lengthen the bladder neck without reducing its capacity.

The urethra and bladder are separated from the pubis to place the urethra as deeply as possible within the pelvic ring. This technique allows for the placement of sutures on the neck, the purpose of which is to “lift” the neck. Intraoperative urethral pressure after suturing is usually higher than 60 cm H2O. If visualization of the urethra is difficult, the pubic symphysis can be dissected and spread apart with retractors to ensure good access. The neck of the bladder is covered with U-shaped sutures using the muscles of the right side first, then the left side, using the “double suturing” principle. Layered (in two layers), “double suturing” type suturing along the midline of the lower transverse incision additionally narrows and lengthens the neck of the bladder. The bladder is drained with a cystostomy drainage for 3 weeks. Ureteral catheters are left for at least 10 days. No catheters are left in the urethra.

The urethra is not manipulated in any way for 3 weeks, then an 8 CH catheter is inserted through it. Careful bougienage may be necessary. Sometimes urethroscopy helps to clarify the anatomical situation. The suprapubic (cystostomy) drainage can be removed only when the urethra is freely catheterized. In this case, the cystostomy drainage is clamped, and the child is allowed to urinate. If the child urinates without difficulty, an ultrasound of the kidneys and ureters or intravenous urography is performed to determine whether there is ureterohydronephrosis. If there is no hydronephrosis or it is present but does not progress compared to preoperative data, the cystostomy tube is removed.

Careful monitoring is carried out until the bladder capacity increases. In addition, regular urine tests are mandatory to avoid missing a urinary infection. If episodes of exacerbation of urinary infection occur frequently, then ultrasound, X-ray examination or cystoscopy is performed to exclude stones or a foreign body. Bladder exstrophy is a rare pathology in pediatric urology. Such complex patients are traditionally concentrated in large clinics that have accumulated extensive experience in the treatment of epispadias and bladder exstrophy. Ensuring acceptable urinary continence in children with bladder exstrophy without compromising renal function and the formation of genitals that are visually indistinguishable from the norm is the direction that characterizes the modern stage of treatment of this severe pathology. Correction of bladder exstrophy requires timely surgical stages of treatment and long-term painstaking monitoring of the patient's condition during the period of bladder growth.

Patients with bladder exstrophy constantly need to solve everyday problems. This is the prevention of exacerbations of pyelonephritis and correction of vesicoureteral reflux, prevention of bladder stones and search for minimally invasive methods of their removal, treatment of strangulated inguinal hernias and correction of cryptorchidism. The second stage of treatment - correction of epispadias can hardly be called easy. Complete reliable elimination of deformation of the cavernous bodies and creation of an extended hermetic urethra in a child with minimal penis size at an early age (1-3 years) also requires special training. Achieving gradual growth and increase in bladder volume to 100-150 ml in 3-4-year-old children, satisfactory urinary retention with dry intervals of 1-3 hours remains a difficult task even for specialists. Good results of exstrophy treatment are the result of several difficult urological and orthopedic operations. It is very important that each surgical intervention is performed in a timely manner according to indications by surgeons with sufficient experience in the treatment of epispadias and bladder exstrophy.