Epispadias and Bladder Exstrophy: Treatment in Adults

In the primary forms of epispadias, dorsal deviation of the penis is always detected with angles of the latter exceeding 50 °. With iatrogenic deviations, the combined dorsolateral deformity with axial rotation of cavernous bodies is most often noted. According to S. Woodhouse (1999), dorsal deformity is observed in 77% of adult patients, unilateral fibrosis of cavernous bodies - in 9% of cases, and in 14% of patients there is a bilateral lesion of cavernous bodies. Complex deformities in adult patients are considered the result of previous reconstructive interventions, including the use of cavernous bodies, in particular the belly coat as a plastic material.

As a rule, treatment of bladder exstrophy (anterior abdominal wall plastic, bladder formation) and the elimination of urinary incontinence are performed in early childhood. Urethroplasty, correction of deviation of the penis - the second stage, carried out in 5-7-year-old children. Most authors adhere to the concept of complete anatomical reconstruction of the pelvic ring in the primary formation of the bladder. Only this approach allows to increase the efficiency of correction of urinary incontinence and maintain the capacity of the bladder, which, in turn, relieves the patient from the crippling derivational techniques - ureterosigmostomii, ureterorectostomy, etc. According to P. Sponseller (1995), the best results are achieved using lateral transverse osteotomy. Existing methods of plastic, eliminating urinary incontinence, are very numerous. In Russia, widely used methods VM. Derzhavina and plastic sphincter no Young-Dees. The latter in various modifications are widely used in Europe. Some authors recommend strengthening the pelvic floor with a synthetic loop, wrapping the formed neck of the bladder with a silicone cuff, a gland flap and a detrusor flap, suspending the urethra on the loop to the anterior abdominal wall. Various variants of sling operations are applied - ТVT, etc. Performing loop plasty of the neck of the bladder and the muscles of the pelvic floor has brought positive results. They also use the Gebel-Shtekel's operations, strengthening the neck of the bladder with a demicose valve of the latter. Relatively satisfactory results were obtained using the pelvic lumbar pelvic floor. Implantation of an artificial sphincter of the bladder in patients of a more mature age is described, but if we take into account previous surgical interventions, this type of treatment is associated with a risk of erosion of the urethra and inferiority of the sphincter. In pediatric practice and in patients of more mature age, periurethral submucosal injections of Teflon and collagen are used to correct urinary incontinence. Nevertheless, despite the significant success of plastic reconstructive surgery in the correction of exstrophy and epispadias, negative results of functional reconstruction of the bladder are observed quite often and the problem of urinary incontinence in such patients remains relevant.

In 1895 J. Cantwell first performed urethroplasty with total epispadias. The essence of the technique was the complete mobilization of the dorsal urethral plate and the location of the tubularized urethra under the cavernous bodies, which had previously been rotated in the dorsal direction and connected in the middle third. Many of the currently existing techniques are various modifications of the Cantwell operation. The incidence of complications in this type of intervention is about 29%.

In 1963, E. Michalowski and W. Modelski proposed a multi-stage version of epispadias correction. Since that time, many variants of phased urethroplasty have been developed using skin, prepubic and islet flaps. Various methods of correction of exstrophy and epispadias have been borrowed from the technique of performing urethroplasty, which are applicable for hypospadias, for example, overhead urethroplasty using a flap of the mucous membrane of the cheek. The multiple surgical treatment of epispadias and bladder exstrophy is ambiguous according to the results, are not devoid of shortcomings and are associated with complications observed in the operative correction of hypospadias. The greatest number of the latter occurs when the Tirsch-Yang method is followed and the displaced islet flap is used. According to P. Caione (2001), the incidence of complications is 66% and 73%, respectively. According to the results of studies, with exstrophy the incidence of complications reaches 64% versus 33% with total epispadias in combination with urinary incontinence.

To eliminate deformities in epispadias and exstrophy, in rare cases, the methods of corpporoplasty used for deformities acquired, for example, in Peyronie's disease, are used. Differences consist in the fact that they, as a rule, are used only with the expressed asymmetry of cavernous bodies and as a plastic material only the skin flap and the dura mater are used. In the overwhelming majority of cases, the technique of ventral rotation of cavernous bodies, first proposed by S. Koff (1984), is performed. Later it was modified. Currently, it is known as Cantwell-Ransley Corpoplasty and consists of rotating cavernous bodies and imposing a cavernous-cavernous at the point of maximum deviation.

The method proposed by M. Mitchell and D. Bagli in 1996 is becoming increasingly widespread, and consists of performing a complete penile dissection and creating new anatomical relationships between the urethra and cavernous bodies.

The principle of operative correction of epispadias using the Mitchell method is based on the fact that the anatomy of the penis in this anomaly is different from that of hypospadias due to various embryogenesis of these conditions.

Hypospadia is a fixation on the path of normal development of the urogenital tract, while epispadia is a gross distortion of its normal development. With epispadias, the urethral plate is completely formed, the deforming process only leads to a disruption of its closure. Cavernous bodies are split, but they have normal innervation and blood supply, although the features of the latter remain the subject of further study.

Unlike the method of S. Perovic (1999), using the Mitchell-Bagli method, there is no violation of the glanuloapical relationship. Of particular interest is the modification of the Mitchell operation proposed by P. Caione in 2000, consisting in creating a half-sleeve that mimics the external sphincter, from the perineal muscular complex and paraprostatic tissues in the neck of the bladder.

The incidence of complications after Mitchell surgery and its various modifications is 11%, and the frequency of fistula development of neo -urethra is 2.4% versus 5-42% for Cantwell-Ransley operation.

The problems of correcting the length of the penis are rather complicated and the problems of correction of the penis length remain unresolved. Unfortunately, interventions performed in childhood aimed at the maximum possible isolation of cavernous bodies, up to their separation from the lower branch of the pubic bone, in conjunction with the correction of curvature in Cantwell-Ransley, do not significantly increase the length of the penis. Moreover, complete mobilization of cavernous bodies is associated with a risk of damage to the cavernous arteries.

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

The method of one-stage urogenital reconstruction (Kovalev-Koroleva's operation)

In 1998, V. Kovalev and S. Koroleva were offered operative treatment of epispadias and bladder exstrophy in adults. Its distinctive feature is the simultaneous execution of an elongating urethra. Corporeal, glanulo-, spongio, sphincter-and abdomenoplasty.

In all cases the technique of full penile preparation was used. With the preservation of the urethral plate, it was separated from the cavernous bodies, mobilized to the region of the seminal tubercle or bladder. Subsequently, dissection of cavernous bodies with excision of the chord and scar tissue was performed. Own urethral plate tubularize, perform bilateral corpototomy. It is considered justified and expedient to perform several bilateral corpototomy (at least two), since after mobilization of the urethral plate, excision of the chord and scar tissue of a single median corpotomy for a complete correction of the penile deformation is not enough. This is due to the combined nature of penile deviation, as well as the direct involvement of intracorporeal factors in its formation in adult patients. As an plastic material for corpporoplasty, an autovenous flap (v. Saphena magna) is used, for which appropriate access is made on the medial surface of the thigh. After performing corpotomy, the difference in the length of the tubularized urethral plate and cavernous bodies becomes evident. With the purpose of lengthening the urethra, an islet vascularized flap is taken on the feeding stem. Two-level corpoplasty can simultaneously eliminate deviation and increase the length of the penis. The isolated islet flap is tubularized and anastomosis is performed with the tubularized own urethral plate (urethro-neuretroanastomosis). The length of the expandable part of the urethra (neurethra) depends on the presence of plastic material and the length of the corpora cavernosa after corpoplasty and ranges from 2 to 6 cm. The formation of an official arbitrary sphincter of the bladder is performed by rotating the vascular muscle flap of the rectus abdominis muscle and transposing it into the cervical Bubble with the creation of a muscular clutch around it. Abdomenoplasty is performed by fixing the flap of the rectus abdominis muscle in the papillary zone, which contributes to replenishing the bony defect of the womb due to diastasia, additional sealing of the bladder and urethral sutures, improving trophism of the tissues, and also gives a vertical directivity to the muscle fibers when they are transposed to the cervical region . At the end of the operative intervention, the rotation of the cavernous bodies and the ventral transposition of the neo-urethra with the formation of the external opening on the head of the penis. In some cases, with a deficit of plastic material and insufficient length of neo-urethra, the outer opening is shaped like a coronary hypospadias. The feeding leg of the urethral flap, when it is moved to the polar surface of the penis, not only improves neo-urethra trophism and seals the seams after tubularization of the flap and urethral anastomoses, thus preventing the formation of fistulas, but also provides a cosmetic effect of the presence of the spongy urethra (spongioplasty). The cosmetic effect is all the more pronounced, the greater the thickness of the feeding leg. The skin defect is replenished with the help of local tissues and displaced vascularized grafts.

In the postoperative period, anticoagulants, disaggregants, angioprotectors, antioxidants, ozonotherapy, laser therapy, vacuum therapy should be prescribed to improve microcirculation and trophic flap. Alpha-adrenoblockers were used to eliminate hyperreflexia of the bladder and sympathetic constrictor influence. In addition, the training of an arbitrary official sphincter of the bladder was performed. The rehabilitation program included sexological training, various methods of psychotherapy, drug correction of psychoemotional disorders.

Results and discussion

Evaluation of the results of operative treatment of epispadias and bladder exstrophy was carried out in terms of one to ten years. A total of 34 patients were operated on. Functional and aesthetic results served as criteria for assessing outcomes of surgical interventions. Sphincteroplasty was performed in 73.5% of cases with preserved reservoir function of the bladder, and lengthening urethro- and corpoplasty to all patients, including those who underwent various kinds of intestinal urinary diversion, since even in the absence of a natural act of urination, the formation of the urethra as an ejaculatory duct is an important component of social and sexual rehabilitation. The cosmetic effect was evaluated based on the appearance of the penis, its length, the shape of the head, the absence or presence of deformity. With the method described, the penis was elongated by 2-2.5 cm, which in a number of cases allowed the extender to be used and an additional 1 cm elongation was achieved.

Visual rectification of the penis in a relaxed state was achieved in all patients. In 80% of patients, the angle of erectile deformation did not exceed 20%, which was regarded as functionally insignificant, not requiring correction. In several cases, the relapse of the deviation was 30 to 45 °. Three patients were reoperated (lengthening corpoplasty). The conical head is marked in 36% of observations. This is considered not a complication, but a characteristic feature of operative treatment of epispadias and bladder exstrophy. All patients were satisfied with the aesthetic results of epispadias treatment and bladder exstrophy.

The functional result was assessed by the preservation of erectile and ejaculatory functions, the quality of urination, the consistency of the continent mechanism. The absence of postoperative erectile dysfunction in this extensive and complex surgical treatment of epispadias and bladder exstrophy can be explained by the peculiarities of the vascular architectonics of the abnormal penis and the operational technique, which consists in creating access to the alveolar envelope at the stage of corpoplasty in the avascular zone. Asthenic ejaculation was noted in 47.1% of patients, 20.6% indicated retarded ejaculation.

A full functioning of the official muscular sphincter was noted in 80% of patients. In 20% of cases, leakage and partial urinary incontinence in orthostasis were noted, which was also regarded as a positive result (compared with the initial total incontinence). Operative treatment of epispadias and bladder exstrophy allowed patients to stop using diapers permanently and switch to episodic (with physical activity) application of the penile clamp.

Ischemia of the head (20.5%) and necrotic changes in the skin of the penis (11.8%) were the most frequent specific complications in the above type of operative treatment of epispadias and bladder exstrophy and a natural consequence of tissue trophism as a result of numerous previous operations and reconstruction proper. However, they are not considered functionally significant, as against the background of a full-fledged complex therapy, in all cases it was possible to preserve the head and skin of the penis without resorting to additional plastic operations.

Urethral fistulas (as a postoperative complication) were found in 6% of patients. This index is lower than in other cases of corporeurethroplasty, but higher than with the primary surgery for exstrophy and epispadias in childhood, which can be explained, as a rule, by an increased volume of repeated surgical treatment of epispadias and bladder exstrophy.

Social adaptation was achieved in all patients. All patients had heterosexual orientation. 88% of patients after complex treatment of epispadias and bladder exstrophy were able to perform coitus, i.e. Fully sexually adapted. The remaining patients used alternative methods of sexual relations. A permanent sexual partner had 44% of patients. Four of them created families, three of them had children. The average satisfaction score for life was 17 ± 2.5 (70.8% of the maximum achievable score).

Conclusion

A full one-stage urogenital reconstruction (Kovalyova Koroleva's operation) with bladder exstrophy and total epispadias in adult patients is considered a pathogenetically grounded method. From the point of view of the preservation of the erectile function, it is safe and justified. The peculiarities of the urethral ventilation help to reduce the incidence of complications (for example, the urethral fistula), which are more common when using other methods. The use of full penile preparation and several types of flaps for organ reconstruction provides the opportunity to freely rotate elongated cavernous bodies along with the glans penis, strengthen the neck of the bladder, lengthen the urethra and perform its transposition, which allows creating new synthopic ratios closest to the anatomical norm

It should be noted that this method of reconstruction does not disturb the glanuloapical relationships, which certainly contributes to a reduction in the number of complications associated with trophic head disorders. Simultaneous implementation of complete urogenital reconstruction allows maximum use of plastic material and provides a satisfactory cosmetic and functional result. The creation of an official muscular sphincter by transposition of the rectus muscle flap is justified not only from the functional muscle (the mechanism of continence and improvement of local trophism), but also from the cosmetic point of view. After a one-stage urogenital reconstruction, all patients with epispadias and exstrophy experienced a significant increase in the level of social and sexual adaptation, which was reflected in the expansion of the range of social and sexual contacts, as well as in the increase in the range of communications. This is based not only on eliminating the primary psychotraumatic factor, but also on increasing self-esteem after successful surgery. In patients who underwent various derivational treatment of epispadias and bladder exstrophy, restoration of the urethra as an ejaculatory duct, taking into account the safety of the libido, ejaculation and orgasm, is considered an important and indispensable stage of social rehabilitation.

Social and sexual adaptation of patients with exstrophy and total epispadia requires combined application of methods of plastic reconstructive surgery and psychological rehabilitation. The use of psycho- and pharmacotherapy to achieve the optimal psycho-emotional background allows you to quickly achieve sexual and social adaptation of this category of patients.

[5], [6], [7], [8], [9], [10]