Epispadias and bladder exstrophy - treatment in adults

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

As a rule, treatment of bladder exstrophy (plastic surgery of the anterior abdominal wall, formation of the bladder) and elimination of urinary incontinence are performed in early childhood. Urethroplasty, correction of penile deviation is the second stage, carried out in children aged 5-7 years. Most authors adhere to the concept of complete anatomical reconstruction of the pelvic ring during the primary formation of the bladder. Only this approach allows increasing the effectiveness of correction of urinary incontinence and preserving the capacity of the bladder, which, in turn, relieves the patient from mutilating derivation techniques - ureterosigmoidostomy, ureterorectostomy, etc. According to P. Sponseller (1995), the best results are achieved using lateral transverse osteotomy. There are many existing plastic techniques that eliminate urinary incontinence. In Russia, the methods of V.M. Derzhavin and sphincter plastic surgery no Young-Dees have become widespread. The latter in various modifications is widely used in Europe. Some authors recommend strengthening the pelvic floor with a synthetic loop, wrapping the formed bladder neck with a silicone cuff, omental flap and detrusor flap, hanging the urethra on a loop to the anterior abdominal wall. Various types of sling operations are used - TVT, etc. Loop plastic surgery of the bladder neck and pelvic floor muscles has yielded positive results. Hebel-Steckel operations are also used, strengthening the bladder neck with a demucosal valve of the latter. Relatively satisfactory results were obtained using loop plastic surgery of the pelvic floor muscles. Implantation of an artificial sphincter of the bladder in older patients has been described, but if previous surgical interventions are taken into account, this type of treatment is associated with the risk of developing urethral erosion and sphincter insufficiency. In pediatric practice and in older patients, periurethral submucosal injections of Teflon and collagen are used to correct urinary incontinence. However, despite significant advances in plastic reconstructive surgery in the correction of extrofnia 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 performed the first urethroplasty for total epispadias. The essence of the technique was the complete mobilization of the dorsal urethral plate and the placement of the tubularized urethra under the cavernous bodies, which were previously rotated in the dorsal direction and connected in the middle third. Many currently existing techniques are various modifications of the Cantwell operation. The complication rate for this type of intervention is about 29%.

In 1963, E. Michalowski and W. Modelski proposed a multi-stage version of epispadias correction. Since then, many versions of staged urethroplasty have been developed using skin, preputial, and insular flaps. Various methods of exstrophy and epispadias correction were borrowed from the technique of urethroplasty used in hypospadias, for example, overlay urethroplasty using a flap from the mucous membrane of the cheek. The various surgical treatments for epispadias and bladder exstrophy are controversial in their results, are not without drawbacks, and are associated with complications observed during surgical correction of hypospadias. The greatest number of the latter occurs when performing the Thiersch-Young technique and using a displaced insular flap of the foreskin. According to P. Caione (2001), the complication rate is 66% and 73%, respectively. According to research results, with exstrophy the complication rate reaches 64% versus 33% with total epispadias combined with urinary incontinence.

To correct the deformation in epispadias and exstrophy, corporoplasty methods used for acquired deformations, such as Peyronie's disease, are rarely used. The differences are that they are usually used only in cases of pronounced asymmetry of the cavernous bodies and only a skin flap and dura mater are used as plastic material. In the vast majority of cases, the ventral rotation technique of the cavernous bodies, first proposed by S. Koff (1984), is used. It was subsequently modified. Currently, it is known as Cantwell-Ransley corporoplasty and consists of rotation of the cavernous bodies and the imposition of a cavernostomy at the point of maximum deviation.

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

The principle of surgical 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 in hypospadias due to the different embryogenesis of these conditions.

Hypospadias is a fixation on the path of normal development of the urogenital tract, while epispadias is a gross distortion of its normal development. In epispadias, the urethral plate is fully formed, the deforming process only leads to a violation of its closure. The cavernous bodies are split, but 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), when using the Mitchell-Bagli method, there is no disruption of the glanuloapical relationships. Of interest is the modification of the Mitchell operation proposed by P. Caione in 2000, which consists of creating a half-coupling simulating the external sphincter from the perineal muscle complex and paraprostatic tissues in the area of the bladder neck.

The complication rate after the Mitchell operation and its various modifications is 11%, and the incidence of neourethral fistulas is 2.4% versus 5-42% with the Cantwell-Ransley operation.

The problems of penis length correction remain quite complex and not fully resolved. Unfortunately, interventions performed in childhood aimed at the maximum possible isolation of the cavernous bodies, up to separating them from the lower branch of the pubic bone, in combination with the correction of curvature according to Cantwell-Ransley, do not provide a significant increase in penis length. Moreover, complete mobilization of the cavernous bodies is associated with the risk of damage to the cavernous arteries.

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Method of one-stage urogenital reconstruction (Kovalev-Koroleva operation)

In 1998, V. Kovalev and S. Koroleva proposed surgical treatment of epispadias and bladder exstrophy in adults. Its distinctive feature is the simultaneous performance of lengthening urethro-, corporo-, glanulo-, spongio-, sphinctero- and abdominoplasty.

In all cases, the technique of complete penile dissection was used. If the urethral plate was preserved, it was separated from the cavernous bodies, mobilized to the area of the seminal tubercle or urinary bladder. Then, the cavernous bodies were dissected with excision of the chord and scar tissue. The proper urethral plate is tubularized, and bilateral corporotomies are performed. It is considered justified and appropriate to perform several bilateral corporotomies (at least two), since after mobilization of the urethral plate, excision of the chord and scar tissue, a single median corporotomy is insufficient for complete correction of the penile deformity. This is due to the combined nature of penile deviation, as well as the direct participation of intracorporeal factors in its formation in adult patients. An autovenous flap (v. saphena magna) is used as a plastic material for corporoplasty, for which an appropriate approach is made on the medial surface of the thigh. After performing corporotomy, the difference in the length of the tubularized urethral plate and the cavernous bodies becomes obvious. In order to lengthen the urethra, an insular vascularized flap on a feeding pedicle is taken. Two-level corporoplasty allows simultaneously eliminating deviation and increasing the length of the penis. The isolated insular flap is tubularized and anastomosed with the tubularized proper urethral plate (urethro-neourethroanastomosis). The length of the extended part of the urethra (neourethra) depends on the availability of plastic material and the length of the cavernous bodies after corporoplasty and ranges from 2 to 6 cm. The formation of an artificial voluntary sphincter of the bladder is carried out by rotating the vascularized muscle flap of the rectus abdominis muscle and transposing it into the bladder neck area with the creation of a muscle cuff around it. Abdominoplasty is performed by fixing the flap of the rectus abdominis muscle in the pubic area, which helps to compensate for the pubic defect due to diastasis of the pubic bones, additionally sealing the sutures of the bladder and urethra, improving tissue trophism, and also gives a vertical direction to muscle fibers during their transposition into the neck area. At the end of the surgical intervention, the cavernous bodies are rotated and the neourethra is ventrally transposed with the formation of an external opening on the head of the penis. In some cases, with a shortage of plastic material and insufficient length of the neourethra, the external opening is formed according to the type of coronal hypospadias. The feeding leg of the urethral flap, when shifted to the polar surface of the penis, not only improves the trophism of the neourethra and seals the sutures after tubularization of the flap and urethral anastomoses, thus preventing the formation of fistulas,but also provides the cosmetic effect of the presence of a spongy body of the urethra (spongioplasty). The cosmetic effect is more pronounced, the greater the thickness of the feeding pedicle. The skin defect is compensated with the help of local tissues and displaced vascularized flaps.

In the postoperative period, it is necessary to prescribe anticoagulants, disaggregants, angioprotectors, antioxidants, ozone therapy, laser therapy, vacuum therapy to improve microcirculation and trophism of the flaps. Alpha-adrenergic blockers were used to eliminate hyperreflexia of the bladder and sympathetic constrictor influence. In addition, training of the arbitrary artificial sphincter of the bladder was performed. The rehabilitation program included sexological training, various psychotherapy techniques, and drug correction of psychoemotional disorders.

Results and discussion

The results of surgical treatment of epispadias and bladder exstrophy were evaluated over periods of one to ten years. A total of 34 patients were operated on. The criteria for evaluating the outcomes of surgical interventions were functional and aesthetic results. Sphincteroplasty was performed in 73.5% of cases with preserved reservoir function of the bladder, and lengthening urethro- and corporoplasty were performed in all patients, including those who underwent various types of intestinal diversion of urine, since even in the absence of a natural act of urination, the formation of the urethra as an ejaculatory canal is an important component of social and sexual rehabilitation. The cosmetic effect was assessed based on the appearance of the penis, its length, the shape of the head, the absence or presence of deformation. Using the described method, an elongation of the penis by 2-2.5 cm was achieved, which made it possible in some cases to use an extender and achieve an additional elongation of 1 cm.

Visual straightening 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 considered functionally insignificant and did not require correction. In several cases, the deviation recurrence was from 30 to 45°. Three patients were reoperated (lengthening corporoplasty). Conical head was noted in 36% of cases. This is considered not a complication, but a characteristic feature of surgical treatment of epispadias and bladder exstrophy. All patients were satisfied with the aesthetic results of the treatment of epispadias and bladder exstrophy.

The functional result was assessed by the preservation of erectile and ejaculatory function, quality of urination, and the viability of the continental 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 architecture of the abnormal penis and the surgical technique, which consists in creating access to the tunica albuginea at the stage of corporoplasty in the avascular zone. Asthenic ejaculation was noted in 47.1% of patients, and retarded ejaculation was reported in 20.6%.

Full functioning of the artificial muscular sphincter was noted in 80% of patients. In 20% of cases, leakage and partial urinary incontinence in orthostasis were noted, which was also assessed as a positive result (compared with the initial total urinary incontinence). Surgical treatment of epispadias and bladder exstrophy allowed patients to abandon the constant use of diapers and switch to episodic (during physical activity) use of a penile clamp.

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

Urethral fistulas (as a postoperative complication) were found in 6% of patients. This figure is lower than in other types of corporourethroplasty, but higher than in primary surgeries for exstrophy and epispadias in childhood, which can be explained, as a rule, by the 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. they were fully sexually adapted. The remaining patients used alternative methods of sexual relations. 44% of patients had a regular sexual partner. Four of them created families, three had children. The average life satisfaction score was 17±2.5 (70.8% of the maximum achievable score).

Conclusion

Complete one-stage urogenital reconstruction (Kovalev-Koroleva operation) for bladder exstrophy and total epispadias in adult patients is considered a pathogenetically justified method. From the point of view of erectile function preservation, it is safe and justified. Features of ventralization of the urethra help to reduce the incidence of complications (for example, urethral fistulas), which are more common when using other methods. The use of complete penile dissection and several types of flaps for organ reconstruction makes it possible to freely rotate the elongated cavernous bodies together with the head of the penis, strengthen the neck of the bladder, lengthen the urethra and perform its transposition, which allows creating new syntopic relationships that are as close as possible to the anatomical norm.

It should be noted that this reconstruction method does not disrupt glanuloapical relationships, which certainly helps to reduce the number of complications associated with trophic disorders of the glans. One-stage complete urogenital reconstruction allows for maximum use of plastic material and provides a satisfactory cosmetic and functional result. The creation of an artificial muscular sphincter by transposing the rectus abdominis muscle flap is justified not only from a functional (continence mechanism and improvement of local trophism) but also from a cosmetic point of view. After one-stage urogenital reconstruction, all patients with epispadias and exstrophy showed 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 spectrum of communications. This is based not only on the elimination of the primary psychotraumatic factor, but also on an increase in the self-esteem of patients after a successful operation. In patients who have undergone various diversion treatments for epispadias and bladder exstrophy, restoration of the urethra as an ejaculatory canal, taking into account the preservation of libido, ejaculation and orgasm, is considered an important and integral stage of social rehabilitation.

Social and sexual adaptation of patients with exstrophy and total epispadias requires the combined use of plastic reconstructive surgery and psychological rehabilitation. The use of psycho- and pharmacotherapy to achieve an optimal psycho-emotional background allows for faster sexual and social adaptation of this category of patients.

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