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Background. Bladder exstrophy (ВE) is a complex combined malformation of the organs of the genitourinary system, the methods of treatment of which have not been fully developed. Materials and methods. When writing a literature review, materials devoted to the problem of BE published in the databases of PubMed, eLIBRARY (the scientific electronic library of Russia), Scopus, Medline, EMBASE, websites and journals of foreign and domestic professional associations were used. Results. Multiple anatomical features of the structure of organs and entire systems make BE one of the most difficult-to-correct congenital malformations in the field of pediatric urology, requiring a comprehensive, systematic approach to diagnosis and treatment. The paper reflects the key, in our opinion, positions of the author’s Kelly, Mainz operations, the approaches of Schrott–Erlangen, CPRE, MSRE, as well as the view of a number of authors on osteotomy. Conclusion. The abundance of primary closure techniques and the difference in views of numerous authors on the required amount ofsurgical intervention presented in thisliterature review are indisputable proof ofthe relevance ofthe problem.

Bladder extrophy - is a difficult malformation for the treatment. Aplasia of one of the cavernous bodies of the penis is an extremely rare pathology. In the available literature, we did not meet reports of correction of bladder extrophy in children with a single cavernous body of the penis.From 1990 to 2020, 545 children (364 boys) with extrophy have been repair. Two (0.5 %) boys only had a classic bladder extrophy combined with the aplasia of one cavernous body. The first patient with classical extrophy and multiple malformations (fingers of the right hand, fingers of the right foot, aplasia of the left kidney, left cavernous body, left testicle and hypoplasia of the left half of the scrotum) the penis with the single cavernous body on the right was somewhat thinner, but formed anatomically correctly with the presence of a urethra and closed prepuce. The primary closure of the bladder was performed with bilateral iliac osteotomy and the joint of pelvic bones at the age of 1 year. The patient had the anatomically properly formed bladder sphincter and urethra, penile correction was not required. After the operation, urinary continence had been achieved.The second boy was with classical extrophy and epispadia of the single cavernous body, he was given stage treatment. At the age of 5 days (2004) the primary closure of the bladder and the connection of the pelvic bones were performed. At the age of 4 years (2008) the formation of the urethra was carried out, the correction of the dorsal deformation of the single cavernous body by excision of the chord and the application of multiple superficial cross-cutting on the tunica albuginea. Bladder neck plastic by Kelly technique in combination with bilateral osteotomy of pelvic bones was performed at the age of 7 years in (2011). The boy of 15 years (2019) had a bladder volume of 350-400 ml. Dry 4-5 hours. Urinate freely with a wide stream. Then a two-stage plastic of the distal urethra was performed. We used the Bracka technique with augmentation of the glans penis and implantation a free graft of the mucous lip. We achieved a good result.Using modern penile correction technologies help to socially adapt patients with rare and difficult malformations.

Background. Important causes of complications of hypospadias repair are the deficit of tissues for plastic surgery, grooveless and small size of glans, obliteration of the urethral platewith varying degrees of scarring. The coronal urethral fistula is the most common complication of urethroplasty. Surgeons continue to search for reliable methods of correction of complications.Materials and methods. Between 2011 and 2019, 85 children aged 2 and 17 years presented with coronal fistula of urethra after hypospadias repair. (TIP) Snodgrass – 78 (91.7 %) and Mathiue – 7 (8.2 %) procedures have been performed them earlier. In our clinic previously operated 28 (32 %) boys, primary surgery of the remaining 57 (67 %) was performed in other medical institutions.Results. All patients (85), conditionally, were divided into two groups. The first group included 39 children (45.8 %), with stitching a fistula, the second group consisted of 46 patients (54.1 %), with augmentation of the urethral plate of the glans and distal urethra with the implantation of a rectangular preputial or oral mucosa free graft. Recurrent urethral fistula after stitching was observed in 10 boys (25.6 %) of the first group, and only in 2 cases (4.3 %) in children with the augmentation of the urethral plate (p