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Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60–120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy.

The article describes a method of surgical formation of a neophallus in a child with burned penis and presents a literature review on treatment techniques and patient management tactics for this pathology. De Castro phalloplasty is actively used in children with aphallia and male karyotype 46,ХY, micropenis, and exstrophy–epispadias complex. Uniqueness of the presented clinical demonstration lies in the fact that it is the first time in Russia De Castro phalloplasty was performed in a child with partial amputation of the penis.

The term exstrophy-epispadias complex refers to a group of midline defects ranging from epispadias to cloacal exstrophy. Bladder exstrophy is the most frequent malformation of this spectrum and it can present as a classical or a variant form. We report a case of a hybrid bladder exstrophy variant having some characteristics of both a duplicate bladder exstrophy and a superior vesical fistula.

Objectives: To study the presentation and management of the exstrophy-epispadias complex in adolescents and adults and to evaluate the outcome of the repair. Subjects and methods: This retrospective review of our medical records of adult patients with an exstrophy-epispadias complex managed between January 2010 and June 2015 included one female and 7 male patients. Only patients aged above 10 years who had not undergone any surgical treatment during childhood were included in the study. All patients were subjected to preoperative biopsy from the urinary bladder plate. None of them underwent cystectomy. Seven patients underwent urinary bladder augmentation using ileocystoplasty, bladder neck reconstruction with para-urethral tissue closure using a wedge of pubic bone, and single-stage epispadias repair. In one patient epispadias repair was not done due to intraoperative hemodynamic instability. Results: All patients are continent but one in whom epispadias repair was not done and who is waiting for epispadias repair. One patient has developed a urethro-cutaneous fistula at the base of the penis. Two patients developed urinary bladder stones, and both of them were managed by minimally invasive methods. All the patients are on clean intermittent self-catheterization. They are all doing well socially and psychologically. Conclusion: Though exstrophy-epispadias in adolescents and adults is a rare entity, the described modified surgical technique provides good results and improves the patients’ social and psychological well being.

We describe the case of a bladder exstrophy–epispadias complex (BEEC), in which a “split” appendix was used simultaneously as a posterior urethral replacement (for strictured Mitchell's repair) as well as a Mitrofanoff channel. Split appendix had been used before for simultaneous Mitrofanoff and Malone's antegrade contrast enema channels to manage neurogenic bladder and bowel. To the best of our knowledge, this is the first reported case for the use of split appendix for urethral reconstruction in a child with BEEC.

Abstract Aim The aim of this report is to present a brief review of the current literature on the management of EEC. Case Report A term male neonate presented at birth with classic bladder exstrophy, a variant of the exstrophy-epispadias complex (EEC). The defect was covered with sterile silicon gauzes and waterproof dressing; at 72 hours of life, primary closure without osteotomy of bladder, pelvis, and abdominal wall was successfully performed. Discussion EEC incidence is approximately 2.15 per 1,00,000 live births; several urological, musculocutaneous, spinal, orthopedic, gastrointestinal, and gynecological anomalies may be associated to EEC. Initial medical management includes use of occlusive dressings to prevent air contact and dehydration of the open bladder template. Umbilical catheters should not be positioned. Surgical repair stages include initial closure of the bladder and abdominal wall with or without osteotomy, followed by epispadias repair at 6 to 12 months, and bladder neck repair around 5 years of life. Those who fail to attain continence eventually undergo bladder augmentation and placement of a catheterizable conduit. Conclusion Modern-staged repair of EEC guarantees socially acceptable urinary continence in up to 80% of cases; sexual function can be an issue in the long term, but overall quality of life can be good.