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Summary Introduction Bladder exstrophy is a rare malformation with functional and psychological issues affecting children's quality of life. This study aims to evaluate the urinary continence and the quality of life of patients operated for bladder exstrophy in our institution. Methods This is a retrospective study including fifteen patients operated for bladder exstrophy between 1995 and 2015. All patients underwent a physical examination and an interview with a psychiatrist. Urinary continence was evaluated by dryness periods. Quality of life was evaluated by the QoL SF-36 scale. Results All patients underwent primary early bladder closure. Posterior osteotomy was performed initially in 6 cases, and during the redo surgery in 7 cases. Leakage was recorded in 4 cases, fistula in 9 patients. Twelve patients had one or more redo surgery. Final bladder closure success rate was of 80%. Additional continence surgical procedures were performed by a Young Dees technique in 8 cases and with bladder enlargement associated with Mitrofanoff continent derivation in 5 cases. Only two patients over fifteen did not use diapers. All the items of QoL score according SF-36 were under the normal value (75%) except physical functioning and physical limitation. These scores go worst with age. Scores were better for continent boys in both physical and psychological items, but the difference was not significant However, significant difference was observed between boys having less than 3 surgeries and those having more than three procedures. Psychological and social scores were better for girls than for boys and differences were significant. Conclusions The risk of urinary incontinence is high in children managed for bladder exstrophy. Boys are more affected than girls with subsequent psychological and social repercussions. In our study, quality of life depends more on number of surgery than continence results. Psychological problems should be screened early for accurate treatment. Level of evidence 3.

Pyloric duplication cyst is a rare congenital anomaly. Few cases have been described in the literature. We report two cases of 2 and 5 year-old girl. The diagnosis was suspected on ultra sonography and upper gastrointestinal series and confirmed by surgery and histopathologic examination. Resection was total in one case and partial in the other. Pyloroplasty was associated. Post operative was unfaithful.

Introduction Over the years, the surgical management of recto-sigmoid Hirschsprung's disease (HD) has evolved radically and at present a single stage transanal pull-through can be done in suitable cases, which obviates the need for multiple surgeries. Aim The aim of this paper was to evaluate the role of transanal pull-through in the management of recto-sigmoid HD in our institution. Material and methods A retrospective analysis (between January 2003 and December 2009) was carried out on all cases of Hirschsprung's reporting to unity of pediatric surgery of Tunis Children's Hospital that were managed by transanal pull-through as a definitive treatment. All selected patients including neonates had an aganglionic segment confined to the rectosigmoid area, confirmed by preoperative barium enema and postoperative histology. Twenty-six children (86%) had their operation done without construction of prior colostomy. Results Transanal pull-through was performed in 31 children. Mean operating time was 150 minutes (range 64 to 300 minutes). No patients required laparotomy because all patients including neonates had an aganglionic segment confined to the rectosigmoid area. Blood loss ranged between 20 to 56 ml without blood replacement. Since all children were given an epidural caudal block, the requirement of analgesia in these cases was minimal. Postoperative complications included perianal excoriation in 7 out of 31 patients lasting from 3 weeks to 6 months. Complete anorectal continence was noted in 21 of 31 (67%) children in follow up of 3-5 years. Conclusions Transanal endorectal pull-through procedure for the management of rectosigmoid HD is now a well-established and preferred approach. Parental satisfaction is immense due to the lack of scars on the abdomen. As regards the continence, a long-term follow-up is necessary to appreciate better the functional results of this surgery.

Situs inversus is a challenge to the physician, both for the diagnostic and for the therapeutic. A combination of partial situs inversus, annular pancreas and polysplenia with bowel malrotation has been reported in a newborn who presented as duodenal obstruction. Situs inversus is rare especially without heart malformation. All the abnor - malities in this combination can be explained on the basis of multiple organ malrotation. It also demonstrates the necessity of meticulous investigation and precise description of the anomaly as far as the management of this case is concerned.