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Background The GAS study is an international RCT to evaluate neurodevelopmental outcome comparing general plus regional anesthesia versus regional anesthesia alone in 722 neonates and infants who had inguinal hernia repair up to 60 weeks of postmenstrual age. This paper comprises a secondary descriptive analysis of hernias, aspects of surgery and outcomes. Methods The incidence of unilateral and bilateral hernias, side preponderance, predictive factors for bilateral hernias and surgical approaches were collated. Follow-up outcome data were examined at 2 years. Results Of 711 eligible patients, there were 679 with hernia data showing that 321 hernias were right-sided, 190 left and 168 bilateral. Male to female ratio was 5:1. Of those with unilateral hernias, 25.8% underwent contralateral exploration and in these cases a patent processus vaginalis was found in 68.9%. Bilateral hernias were more common in younger and female patients. At 2 years there was a recurrence rate of 0.99% and in 2.7% of patients a hernia was evident on the contralateral side (metachrony), and this was unrelated to the anesthesia technique. Conclusions Bilateral hernias are associated with lower gestational age at birth and female gender. There was a low incidence of complications and the anesthesia technique did not affect surgical outcome. Level of evidence Level 1 evidence from prospective treatment study.

Background/purpose Currarino syndrome (CS) phenotype, initially described as the triad of hemisacrum, anorectal malformation (ARM) and presacral mass, can be extremely variable. The triad is often incomplete and 3 main CS phenotypical subtypes have been described: Complete, Mild and Minimal. Various associated malformations are often present. Mutations in the MNX1 gene are the main genetic background of CS, although they are not present in almost half of the cases. Aim of our study is to analyze the distribution of the 3 CS subtypes and the incidence of associated malformations in a large sample of patients and to add information about the role of the genetic testing in guiding the diagnostic and prognostic evaluation of CS patients. Methods A multicentre retrospective data collection was performed. CS patients' phenotype was accurately analyzed according to a diagnostic-therapeutic standardized data collection sheet. The distribution of the three CS types and the frequency of each associated malformation were calculated. The phenotype of the patients with a known genetic anomaly was compared to the phenotype of the population with no genetic diagnosis, in order to determine whether the presence of a known genetic defect could correlate with a more severe CS phenotype. Results Data from 45 patients were analyzed. Twenty patients (44.5%) presented a Complete CS type, 19 (42.2%) a Mild CS and 6 (13.3%) a Minimal CS. In addition to the classical triad elements, 38 (84.5%) patients showed associated anomalies. The group of patients who resulted positive for a MNX1 mutation comprised a higher number (56.5%) of Complete CS cases than the group of patients that did not carry any MNX1 mutation (13%) ( p = 0.0085). We could not find any relationship between CS subtype and the number of associated anomalies ( p = 0.5102). Conclusions The presence of a MNX1 mutation seems to correlate with a more severe CS phenotype. MNX1 seems the main responsible for the expression and the severity of the CS triad, while the associated anomalies appear to be prevalently determined by genes sited on different loci. A thorough multidisciplinary diagnostic overview of CS patients should always include genetic counseling and analysis, both in postnatal and prenatal settings. Type of study Retrospective Study. Level of evidence II.

Background/Purpose Surgical resection or strictureplasty (SP) are different options for intestinal Crohn disease (CD) strictures. The aim of this article is evaluation of long-term outcome of SP and resection. Methods From 1996 to 2011, 39 patients (23 male, 16 female) with symptomatic ileal and ileocolonic CD strictures resistant to medical/nutritional therapy and treated with surgery in 2 different surgical units were reviewed. The mean age at diagnosis was 11.82 years (range, 4-17 years). Mean age at surgery was 15.94 years (range, 4-24 years). Mean follow-up was 6.88 years (range, 0.5-15 years). Patients underwent resection (group A) or different SP techniques (group B). Results Twenty patients underwent intestinal resection (ileal or ileocolonic resection), and 19 patients underwent SP (jejunal, ileal, or ileocolic). Early postsurgical complications were observed in 2 patients of group A. Follow-up of group A patients revealed that 1 patient needed emergency treatment after 8 months surgery because of adhesions and 1 patient developed recurrence treated with medical therapy. In the follow-up group B, 3 patients experienced disease recurrence, 2 of them at the site of previous surgery. Conclusions At long-term follow-up, no significant difference in relapsing rate was observed between the 2 groups. Strictureplasty and resection represent an effective treatment of pediatric CD strictures. Strictureplasty could represent the first option for intestinal preservation.

Background Surgeons are at risk for developing work-related musculoskeletal symptoms (WMS). The present study aims to examine the physical factors and their association with WMS among pediatric laparoscopic surgeons. Methods A questionnaire consisting of 21 questions was created and mailed to 25 pediatric laparoscopic surgeons (LG). 23/25 surgeons (92%) completed the survey. The questionnaire was analyzed and then split into 2 groups. Group 1 (LG1) included surgeons with greater laparoscopic experience, and group 2 (LG2) included surgeons with less important laparoscopic experience. In addition, we constructed and sent to the same surgeons a similar questionnaire focused on WMS after an open procedure (OG) with the aim to compare results of LG with OG. Results The prevalence rate of WMS with shoulder symptoms was 78.2% in surgeons that performed laparoscopy for more than 10years, with 60.8% also reporting other pain. In 66.6% this pain is evident only after a long-lasting procedure. Forty-four percent of these surgeons require painkillers at least twice a week. Fifty percent of these surgeons also suffer at home. Fifty-five and one half percent of surgeons indicate that this pain is related to their laparoscopic activity. Forty-three and a half percent think that laparoscopy is beneficial only for the patient but has a bad ergonomic effect for surgeons. Sixty-five and two-tenths percent think that robotic surgery can be helpful to improve ergonomics. Comparing the groups, WMS occur more frequently in LG (78.2%) than in OG (56.5%), but this difference was not statistically significant (χ 2 =0.05). In addition, WMS occur more frequently in LG1 (84.6%) than in LG2 (70%), but this difference was not statistically significant (χ 2 =0.05). Conclusions These results confirmed a strong association between WMS and the number of laparoscopic procedures performed. Skilled laparoscopic surgeons have more pain than less skilled laparoscopic surgeons. WMS in the same group of surgeons are more frequent after laparoscopy than after open procedures. The majority of surgeons refer to shoulder symptoms.

Purpose: The aim of this study is to demonstrate the feasibility and usefulness of mechanical suturing in children for low rectal anastomosis. Methods: The study group includes 31 children operated on from January 1993 to July 1996 by the same senior surgeon, performing the modified Duhamel procedure for Hirschsprung's disease in 17 children, intestinal neuronal dysplasia in seven, and the Knight-Griffen procedure in seven pediatric patients with chronic ulcerative colitis. Results: In all the cases the technique of "viscero-synthesis" was performed using the mechanical stapler. A circular stapler has been used for the end-to-end and the end-to-side anastomosis between the anal canal or the back wall of the rectum with the pulled viscus, while a linear endoscopic stapler (GIA) has been used for the consolidation of the rectocolic wall in the modified Duhamel technique. Conclusions: The results obtained demonstrate that the mechanical staplers in children are safe and effective in low rectal anastomosis, sparing operative time and reducing the risk of anastomotic dehiscence; however, the size of circular instruments limits its use in neonates and small infants.

Perineal sepsis is a life-threatening complication of acute leukemia. Although conservative management (antibiotics, incision, and drainage, alone or in combination) is considered the criterion standard, it provides an outcome that is not fully satisfactory, with an overall mortality of roughly 30%. This report presents a case series of 4 children who underwent early defunctioning colostomy for the treatment of perineal sepsis during leukemia. This management proved to be successful and allowed prompt reestablishment of chemotherapy, thus improving overall results. Routine application of this "aggressive" management in these cases will presumably increase overall survival of children with leukemia.

Purpose This article is aimed at describing a series of 70 patients who underwent a redo pull-through in the period between January 1991 and January 2007 and to compare them with a series of consecutive patients with Hirschsprung disease who underwent a single pull-through. Methods The notes were reviewed, and a comprehensive interview based on a disease-specific questionnaire for continence and quality of life was submitted to all eligible patients. We used a number of consecutive patients who underwent a single pull-through for Hirschsprung disease as control group. Results Seventy patients were included. Residual aganglionosis in pulled-through bowel or retained aganglionic rectum represented the indications to reoperation in most cases. Intestinal obstruction represented the leading symptom in more than 60% of patients. We adopted either the endorectal pull-through, the Duhamel, or the Swenson procedure. Thirty-six patients were assessed for long-term outcome. One third experienced complications. Long-term continence was satisfactory in 70%. Enterocolitis, soiling, and perineal excoriations were complained by 30%. Excellent to good perspectives were described by 94%, and excellent to good cosmetic results by 22%. The only significant difference with control group (109 patients) involved cosmetic appearance, whereas soiling, although more frequent, could not be considered significantly increased. Conclusions Patients who experience failure of a pull-through must go through multiple procedures to achieve definitive results. Although overall outcome does not significantly differ from that of a single effective pull-through, great efforts should be made to minimize complications and avoid the need for a redo, which requires experienced surgeons and highly committed families for a longer course of the disease.

Background/Purpose: Vesico-ureteric reflux (VUR) is a common problem in children with neuropathic bladder. Lesserdegree VUR may be manageable by intermittent catheterization or by anticholinergics, but higher grades usually require surgical treatment. If left untreated, two thirds of such patients may experience deterioration of the upper renal tracts. The aim of this study was to compare the results of the STING (Subureteric Teflon Injection) technique with surgical ureteric reimplantation as treatment for VUR in neuropathic bladder. Methods: From January 1981 to December 1996, 58 children with NB (81 ureters) were treated for VUR. STING and Cohen ureteroneocystotomy were performed in 40 and 41 ureters, respectively. Mean age was 4.5 years (STING) and 5.1 years (Cohen). Results: Twenty-nine of 40 refluxing ureters (72.5%) were cured by STING, whereas Cohen eradicated reflux in 39 of 41 ureters (95.5%). No complications were observed in either group. All the ureters in which STING failed were treated successfully by Cohen ureteroneocystostomy. The 2 ureters still refluxing after surgical reimplantation were cured successfully by a single STING. The mean follow-up was 6.8 years in the Cohen group and 4.8 years in the STING group. During follow-up, no recurrence was observed in patients cured by open reimplantation. In the STING group, 2 previously cured ureters showed recurrence of VUR: both were treated successfully by a further STING. Conclusions: Open ureteral reimplantation is more effective than STING in correcting VUR in children with neuropathic bladder dysfunction. Nevertheless, the good success rate, the relative technical simplicity, outpatient nature, and rapid recovery point to STING as a safe and effective procedure for the initial treatment of VUR. Failure of STING does not preclude a successful open operation.

Introduction Long-term results after surgical treatment of Hirschsprung disease (HSCR) are not always as good as a surgeon may perceive. Several studies have tried to correlate preoperative features to the surgical outcome of HSCR, but none came to definitive conclusions. Our study is aimed at identifying risk factors of poorer long-term outcome after surgery for HSCR. Materials and Methods One hundred two patients with HSCR were included. Eighty had rectosigmoid aganglionosis and 22 had total colonic aganglionosis (TCSA). Preoperative variables were sex, associated anomalies, delayed meconium passage, preoperative enterocolitis, preoperative bowel obstruction, age at surgery, and number of pull-through procedures performed. Outcome measures were surgical complications, postoperative enterocolitis, perineal excoriations, constipation, continence, psychological self acceptance, and patients' perspectives. These were evaluated with regard to preoperative features and length of aganglionosis. Results Results of patients with TCSA proved to be significantly worse than those of patients with classic HSCR (constipation and poor continence excluded). Male patients with TCSA proved to have a significantly higher incidence of complications (100% vs 38.5%) and poor psychological self acceptance (100% vs 46.2%). Patients with associated anomalies (central nervous system) experienced a significantly higher incidence of postoperative constipation (43% vs 13%). Patients who described failure or delayed meconium passage complained of significantly worse continence (28.3% vs 7.1% of fair to poor continence). Preoperative enterocolitis proved to be significantly correlated to postoperative enterocolitis (16.4% vs 37.1%) and perineal excoriations (13.7% vs 30.9%). Patients with preoperative intestinal obstructions complained of significantly worse psychological self acceptance (37.8% vs 12.5%). Conclusions Length of aganglionosis has the greatest impact on overall surgical outcome of HSCR. Other minor risk factors have been identified, namely, male sex, associated central nervous system anomalies, failure to pass meconium, and preoperative enterocolitis occurrences. Age at surgery and redo procedures proved not to significantly interfere with the outcome. Our study provides comprehensive and useful data to inform parents and families of a baby with HSCR with regard to expected results and long-term outcomes of surgery basing on preoperative features. Further studies on larger series are strongly recommended.

Purpose Diagnostic laparoscopy has changed the surgical approach to nonpalpable testes (NPT). The aim of this study was to determine the value of laparoscopy in managing patients with NPT and to suggest guidelines for the interpretation of laparoscopic findings. The authors report the results of a multicenter study of the Italian Society of Video Surgery in Infancy on laparoscopic management of NPT. Methods Between 1993 and 2001, the authors collected records of 364 patients with NPT who underwent laparoscopy, for a total of 388 testicular units. Results Intraabdominal testes were found in 124 (34%) cases, for a total of 137 testes; 83 testicular units were classified as low and 54 as high. A total of 155 (43%) patients had cord structures entering the internal inguinal ring, 79 (22%) had intraabdominal blind-ending cord structures, and 6 (1%) had testicular agenesia. Conclusions Laparoscopy is a valuable tool in diagnosing and treating more than 50% of cases of NPT. The laparoscopic evaluation of abdominal testes can provide indications for the most suitable surgical technique; moreover, in 23% of patients, it makes abdominal exploration unnecessary. The value of laparoscopy is even greater if the anatomical aspect of the internal ring and spermatic cord structures is carefully evaluated.

The authors present the investigations and surgical treatment of two cases of duodenal cystic duplication. Abdominal pain and gastroesophageal reflux were the most important symptoms and signs associated with an history of recurrent acute pancreatitis. Computed tomography scan, ultrasound examination, and cholangiography confirmed preoperatively the diagnosis, and a transduodenal surgical approach was carried out in both children. A simple marsupialization of the cyst was performed in the former, and a sphincterotomy with papillosphincteroplasty was associated in the latter. The diagnosis was confirmed by microscopy, and both the children are asymptomatic after a 14 and 18 months of follow-up. This report focuses on the importance of the cholangiopancreatography for every child presenting with recurrent, unexplained bouts of acute pancreatitis, and underlines the technical surgical aspects on the basis of the anatomic identification of the malformation.