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BACKGROUND Cross-sectional imaging is required to assess disease prior to surgery for neuroblastoma (NBL), and both magnetic resonance imaging (MRI) and computed tomography (CT) scan are considered acceptable. We had concerns that MRI was underestimating disease extent, so from early 2016 we have systematically used MRI and CT before all abdominal NBL resections. The aim of this retrospective study was to establish which imaging modality is more accurate in determining disease extent, particularly after chemotherapy. METHODS Abdominal MRI and CT scans for all children with abdominal NBL referred for surgery from January 2016 to February 2018 were retrospectively reviewed to evaluate the extent of disease and the presence of imaging-defined risk factors (IDRFs). RESULTS Thirty-one patients were eligible for consideration of surgery post disease reassessment with MRI/MIBG. Twenty-four of 31 children were included. CT was performed a median of 15 (range, 1-47) days after MRI. MRI underestimated IDRFs compared with CT in 13 of 24 patients (54%). Seventeen of 24 patients underwent surgery, and operative findings had 100% correlation with CT imaging. Notably, there were fundamental changes in management post CT in 6 of 24 patients (25%). CT did not underestimate disease compared with MRI in any patient. CONCLUSION MRI underestimated the extent of the disease in half of our patients considered for NBL resection. This may be due in part to tumor fibrosis, calcification, and chemotherapy. Preoperative CT scan is the best imaging modality to identify all IDRFs after chemotherapy to ensure safe surgery.

Introduction Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF. Materials and Methods The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9. Results Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items. Conclusion Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

AIM OF THE STUDY: Complex tracheo-oesophageal fistulae (TOF) are rare congenital or acquired conditions in children. We discuss here a multidisciplinary (MDT) approach adopted over the past 5 years. METHODS: We retrospectively collected data on all patients with recurrent or acquired TOF managed at a single institution. All cases were investigated with neck and thorax CT scan. Other investigations included flexible bronchoscopy and bronchogram (B&B), microlaryngobronchoscopy (MLB) and oesophagoscopy. All cases were subsequently discussed in an MDT meeting on an emergent basis if necessary. MAIN RESULTS: 14 patients were referred during this study period of which half had a congenital aetiology and the other half were acquired. The latter included button battery ingestions (5/7) and iatrogenic injuries during oesophageal atresia (OA) repair. Surgical repair was performed on cardiac bypass in 3/7 cases of recurrent congenital fistulae and all cases of acquired fistulae. Post-operatively, 9/14 (64%) patients suffered complications including anastomotic leak (1), bilateral vocal cord paresis (1), further recurrence (1), and mortality (1). Ten patients continue to receive surgical input encompassing tracheal/oesophageal stents and dilatations. CONCLUSIONS: MDT approach to complex cases is becoming increasingly common across all specialties and is important in making decisions in these difficult cases. The benefits include shared experience of rare cases and full access to multidisciplinary expertise. ispartof: PEDIATRIC SURGERY INTERNATIONAL vol:35 issue:1 pages:97-105 ispartof: location:ITALY, Taormina status: published

To assess incidence and natural history of umbilical hernia following sutured and sutureless gastroschisis closure. With audit approval, we undertook a retrospective clinical record review of all gastroschisis closures in our institution (2007–2013). Patient demographics, gastroschisis closure method and umbilical hernia occurrence were recorded. Data, presented as median (range), underwent appropriate statistical analysis. Fifty-three patients were identified, gestation 36 weeks (31–38), birth weight 2.39 kg (1–3.52) and 23 (43 %) were male. Fourteen patients (26 %) underwent sutureless closure: 12 primary, 2 staged; and 39 (74 %) sutured closure: 19 primary, 20 staged. Sutured closure was interrupted sutures in 24 patients, 11 pursestring and 4 not specified. Fifty patients were followed-up over 53 months (10–101) and 22 (44 %) developed umbilical hernias. There was a significantly greater hernia incidence following sutureless closure (p = 0.0002). In sutured closure, pursestring technique had the highest hernia rate (64 %). Seven patients underwent operative hernia closure; three secondary to another procedure. Seven patients had their hernias resolve. One patient was lost to follow-up and seven remain under observation with no reported complications. There is a significant umbilical hernia incidence following sutureless and pursestring sutured gastroschisis closure. This has not led to complications and the majority have not undergone repair.

Antegrade continence enema (ACE) revolutionised the lives of children with chronic constipation and soiling. Parents often ask how long the ACE will be required. We looked at our patients 5 years after ACE formation to answer the question. We reviewed clinical notes of all patients undergoing ACE procedure during January 1990 to December 2010. Only patients with >5 years follow-up were included. Data are given as median (range). 133 patients were included with >5 years of follow-up. Primary pathology was anorectal anomaly (ARA) 64 (48 %); spinal dysraphism (SD) 40 (30 %); functional constipation (FC) 14 (10 %); Hirschsprung’s Disease (HD) 10 (8 %) and others 5 (4 %). Median follow-up was 7 years (5–17 years). Overall 74 % still use their ACE; whilst 26 % no longer access their stoma, of whom 47 % recovered normal colonic function. 50 % of HD patient recover colonic function. FC has the highest failure rate at 21 %. Overall 86 % achieved excellent clinical outcome with 74 % of patient still using their ACE at 5 years. HD has the highest recovery rate of 50 %. FC has a more unreliable clinical outcome with 21 % recovered colonic function and 21 % failed. Outcome varied dependent on the background diagnosis.

PURPOSE: Radiologically inserted gastrojejunal tubes (RGJ) and surgical jejunostomy (SJ) are established modes of jejunal feeding. The aim of the study is to review nutritional outcomes, complications and the practical consideration to enable patients and carers to make informed choice. METHODS: Retrospective review of patient notes with a RGJ or SJ in 2010, with detailed follow-up and review of the literature. RESULTS: Both RGJ and SJ are reliable modes to provide stable enteral nutrition. Both have complications and their own associated limitations. CONCLUSIONS: The choice has to be tailored to the individual patient, the social care available, the inherent medical disease and risk/benefit of repeated anaesthetic and radiation exposure. RGJ and SJ are important tools for nutritional management that achieve and maintain growth in a complex group of children. The risk and benefits should be reviewed for each individual patient. ispartof: PEDIATRIC SURGERY INTERNATIONAL vol:34 issue:9 pages:951-956 ispartof: location:Germany status: published

Aim Regular anal dilatations are commonly recommended in the postoperative management following posterior sagittal anorectoplasty (PSARP) in anorectal malformations (ARM). We hypothesized that routine postoperative dilatations may not affect surgical outcomes following PSARP. We compare surgical outcomes of routine postoperative dilatations versus no routine postoperative dilatations from two United Kingdom tertiary pediatric surgical centers. Materials and Methods This is retrospective records review of patients undergoing definitive surgery for ARM in two tertiary surgical centers in the UK over 5 years. Center A used a protocol of routine postoperative dilatations, and center B used a protocol, which used dilatations only for clinical indications of stricture. Data collected included ARM type, operative procedures, and postoperative interventions. All post-operative interventions under general anesthesia (GA) were compared between groups. Results From 2011 to 2015, 49 procedures (46 PSARPs) were performed in center A and 54 (52 PSARPs) in center B. Median follow up period was 31 months (interquartile range [IQR] 18–48). The first postoperative anal calibration under GA was documented for 43 (86%) patients in center A and for 42 (78%) patients in center B. Following this, center A followed routine postoperative dilatation (RPD) at home, and center B reserved further dilatations for specific indications. RPD was performed for 100% of patients in center A versus 8% in center B. Further anal dilatations under GA were performed in 19 (38%) children in center A and in 17 (34%) children in center B (p = 0.68). In center A, 10 patients (22%) needed further surgery versus 14 (28%) in center B (p = 0.48). Conclusion The use of routine postoperative dilatations does not significantly improve surgical outcomes following PSARP in ARM.

Percutaneous placement of gastrostomy was first described in 1980. Since then it has become widely used in pediatric surgery throughout the world. Inherent in the technique is the possibility of inadvertent damage to adjacent anatomical structures, most commonly the transverse colon. Management previously had involved laparotomy and correction of the gastrocolic fistula. Here we describe a novel laparoscopic approach to the repair of a gastrocolic fistula following percutaneous gastrostomy, avoiding the morbidity of laparotomy in an immunocompromised patient with a rotund abdomen secondary to steroid usage.

Background/Purpose Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use in MH repair to curb recurrence. A systematic review and meta-analysis were performed to study the recurrence and complications associated with minimally invasive surgery and the use of patch. Methods We retrospectively reviewed all cases of MH who underwent first-time repair in 2012-2017 in our institution to determine recurrence and complication rate. A MEDLINE search related to minimally invasive surgery (MIS) and patch repair of MH was conducted for systematic review. Eligible articles published from 1997-2017 with follow-up data available were included. Primary outcomes measured were recurrence and complication. Meta-analysis to compare open versus MIS and primary versus patch repair in the MIS group were performed in comparative cohorts. Continuous data were presented as median (range), and statistical significance was P Results In our institution, 12 consecutive patients aged 17-month-old (22 days-7 years), underwent laparoscopic patch repair of MH, with one conversion to laparotomy. No recurrence or significant complication occurred over a follow-up period of 8 months (1-48 months). Thirty-six articles were included from literature review and were combined with the current series. All were retrospective case reports or series, of which 6 were comparative cohorts with both MIS and open repairs. A total of 296 patients from 37 series were ultimately used for analysis: 80 had open repair (4 patch) and 216 had MIS repair (32 patch), with a patch rate of 12%. There were 13 recurrences (4%): no difference between open and MIS repairs (4/80 vs 9/216, p = 0.75); recurrence rate following primary repair was 13/260 (5%), but no recurrence occurred with 36 patch repairs. Meta-analysis showed no difference in recurrence between open and MIS repair (p = 0.83), whereas patch repair was associated with 14% less recurrence compared with primary repair, although it did not reach statistical significance (p = 0.12). There were 13 complications (5%): no difference between open and MIS repairs (5/80 vs 8/216, p = 0.35). One small bowel obstruction occurred in a patient who had laparoscopic patch repair. Conclusion In MH, recurrence and complication rates are comparable between MIS and open repairs. Use of patch appeared to confer additional benefit in reducing recurrence. Type of Study Systematic review Level of Evidence 3A

Aim The objective of the study is to describe management of exomphalos major and investigate the effect of congenital cardiac anomalies. Methods A single-center retrospective review (with audit approval) was performed of neonates with exomphalos major (fascial defect ≥ 5cm ± liver herniation) between 2004 and 2014.Demographic and operative data were collected and outcomes compared between infants who had primary or staged closure. Data, median (range), were analyzed appropriately. Results A total of 22 patients were included, 20 with liver herniation and 1 with pentalogy of Cantrell. Gestational age was 38 (30–40) weeks, birth weight 2.7 (1.4–4.6) kg, and 13 (60%) were male. Two were managed conservatively due to severe comorbidities, 5 underwent primary closure, and 15 had application of Prolene (Ethicon Inc) mesh silo and serial reduction. Five died, including two managed conservatively, none primarily of the exomphalos. Survivors were followed up for 38 months (2–71). Cardiac anomalies were present in 20 (91%) patients: 8 had minor and 12 major anomalies. Twelve (55%) patients had other anomalies. Primary closure was associated with shorter length of stay (13 vs. 85 days, p = 0.02), but infants had similar lengths of intensive care stay, duration of parenteral feeds, and time to full feeds. Infants with cardiac anomalies had shorter times to full closure (28 vs. 62 days, p = 0.03), but other outcomes were similar. Conclusion Infants whose defect can be closed primarily have a shorter length of stay, but other outcomes are similar. Infants with more significant abdominovisceral disproportion are managed with staged closure; the presence of major cardiac anomalies does not affect surgical outcome.

Intussusception is a common abdominal emergency in infants and young children, with a peak incidence between 5 and 7 months of age (70 % of cases present between 3 and 13 months). Treatment is reduction, usually by pneumatic or hydrostatic enema. However, surgery is required when enema reduction fails or there is radiologic indication of doubt or risk regarding reduction. Laparoscopy may be diagnostic, providing confirmation of reduction or persistent intussusception (where doubt exists), or it may be interventional, allowing a minimally invasive approach to reduction. In the event that laparoscopic reduction is unsuccessful, it also enables a focused and minimal incision for open surgery.

Esophageal atresia/tracheo-esophageal fistula (EA/TEF) has an incidence of approximately 1:3,500. The incidence of malrotation is thought to be 1:200-500. We attempted to define the incidence of a combination and discuss the implications.This was a retrospective review of all patients admitted to a single institution with a diagnosis of EA or EA/TEF or TEF between April 1981 and January 2013. Patients were included if the position of the duodeno-jejunal flexure (DJF) was determined by upper GI contrast study (UGIS), surgery or post-mortem.Case notes were reviewed for 235 patients. In the EA type A group, 3/28 (11 %; 95 % CI 3.7-27.2 %) had malrotation, significantly higher than the reported incidence of malrotation in the general population (p = 0.0008). All three patients in this group were symptomatic with one patient found to have a volvulus at emergency surgery. In the type C group, 6/196 (3 %, 95 % CI 1.4-6.5 %) had malrotation, significantly higher than the incidence reported for the general population (p = 0.0033) but not significantly different to that of the type A group (p = 0.0878). There were no patients with malrotation identified in any other EA/TEF type. In total, 9/235 (3.8 %; 95 % CI 2.0-7.2 %) patients with EA had malrotation, significantly higher than the 5/1,050 (0.48 %) reported for the general population (p = 0.0002).There is a high incidence of malrotation in patients with pure EA. In the type A group an attempt to identify the DJF position at gastrostomy siting and/or performance of UGIS in the neonatal period should be undertaken. There should also be a low threshold for UGIS in all EA/TEF patients.

Aortopexy is the most effective treatment for severe tracheomalacia associated with esophageal atresia with distal tracheo-esophageal fistula (EA/TOF). In the last few years, the thoracoscopic approach has been proposed, but the number of patients treated is limited. The purpose of this study is to review our initial experience with thoracoscopic aortopexy.A retrospective review of medical records was performed on EA/TOF patients undergoing thoracoscopic aortopexy at the Great Ormond Street Hospital for Sick Children (London, United Kingdom) from January 2009 to May 2012. Patient demographics, indication, perioperative course, and long-term results when available were noted.Four patients underwent a successful thoracoscopic aortopexy, with no operation being converted. No morbidity or mortality was associated with the procedure. Length of postoperative stay ranged from 2 to 4 days. All patients were relieved of their symptoms, and no recurrence was noted.Thoracoscopic aortopexy is a feasible and successful treatment for severe tracheomalacia in EA/TOF patients. The complication rate may be lower than after the open procedure and is more satisfactory in terms of cosmetic appearance. However, we need a larger and prospective study with a longer follow-up to confirm these preliminary results.

To determine whether fluoroscopic balloon dilation (FBD) is a safe and effective method of treating esophageal anastomotic stricture after surgical repair in an unselected patient population.With ethics committee approval, records for 103 consecutive patients who underwent FBD with our interventional radiology service (1999-2011) were reviewed retrospectively. Patients underwent diagnostic contrast material-enhanced study prior to the first dilation. Dilations were performed by using general anesthesia. Outcomes were number and/or frequency of dilations, clinical effectiveness and response to dilations, esophageal perforation, requirement for surgery, and mortality. Data were expressed as mean ± standard deviation (with range). Comparisons were conducted by using the Fisher exact test and log-rank test. The significance level was set at P.05.One hundred three patients (61 male patients, 59%) underwent 378 FBD sessions (median, two dilations per patient; range, 1-40 dilations). The median age at first FBD was 2.2 years (range, 0.1-19.5 years). The balloon catheter diameters ranged from 4 to 20 mm. FBD was successful in 93 patients (90%): 44 (47%) after single dilation and 49 (53%) after multiple dilations. There was no difference in the proportion of patients who required one dilation and were younger than 1 year versus those who were 1 year of age and older (P.99; odds ratio, 1.07 [range, 0.43-2.66]). Ten patients (10%) required further procedures: Three underwent stent placement, three underwent esophageal stricture resection, and four underwent esophageal reconstruction. Four esophageal perforations (1%) developed after FBD. Antireflux surgery was performed in 18 patients (17%). There were no deaths.FBD for anastomotic strictures after esophageal atresia repair is feasible and acceptably safe and provides relief of symptoms in most patients (90%); however, about half require more than one dilation, and surgery is best predicted if more than 10 dilations are required.

Purpose To assess testicular atrophy and non-scrotal position after laparoscopic second stage Fowler-Stephens orchidopexy. Material and Methods Retrospective case review of all consecutive laparoscopic second stage Fowler-Stephens orchidopexies performed at our institutions between January 2000 and May 2009. Results Eighty-nine children, aged between 14 months and 18 years (median 3 years 8 months), underwent a laparoscopic second stage Fowler-Stephens orchidopexy for 109 intra-abdominal testes (20 bilateral cases, 69 unilateral: right 31, left 38). In 10 of the 20 boys with bilateral intra-abdominal testes, a laparoscopic second stage Fowler-Stephens orchidopexy was performed synchronously; in the other 10 children the second stages took place on separate occasions. Five boys (8 testes) were lost to follow-up. The remaining 84 children (101 testes) were reviewed in outpatient clinic for up to 8 years (median 9 months). Testicular atrophy was identified in 12 of 101 testicles (12%). This included 8 of 66 (12%) unilateral cases and 4 of 35 (11%) bilateral intra-abdominal testes. Testicular ascent was noted in 11 of 101 testicles (11%), which comprised 6 (9%) unilateral and 5 (14%) bilateral cases. Of the 16 bilateral testicles brought to the scrotum during the same procedure 3 (18%) atrophied and 5 (31%) ascended, compared to 1 (5%) atrophy and 1 (5%) ascent amongst the 19 testes brought to the scrotum on separate occasions. These differences were not statistical significant (Fisher's exact test: p = 0.31 and p = 0.07, respectively). Overall, 78 of the 101 (78%) testicles achieved satisfactory outcomes on follow up. Conclusions Our series of laparoscopic second stage Fowler-Stephens orchidopexy is one of the largest studies to date, documenting a successful outcome in 78%. Testicular atrophy occurred in 12% and ascent in 11%. This is vital information when counselling parents.

Achalasia is a rare esophageal motility disorder: its optimal treatment in children is still a matter of debate. Records of children treated for achalasia, over an 18-year period, were reviewed.Forty-eight children (median age at diagnosis 10 years; range 3-17 years) were identified. Twenty-eight patients were initially treated with Heller's myotomy (HM) and 20 with balloon dilatation (BD). At last follow-up (median 3 years; range 1-5.5 years), 43.8% (21/48) of children were symptom free. The number of asymptomatic children was significantly higher among those treated initially with HM compared to BD (HM 15/28, 53.6% BD 6/20, 30%, p 0.05). All children who underwent BD required HM due to symptom recurrence. The median (range) total number of procedures was significantly higher in the BD group (BD 3 (1-7); HM 1 (1-5); p 0.05) with a shorter time to the second intervention (BD 14 months, 95%CI 4-24; HM 58 months, 95%CI 38-79; p 0.05). Of 108 procedures, esophageal perforation occurred in two children after HM (two out of 48 HM procedures in total, 4%) and one child after BD (1/60, 1.7%).Less than half of children with achalasia are symptom free after initial treatment with either BD or HM. HM, however, when performed as first procedure, provided longer symptom-free period and reduced need for subsequent intervention. What is Known: • Balloon dilatation (BD) and Heller's myotomy (HM) are safe and effective treatment options for achalasia. • Controversy, however, exists regarding the most effective initial therapeutic approach. What is New: • HM with or without fundoplication may represent the initial therapeutic approach of choice. • Initial BD may negatively affect the outcome of a subsequent HM.

In our institution, some children routinely receive parenteral nutrition (PN) following surgery for duodenal atresia/stenosis, while others do not. Our aim was to compare growth and infection rate between these two treatment strategies. This was a retrospective study of all children undergoing surgery for duodenal atresia/stenosis over 7 years. Of the 54 children, 19 commenced PN soon after surgery (the ‘Initial PN’ group). Of the remaining 35 children, 13 (37 %) subsequently required PN (the ‘Delayed PN’ group). The remaining 22 never received PN (the ‘Never PN’ group). The proportion of patients experiencing clinically suspected sepsis was higher in those receiving PN (‘Initial’ plus ‘Delayed’; 41 %) compared with those who never received PN (14 %; p = 0.04). The ‘Initial PN’ and ‘Never PN’ groups did not show a significant change in weight Z score over time. However, the ‘Delayed PN’ group showed a significant decrease in weight Z scores from the time of operation to the time of achieving full enteral feeds, and failed to catch up by the time of last follow-up. Children with duodenal atresia/stenosis can be managed without PN. However, a third of these children subsequently require PN, lose weight centiles, and have a high rate of sepsis.

Aim “Buried bumper” is a complication of percutaneous gastrostomy related to the internal flange getting buried into the wall of the stomach. The aim of this study is to evaluate the management of this complication. Methods The surgical and interventional radiology database in our hospital from August 1999 to May 2011 was analyzed. There were 2,007 patients who underwent percutaneous gastrostomy insertion. Notes for patients with buried bumper were reviewed. A telephonic interview with the parents of these children was performed with focused assessment of the care of the gastrostomy tube before the episode of buried bumper. Continuous data are reported as median (range). Results Twenty children developed buried bumper after gastrostomy insertion. They had a primary diagnosis of neurological ( n = 14), metabolic ( n = 3), or endocrine ( n = 3) disorders. The age at presentation was 5.7 years (2 to 18 years); 2.5 years (1 month to 5 years) after gastrostomy insertion. Ten children (50%) presented with symptoms related to buried bumper which included leakage around the gastrostomy ( n = 4), pus, discharge or bleeding from the site ( n = 5), stiffness on feeding ( n = 3), and unable to push the flange ( n = 1) (three children had more than one symptom). Ten children (50%) were asymptomatic and underwent routine change or removal of gastrostomy. In nine children, there was an attempt to remove the flange by interventional radiology but this was successful only in one. In the remaining 19 children, 4 had endoscopic removal while 15 children developed an inflammatory mass and required a laparotomy ( n = 12) or laparoscopic-assisted excision ( n = 3). Conclusion Buried bumper is a rare complication of percutaneous gastrostomy. Inadequate postoperative care without appropriate mobilization is a factor leading to this preventable complication. Endoscopic removal is possible, failing which laparoscopic surgery should be considered.

Midgut volvulus due to malrotation may result in loss of the small bowel. Until now, after derotation of the volvulus, pediatric surgeons do not deal with the mesenteric thrombosis, which causes continuing ischemia of the intestine. On occasion, a “second look” laparotomy is performed in the hope that some improvement in blood supply to the intestine has occurred. We describe a new combined treatment to restore intestinal perfusion based on digital massage of the superior mesenteric vessels after derotation and systemic infusion of tissue-type plasminogen activator. This new therapy has been successful in 2 neonates with severe intestinal ischemia due to volvulus.

Purpose The purpose of this study is to evaluate (1) the predictive value of fetal bowel dilatation (FBD) for intestinal atresia in gastroschisis and (2) the postnatal management and outcome of this condition. Methods A retrospective review of all gastroschisis cases diagnosed in our fetal medicine unit between 1992 and 2010 and treated postnatally in our center was performed. Results One hundred thirty cases had full postnatal data available. Intestinal atresia was found at surgery in 14 neonates (jejunum, n=6; ileum, n=3; ascending colon, n=3; multiple, n=2). Polyhydramnios and FBD were more likely in the atresia group compared with infants with no atresia ( P = .0003 and P = .005, respectively). Fetal bowel dilatation had 99% negative predictive value (95% confidence interval, 0.9-0.99) and 17% positive predictive value (95% confidence interval, 0.1-0.3) for atresia. Treatment of intestinal atresia included primary anastomosis (n = 5), delayed anastomosis (n = 2), and stoma formation followed by anastomosis (n = 7). Infants with atresia had longer duration of parenteral nutrition, higher incidence of sepsis, and cholestasis compared with infants with no atresia ( P = .0003). However, the presence of atresia did not increase mortality. Conclusions Polyhydramnios and FBD are associated with atresia. Absence of FBD in gastroschisis excludes intestinal atresia. In our experience, atresia is associated with a longer duration of parenteral nutrition but does not influence mortality. These findings may be relevant for antenatal counseling.

Purpose Various pull-through techniques, both open and laparoscopic, have been performed for Hirschsprung disease. Our study compares open and laparoscopic Duhamel pull-through. Methods After ethical approval, we reviewed all children (n = 181) with Hirschsprung disease admitted to our institution between 1999 and 2009. We excluded total colonic aganglionosis (n = 14), previous pull-through done elsewhere (n = 33), or follow-up performed abroad (n = 58). Open and laparoscopic pull-through were done in the same period according to surgeon preference. Data were analyzed using χ 2 or Mann-Whitney U test. Results Seventy-six children had a Duhamel pull-through for rectosigmoid aganglionosis. Operative time, time to full feeds, and length of hospital stay were similar in each group. Open (n = 41) Fifteen children (37%) required 33 further procedures. Fourteen had procedures for persistent constipation, including redo Duhamel (n = 2), stoma formation (n = 2), spur division (n = 2), and dilatation/stretch/Botox/rectal biopsy/manual evacuation (n = 23). Three children had other procedures (adhesiolysis [n = 2] and incisional hernia repair [n = 1]). Laparoscopic (n = 35) Fourteen children (40%) required 30 further procedures. Eleven had procedures for persistent constipation, including redo Duhamel (n = 1), stoma formation (n = 4), spur division (n = 9), and dilatation/stretch/rectal biopsy (n = 8). Three children had other procedures (adhesiolysis [n = 1] and incisional hernia repair [n = 2]). There were 4 conversions. Conclusion Open and laparoscopic Duhamel pull-through have similar outcomes. We show that the techniques have comparable operative times and hospital stay.

Aim In ventilator-dependent infants with complex comorbidities, severe gastro-oesophageal reflux (GOR) may contribute to prolonging the period of ventilation. It is often difficult to predict whether antireflux surgery will improve the respiratory status of an infant and assist with weaning off the ventilator. The aim of this study was to review the outcomes in a cohort of ventilator-dependent infants who underwent fundoplication to help wean them off ventilation. Methods Between January 2006 and December 2010, out of 596 infants who underwent fundoplication for symptoms of GOR, 26 were ventilator dependent before surgery; 13 patients had an emergency fundoplication following an acute life-threatening event (n = 5, 19%) or an acute deterioration of respiratory status (n = 8, 31%). Fundoplication was planned in the rest of the group (n = 13, 50%) with the aim of improving respiratory status and weaning from ventilation. The median age at surgery was 5.8 months (range: 0.8 to 19.4 months). The median weight at surgery was 6.3 kg (range: 4 to 15.1 kg). Data were collected for each infant on comorbidities, pre- and postoperative ventilation status, pre- and postoperative GOR symptoms, and survival. Results All infants underwent a Nissen fundoplication with no intraoperative morbidity or mortality. Of these, 12 infants had a laparoscopic fundoplication; 14 infants had an open fundoplication. Postoperatively, all infants received invasive positive pressure ventilation in the intensive care unit (ICU). All infants were successfully weaned from ventilation. The median time to extubation was 4 days (range: 2 to 18 days). The median postoperative ICU stay was 9 days (range: 3 to 52 days). Of the patients, 9 (34%) had a recurrence of symptoms following fundoplication; 5 (19%) subsequently underwent revision of fundoplication and 1 (3.8%) underwent oesophago-gastric dissociation; and 10 (38%) died within the study period. Conclusion In infants with severe GOR, ventilator dependence, and complex comorbidities, fundoplication may be a useful procedure to assist weaning off ventilator dependence. Rates of symptom recurrence, of revision of fundoplication, and of mortality within this cohort were higher than expected. These data reflect the challenges of patient selection in high-risk groups.

Background/Purpose A diverting jejunostomy without bowel resection is an option for surgical management of extensive necrotizing enterocolitis (NEC). We aimed to determine outcomes of infants who underwent this operation. Methods We collected clinical and outcome data on infants undergoing a diverting jejunostomy with no bowel resection as a primary procedure for extensive NEC. Data are median (range). Results Seventeen neonates underwent a diverting jejunostomy. Eleven (65%) had multifocal disease, whereas 6 (35%) had pan-intestinal involvement. Perforation was seen in 7 (41%), all with multifocal disease. The stoma was placed 12 cm (8-45) from the duodenojejunal flexure. Six infants (35%) died, 4 of these within a day of operation, owing to persisting instability. Intestinal continuity was achieved in all survivors after 52 (17-83) days, and only 1 infant (9%) had a colonic stricture. Seven infants recovered without the need for further intestinal resection distal to the jejunostomy. In those that survived, parenteral nutrition was needed for 2.2 months (1.3-18.0). A single patient had short bowel syndrome. Conclusions A diverting jejunostomy is a useful surgical procedure that allows high survival and enteral autonomy in the treatment of extensive NEC. In most patients, the affected intestine recovers without further intestinal resection.

Objectives Improved survival in infants with esophageal atresia (EA) with a birth weight

Provision of anesthesia for thoracoabdominal surgery in the neonate presents a number of specific challenges to the anesthesiologist. Many neonates are born at term in good condition, with or without an antenatal diagnosis, but many others are born premature and/or of low birth weight with associated cardiac abnormalities, pulmonary hypertension, and/or complications relating to the specific surgical diagnosis. Many of the principles of neonatal surgery are discussed elsewhere—this chapter considers specific conditions that require thoracic or abdominal surgery in the neonatal period, and the anesthetic and surgical requirements for each. There are very few randomized controlled trials to guide management. Most management strategies are based on retrospective case reviews or expert opinion. The use of minimally invasive techniques is becoming increasingly common and these approaches are also discussed.

Gastric transposition is an established method of esophageal replacement in children, and the use of minimally invasive techniques avoids the trauma of open access. The objective of this study was to compare outcomes of minimally invasive versus open gastric transposition in children.All cases of attempted laparoscopic-assisted gastric transposition at Great Ormond Street Hospital (GOSH), London, United Kingdom, between 2003 and 2012 were retrospectively reviewed. A comprehensive literature search was completed on MEDLINE for minimally invasive gastric transposition in children, and postoperative outcomes were collated. The outcomes from the retrospective review (single-center, GOSH) and the literature search (multicenter) were compared with those of the largest study on open gastric transposition consisting of 192 cases performed at GOSH.In this retrospective review of 19 patients (mean age, 3.5 years; range, 0.4-15 years), the indications were long-gap esophageal atresia, postoperative, caustic, and idiopathic esophageal stricture, and esophageal dysmotility. Three cases were converted to laparotomy and excluded from subsequent analysis. There were one anastomotic leak, two strictures, and no deaths in this series. The literature search yielded a further 50 cases for comparison. Single-center (n=16) and multicenter (n=66) comparison of minimally invasive versus open technique (n=192) showed no difference in leak (6.3% and 16.7%, respectively, versus 12.0%; P=.701 and P=.398), stricture (12.5% and 15.2% versus 20.8%; P=.535 and P=.370), and mortality rates (0% and 1.5% versus 4.7%; P=1.000 and P=.461).Minimally invasive gastric transposition is a safe and acceptable alternative to open surgery in children.

Background The optimal management of oesophageal achalasia remains unclear in the paediatric population due to the rarity of the disease. This study reviews the institutional experience of the laparoscopic Heller's cardiomyotomy (HC) procedure and attempts to define the most appropriate treatment. Methods A retrospective review of children undergoing HC at a single institution was performed. Demographics, pre-operative investigations, and interventions were reviewed. Post-operative outcomes and follow up were evaluated. Data is expressed as median (range). Results Twenty-eight children were included (13 male, 15 female) whose median age was 13 (3.2–17.4) years. Nine children underwent a pre-operative oesophageal balloon dilatation (OBD) a median of 1(1–6) times. Others included botulinum toxin injection (n=1) and Nifedipine (n=1). All had a pre-operative upper gastrointestinal contrast series, and twenty-five had upper gastrointestinal endoscopy and manometry. All had laparoscopic HC with no conversions, and ten had a concomitant fundoplication. Post-operative intervention occurred in eight (28%) incorporating OBD (n=7), of whom four required a redo HC. One patient underwent a redo without intervening OBD. Follow-up was for a median of 0.83 (0–5) years with fourteen children discharged from surgical follow-up. Twenty-seven have thus far had a good outcome. Conclusion This study comprises the largest series of paediatric laparoscopic HC reported to date. It is effective with or without a fundoplication and is the best long term treatment modality available. OBD for persisting symptoms following HC may obviate the need for redo myotomy.

Purpose To evaluate outcomes in critically ill neonates with necrotising enterocolitis (NEC) undergoing a laparotomy in the neonatal intensive care unit (NICU). Methods This is a retrospective review of neonates diagnosed with NEC who underwent a laparotomy on NICU between 2001 and 2011. Demographic, diagnostic, operative and outcome data were analysed. Nonparametric comparison was used. Data are reported as median (range). Results 221 infants with NEC were referred for surgical evaluation; 182 (82%) underwent surgery; 15 (8%) required a laparotomy on NICU. Five had NEC totalis, 4 multifocal disease and 6 focal disease. Five had an open and close laparotomy, 8 stoma with/without bowel resection and 2 bowel resection and primary anastomosis. Ten (67%) died at a median of 6.5-hours (2–72) postoperatively; 2 died at 72 and 264-days. The 30-day mortality rate was higher (p=0.01) among infants undergoing a laparotomy on NICU (10/15; 67%) than in theatre (54/167; 32%). There was no significant difference in mean Paediatric Index of Mortality 2 Scores between survivors and nonsurvivors (p=0.55). Three (20%) infants remain alive with no or minimal disability at 1.4 (0.5–7.5) years. Conclusion Laparotomy for NEC on NICU is a treatment option for neonates who are too unstable to transfer to theatre. However, with 67% dying within 6.5-hours and a further 13% after months in hospital, we must consider whether surgery is always in their best interests. Development of a prediction model to help distinguish those at highest risk of long-term morbidity and mortality could help with decision making in this difficult situation.

Morgagni diaphragmatic hernia can be repaired laparoscopically. The aim of this study is to evaluate the outcome of this minimally invasive approach.A retrospective review was conducted on all consecutive children who underwent repair of Morgagni hernia from January 2002 to December 2011 in our hospital. Data are expressed as median (range).There were 12 children with Morgagni hernia. Age at surgery was 7.5 months (2-125). Associated malformations were present in 7 children (58 %). All children underwent initial laparoscopic approach. Two children (16 %) underwent conversion to open surgery. The hernia was closed primarily in 11 children (92 %), using a polyester patch in 1 (8 %). There were no intraoperative or immediate postoperative complications. Five children (42 %), all repaired initially without a patch, had a recurrence of the Morgagni hernia. The repair of the recurrent hernia was performed laparoscopically in four out of the five children, and a patch was used in two patients with no further recurrences or complications.There is a high rate of recurrence after laparoscopic Morgagni hernia repair. This is exclusively associated with laparoscopic repair without patch, and it is in contrast with the low recurrence rate reported previously. More frequent use of patch may be beneficial.

Indications for laparoscopic inguinal hernia repair in infants and children remain controversial. The purpose of this study is to compare clinical features and outcome of laparoscopic inguinal hernia repair in infants with older children. Retrospective single centre review of all patients

Aim of the study: the aim of this study was to report incidence and clinical outcomes of recurrent necrotizing enterocolitis (NEC). Methods: review of infants treated for recurrent episode(s) of NEC at a tertiary Neonatal Surgical Intensive Care Unit over 8 years (January 2002 to February 2011). Demographic, clinical, radiological, and operative data were analyzed and compared using Mann-Whitney or Fisher's exact tests. Data are reported as median (range). Results: a total of 212 consecutive infants were referred for surgical evaluation and treatment of NEC (Bell stage II or III). Of these patients, 22 (10%) had suspected recurrent NEC: in 11 of these the primary episode was Bell stage I successfully treated before coming to our institution (suspected recurrent NEC); in the remaining 11, the primary episode was confirmed (Bell stage II or III) NEC successfully treated in our hospital. Birth weight, gestational age at birth, corrected gestational age, weight on admission, gender, need for surgery, stricture, and mortality rates were similar between infants with recurrent NEC and those with a single episode. Long-term parenteral nutrition (PN) dependency (>28 days) was significantly more common following recurrent NEC compared with a single episode. Among the infants with recurrent NEC, medical therapy alone was not successful in the majority (82%) of cases during the first episode and all required surgery during the recurrent episode. Conclusion: infants (10%) referred for surgical treatment of NEC develop recurrence of the disease. Surprisingly, these infants have similar mortality and stricture rates to those with a single episode. However, the incidence of long-term PN dependency was significantly increased in those with recurrent episodes of NEC

Aim of the Study The aim of this study was to investigate the factors associated with mortality in infants referred for the surgical treatment of advanced necrotizing enterocolitis (NEC). Methods Retrospective review of all infants with confirmed (Bell stage II or III) NEC treated in our unit during the past 8 years (January 2002 to December 2010). Data for survivors and nonsurvivors were compared using Mann-Whitney test and Fisher's exact test and are reported as median (range). Results Of the 205 infants with NEC, 35 (17%) were medically managed; 170 (83%) had surgery; 66 (32%) infants died; all had received surgery. Survivors and nonsurvivors were comparable for gestational age, birth weight, and gender distribution. Overall mortality was 32%, the highest mortality was in infants with pan-intestinal disease (86%) but remained significant in those with less severe disease (multifocal 39%; focal disease 21%). The commonest cause of mortality was multiple organ dysfunction syndrome and nearly half of the nonsurvivors had care withdrawn. Conclusion Despite improvement in neonatal care, overall mortality (32%) for advanced NEC has not changed in 10 years. Mortality is significant even with minimal bowel involvement.

Aim of the Study Stomal stenosis is the commonest complication of the antegrade colonic enema (ACE) procedure, reportedly occurring in 25–55% of patients. As such, a simple ACE stopper device (a small silicone plug sited in the ACE conduit between catheterisations) was designed to prevent stenosis. We performed a long-term follow-up study to determine the effectiveness of the stopper device. Methods A retrospective case note review was performed of all patients who successfully underwent a primary ACE procedure over an 8.5-year period (January 2002 to June 2010). The inclusion criteria were (i) a minimum of 6 months follow-up, (ii) simple appendicostomy, (iii) caecal/colonic flap. In all patients an ACE stopper was sited in the conduit for at least 4 months and removed only for catheterisation. Data are mean±SEM. Main Results 38 children were included in our study. Mean age at surgery was 9.6±0.5 years. Surgery was performed in 22 patients for incontinence and in 16 for chronic constipation. 31 underwent an appendicostomy and 7 had a caecal/colonic flap; all received an ACE stopper. The mean follow-up was 2.6±0.3 years. Only 3 patients (8%) developed stomal stenosis. The first occurred 6 months postoperatively, resulting from an ACE stopper which was too small and consequently persistently fell out. This conduit required dilatation. The second occurred at 27 months secondary to a stomal infection and required surgical revision. The third occurred 8 months postoperatively for no obvious cause, and was treated with dilation. 1 patient experienced stomal leakage. Conclusion The ACE stopper is a simple yet highly effective method of preventing stomal stenosis. We recommend using the stopper in all ACE patients.

Background/Purpose Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TOF) can be repaired thoracoscopically, but this may cause hypercapnia, acidosis, and reduced cerebral oxygenation. We evaluated the effect of thoracoscopy in infants on cerebral oxygen saturation (cSO 2 ), arterial blood gases, and carbon dioxide (CO 2 ) absorption. Methods Eight infants underwent thoracoscopy (6 CDH and 2 EA/TOF). Serial arterial blood gases were taken. Regional cSO 2 was measured using near-infrared spectroscopy. Absorption of insufflated CO 2 was calculated from exhaled 13 CO 2 / 12 CO 2 ratio measured by mass spectrometry. Results CO 2 absorption increased during thoracoscopy with a maximum 29% ± 6% of exhaled CO 2 originating from the pneumothorax. Paco 2 increased from 9.4 ± 1.3 kPa at the start to 12.4 ± 1.0 intraoperatively and then decreased to 7.6 ± 1.2 kPa at end of operation. Arterial pH decreased from 7.19 ± 0.04 at the start to 7.05 ± 0.04 intraoperatively and then recovered to 7.28 ± 0.06 at end of operation. Cerebral hemoglobin oxygen saturation decreased from 87% ± 4% at the start to 75% ± 5% at end of operation. This had not recovered by 12 (74% ± 4%) or 24 hours (73% ± 3%) postoperatively. Conclusions This preliminary study suggests that thoracoscopic repair of CDH and EA/TOF may be associated with acidosis and decreased cSO 2 . The effects of these phenomena on future brain development are unknown.

Objectif Evaluer les resultats de l’approche coelioscopique des malrotations intestinales. Methode Etude retrospective monocentrique des patients ayant eu une coelioscopie entre avril 2007 et janvier 2014 devant un diagnostic radiologique de malrotation intestinale. Les donnees sont en mediane (range). Resultats 44 patients ont ete operes a 7mois (1jour-16ans). 33 (75%) patients etaient symptomatiques. 37 (84%) coelioscopies etaient menees a terme. 7 (16%) etaient converties pour difficulte d’evaluer le mesentere ou en raison du volvulus. La duree etait de 85min (30–190). La duree de realimentation etait de 4jours (1–27) et d’hospitalisation de 5jours (1–118). Le suivi etait de 11,4mois (1mois-5,2ans). 4 (9%) patients ont eu une reprise : 3 (7%) pour volvulus et 1 (2%) pour procedure incomplete. Conclusion Il s’agit d’une des plus grandes series de Ladd coelioscopique. Le taux de conversion (8–33%), de reprise (2–19%) et de volvulus post Ladd (0–19%) est similaire aux series publiees. La procedure de Ladd coelioscopique est realisable avec peu de complications. Elle pourrait permettre de reduire le risque d’occlusion sur brides lie a la voie ouverte. Un seuil de conversion bas est fondamental en cas de difficulte a evaluer la racine du mesentere.

Objectif Evaluer notre experience preliminaire d’aortopexie par thoracoscopie pour le traitement de la tracheomalacie severe associee a l’atresie de l’œsophage avec fistule oesotracheale (AO-FOT). Methode Etude retrospective des patients operes d’une AO-FOT qui ont eu une aortopexie thoracoscopique entre janvier 2009 et mai 2012. Les donnees sont en moyennes (range). Resultats 4 patients ont ete traites par thoracoscopie. Aucune procedure n’a ete convertie. L’âge a l’intervention etait de 11mois (3–27) et le poids de 8,4 Kgs (4,9–10,9). La duree d’intervention etait de 128min (95–150). Aucune morbi-mortalite n’etait associee a l’intervention. La duree maximale d’utilisation de morphinique en postoperatoire etait de 48h. La duree de sejour postoperatoire etait de 4 jours (2–7). Les symptomes ont disparu chez tous les patients et aucun n’a recidive. Conclusion L’aortopexie thoracoscopique permet de traiter avec succes une tracheomalacie severe associee a une AO-FOT. Le taux de complication pourrait etre moins eleve que par voie open avec un avantage esthetique certain.

Introduction Management of oesophageal atresia (OA) and trachea-oesophageal fistula (TOF) in babies of low birth weight is challenging especially when associated with other anomalies. Birth weight of