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The diagnosis and treatment of conjoined twins are reviewed. Conjoined twins are commonly known as Siamese twins. This means that the bodies of both twins are joined, and they usually share some organs.

Long term outcomes of gastric transposition (GT) for complex esophageal atresia (EA) are poorly reported. We aimed to perform comprehensive long term follow up of adults who had been treated with GT for EA as children. Consecutive patients who underwent GT for EA in childhood aged > 18 years old were identified alongside age matched patients who had primary repair (PR). Type of EA, comorbidities and details of surgery were recorded. Telephone interviews included medical history, current symptoms — including gastrointestinal symptom rating scale (GSRS), morbidity and health related quality of life (HRQoL) using gastrointestinal quality of life index (GIQLI). 32 participants were interviewed in each group (mean age 29 years). BMI (19.9 ± 3.5) was significantly lower (p = 0.0006) in GT group. 6/32 (19%) still required supplementary feeding. Adult morbidity included anastomotic stricture (34%), chronic respiratory disease (28%), dumping symptoms (25%), anemia (47%) and depression (19%). 3 patients required major revision surgery. Participants in both groups report regular upper gastrointestinal symptoms (GSRS: GT = 2.1, PR = 2.0) and were more symptomatic than the normal population (1.4) but not statistically different from each other. HRQoL (GIQLI = 113) was lower than after PR (122) but not significantly different (p = 0.29) and the normal population (125). 23% of GT participants had higher than normal HRQoL. GT for EA is associated with significant morbidity and symptoms, including issues previously unreported in adulthood such as mental health problems. This mandates long term follow up and quality transition of these patients into adult care. Retrospective study. Level III. [ABSTRACT FROM AUTHOR]

Abstract Aim of the study Divided colostomy (DC) has been recommended in anorectal malformations (ARMs) with previously reported advantages of decreasing overflow into the distal limb and urinary tract infections (UTIs). Skin bridge loop colostomy (LC) is a technically easier alternative without an increase in these complications. We report our institutional experience of LC in ARM. Methods Retrospective study (Institution-approved Clinical Audit) reviewing the clinical records of all patients with ARM undergoing stoma formation in a single UK tertiary pediatric surgical center (2000–2015). Data collected included type of ARM, associated anomalies, type and level of colostomy, time to stoma closure, complications and UTIs. Results One hundred and eighty-two (95 female) patients underwent colostomy formation for ARM. The vast majority (171/ 94%) underwent LC; 9 (5%) had a divided colostomy (DC) and 2 (1%) had no available data. The spectrum of defects in girls included rectovestibular (62/65%), rectovaginal (4/4%) and cloaca (29/31%). In boys, 71 (82%) had a fistula to the urinary tract and 16 (18%) presented with a perineal fistula. Urological abnormalities coexisted in 87 (47.8%) patients. Thirty five (21%) patients developed UTIs. Among the 19 girls who developed UTI, 8 had rectovestibular fistula and 11 had cloaca. Of the 16 boys who developed UTI, 14 had a fistula to the urinary tract and 11 had an independent urological abnormality. The mean time from stoma formation to stoma closure was 10 (3–52) months. Complications were reported in 22 (12%) LCs. Fifteen patients (9%) developed a stoma prolapse following LC with 10 (6%) requiring surgical revision. Conclusions This is the largest reported series of outcomes following LC for ARM. LC is easier to perform and to close, requiring minimal surgical access, with comparable complications and outcomes to those published for DC. Type of study Retrospective comparative study. Level of evidence III. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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%).Conclusion: 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. [ABSTRACT FROM AUTHOR]

Background/Purpose To report outcomes of a standardised technique for intestinal anastomosis in infants and children. Methods Data were prospectively collected on all paediatric intestinal anastomosis by a single surgeon over a 21 year period. Anastomoses were constructed using an end-to-end extramucosal technique with interrupted polypropylene sutures. Demographic and clinical data were recorded. Results Six-hundred and thirteen anastomoses were constructed in 550 patients. Median age at time of anastomosis was 6 months (range 1 day–226 months). The most common reason for anastomosis was stoma closure (n = 271, 49%). For those patients that required multiple anastomoses the most common pathology was acute NEC (n = 22/41, 54%). One-hundred and one (18.4%) patients passed stool within 24 hours of surgery, 175 (31.8%) between 24–48 hours and 95 (17.3%) between 48–72 hours. Anastomotic complications occurred in 7 patients (1.3%) including anastomotic leakage (n = 5, 0.9%) and anastomotic stricture (n = 2, 0.4%). The majority of anastomotic leakages (80%) followed resection of acute NEC. Conclusions The interrupted extramucosal anastomosis is safe and effective. The return of bowel function is rapid and the complication rate acceptable. We recommend this technique be used for all intestinal anastomoses in children and infants. [ABSTRACT FROM AUTHOR]

Objectives Improved survival in infants with esophageal atresia (EA) with a birth weight < 1,500 g or a major cardiac anomaly has been reported when compared with the original Spitz classification proposed in 1994. Aim We reviewed outcome data for infants born over the last decade in our institution to update previously reported survival statistics. Materials and Methods The records of all neonates (n = 200) with a diagnosis of EA managed in a single institution between 2001 and 2011 were reviewed and compared with data from the original Spitz study and the subsequent reported cohort from the same institution. Data were obtained on birth weight, presence of a major cardiac anomaly, and survival. Differences in survival were compared using the Yates-corrected chi-square test. Local ethical study approval was obtained. Results Infants born over the last decade had a comparable overall survival rate of 93% (186/200) versus 92.6% (174/188) in the previously reported cohort (1993-2004). We demonstrate an improved survival as compared to the Spitz cohort (87.6%, 326 /372, p = 0.06) and a statistically significant improvement in survival in Group II (p = 0.01). Within this group, 12/51 neonates had a birth weight < 1,500 g and 39/51 had major cardiac anomalies. Of interest, of the nine deaths in Group II, eight were in the subgroup with major cardiac anomalies. Conclusion The survival of neonates in Group II has significantly improved. Mortalities within this group were predominantly in the subgroup with major cardiac anomalies suggesting birth weight is of less significance than in previous years reflecting recent advances in neonatal care. We propose an updated prognostic classification that makes a distinction between cardiac and low-birth-weight infants. [ABSTRACT FROM AUTHOR]

Background Straining at stool is an automatic reflex in babies and implies the presence of rectal sensation. We hypothesised that early reported rectal sensation would predict future continence in children with anorectal anomalies. Aim of the study The aim of this study is to determine if early straining at stool was a useful predictor of future continence in infants born with high anorectal malformations. Methods A retrospective case note review of prospectively collected clinical information was performed with institutional review board approval. All patients with intermediate/high anorectal malformation operated on by a single surgeon from 1984 to 2010 were included. After stoma closure, parents were asked: • Do you see your baby straining to pass stool? • Can you tell when your baby is about to pass stool? The responses were noted within the first year of stoma closure and then all patients were followed up until they were at least 3 ½ years old and continence could be assessed using the Krickenbeck outcome classification. Data were compared using Fisher's exact test and sensitivity, specificity and positive predictive value (PPV) were calculated. Main results Forty-eight patients were included in the study. Sixteen (33%) were female (12 cloacal malformation, 3 rectovaginal fistula, 1 rectal atresia) and 32 (66%) were male (6 rectovesical fistulae, 22 rectourethral fistulae, 4 no fistula). Median follow-up was 9.7 years (range 3.5–17.9). Twenty-one children were noted by their parents to exhibit early straining at stool after stoma closure. Twenty of them achieved long term continence. The sensitivity of early straining as a predictor for long term continence was 77%, specificity 95% and positive predictive value 95%. Conclusion The presence of early rectal sensation reported by parents is a good predictor of long term continence. This allows more informed discussion with families in the early years of life. [ABSTRACT FROM AUTHOR]

The various stages of the separation are carefully planned but despite this, variations which will change the schedule of the procedure may exist. In general the operation commences on the opposite side from the main procedure and then the twins are turned for the remainder of the operation. Each type of conjoined twin is different but basically thoracopagus involves the hearts, omphalopagus involves the liver and small intestine and ischiopagus involves the large intestine and genito-urinary system. Our results are presented together with interesting cases from which lessons have been learned. [ABSTRACT FROM AUTHOR]

Conjoined twins represent a great challenge for most pediatric specialists including pediatric surgeons, anesthetists, neonatologists, urologists, neurosurgeons, and orthopedic surgeons. This anomaly can be classified according to the type of twins׳ fusion. Various organs can be fused making the separation difficult. Conjoined twins are usually diagnosed antenatally by ultrasound. Detailed fetal echocardiography is necessary to counsel the parents during pregnancy. Postnatally, the majority of the conjoined twins can be thoroughly investigated using various imaging techniques. This allows careful planning of the operation. However, in approximately one-third of the patients an urgent operation is required at birth without a complete assessment of the joining. This is associated with a poorer outcome. [ABSTRACT FROM AUTHOR]

Background: 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. Materials and Methods: 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. Results: 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). Conclusion: Minimally invasive gastric transposition is a safe and acceptable alternative to open surgery in children. [ABSTRACT FROM AUTHOR]

Aims Oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF) is the most common congenital anomaly of the oesophagus. There is limited literature suggesting a linear relationship between increasing gap length and the incidence of all major complications. We sought to assess whether measured gap length at the time of surgery was related to outcomes in our patients. =section-title= Methods All patients with a diagnosis of OA ± TOF who underwent repair under a single surgeon between 1983 and 2012 were included. The length between the oesophageal pouches was measured at the time of surgery. Patients were then divided into three groups; short ≤ 1 cm, intermediate > 1-≤2 cm and long > 2-≤5 cm. Outcome measures were anastomotic leak, strictures requiring dilatation, gastrooesophageal reflux disease (GORD) and need for fundoplication. =section-title= Results 122 patients were included in the study. The outcomes for patients with short (n = 53), intermediate (n = 51) and long gaps (n = 18) were as follows: anastomotic leak -- 1.9%, 2%, 5.5% (P = 0.66), strictures requiring dilatation -- 32%, 33%, 50% (P = 0.67), GORD -- 51%, 59%, 72% (P = 0.58) and need for fundoplication -- 11%, 20%, 44% (*P = 0.02). There were no deaths related to the repair. =section-title= Conclusions Measured gap length at the time of surgery did not have a linear relationship with leak or stricture rate. Our experience suggests that when primary repair is possible absolute gap length is irrelevant to the development of post-operative complications. There is however a significant increase in the need for fundoplication in those with a long gap. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: 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. Patients and Methods: 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. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: Gastroschisis neonates have delayed time to full enteral feeds (ENT), possibly due to bowel exposure to amniotic fluid. We investigated whether delivery at <37weeks improves neonatal outcomes of gastroschisis and impact of intra/extra-abdominal bowel dilatation (IABD/EABD). Methods: A retrospective review of gastroschisis (1992–2012) linked fetal/neonatal data at 2 tertiary referral centers was performed. Primary outcomes were ENT and length of hospital stay (LOS). Data (median [range]) were analyzed using parametric/non-parametric tests, positive/negative predictive values, and regression analysis. Results: Two hundred forty-six patients were included. Thirty-two were complex (atresia/necrosis/perforation/stenosis). ENT (p<0.0001) and LOS (p<0.0001) were reduced with increasing gestational age. IABD persisted to last scan in 92 patients, 68 (74%) simple (intact/uncompromised bowel), 24 (26%) complex. IABD or EABD diameter in complex patients was not significantly greater than simple gastroschisis. Combined IABD/EABD was present in 22 patients (14 simple, 8 complex). When present at <30weeks, the positive predictive value for complex gastroschisis was 75%. Two patients with necrosis and one atresia had IABD and collapsed extra-abdominal bowel from <30weeks. Conclusion: Early delivery is associated with prolonged ENT/LOS, suggesting elective delivery at <37weeks is not beneficial. Combined IABD/EABD or IABD/collapsed extra-abdominal bowel is suggestive of complex gastroschisis. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Revisional oesophageal reconstructive surgery carries uncommon and unusual risks related to previous surgery. To provide maximum anatomical detail and facilitate successful outcome, we report a standardised pre-operative investigative strategy for all such patients. Methods: Prospective 8-month cohort study following the introduction of this strategy. All patients underwent high resolution thoracic contrast CT scan and micro-laryngo-bronchoscopy by a paediatric ENT surgeon in addition to upper gastrointestinal contrast study, oesophagoscopy, and echocardiogram. Results: Seven children (median age 5.6months [range 2.2–60]) completed the pathway. Four were referred with recurrence of a previously divided tracheo-oesophageal fistula (3 congenital, 1 acquired) and 3 (all with oesophagostomy) for oesophageal replacement for congenital isolated oesophageal atresia (OA, n=1) and failed repair of OA with distal TOF with wide gap (n=2). Overall, unanticipated findings were demonstrated in 6/7 children and comprised severe tracheomalacia and right main bronchus stenosis requiring aortopexy (n=1), vocal cord palsy (n=2), extensive mediastinal rotation (n=1), proximal tracheal diverticulum (n=1), severe subglottic stenosis requiring airway reconstruction (n=1), proximal tracheal diverticulum (n=1), right sided aortic arch (n=1) and left sided aortic arch (previously reported to be right sided, n=1). Conclusions: This standardised approach for this complex group of patients reveals a high incidence of unexpected anatomical and functional anomalies with significant surgical and possible medico-legal implications. We recommend these investigations during the pre-operative work-up prior to all revisional oesophageal surgery. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

Abstract: Background/Purpose: Video-assisted thoracoscopic surgery (VATS) resection of mediastinal neurogenic tumors is still controversial in children. The aim of this study was to review the cases of VATS resection of such tumors in children from 3 institutions located in different countries. Methods: This retrospective study included 17 children treated between July 1995 and February 2011. Medical charts were reviewed for collection of data on age, sex, histologic type of tumor, clinical manifestations, age and weight at surgery, tumor size, duration of thoracic drainage, surgical complications, tumor recurrence, and mortality. Results: Thirteen (76.5%) males and 4 (23.5%) females were studied. Median age was 16 months (range, 10.6-60 months), and median weight was 11.9 kg (range, 9.3-27.4 kg). Ten children had neuroblastoma (58.8%), 4 had ganglioneuroma (23.5%), and 3 had ganglioneuroblastoma (17.7%). The median duration of the operation was 90 minutes (range, 45-180 minutes), with complete thoracoscopic resection in all cases. Two children (11.8%) developed Horner syndrome postoperatively. No deaths were reported, and no recurrence was noted during a median follow-up period of 16 months (range, 8.9-28.6 months). Conclusions: Video-assisted thoracoscopic surgery resection of mediastinal neurogenic tumors in children produced good results, with no recurrence and minimal postoperative complications. The major advantages of this approach are the avoidance of thoracotomy complications and the enhanced surgical accuracy provided by improved visualization. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

Abstract: Purpose: Neuroblastoma (NB), ganglioneuroblastoma (GNB), and ganglioneuroma (GN) are neuroblastic tumours (NT) of sympathetic nervous system origin. Brain lipid-binding protein (BLBP) has potential morphogenic activity during nervous system development but has not been studied in these tumours. We analyzed the expression of BLBP in NT according to histological subtypes and extent of differentiation. Methods: Thirty cases of NT (10 each of NB, intermixed GNB, and GN) were identified from the histopathology archive of a single center. Tissue sections were obtained from representative paraffin blocks and immunohistochemistry for BLBP performed. Results: Brain lipid-binding protein was not expressed in any NB case. In all cases of GN, BLBP was strongly expressed in the cytoplasm of mature ganglion cells but negative in Schwannian stroma. In the intermixed GNB, there was similar strong BLBP immunoreactivity in the cytoplasm of fully differentiated and differentiating ganglion cells but no BLBP expression in immature neuroblasts. Conclusion: Brain lipid-binding protein is strongly expressed in mature and maturing ganglion cells in NT (GN and GNB), whereas it is absent in poorly differentiated neuroblasts of GNB and NB. Cytoplasmic expression of BLBP in NT increases as the cells undergo neural differentiation and is therefore associated with the extent of tumour differentiation and favorable histology. [Copyright &y& Elsevier]