A Novel Standard for Systematic Reporting of Neuroblastoma Surgery: The International Neuroblastoma Surgical Report Form (INSRF): A Joint Initiative by the Pediatric Oncological Cooperative Groups SIOPEN∗, COG∗∗, and GPOH∗∗∗.

OBJECTIVE: To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBL-related surgical procedures and their outcomes. SUMMARY OF BACKGROUND DATA: NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated. Various surgical guidelines exist within the protocols of the different cooperative groups, although there is no standardized operative report form to document the surgical treatment of NBL. METHODS: After analyzing the treatment protocols of the SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology pediatric cooperative groups, important variables were defined to completely describe surgical biopsy and resection of NBL and their outcomes. All variables were discussed within the Surgical Committees of SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology. Thereafter, joint meetings were organized to obtain intercontinental consensus. RESULTS: The "International Neuroblastoma Surgical Report Form" provides a structured reporting tool for all NBL surgery, in every anatomical region, documenting all Image Defined Risk Factors and structures involved, with obligatory reporting of intraoperative and 30 day-postoperative complications. CONCLUSION: The International Neuroblastoma Surgical Report Form is the first universal form for the structured and uniform reporting of NBL-related surgical procedures and their outcomes, aiming to facilitate the postoperative communication, treatment planning and analysis of surgical treatment of NBL.

ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management.

INTRODUCTION: Evidence supporting best practice for long-gap esophageal atresia is limited. The European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) organized a consensus conference on the management of patients with long-gap esophageal atresia based on expert opinion referring to the latest literature aiming to provide clear and uniform statements in this respect. MATERIALS AND METHODS: Twenty-four ERNICA representatives from nine European countries participated. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing items on perioperative, surgical, and long-term management, and literature review. The 2-day conference was held in Berlin in November 2019. Anonymous voting was conducted via an internet-based system using a 1 to 9 scale. Consensus was defined as ≥75% of those voting scoring 6 to 9. RESULTS: Ninety-seven items were generated. Complete consensus (100%) was achieved on 56 items (58%), e.g., avoidance of a cervical esophagostomy, promotion of sham feeding, details of delayed anastomosis, thoracoscopic pouch mobilization and placement of traction sutures as novel technique, replacement techniques, and follow-up. Consensus ≥75% was achieved on 90 items (93%), e.g., definition of long gap, routine pyloroplasty in gastric transposition, and avoidance of preoperative bougienage to enable delayed anastomosis. Nineteen items (20%), e.g., methods of gap measurement were discussed controversially (range 1-9). CONCLUSION: This is the first consensus conference on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia. Substantial statements regarding esophageal reconstruction or replacement and follow-up were formulated which may contribute to improve patient care.

Insufflation in minimally invasive surgery: Is there any advantage in staying low?

AIM: Minimally invasive repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) and congenital diaphragmatic hernia (CDH) is feasible and confers benefits compared to thoracotomy or laparotomy. However, carbon dioxide (CO2) insufflation can lead to hypercapnia and acidosis. We sought to determine the effect of lower insufflation pressures on patients' surrogate markers for CO2 absorption - arterial partial pressure of CO2 (PaCO2), end tidal CO2 (EtCO2) and pH. METHODS: Single center retrospective review, including neonates without major cardiac anomaly. Selected patients formed 2 groups: Historical pressure (HP) group and low pressure (LP) group. We reported on the patients' preoperative characteristics that potentially confound the degree of CO2 absorption or elimination. Outcome measures were perioperative PaCO2, EtCO2, arterial pH and anesthetic time. RESULTS: 30 patients underwent minimally invasive surgery for CDH and 24 patients for EA/TEF with similar distribution within the HP and LP group. For CDH patients as well as for EA/TEF patients, there were no significant differences in their preoperative characteristics or surgery duration comparing HP and LP groups. With a decrease in insufflation pressure in CDH patients, there were a significant decrease (p = 0.002) in peak PaCO2 and an improvement in nadir pH (p = 0.01). For the EA/TEF patients, the decrease in insufflation pressure was associated with a significant decrease (p = 0.03) in peak EtCO2. Considering all 54 patients, we found EtCO2 to be highly significantly inversely correlated with pH and positively correlated with intraoperative PaCO2 (p < 0.001). Baseline Hb was inversely correlated with mean EtCO2 (p < 0.001). CONCLUSION: With lower insufflation pressures, CDH patients had significantly improved hypercapnia and acidosis, while EA/TEF patients had significantly reduced EtCO2. EtCO2 was correlated with acidosis and hypercapnia. TYPE OF STUDY: Retrospective case control study. LEVEL OF EVIDENCE: Level III.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework.

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.

Potential Benefits of Laparoscopic Repair of Duodenal Atresia: Insights from a Retrospective Comparative Study.

INTRODUCTION: Congenital duodenal obstruction (CDO) repair can be performed open or laparoscopically. We aimed to determine the potential benefit of laparoscopic repair regarding tolerance of enteral feeding, postoperative pain, hospital stay, and complication rate. MATERIALS AND METHODS: In a single-center retrospective cohort study, we compared neonates with isolated CDO operated open versus laparoscopically from 2010 to 2019. No transanastomotic tubes were used, and anastomoses were created in a side-to-side fashion in all cases. An early feeding policy is applied for all cases operated at our institution. Statistical comparison was performed using the Mann-Whitney's test or Fisher's exact test where appropriate. RESULTS: Forty-one patients analyzed were similar regarding body weight, gestational age, and proportion of patients with trisomy 21. Median follow-up was 21 months. Four (20%) out of 20 laparoscopic procedures started laparoscopically were converted to open. Comparing the 21 open with the 16 laparoscopically completed patients, median anesthetic duration was shorter by 18% in the open versus laparoscopic completed group (218 vs. 179 minutes, respectively; p = 0.025). Median postoperative time to full enteral feeds was shorter by 4 days in the first group (7 vs. 11 days, respectively; p = 0.028). In accordance, the median duration of parenteral nutrition (PN) was less than half in the laparoscopic completed compared with the open group (5 vs. 11.5 days, respectively; p = 0.031). Postoperative opioids were required for only half the duration in the laparoscopically completed group compared with open (2 vs. 4 days, respectively; p = 0.026). Outcomes such as length of stay, the occurrence of strictures or adhesions requiring reintervention, or line sepsis were similar in both groups. CONCLUSION: Patients undergoing laparoscopic CDO repair at our institution benefited from shorter time to full enteral feeds, and reduced the need for PN as well as postoperative pain medication.

Variations in the Detection of Anorectal Anomalies at Birth among European Cities.

INTRODUCTION: The diagnosis of anorectal malformations (ARMs) is made at birth by perineal examination of the newborn, yet small series reported late diagnosis in almost 13%. No large series to date have looked into the magnitude of missed ARM cases in the neonatal period across Europe. This study aimed to define the rate of missed ARM at birth across four United Kingdom and European Union centers. MATERIALS AND METHODS: All ARM cases treated at two United Kingdom tertiary centers in the past 15 years were compared with two tertiary European centers. Demographic and relevant clinical data were collected. Late diagnosis was defined as any diagnosis made after discharge from the birth unit. Factors associated with late diagnosis were explored with descriptive statistics. RESULTS: Across the four centers, 117/1,350, 8.7% were sent home from the birth unit without recognizing the anorectal anomaly. Missed cases showed a slight female predominance (1.3:1), and the majority (113/117, 96.5%) were of the low anomaly with a fistula to the perineum. The rate of missed ARM cases was significantly higher in the United Kingdom centers combined (74/415, 17.8%) compared with those in the European Union (43/935, 4.6%) (p < 0.00001), and this was independent of individual center and year of birth. CONCLUSION: Significant variation exists between the United Kingdom and other European countries in the detection of ARM at birth. We recommend raising the awareness of accurate perineal examination at the time of newborn physical examination. We feel this highlights an urgent need for a national initiative to assess and address the timely diagnosis of ARM in the United Kingdom.

Congenital intrathoracic stomach can be safely managed laparoscopically.

PURPOSE: Congenital intrathoracic stomach (CIS) is an uncommon pediatric surgical diagnosis where > 2/3rd of the stomach lies within the chest through a hiatus defect. We reviewed our recent experience with this condition. METHODS: A retrospective single-center review of children with a diagnosis of CIS (2007-2018) was performed. Patient demographics, presentation, imaging and management were assessed. Results are expressed as median (range). RESULTS: Eleven patients (6 girls) were identified with onset of symptoms at 2 (0-26) months of age. Presenting symptoms were vomiting (8/11), respiratory symptoms (4/11) and failure to thrive (2/11). Two patients had Marfan's syndrome. An upper gastrointestinal contrast study demonstrated gastric herniation in all. All were corrected laparoscopically with hiatus repair and fundoplication [age at surgery 10.5 (1.5-34.5) months]. A concurrent gastrostomy was done in children ≤ 6 months (n = 5). Enteral feeds were commenced on post-operative day one in 9 and second post-operative day in 2. At 7 (0-95) months follow-up, all were on full enteral feeds. One patient had a recurrence 6 months post-operatively, which was re-operated laparoscopically without any further recurrence. CONCLUSION: This is the largest reported series of children with CIS. All could be managed laparoscopically with no conversions and a low recurrence.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Diagnostics, Preoperative, Operative, and Postoperative Management.

INTRODUCTION: Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature. MATERIALS AND METHODS: Nineteen ERNICA representatives from nine European countries participated in the conference. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing the domains diagnostics, preoperative, operative, and postoperative management, and literature review. The 2-day conference was held in Berlin in October 2018. Anonymous voting was conducted via an internet-based system. Consensus was defined when 75% of the votes scored 6 to 9. RESULTS: Fifty-two items were generated with 116 relevant articles of which five studies (4.3%) were assigned as level-1evidence. Complete consensus (100%) was achieved on 20 items (38%), such as TEF closure by transfixing suture, esophageal anastomosis by interrupted sutures, and initiation of feeding 24 hours postoperatively. Consensus ≥75% was achieved on 37 items (71%), such as routine insertion of transanastomotic tube or maximum duration of thoracoscopy of 3 hours. Thirteen items (25%) were controversial (range of scores, 1-9). Eight of these (62%) did not reach consensus. CONCLUSION: Participants of the conference reached significant consensus on the management of patients with EA/TEF. The consensus may facilitate standardization and development of generally accepted guidelines. The conference methodology may serve as a blueprint for further conferences on the management of congenital malformations in pediatric surgery.

Role of Routine Dilatations after Anorectal Reconstruction-Comparison of Two Tertiary Centers.

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.

Laparoscopic Ladd's procedure for malrotation in infants and children is still a controversial approach.

BACKGROUND: Open Ladd's procedure is the gold standard for the correction of intestinal malrotation and laparoscopic approach remains controversial. This study aimed to evaluate our experience in laparoscopic management of malrotation. METHODS: Single center retrospective study including patients who underwent a laparoscopic assessment of intestinal malrotation with correction if appropriate between 2007 and 2017. RESULTS: Sixty-five patients (median age 7 months) had a laparoscopic assessment with and without correction of malrotation. Forty-five (69%) were symptomatic, including 16 (25%) with a midgut volvulus. The procedure was completed laparoscopically in 55 (86%) patients in 110 min (30-190). Conversions happened more frequently at the beginning of the experience. With a follow-up of 12.5 months (8 days-5.3 years), morbidity rate was 15% and 4 (6%) patients underwent a redo surgery, all in the first 5 months after surgery, compared with 3/53 (6%) in a contemporaneous group undergoing open Ladd's. CONCLUSION: This is the largest series reported so far of the laparoscopic management of malrotation. Laparoscopic Ladd's procedure is reliable but still exposes to open conversion which may be in part owing to a learning curve. A low conversion threshold is important in cases with volvulus. The redo rate is similar to that of the open procedure. LEVEL OF EVIDENCE: Level III retrospective comparative treatment study.

Surgical jejunostomy and radiological gastro-jejunostomy tube feeding in children: risks, benefits and nutritional outcomes.

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.

Morgagni hernia repair in children over two decades: Institutional experience, systematic review, and meta-analysis of 296 patients.

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<0.05. 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.

The outcome of critically ill neonates undergoing laparotomy for necrotising enterocolitis in the neonatal intensive care unit: a 10-year review.

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.

Is early delivery beneficial in gastroschisis?

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.

Fluoroscopic balloon dilation of esophageal atresia anastomotic strictures in children and young adults: single-center study of 103 consecutive patients from 1999 to 2011.

PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Hypercapnia and acidosis during open and thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia: results of a pilot randomized controlled trial.

OBJECTIVE: We aimed to evaluate the effect of thoracoscopy in neonates on intraoperative arterial blood gases, compared with open surgery. BACKGROUND: Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TEF) can be repaired thoracoscopically, but this may cause hypercapnia and acidosis, which are potentially harmful. METHODS: This was a pilot randomized controlled trial. The target number of 20 neonates (weight > 1.6 kg) were randomized to either open (5 CDH, 5 EA/TEF) or thoracoscopic (5 CDH, 5 EA/TEF) repair. Arterial blood gases were measured every 30 minutes intraoperatively, and compared by multilevel modeling, presented as mean and difference (95% confidence interval) from these predictions. RESULTS: Overall, the intraoperative PaCO2 was 61 mm Hg in open and 83 mm Hg [difference 22 mm Hg (2 to 42); P = 0.036] in thoracoscopy and the pH was 7.24 in open and 7.13 [difference -0.11 (-0.20 to -0.01); P = 0.025] in thoracoscopy. The duration of hypercapnia and acidosis was longer in thoracoscopy compared with that in open. For patients with CDH, thoracoscopy was associated with a significant increase in intraoperative hypercapnia [open 68 mm Hg; thoracoscopy 96 mm Hg; difference 28 mm Hg (8 to 48); P = 0.008] and severe acidosis [open 7.21; thoracoscopy 7.08; difference -0.13 (-0.24 to -0.02); P = 0.018]. No significant difference in PaCO2, pH, or PaO2 was observed in patients undergoing thoracoscopic repair of EA/TEF. CONCLUSIONS: This pilot randomized controlled trial shows that thoracoscopic repair of CDH is associated with prolonged and severe intraoperative hypercapnia and acidosis, compared with open surgery. These findings do not support the use of thoracoscopy with CO2 insufflation and conventional ventilation for the repair of CDH, calling into question the safety of this practice. The effect of thoracoscopy on blood gases during repair of EA/TEF in neonates requires further evaluation. (ClinicalTrials.gov Identifier: NCT01467245).

Recurrence rate of Morgagni diaphragmatic hernia following laparoscopic repair.

PURPOSE: Morgagni diaphragmatic hernia can be repaired laparoscopically. The aim of this study is to evaluate the outcome of this minimally invasive approach. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

Management of a complication of percutaneous gastrostomy in children.

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.

Outcomes of diverting jejunostomy for severe necrotizing enterocolitis.

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.

Decreased cerebral oxygen saturation during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia in infants.

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.