Short and Medium Term Outcomes of Open and Laparoscopic Assisted Oesophageal Replacement Procedures.

AIM OF THE STUDY: We describe the short- and medium-term outcomes following open and laparoscopic assisted oesophageal replacement surgery in a single tertiary paediatric surgical centre. METHODS: A retrospective review (institutional audit approval no. 3213) on patients who underwent open or laparoscopic-assisted oesophageal replacement (OAR vs. LAR) at our centre between 2002 and 2021 was completed. Data collected (demographics, early complications, stricture formation, need for oesophageal dilatations, and mortality) were analysed using GraphPad Prism v 9.50 and are presented as median (IQR). RESULTS: 71 children (37 male) had oesophageal replacement surgery at a median age of 2.3 years (IQR 4.7 years). 51 were LAR (6 conversions). Replacement conduit was stomach (n = 67), colon (n = 3), or jejunum (n = 1). Most gastric transpositions had a pyloroplasty (46/67) or pyloromyotomy (14/67). Most common pathology was oesophageal atresia (n = 50 including 2 failed transpositions), caustic injury (n = 19 including 3 due to button battery), stricture of unknown cause (n = 1), and megaoesophagus (n = 1). There were 2 (2.8 %) early postoperative deaths at 2 days (major vessel thrombosis), 1 month (systemic sepsis), and one death at 5 years in the community. The rate of postoperative complications were comparable across LAR and OAR including anastomotic leak, pleural effusions, or early strictures. More patients with caustic pathology needed dilatations (60 % vs 30 % in OA, p = 0.05). CONCLUSIONS: Outcomes of open and laparoscopic-assisted oesophageal replacement procedures are comparable in the short and medium term. Anastomotic stricture is higher in those with caustic injury. LEVEL OF EVIDENCE: IV.

Laparoscopic inguinal hernia repair (LIHR): the benefit of the double stitch in the largest single-center experience.

AIM: To review our experience of laparoscopic inguinal hernia repair (LIHR) regarding complication rates, the practice of closing the asymptomatic patent processes vaginalis (PPV), and comparison of complication rates between pre-term (< 37 week gestation) and term infants. METHODS: Retrospective review of LIHR performed between 2009 and 2021. Repair was performed by intracorporal single or double purse string/purse string + Z-stitch using a non-absorbable suture. Data were analyzed using Chi-squared/Mann-Whitney and are quoted as median (range). RESULTS: 1855 inguinal rings were closed in 1195 patients (943 (79%) male). 1378 rings (74%) were symptomatic. 492 (41%) patients were pre-term. Corrected gestational age at surgery was 55 weeks (31 weeks-14.6 years) and weight 5.9 kg (1-65.5). Closure of contralateral PPV was higher in the premature group (210/397 [53%] vs. 265/613 [43%] p = 0.003). There were 23 recurrences in 20 patients, of whom 10 had been born prematurely. The only factor significantly associated with a lower recurrence was use of a second stitch (p = 0.011). CONCLUSION: This is the largest single-center reported series of LIHR. LIHR is safe at any age, the risk of recurrence is low, and can be corrected by re-laparoscopy. Use of a Z-stitch or second purse string is associated with a significantly lower rate of recurrence.

Long-Term Functional Outcomes and Multidisciplinary Management after Ileorectal Duhamel Pull-Through for Total Colonic Aganglionosis-20-Year Experience in a Tertiary Surgical Center.

INTRODUCTION: Management of patients with total colonic aganglionosis (TCA) is challenging for pediatric surgeons. The purpose of this study was to review our institution's 20-year experience regarding long-term outcomes and multidisciplinary team management of these patients after an ileorectal Duhamel pull-through procedure. MATERIALS AND METHODS: Retrospective review was conducted for all patients diagnosed with TCA in our tertiary institution. Data were collected on demographics, clinical presentation, complications, need for additional surgery, and long-term effects on bowel function. RESULTS: Of a total of 202 patients with Hirschsprung's disease, 13 were diagnosed with TCA (6.4%). Clinical presentation was variable. Eleven presented in neonatal period with distal bowel obstruction and two presented with constipation in early infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow-up was 13 years. Eleven are toilet trained, of whom five are fully continent. Six continue to have problems with bowel continence or constipation. One developed recurrent episode of Hirschsprung's associated enterocolitis. Two patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, pediatric psychologists, and clinical nurse specialists. CONCLUSION: TCA can be associated with significant long-term morbidity. Nearly half of the patients in this series have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with inputs of a multidisciplinary team has greatly helped manage these complex patients in our institution.

Ultrasonographic features associated with previous torsion and the impact of surgery in managing neonatal ovarian cysts: a 20-year single-centre retrospective study.

PURPOSE: To identify markers of previous ovarian torsion and outline the outcomes according to US appearance and operative management. METHODS: A retrospective single-centre review of neonatal ovarian cysts from January 2000 to January 2020. Data on postnatal cyst size and sonographic features and operative treatment were co-related with outcomes of ovarian loss and histology. RESULTS: 77 females were included with 22 simple and 56 complex cysts, one patient had bilateral cysts. 9/22 (41%) simple cysts regressed spontaneously in a median of 13 weeks (8-17). Complex cysts regressed spontaneously less frequently, 7/56(12%, P = 0.01), in 13 weeks (7-39). 38/56 (68%) complex and 12/22 (55%) simple cysts were treated operatively. 21/22 (95%) ovaries with initially simple cyst were salvaged compared to 20/56(36%) with initially complex cyst (P < 0.001). A fluid-debris level in 23/26 complex cysts was most associated with ovarian loss (P = 0.0006). Presence of viable ovarian stromal tissue was seen in 8/20 (40%) excised specimens during ovarian sparing procedures and in 5/30 (17%) oophorectomies for necrotic appearing ovaries. CONCLUSIONS: Fluid-debris level on US is significantly associated with ovarian loss likely due to previous torsion. Simple cysts are viable and often regress spontaneously. The finding of viable ovarian stromal tissue in resected specimens supports attempting ovarian preservation wherever possible.

Shortwave Infrared Imaging Enables High-Contrast Fluorescence-Guided Surgery in Neuroblastoma.

Fluorescence-guided surgery is set to play a pivotal role in the intraoperative management of pediatric tumors. Shortwave infrared imaging (SWIR) has advantages over conventional near-infrared I (NIR-I) imaging with reduced tissue scattering and autofluorescence. Here, two NIR-I dyes (IRDye800CW and IR12), with long tails emitting in the SWIR range, were conjugated with a clinical-grade anti-GD2 monoclonal antibody (dinutuximab-beta) to compare NIR-I and SWIR imaging for neuroblastoma surgery. A first-of-its-kind multispectral NIR-I/SWIR fluorescence imaging device was constructed to allow an objective comparison between the two imaging windows. Conjugates were first characterized in vitro. Tissue-mimicking phantoms, imaging specimens of known geometric and material composition, were used to assess the sensitivity and depth penetration of the NIR-I/SWIR device, showing a minimum detectable volume of ∼0.9 mm3 and depth penetration up to 3 mm. In vivo, fluorescence imaging using the NIR-I/SWIR device showed a high tumor-to-background ratio (TBR) for both dyes, with anti-GD2-IR800 being significantly brighter than anti-GD2-IR12. Crucially, the system enabled higher TBR at SWIR wavelengths than at NIR-I wavelengths, verifying SWIR imaging enables high-contrast delineation of tumor margins. This work demonstrates that by combining the high specificity of anti-GD2 antibodies with the availability and translatability of existing NIR-I dyes, along with the advantages of SWIR in terms of depth and tumor signal-to-background ratio, GD2-targeted NIR-I/SWIR-guided surgery could improve the treatment of patients with neuroblastoma, warranting investigation in future clinical trials. SIGNIFICANCE: Multispectral near-infrared I/shortwave infrared fluorescence imaging is a versatile system enabling high tumor-to-background signal for safer and more complete resection of pediatric tumors during surgery.

Minimal access surgery for congenital diaphragmatic hernia: surgical tricks to facilitate anchoring the patches to the ribs.

OBJECTIVE: Minimal Access Surgery (MAS) for Congenital Diaphragmatic Hernia (CDH) repair is well described, yet only a minority of surgeons report this as their preferred operative approach. Some surgeons find it particularly difficult to repair the defect using MAS and convert to laparotomy when a patch is required. We present in this study our institutional experience in using an easy and relatively cheap methodology to anchor the patch around the ribs using Endo Close™. This device has an application in MAS for tissue approximation using percutaneous suturing. METHODS AND TECHNIQUE: We retrospectively reviewed our database for patients undergoing MAS repair of CDH between 2009 and 2021. Outcome measures included length of surgery and recurrence rates after patch repair. Endo Close™ was used in all patients who required patch repair. We declare no conflict of interest and to not having received any funding from Medtronic (UK). The technique is as follows: (1) The edges of the diaphragm are delineated by dissection. When primary suture repair of the diaphragmatic hernia was unfeasible without tension, a patch was used. (2) The patch is anchored in place by two corner stitches at the medial and lateral borders. (3) The posterior border of the patch is fixed to the diaphragmatic edge by running or interrupted stitches. (4) For securing the anterior border, a non-absorbable suture is passed through the anterior chest wall and the patch border is taken with intracorporeal instruments. (5) Without making another stab incision, the Endo Close™ is tunnelled subcutaneously through the anterior chest wall. (6) The suture end is pulled through the Endo Close™ and the knot is tied around the rib. This procedure can be performed as many times as required to secure the patch. RESULTS: 58 patients underwent MAS surgery for repair of CDH between 2009 and 2021. 48 (82%) presented with a left defect. 34 (58%) had a patch repair. The length of patch repair surgery for CDH ranged from 100-343 min (median 197). There was only one patient (3%) in the patch repair cohort that had a recurrent hernia, diagnosed 12 months after the initial surgery. CONCLUSIONS: In our experience, MAS repair of CDH is feasible. We adopted a low threshold in using a patch to achieve a tension-free repair. We believe that the Endo Close™ is a cheap and safe method to help securing the patch around the ribs.

Thoracoscopic vs open repair of congenital diaphragmatic hernia after extracorporeal membrane oxygenation: a comparison of intra-operative data.

PURPOSE: ECMO is an escalation treatment for hypoxic respiratory failure in patients with CDH. Open repair has been advocated after ECMO indicating that physiological changes associated to thoracoscopic repair were not well tolerated. METHODS: We have performed a retrospective review of all patients who underwent ECMO prior CDH repair over a 7 year period (2015-2021). Outcome measures were intra-operative Ph, PCO2, PO2 and FiO2 at 30 min, 1 h 30 min, and 2 h 30 min of surgery, operative time and recurrence rate. Data are shown in median (range). RESULTS: Eleven patients required ECMO prior CDH repair. Six of eleven (55%) were done thoracoscopically (Group A) and five of eleven (45%) via laparotomy (Group B). Two of six (33%) patients (Group A) were converted to a laparotomy, one of six (16%) patient developed a recurrence, and there was no recurrence in Group B. Two of five (40%) patients died within the first 60 days of life, whilst there was no death in Group A. Intra-operative values are shown below. CONCLUSION: Whilst this is a preliminary report of a limited number of patients, there is no obvious difference of intra-operative blood gas parameters during surgical repair in patients after ECMO. Thoracoscopic CDH repair may be considered in patients after ECMO.

Maintaining a minimally invasive surgical service during a pandemic.

PURPOSE: The safety of minimally invasive surgery (MIS) was questioned in the COVID-19 pandemic due to concern regarding disease spread. We continued MIS during the pandemic with appropriate protective measures. This study aims to assess the safety of MIS compared to Open Surgery (OS) in this setting. METHODS: Operations performed during 2020 lockdown were compared with operations from the same time-period in 2019 and 2021. Outcomes reviewed included all complications, respiratory complications, length of stay (LOS) and operating surgeon COVID-19 infections (OSI). RESULTS: In 2020, MIS comprised 52% of procedures. 29% of MIS 2020 had complications (2019: 24%, 2021: 15%; p = 0.08) vs 47% in OS 2020 (p = 0.04 vs MIS). 8.5% of MIS 2020 had respiratory complications (2019: 7.7%, 2021: 6.9%; p = 0.9) vs 10.5% in OS 2020 (p = 0.8 vs MIS). Median LOS[IQR] for MIS 2020 was 2.5[6] days vs 5[23] days in OS 2020 (p = 0.06). In 2020, 2 patients (1.2%) were COVID-19 positive (MIS: 1, OS: 1) and there were no OSI. CONCLUSION: Despite extensive use of MIS during the pandemic, there was no associated increase in respiratory or other complications, and no OSI. Our study suggests that, with appropriate protective measures, MIS can be performed safely despite high levels of COVID-19 in the population.

Standardizing the surgical management of benign ovarian tumors in children and adolescents: A best practice Delphi consensus statement.

AIM: No widely agreed consensus protocols exist for the management of benign ovarian tumors (BOT) in children. This presents a substantial risk for suboptimal management. We aimed to generate multispecialty consensus guidance to standardize surgical management and provide a clear follow-up protocol for children with BOTs. METHODS: Prospective two-round confidential e-Delphi consensus survey distributed among multispecialty expert panel; concluded by two semistructured videoconferences. MAIN RESULTS: Consensus was generated on these core outcome sets: preoperative/intraoperative management; follow-up; adolescent gynecology referral. (1) Children with BOTs should receive the same management as other patients with potentially neoplastic lesions: Preoperative discussion at a pediatric oncology multidisciplinary meeting to risk stratify tumors, and management by health professionals with expertise in ovarian-sparing surgery and laparoscopy. (2) Ovarian-sparing surgery for BOTs should be performed wherever possible to maximize fertility preservation. (3) Ovarian masses detected during emergency laparoscopy/laparotomy should be left in situ wherever feasible and investigated appropriately (imaging/tumor markers) before resection. (4) Follow-up should be undertaken for all patients after BOT resection. Patients should be offered referral to adolescent gynecology to discuss fertility implications. CONCLUSION: This best practice Delphi consensus statement emphasizes the importance of managing children with BOTs through a well-defined oncological MDT strategy, in order to optimize risk stratification and allow fertility preservation by ovarian-sparing surgery wherever possible.

Long-term outcomes following failure of Nissen fundoplication.

PURPOSE: To determine the management and outcomes of patients with gastro-oesophageal reflux (GOR) that requires further intervention following failure of Nissen fundoplication (NF). METHODS: After institutional audit department approval, a retrospective review of paediatric patients who had further intervention following failure of primary NF between January 2006 and December 2015 for GOR at our centre was performed. Data are presented as median (range). RESULTS: Of 820 patients who underwent NF, 190 (23%) received further procedures for GOR management at a median of 21 months of age (6-186); 90/190 (47%) had gastro-jejunal feeding (GJ). Of these, 67 (74%) remained on GJ feeds up to a median of 48 months and 23/90 (26%) had a second NF after GJ feeding. 97/190 (51%) had a redo fundoplication without having had a GJ; thus, 120/190 (63%) of patients having a further procedure went on to have a second NF after a median period of 15 months (1-70 months). Three patients (2%) had early emergency wrap revision 4 days after first fundoplication (we classed this as an 'early complication'). Of the seven patients who failed a 3rd NF, 4 continued GJ feeding, 2 of had oesophagogastric dissociation; 2 had 4th NF of which 1 was successful and 1 patient had gastric pacemaker and is successfully feeding orally. Patients who were finally successfully managed with GJ underwent 2 (2-5) tube changes/year. We found patients who had a previous GJ were more likely to have failure of the redo fundoplication than those who had not to have the GJ (16/24 vs. 30/90, p = 0.005). CONCLUSION: The chance of success decreases with every further attempt at fundoplication. The only factor significantly associated with failure of redo fundoplication was whether the patient previously had a GJ tube. In patients with failed fundoplications, when symptom free on jejunal feedings, further anti-reflux surgical intervention should be avoided. A randomized prospective study is needed for patient selection.

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.

Foreign body ingestion during the COVID-19 pandemic: a retrospective single centre review.

Introduction: During the COVID-19 pandemic in 2020, there were many changes in the provision of healthcare as well as home and educational environments for children. We noted an apparent increase in the number of children presenting with ingested foreign bodies and due to the potential impact of injury from this, further investigated this phenomenon. Method: Using a prospective electronic record, data were retrospectively collected for patients referred to our institution with foreign body ingestion from March 2020 to September 2020 and compared with the same period the year prior as a control. Results: During the 6-month pandemic period of review, it was observed that 2.5 times more children were referred with foreign body ingestion (n=25) in comparison to the control period (n=10). There was also a significant increase in the proportion of button battery and magnet ingestions during the COVID-19 pandemic (p 0.04). Conclusion: These findings raise concerns of both increased frequency of foreign body ingestion during the COVID-19 pandemic and the nature of ingested foreign bodies linked with significant morbidity. This may relate to the disruption of home and work environments and carries implications for ongoing restrictions. Further awareness of the danger of foreign body ingestion, especially batteries and magnets, is necessary (project ID: 2956).

Outcomes in Hirschsprung's disease with coexisting learning disability.

This study describes functional and health-related quality of life (HRQoL) outcomes in patients with Hirschsprung's disease (HSCR) with associated learning disability or neurodevelopmental delay (LD), completing a core outcome set for HSCR. This was a cross-sectional study from a tertiary pediatric surgery center. Patients treated between 1977 and 2013 were prospectively contacted to complete an outcomes survey. Children under 12 and older patients with LD were assisted to complete these by a proxy. Bowel and urologic function were assessed (Rintala's BFS and modified DanPSS) along with HRQoL (PedsQL/GIQLI/SF-36). Thirty-two patients with LD were compared to 186 patients with normal cognition. Patients with LD had 76% survival over the follow-up period, compared to 99% in the remainder of the cohort. Poor functional outcomes were common in the patients with LD, considerably higher than cognitively normal patients: with weekly issues withholding stool, soiling and fecal accidents in over half of patients surveyed (44-60%), and urinary incontinence in 46%. Use of permanent stoma was significantly higher (22% vs. 4%; p = 0.001). HRQoL was worse in domains of physical functioning in adults and children but not for social or emotional domains in adults. Subgroup analysis of patients with Down syndrome suggested similar functional results but better QoL. Multivariate analysis demonstrated a dramatically higher incidence of poor continence outcomes in patients with LD (adjusted OR 9.6 [4.0-23]).Conclusions: We provide LD-specific outcomes showing inferior function but similar HRQoL to other patients with HSCR, this is much needed in the counselling of families of these children. What is Known: • Hirschsprung's disease is commonly associated with syndromes or other anomalies with resultant cognitive impairments. • The outcomes for these patients specifically have been poorly described in the literature. What is New: • Objective functional and quality of life surveys demonstrate significant differences from patients without cognitive impairment. • Patients with learning disability Patients with associated LD were almost ten times more likely to have an associated poor functional outcome, with very little impact on proxy-reported quality of life.

Long-term surgical and patient-reported outcomes of Hirschsprung Disease.

BACKGROUND: Information is needed regarding the complex relationships between long-term functional outcomes and health-related quality of life (HRQoL) in Hirschsprung's Disease (HSCR). We describe long-term outcomes across multiple domains, completing a core outcome set through to adulthood. METHODS: HSCR patients operated at a single center over a 35-year period (1978-2013) were studied. Patients completed detailed questionnaires on bowel and urologic function, and HRQOL. Patients with learning disability (LD) were excluded. Outcomes were compared to normative data. Data are reported as median [IQR] or mean (SD). RESULTS: 186 patients (median age 28 [18-32] years; 135 males) completed surveys. Bowel function was reduced (BFS 17 [14-19] vs. 19 [19-20], p < 0•0001;η2 = 0•22). Prevalence and severity of fecal soiling and fecal awareness improved with age (p < 0•05 for both). Urinary incontinence was more frequent than controls, most of all in 13-26y females (65% vs. 31%,p = 0•003). In adults, this correlated independently with constipation symptoms (OR 3.18 [1.4-7.5],p = 0.008). HRQoL outcomes strongly correlated with functional outcome: 42% of children demonstrated clinically significant reductions in overall PedsQL score, and poor bowel outcome was strongly associated with impaired QOL (B = 22•7 [12•7-32•7],p < 0•001). In adults, GIQLI scores were more often impacted in patients with extended segment disease. SF-36 scores were reduced relative to population level data in most domains, with large effect sizes noted for females in General Health (g = 1.19) and Social Wellbeing (g = 0.8). CONCLUSION: Functional impairment is common after pull-through, but bowel function improves with age. Clustering of poor functional outcomes across multiple domains identifies a need for early recognition and long-term support for these patients.

Above and Beyond Robotic Surgery and 3D Modelling in Paediatric Cancer Surgery.

Although the survival rates for children's cancers have more than doubled in the last few decades, the surgical practise has not significantly changed. Among the most recent innovations introduced in the clinic, robotic surgery and augmented reality are two of the most promising, even if they are not widespread. The increased flexibility of the motion, the magnification of the surgical field and the tremor reduction provided by robotic surgery have been beneficial to perform complex oncological procedures in children. Besides, augmented reality has been proven helpful in planning for tumour removal, facilitating early discrimination between cancer and healthy organs. Nowadays, research in the field of surgical oncology is moving fast, and new technologies and innovations wich will help to shape a new way to perform cancer surgery. Paediatric surgeons need to be ready to adopt these novel devices and intraoperative techniques to allow more radical tumour resections with fewer complications. This review aims to present the mechanism of action and indications of several novel technologies such as optical imaging surgery, high definition cameras, and intraoperative loco-regional treatments. We hope this will enhance early adoption and more research on how to employ technology for the benefit of children.

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.

Comparison Between Diffusion-Weighted MRI and 123 I-mIBG Uptake in Primary High-Risk Neuroblastoma.

BACKGROUND: High-risk neuroblastoma (HR-NB) has a variable response to preoperative chemotherapy. It is not possible to differentiate viable vs. nonviable residual tumor before surgery. PURPOSE: To explore the association between apparent diffusion coefficient (ADC) values from diffusion-weighted magnetic resonance imaging (DW-MRI), 123 I-meta-iodobenzyl-guanidine (123 I-mIBG) uptake, and histology before and after chemotherapy. STUDY TYPE: Retrospective. SUBJECTS: Forty patients with HR-NB. FIELD STRENGTH/SEQUENCE: 1.5T axial DW-MRI (b = 0,1000 s/mm2 ) and T2 -weighted sequences. 123 I-mIBG scintigraphy planar imaging (all patients), with additional 123 I-mIBG single-photon emission computed tomography / computerized tomography (SPECT/CT) imaging (15 patients). ASSESSMENT: ADC maps and 123 I-mIBG SPECT/CT images were coregistered to the T2 -weighted images. 123 I-mIBG uptake was normalized with a tumor-to-liver count ratio (TLCR). Regions of interest (ROIs) for primary tumor volume and different intratumor subregions were drawn. The lower quartile ADC value (ADC25prc ) was used over the entire tumor volume and the overall level of 123 I-mIBG uptake was graded into avidity groups. STATISTICAL TESTS: Analysis of variance (ANOVA) and linear regression were used to compare ADC and MIBG values before and after treatment. Threshold values to classify tumors as viable/necrotic were obtained using ROC analysis of ADC and TLCR values. RESULTS: No significant difference in whole-tumor ADC25prc values were found between different 123 I-mIBG avidity groups pre- (P = 0.31) or postchemotherapy (P = 0.35). In the "intratumor" analysis, 5/15 patients (prechemotherapy) and 0/14 patients (postchemotherapy) showed a significant correlation between ADC and TLCR values (P < 0.05). Increased tumor shrinkage was associated with lower pretreatment tumor ADC25prc values (P < 0.001); no association was found with pretreatment 123 I-mIBG avidity (P = 0.17). Completely nonviable tumors had significantly lower postchemotherapy ADC25prc values than tumors with >10% viable tumor (P < 0.05). Both pre- and posttreatment TLCR values were significantly higher in patients with >50% viable tumor than those with 10-50% viable tumor (P < 0.05). DATA CONCLUSION: 123 I-mIBG avidity and ADC values are complementary noninvasive biomarkers of therapeutic response in HR-NB. LEVEL OF EVIDENCE: 4. TECHNICAL EFFICACY STAGE: 3.

The Compassionate Communities Connectors model for end-of-life care: a community and health service partnership in Western Australia.

BACKGROUND: There is an international drive towards increasing provision of community-led models of social and practical support for people living with advanced illness. AIM: This feasibility project aims to develop, implement and evaluate a model of community volunteers, identified as Compassionate Communities Connectors, to support people living with advanced life limiting illnesses/palliative care needs. The aims also include the development and evaluation of a training programme for volunteers and assessment of the feasibility, acceptability and preliminary effectiveness of this model of care. METHODS: The approach seeks to map and mobilise people's personal networks of care through the Connectors enlisting Caring Helpers (community volunteers). Up to 10 Connectors will be trained to work with at least 30 families selected by the palliative care service as requiring support. The primary outcome is the effect of the intervention on social connectedness. Secondary outcomes are the intervention's effect on unplanned hospital utilisation, caregiver support needs, advance care plans and satisfaction with intervention for patients/carers, volunteers and service providers. CONCLUSION: It is expected that this intervention will enhance patient, carer and family social, psychological and practical support and reduce the need for dying people to be admitted to a hospital.