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Aim of the Study: Abdominal lymphatic malformations (LM) are a challenging clinical entity. Complete excision can be impossible due to the infiltrative nature of some lesions and high rate of complications. Sclerotherapy may require multiple episodes of general anaesthesia and an inability to be definitive in terms of diagnosis and treatment. Subsequently we have adopted a newer algorithm of treatment involving a joint procedure with interventional radiology to enable an intra-operative treatment plan based on the findings. We present our early results., Method: A retrospective observational study in a single tertiary paediatric centre from January 2019 to December 2023. The details of each patient were recorded along with their treatment pathway and outcome., Results: 12 children underwent treatment for abdominal LM. Under GA and laparoscopic vision, pigtail catheters were inserted into the lesion to effect maximal drainage and assessment of surgical anatomy. If resection was feasible this was undertaken through a laparotomy and if not, the drains were retained and sclerotherapy was started. Three underwent drainage with sclerotherapy without resection and 9 underwent drainage with resection. Of the 3 who underwent sclerotherapy 2 have had further treatment and remain under review at a mean follow up of 7.6 months. Of the other 9 there were no signs of recurrence of the LM at a mean follow up of 29 (range 6-56) months., Conclusion: We have found a combined procedure involving IR-guided drainage under laparoscopic vision enabling curative surgical resection to be undertaken, where feasible, with currently no significant morbidity. For those in whom this is not possible then direct intralesional sclerotherapy is the next most useful treatment modality for longer term control of symptoms., Level of Evidence: II., (Copyright © 2024. Published by Elsevier Inc.)

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., Competing Interests: Conflicts of interest The authors have no competing interests to declare. There has not been any financial support for the conduct of this research., (Copyright © 2023. Published by Elsevier Inc.)

Background: Cloacal exstrophy (CE) is the most severe end of the Exstrophy-Epispadias Complex malformations spectrum. Improvements in postnatal management and well-established operative techniques have resulted in survival rates approaching 100%. This systematic review aims to define the prevalence of long-term active medical problems affecting CE patients after the first decade of life., Methods: PubMed/Medline, Embase, Scopus, and ISI Web of Knowledge databases were used for the literature search. Original articles related to medical, surgical, and psychosocial long-term problems in CE patients >10 years of age were included in the study. Quality assessment of the articles was performed through the Newcastle-Ottawa Scale. Prevalence estimates and 95% CI were assessed for each outcome., Results: Twelve studies were included. The most common long-term active problems identified were: urinary incontinence with a prevalence ranging from 9.1% to 85%; sexual function issues related to vaginal anomalies with a prevalence ranging from 8.3% to 71.3%, and uterine anomalies, with a prevalence from 14.3% to 71%; gender identity issues in 46, XY patients raised female had a prevalence from 11.1% to 66.7%. There is no documented history of paternity. Impairment of ambulatory capacity was recorded in 13.8% of patients. Only one paper studied psychological well-being, reporting significantly higher levels of depression among gender reassigned patients., Conclusions: Teenagers and adults born with CE have well defined long-term problems compared to the general population. Recognition and expert management are crucial to improve care and quality of life during and after the transition into adulthood., (Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.)

Background: The employment of fluorescence imaging has gained popularity in many fields of adult surgery where it has demonstrated great potentials to improve both surgical and oncological outcomes while minimizing anesthetic time and lowering health-care costs. However, the clinical application of fluorescence-guided surgery (FGS) in pediatrics is just at the initial phase., Material and Methods: A systematic review of current clinical uses of FGS in pediatric surgery was performed along with a discussion on its advantages, limitations and future developments., Results: 21 studies were included: 9 retrospective and 1 prospective study, 8 case reports, 2 case series and a review article reporting authors' institutional experience. Great emphasis was given to surgical resection of hepatoblastoma and its metastasis (n = 6), real-time imaging of the biliary tree (n = 3) and urogenital system (n = 2). Other current uses concern the assessment of blood perfusion (intestine, n = 3; myocutaneous flap, n = 1; transplanted liver, n = 1) and lymphatic flow imaging (n = 4)., Conclusion: Despite a paucity of clinical studies evaluating its role in pediatric surgery, FGS has shown promising results in helping guide tumor resection and improving the accuracy of anatomical delineation., Type of Study: Review article., Level of Confidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Sacrococcygeal teratoma (SCT) is the most common teratoma in neonates and arises from the coccyx. SCT with intraspinal invasion is extremely rare and only reported in a few cases., Methods: 37 patients with SCT were identified at our institution between 2000 and 2018. Three of these patients had SCT with intraspinal extension. A literature review for intraspinal extension associated with SCT, including mode of diagnosis, presentation, surgical approach and neurological sequelae, between 1993 and 2018 was also conducted., Results: The authors report three cases of infants who were antenatally and/or postnatally diagnosed with a sacrococcygeal teratoma extending into the spinal canal. We illustrate the challenges of accurate diagnosis and therapeutic management. Postnatal magnet resonance imaging (MRI) was the best method to define spinal anatomy and extension of the tumors prior to surgery. Management with a multidisciplinary team approach including neuroradiology, neurosurgery and general surgery was used in our two most recent patients. The literature review yielded 6 cases of SCT with intraspinal extension., Conclusion: Intraspinal extension in SCT is rare but should be excluded at birth before attempting any resection. In case of positive spinal invasion on Ultrasonography (US), MRI is essential to plan for surgery and possible laminectomy to be able to perform a radical resection of this congenital tumor. We recommend this multidisciplinary approach., Level of Evidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Recurrence of congenital diaphragmatic hernia (CDH) was retrospectively evaluated after correction with or without a patch in an institution where tension-free repair is advocated., Methods: Demographics and outcomes of patients with a postero-lateral CDH repaired (2000-2016) were analyzed (univariate tests and binary logistic regression adjusting for time since start of study, gender, defect side, liver herniation, patch, surgical approach, absence of postero-lateral rim and length of follow-up)., Results: Of 203 patients, 107 received a patch (P), and 96 were not patched (NP). Groups were not different for gestational age birthweight, gender, defect side and minimally invasive approach rate. Preoperative ECMO incidence (P:29.9% vs. NP:2.1%, p < 0.01), liver herniation (P:57.0% vs. NP:22.9%, p < 0.01) and absence of a postero-lateral rim (P:61.7% vs. NP:8.3%, p < 0.01) were higher in the P group. The mortality rate was 10.8% (P:15.0% vs. NP:6.2%, p = 0.07). Recurrence was not different (P:9.3% vs. NP:4.2%, p = 0.15). Multivariate analysis showed that recurrence was higher after thoracoscopy compared to open (OR = 12.2 [2.2-68], p < 0.01); neither the use of patch (OR = 2.3, [0.5-10.4], p = 0.28) nor any other factors were associated with recurrence., Conclusion: In this single centre series where tension-free repair was advocated, patch repair of CDH was not associated with higher recurrence, though access route was., Type of Study: Cohort Study., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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 (CO 2 ) insufflation can lead to hypercapnia and acidosis. We sought to determine the effect of lower insufflation pressures on patients' surrogate markers for CO 2 absorption - arterial partial pressure of CO 2 (PaCO 2 ), end tidal CO 2 (EtCO 2 ) 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 CO 2 absorption or elimination. Outcome measures were perioperative PaCO 2 , EtCO 2 , 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 PaCO 2 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 EtCO 2 . Considering all 54 patients, we found EtCO 2 to be highly significantly inversely correlated with pH and positively correlated with intraoperative PaCO 2 (p < 0.001). Baseline Hb was inversely correlated with mean EtCO 2 (p < 0.001)., Conclusion: With lower insufflation pressures, CDH patients had significantly improved hypercapnia and acidosis, while EA/TEF patients had significantly reduced EtCO 2 . EtCO 2 was correlated with acidosis and hypercapnia., Type of Study: Retrospective case control study., Level of Evidence: Level III., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Aim: Symptomatic tracheobronchomalacia can be fatal. Successful treatment includes aortopexy. We report outcomes of the thoracoscopic approach in a single centre., Methods: All patients undergoing thoracoscopic aortopexies from 2009 to 2018 were retrospectively reviewed. Data was reported as median (interquartile range). Risk factors for subsequent tracheostomy were analyzed with logistics regression model, p < 0.05 as significant., Results: Twenty-one patients with mid to distal tracheomalacia (n = 17) and bronchial involvement (n = 4) were determined on bronchoscopy, tracheobronchogram, or CT thorax. Preoperative patient demographics and comorbidities, e.g., gastro-oesophageal reflux disease, prematurity, and cardiac anomalies were recorded. Indications for thoracoscopic aortopexy were apparent life-threatening event(s) (n = 14), recurrent chest infections (n = 5), and failure to wean invasive ventilation (n = 2). Thoracoscopic aortopexies (n = 20) with conversion to open (n = 1) were performed. Intraoperative bleeding (n = 2) occurred, and chest tube (n = 1) was inserted for monitoring. Intraoperative bronchoscopy (n = 17) confirmed improvement of tracheomalacia. Anesthetic time was 140 (90-160) minutes. Postoperatively, 2 patients had dehiscence of the aorta from the sternum. They underwent redo open aortopexy with posterior tracheopexy, and 1 required subsequent tracheostomy. Another 2 patients required tracheostomies. Potential risk factors for subsequent tracheostomy were investigated, and only the association of tracheobronchomalacia was close to significance (OR 16 (95% CI 0.95-267.03), p = 0.05). Follow up duration was 365 (72-854) days. Symptoms resolution occurred in n = 17 (81%) of patients., Conclusion: Different modalities were used to delineate the site of tracheobronchomalacia and its etiology. Tracheomalacia with bronchial involvement may be a risk factor for subsequent tracheostomy., Level of Evidence: Level 3 (Case Series)., (Copyright © 2019. Published by Elsevier Inc.)

Background: Surgical management of Hirschsprung disease (HD) involves fully excising the transition zone (TZ). The literature suggests that resection of ≥5 cm of ganglionic bowel proximal to the aganglionic segment is sufficient. Our primary aim was to evaluate the lengths of the TZ in a cohort of consecutive patients with HD. We reviewed the impact this had on the need for revision surgery. We hypothesized that the TZ can be highly variable and may lead to a TZ pull-through when the proximal donut is not reviewed intraoperatively., Methods: A retrospective review was conducted for all patients undergoing primary pull-through surgery between January 2012 and September 2018. Data was collected on demographics, need for staged surgery, and complications following surgery., Results: Forty-eight patients were eligible for inclusion. 11/48 (23%) patients presented late (>6 months). 27/48 (56%) patients needed a stoma prior to definitive surgery. The median age at pull-through was 6 months (1-84 months). The median TZ length was 1.7 cm (0.3-22.9 cm). 11/48 (23%) had a TZ >5 cm. 36/48 (75%) patients did not have intraoperative review of the donut resulting in three TZ pull-throughs., Conclusions: We would advocate circumferential intraoperative frozen section review of the proximal donut to minimize the risk of a TZ pull-through., Level of Evidence: Level III., (Copyright © 2019. Published by Elsevier Inc.)

Aim: Perineal hemangiomas rarely occur in patients with anorectal malformations (ARMs), but they can pose a significant challenge and warrant special attention. Surgical incision of posterior sagittal anorectoplasty (PSARP) may involve the hemangioma site resulting in hemorrhage, damage to blood supply, leading to complications and adversely affecting outcome. The aim of this study was to review the experience of the ARM-Net Consortium in the management of perineal hemangioma associated with ARM and evaluate treatment strategies., Materials and Methods: Data on all patients with ARM and a perineal hemangioma located in the planes of the PSARP dissection who were managed at participating ARM-Net centers were collected retrospectively by questionnaire, as follows: ARM type, hemangioma distribution and penetration, imaging findings, medical/surgical management, timing of definitive repair, complications and outcome., Results: Ten patients from eight centers were included. Three patients each had a rectobulbar or rectovestibular fistula, 2 had a rectoperineal fistula, and one had a rectoprostatic fistula; in one patient, the hemangioma was too disfiguring to determine malformation type. Mean follow-up time was 36.6 months (median 29 months). Colostomies were performed before definitive repair in 8 patients. Five patients received systemic beta-blockers before PSARP: 3 were operated uneventfully following partial/complete involution of the hemangioma, and 2 are awaiting surgery. The two patients with rectoperineal fistula were managed expectantly. The remaining 3 patients underwent surgery with no preoperative medical treatment, and all had complications: mislocated neoanus in three and complete perineal dehiscence in one., Conclusion: Attempting PSARP in the presence of a perineal hemangioma may lead to complications and adversely affect outcome. This study confirms the benefits of beta blocker treatment before surgical reconstruction., Level of Evidence: Treatment study, level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: The impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown., Methods: An ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered., Results: Overall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%). Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation., Conclusions: Differences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions., Type of Study: Treatment study., Level of Evidence: III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2018. Published by Elsevier Inc.)

Aim: We investigated the natural history of fetal ovarian cysts to estimate the risk of torsion according to size., Methods: Cases were identified from 1/1/2000 until 1/1/2015. Data were collected pre- and postnatally on cyst size and sonographic features until an outcome of surgery, torsion, or resolution. Fisher's exact test for categorical data and logistic regression for continuous data were used to test the significance of size on torsion; P value <0.05 was considered significant., Results: 37 patients with unilateral ovarian cysts were included. 12 (32%) resolved spontaneously prenatally, 14 (38%) resolved spontaneously postnatally, 5 (14%) underwent surgery postnatally and 6 (16%) cases underwent torsion. Rate of torsion increased with size from 0% (n=0) in cysts ≤20mm to 33% (n=2) in cysts >50mm; however, the overall trend failed to reach statistical significance (P=0.1). Cysts of 0-40mm had a significantly higher rate of spontaneous resolution (90% vs. 44% in >40mm, P=0.003), but the rate of torsion was not significantly different (10% in 0-40mm vs. 25% in >40mm, P=0.26). The median time to postnatal resolution was 10 (5-27) weeks in those treated conservatively., Conclusion: Cysts >40mm are significantly less likely to resolve spontaneously; however torsion showed no significant correlation with cyst size. No complications were observed in cysts <20mm., Level of Evidence: IV, case series with no comparison group., (Copyright © 2018. Published by Elsevier Inc.)

Objectives: To develop the first consensus to standardize the management of patients with Anorectal Malformations (ARMs) transitioning from childhood to adulthood., Methods: A dedicated task force of experts performed an extensive literature review and multiple meetings to define the most important aspects of transition of care. The findings were discussed with all ARM-net consortium members and a set of practical recommendations agreed upon at the annual meeting in 2016., Result: We defined seven domains that are essential to provide an effective and practical transition process. Within each domain we have developed a set of key recommendations that are important to be considered for ARM patients entering the age of transition., Conclusions: It is crucial that transition begins at an early age with regular and well-structured follow-up. Cooperation with a selected multidisciplinary team of pediatric and adult practitioners is required to prepare patients and families for effective transition to adult care and to reduce long term morbidity., Type of Study: Review/Consensus paper., Level of Evidence: III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Introduction: The aim of this study was to investigate readmissions within 30days of operation (ReAd) in the setting of a tertiary pediatric surgical practice in the UK., Methods: Using Hospital Episode Statistics, cases that were readmitted within 30days of primary operation were identified retrospectively. Demographics including age, gender, preexisting comorbidities, diagnosis on primary admission and the treatment, length of stay, and diagnosis on readmission with treatment, including further surgical intervention, were collected from discharge summaries and hospital notes. Neonates were excluded from this study. Comorbidities, involving one or more systems, were also identified for each case of readmission. ReAds were classified into emergency and elective cohort depending on the nature of the primary operation. Outcomes were compared between these two groups. Data were quoted as median (range) unless indicated otherwise. Data were analyzed using SPSS software Desktop 22.0, using Mann-Whitney U and Chi-Squared tests, with a consideration that a P≤0.05 was significant., Results: A total of 2378 procedures were performed during the study period. Elective cases, including day cases, accounted for 77% (n=1837) of all cases. The remaining 23% (n=541) were emergency cases. Total unplanned readmission rate within 30days (ReAd) was 2%. Further surgical procedures were required in 38%. Having excluded neonates, the most common primary procedure leading to readmission within 30days was appendicectomy (26%). Overall, the most common cause for readmission within 30days was postoperative infection (30%). The ReAd in emergency cohort was 3.5% in comparison to 1.5% in elective, which was significantly different (P value=0.007)., Conclusion: Readmission within thirty days of primary procedure in pediatric surgery has little published data. An efficient discharge planning may play a vital role in preventing unwanted readmission. Elective operations had a significantly lower readmission rate than emergency operations. Having excluded neonates, appendicectomy was found to be the most common operation associated with readmission in the pediatric surgical practice. Although widely used as quality care indicator in adults, more studies are required to validate readmission rate as a quality of care indicator in pediatric surgery practice., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: We aimed to define characteristics and needs of Facebook users in relation to congenital anomalies., Methods: Cross-sectional analysis of Facebook related to four congenital anomalies: anorectal malformation (ARM), congenital diaphragmatic hernia (CDH), congenital heart disease (CHD) and hypospadias/epispadias (HS/ES). A keyword search was performed to identify relevant Groups/Pages. An anonymous survey was posted to obtain quantitative/qualitative data on users and their healthcare needs., Results: 54 Groups and 24 Pages were identified (ARM: 10 Groups; CDH: 9 Groups, 7 Pages; CHD: 32 Groups, 17 Pages; HS/ES: 3 Groups), with 16,191 Group members and 48,766 Page likes. 868/1103 (79%) of respondents were parents. Male:female ratio was 1:10.9. 65% of the users were 26-40years old. Common reasons for joining these Groups/Pages included: seeking support, education, making friends, and providing support to others. 932/1103 (84%) would like healthcare professionals (HCPs) to actively participate in their Group. 31% of the respondents felt that they did not receive enough support from their healthcare system. 97% of the respondents would like to join a Group linked to their primary hospital., Conclusions: Facebook Groups/Pages related to congenital anomalies are highly populated and active. There is a need for HCPs and policy makers to better understand and participate in social media to support families and improve patient care., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The aim of the study was to assess current international practice in the long term follow-up, in managing active problems and transition of care for teenagers born with anorectal malformations (ARM)., Methods: An original survey was administered to delegates attending two large colorectal surgical meetings in 2015. The 21 questions covered long term follow-up, specific issues for teenagers and transition of care., Results: 96/236 delegates completed the survey. Follow-up was routinely suspended before 10 year of age by 33% of respondents. 90% of them did not use a scoring system to assess or risks stratify patients, despite 81% stating that an objective score would be beneficial. 40% of respondents felt that >30% of their teenagers had ongoing active medical or psychosocial issues. 42% thought their patients were not ready to be transitioned. The process of transition should start around 13-16 years according to 54% of respondents. 72% had no protocol for transition and 82% did not hold multidisciplinary meetings with adult practitioners before transition., Conclusions: International consensus on the following aspects of the care in ARM is needed: structured long term follow-up, objective assessment and risk stratification scores, pathways of transition and methods to prepare patients, parents and adult practitioners., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Idiopathic intussusception is one of the most common causes of small bowel obstruction in children. In the event of failed radiological reduction, laparotomy remains the treatment of choice. There is still no agreement in pediatric surgery about safety and effectiveness of the use of minimally invasive surgery in this common pediatric condition. By reviewing available data we aimed to establish whether laparoscopy should be the primary technique in the surgical reduction of intussusception., Methods: A systematic review of all publications on the laparoscopic treatment of pediatric intussusception from January 1990 to April 2012 was performed. The following variables were analyzed: age, laparoscopic success rate, reason for conversion, enterotomy rate, operative time, complications, and length of stay (LOS)., Results: Ten retrospective studies treating 276 cases of laparoscopically reduced intussusception were identified. A total of 80 conversions corresponded to a 71.0% laparoscopic success rate. Only one case of intraoperative iatrogenic intestinal perforation was reported (0.4%). Postoperative complications occurred in 8 patients (2.9%), and adhesive small bowel obstruction was reported in 1 case (0.4%). Recurrence rate after laparoscopy was 3.6%. Three of 10 papers compared results between laparoscopic and open reduction of intussusception showing a shorter mean LOS in the former group (4.0 vs. 7.1 days, p<0.01)., Conclusion: Laparoscopy is safe and effective in the treatment of pediatric intussusception. Tertiary centers with adequate minimally invasive skills should establish laparoscopy as the primary surgical technique in the treatment of this condition., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background/objective: Renal failure remains one of the most significant causes of morbidity in patients with anorectal malformations (ARM). In the modern era, an increasing number of children born with ARM and genito-urinary (GU) anomalies reach adulthood and require continued multidisciplinary care for the rest of their life. The aim of this study is to present our institutional experience in the management of pediatric chronic renal failure related to severe GU anomalies and anorectal malformations., Methods and Results: Three hundred twenty-one patients with ARM have been followed at our institution since 1987. Six patients developed end-stage renal disease (ESRD) and received a kidney transplant at different ages. One patient is currently followed for mild, progressive chronic renal failure. These seven cases are reported along with a broad discussion concerning etiology of renal failure, neonatal surgical management, pediatric dialysis, urologic issues, and kidney transplantation., Conclusion: Complex GU anomalies associated with ARM require a long-term approach by specialized pediatric and adult clinicians to optimize the care of this selected population of patients., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Prune belly syndrome (PBS), megacystis-microcolon-intestinal hypoperistalsis (MMIH), and omphalocele-exstrophy of the bladder-imperforate anus-spine abnormalities complex (OEIS) are rare congenital malformations of the newborn that lead to incomplete formation of the gastrointestinal and genitourinary tract systems. To date, incomplete mesodermal development is identified as the cause for all these complex genetic syndromes even if the etiology is still unknown. We present an original case sharing characteristics common to PBS, MMIH, and OEIS complex, without a clear inclination toward any particular one. This case hints toward a common pathway in the creation of the 3 syndromes. We hypothesize that they are a spectrum of malformations based on the time frame when the mesoderm fails to create a normal interaction between infraumbilical mesoderm, urorectal septum, lumbosacral somites in the formation of the abdominal wall and the genitourinary and lower gastrointestinal tracts., (Published by Elsevier Inc.)

Massive subcutaneous emphysema (SE), pneumomediastinum (PM), and pneumopericardium (PP) are rare conditions in the pediatric population. Air leak syndrome is a constellation of disorders that include SE, PM, PP, and pulmonary interstitial emphysema. In children, SE, PM, and PP are associated with obstructive airway disease most often in the case of asthma. Management may be conservative or involve invasive procedures that require surgical intervention. Here, we describe a case of massive SE, PM, and PP in a 10-year-old child after placement of a peripherally inserted central line and review the literature.