Graft dilatation and Barrett's esophagus in adults after gastric pull-up and jejunal interposition for long-gap esophageal atresia.

BACKGROUND: Esophageal replacement (ER) with gastric pull-up (GPU) or jejunal interposition (JI) used to be the standard treatment for long-gap esophageal atresia (LGEA). Changes of the ER grafts on a macro- and microscopic level however, are unknown. AIM: To evaluate long-term clinical symptoms and anatomical and mucosal changes in adolescents and adults after ER for LGEA. METHODS: A cohort study was conducted including all LGEA patients ≥ 16 years who had undergone GPU or JI between 1985-2003 at two tertiary referral centers in the Netherlands. Patients underwent clinical assessment, contrast study and endoscopy with biopsy. Data was collected prospectively. Group differences between JI and GPU patients, and associations between different outcome measures were assessed using the Fisher's exact test for bivariate variables and the Mann-Whitney U-test for continuous variables. Differences with a P-value < 0.05 were considered statistically significant. RESULTS: Nine GPU patients and eleven JI patients were included. Median age at follow-up was 21.5 years and 24.4 years, respectively. Reflux was reported in six GPU patients (67%) vs four JI patients (36%) (P = 0.37). Dysphagia symptoms were reported in 64% of JI patients, compared to 22% of GPU patients (P = 0.09). Contrast studies showed dilatation of the jejunal graft in six patients (55%) and graft lengthening in four of these six patients. Endoscopy revealed columnar-lined esophagus in three GPU patients (33%) and intestinal metaplasia was histologically confirmed in two patients (22%). No association was found between reflux symptoms and macroscopic anomalies or intestinal metaplasia. Three GPU patients (33%) experienced severe feeding problems vs none in the JI group. The median body mass index of JI patients was 20.9 kg/m2 vs 19.5 kg/m2 in GPU patients (P = 0.08). CONCLUSION: The majority of GPU patients had reflux and intestinal metaplasia in 22%. The majority of JI patients had dysphagia and a dilated graft. Follow-up after ER for LGEA is essential.

Airway Epithelial Cultures of Children with Esophageal Atresia as a Model to Study Respiratory Tract Disorders.

Esophageal atresia (EA) is a rare birth defect in which respiratory tract disorders are a major cause of morbidity. It remains unclear whether respiratory tract disorders are in part caused by alterations in airway epithelial cell functions such as the activity of motile cilia. This can be studied using airway epithelial cell culture models of patients with EA. Therefore, the aim of this study was to evaluate the feasibility to culture and functionally characterize motile cilia function in the differentiated air-liquid interface cultured airway epithelial cells and 3D organoids derived from nasal brushings and bronchoalveolar lavage (BAL) fluid from children with EA. We demonstrate the feasibility of culturing differentiated airway epithelia and organoids of nasal brushings and BAL fluid of children with EA, which display normal motile cilia function. EA patient-derived airway epithelial cultures can be further used to examine whether alterations in epithelial functions contribute to respiratory disorders in EA.

Bacterial Colonization of the Lower Airways in Children With Esophageal Atresia.

BACKGROUND: Esophageal atresia (EA) is most often accompanied by some degree of tracheomalacia (TM), which negatively influences the airway by ineffective clearance of secretions. This can lead to lower airway bacterial colonization (LABC), which may cause recurrent respiratory tract infections (RTIs). This study aims to evaluate the prevalence and specific pathogens of LABC in EA patients. METHODS: A 5-year retrospective single-site cohort study was conducted including all EA patients that had undergone an intraoperative bronchoalveolar lavage (BAL) during various routine surgical interventions. Concentrations of greater than 10 cfu were considered evidence of LABC. RESULTS: We recruited 68 EA patients, of which 12 were excluded based on the exclusion criteria. In the remaining 56 patients, a total of 90 BAL samples were obtained. In 57% of the patients, at least 1 BAL sample was positive for LABC. Respiratory symptoms were reported in 21 patients at the time of the BAL, of which 10 (48%) had LABC. Haemophilus influenzae (14%) and Staphylococcus aureus (16%) were most frequently found in the BAL samples. The number of respiratory tract infections and the existence of a recurrent fistula were significantly associated with LABC ( P = 0.008 and P = 0.04, respectively). CONCLUSIONS: This is the first study showing that patients with EA have a high prevalence of bacterial colonization of the lower airways which may be a leading mechanism of severe and recurrent respiratory complications.

Primary Posterior Tracheopexy in Esophageal Atresia Decreases Respiratory Tract Infections.

Background: Esophageal atresia (EA) is often accompanied by tracheomalacia (TM). TM can lead to severe respiratory complaints requiring invasive treatment. This study aims to evaluate if thoracoscopic primary posterior tracheopexy (PPT) can prevent the potential sequelae of TM in patients with EA. Methods: A cohort study including all consecutive EA patients treated between 2014 and July 2019 at the Wilhelmina Children's Hospital was conducted. Two groups were distinguished: (group 1) all EA patients born between January 2014 and December 2016 and (group 2) all EA patients born between January 2017 and July 2019, after introduction of PPT. In the latter group, PPT was performed in EA patients with moderate (33-66%) or severe (67-100%) tracheomalacia, seen during preoperative bronchoscopy. Group differences were assessed using the Fisher's exact test for bivariate variables and the Mann-Whitney U-test for continuous variables. Results: A total of 64 patients were included in this study (28 patients in group 1; 36 patients in group 2). In group 2, PPT was performed in 14 patients. Respiratory tract infections (RTIs) requiring antibiotics within the first year of life occurred significantly less in group 2 (61 vs. 25%, p = 0.004). Brief resolved unexplained events (BRUEs) seemed to diminish in group 2 compared to group 1 (39 vs. 19%, p = 0.09). Conclusion: Thoracoscopic primary posterior tracheopexy decreases the number of respiratory tract infections in EA patients. The clinical impact of reducing RTIs combined with the minimal additional operating time and safety of PPT outweighs the risk of overtreatment.

Thoracoscopic Repair of Esophageal Atresia.

Background: Esophageal atresia (EA) is a rare congenital malformation of the esophagus. Surgical treatment is required to restore the continuity of the esophagus. This can be performed through thoracotomy. However, an increasing number of hospitals is performing minimal invasive surgery (MIS). In this article, we describe the technique of thoracoscopic repair of EA in neonates in more detail and show the outcome of a patient cohort operated by young pediatric surgeons in training. Methods: Between 2014 and 2019 correction was performed in 64 EA type C patients at the UMC Utrecht, Wilhelmina Children's Hospital, mainly by young pediatric surgeons in training. Results: All patients were corrected through MIS, 3 days after birth. The median operation duration was 181 (127-334) minutes. Nasogastric tube feeding was started on the first postoperative day, and oral feeding 6 days postop. Postoperative complications included leakage (14.1%), stenosis (51.1%), and recurrent tracheoesophageal fistula (7.8%). Conclusion: Thoracoscopic repair of EA can be performed safely, with good outcome and all the benefits of MIS. However, it remains a challenging procedure and should be performed only in pediatric centers with a vast experience in MIS, especially when training young pediatric surgeons. These centers must have access to a multidisciplinary team of neonatologists, pediatric anesthesiologists, surgeons, and ENT specialists to ensure the best possible care in hemodynamic, respiratory, and cerebral monitoring and gastrointestinal and developmental outcome.

Childhood outcome after correction of long-gap esophageal atresia by thoracoscopic external traction technique.

BACKGROUND: Thoracoscopic external traction technique (TTT) is a relatively new surgical intervention for patients with long-gap esophageal atresia (LGEA) that preserves the native esophagus. The major accomplishment with TTT is that esophageal repair can be achieved within days after birth. This study evaluates the childhood outcome in LGEA patients treated with TTT, including gastrointestinal outcome, nutritional status and Health-Related Quality of Life (HRQoL). METHODS: A cohort study including all LGEA patients that underwent TTT between 2006-2017 was conducted. Patients and/or their parents were invited to fill out questionnaires regarding reflux symptoms and HRQoL. RESULTS: TTT was successful in 11/13 patients (85%). Esophageal anastomosis was accomplished at a median age of 12 days (range 7-138), first oral feeding was started at a median of 16 days postoperatively (range 5-37). All patients required multiple dilatations and 10 patients required anti-reflux surgery. At median follow-up of seven years, five patients reported mild and one moderate reflux complaints. All patients but one reached age-appropriate oral diet. Most patients (80%) were within normal growth range. Overall HRQoL was comparable to healthy controls. CONCLUSION: TTT provides acceptable results in childhood. Oral feeding can be started as soon as two weeks postoperatively. Almost all patients are able to eat an age-appropriate oral diet. Overall HRQoL was comparable to healthy controls.

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.

Five-Year Outcome of Laparoscopic Fundoplication in Pediatric GERD Patients: a Multicenter, Prospective Cohort Study.

BACKGROUND: Gastroesophageal reflux disease (GERD) is a common disease in children. When drug treatment fails, laparoscopic anti-reflux surgery (LARS) is considered. Short-term follow-up studies report high success rates; however, few studies report long-term results. The aim of this study was to describe the long-term effects of LARS in pediatric patients. METHODS: A prospective, multicenter study of 25 laparoscopic fundoplication patients was performed. At 3 months and 1, 2, and 5 years postoperatively, patients and caregivers were asked to complete the gastroesophageal reflux symptom questionnaire to assess symptoms and the PedsQL™ to assess health-related quality of life (HRQoL). RESULTS: Reflux symptom severity was still significantly improved 5 years after LARS compared with preoperative levels (p < 0.0001). However, 26% of patients reported moderate or severe reflux symptoms. Dysphagia was reported in 13% of patients 5 years after LARS and was more common in children with neurologic impairment and children who underwent a Nissen procedure. The increase in HRQoL 3 months postoperatively appears to decline over time: 5 years after surgery, HRQoL was lower, though not significantly, than 3 months postoperatively. HRQoL at 5 years was still higher, though also not significantly, than preoperative levels. The presence of reflux symptoms after surgery was not significantly associated with lower HRQoL. CONCLUSIONS: LARS is effective for therapy-resistant GERD in children. Five years after surgery, reflux symptoms are still improved. However, we observed a decline in symptom-free patients over time. The initial increase in HRQoL shortly after LARS appears to decline over time. TRIAL REGISTRATION: Dutch national trial registry Identifier: 2934 ( www.trialregister.nl ).

Two-Year Outcome after Laparoscopic Fundoplication in Pediatric Patients with Gastroesophageal Reflux Disease.

Introduction: Many studies on short-term efficacy of laparoscopic antireflux surgery (LARS) have shown good to excellent results on reflux symptom control and health-related quality of life (HRQoL). Prospective studies on the long-term efficacy, however, are scarce and indicate that the efficacy of symptom control may decline over time. The aim of this study is to assess the 2-year outcome on reflux symptoms and HRQoL after LARS. Materials and Methods: Between 2011 and 2013, 25 children (12 males, median age 6 [2-18] years) with proton pump inhibitor-resistant gastroesophageal reflux disease were included in a prospective longitudinal cohort study. To assess reflux symptoms and HRQoL, patients and/or their caregivers were asked to fill out the validated age-appropriate gastroesophageal reflux symptom questionnaire and Pediatric Quality of Life Inventory™ before, 3 months, 1 year, and 2 years after LARS. Results: Two years after LARS, 29% of patients had moderate to severe reflux symptoms compared with 92% (P < .001) before operation and 12% 3-4 months after operation (P = .219). The significant increase in HRQoL shortly after fundoplication (80.0 compared with 69.5 (P = .004)) is not observed after 2 years (72.0 compared with 69.5, P = .312). Correlation between the impaired HRQoL scores and the recurrence of symptoms could not be verified. Conclusions: Although the efficacy of LARS tends to deteriorate after 2 years, LARS is still effective in controlling reflux symptoms in the majority of patients. The short-term improvement in HRQoL after LARS appears to be transient.

Gastro-esophageal Reflux After Laparoscopic Gastrostomy Placement in Children.

OBJECTIVES: Gastrostomy placement is frequently performed in pediatric patients who require long-term enteral tube feeding. Evidence on the influence of gastrostomy placement on gastro-oesophageal reflux disease has been inconsistent. The aim of this study was to investigate the influence of gastrostomy on gastro-oesophageal reflux. METHODS: A prospective, longitudinal cohort study was performed including 50 patients who underwent laparoscopic gastrostomy between May 2012 and April 2014. Before and 3 months after surgery 24-hour multichannel intraluminal impedance pH monitoring was performed and caregivers filled out reflux symptom questionnaires. RESULTS: Gastro-oesophageal reflux symptoms were present in a comparable number of patients before (44%) and after gastrostomy placement (40%; P = 0.73). Twenty-five of the patients (50%) underwent both the preoperative and postoperative tests and were included in impedance-pH analysis. Acid exposure time (percentage of time with pH below 4) did not change significantly after gastrostomy placement: from 6.2% (3.0-18.1) to 6.1% (2.6-14.9). The number of reflux episodes did not significantly change, for either liquid [mean difference 4.3 (-4.5 to 13.2)] or mixed liquid-gas reflux [mean difference 2.0 (-9.3 to 13.3)]. Before gastrostomy placement, 18 out of 25 patients had pathological reflux (72%) on pH-impedance measurement. In 4 patients, pathological reflux dissolved, whereas 4 patients newly developed pathological reflux. A low preoperative weight-for-height percentile was associated with increased acid exposure after gastrostomy placement. CONCLUSIONS: Overall, gastrostomy placement was not associated with an increase in acid exposure on 24-hour multichannel intraluminal impedance pH monitoring. Similarly, the prevalence of gastro-oesophageal reflux-related symptoms did not change after gastrostomy.

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.

Health-related quality of life in children after laparoscopic gastrostomy placement.

INTRODUCTION: A gastrostomy placement (GP) is an established treatment to provide enteral feeding in pediatric patients with feeding difficulties aiming to improve nutritional status and health-related quality of life (HRQoL). The aim of this study was to evaluate HRQoL in children with severe feeding difficulties who have undergone GP. MATERIALS AND METHODS: A cross-sectional study was performed including 128 patients who had undergone laparoscopic GP (2004-2011). HRQoL was evaluated using the validated Pediatric Quality of Life 4.0 Inventory. Multiple regression analysis was performed to identify predictors of HRQoL. RESULTS: After a mean follow-up of 4.0 years (interquartile range 2.9-6.2) after GP, mean HRQoL was 53.0 out of 100 (standard deviation 21.1). HRQoL was significantly lower in children with neurologic impairment, with a mean difference of -21.4 points between neurologically impaired and neurologically normal children (p < 0.001). HRQoL was also lower in children with cardiac disease (-19.0 points; p = 0.01) and in children with a history of previous gastrointestinal surgery (-15.2 points; p = 0.03). Feeding through a gastrojejunostomy tube (-33.0 points; p = 0.01) and higher age at the time of operation (-1.2 points per year; p = 0.03) were also associated with lower HRQoL. GP-related complications requiring reintervention were associated with lower HRQoL, although this association was not statistically significant (p = 0.06). CONCLUSIONS: Children with severe feeding difficulty, who have undergone GP, have significantly lower HRQoL compared to a healthy pediatric population. Neurologic impairment, cardiac disease, a history of gastrointestinal surgery, older age, and the need for jejunal feeding through the gastrostomy were predictive of even lower HRQoL.

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.

The Effect of Gastrostomy Placement on Health-Related Quality of Life in Children.

BACKGROUND AND PURPOSE: A gastrostomy placement (GP) aims to improve nutritional status and health-related quality of life (HRQoL) in children who require long-term enteral tube feeding. We evaluated the effect of GP on HRQoL. METHODS: A prospective, longitudinal cohort study was performed including patients referred for laparoscopic GP. Children and/or caregivers were asked to fill out the validated PedsQL™ questionnaire before and 3 months after surgery. The aim was to compare preoperative with postoperative HRQoL and to identify predictors of HRQoL. RESULTS: Fifty patients were included with a median age of 3.4 years (interquartile range 1.4-5.6). After GP, total HRQoL did not significantly increase (p = 0.30). However, psychosocial health significantly increased: 55.8 (standard deviation ±20.8) to 61.2 (±19.6; p = 0.03) on a 100-point scale. This was mainly owing to an increase in social HRQoL: 58.2 (±32.3) to 68.3 (±27.9; p = 0.04). HRQoL both before and after GP was significantly lower in children with neurologic impairment (p < 0.0005). However, neurologic impairment did not influence the effect of surgery on HRQoL (p = 0.66). Low preoperative body mass index was a predictor for improvement in HRQoL after GP. CONCLUSIONS: After GP in children, psychosocial HRQoL improved significantly. This was mainly owing to an improvement in social HRQoL. LEVEL OF EVIDENCE: IV.

Error traps and culture of safety in esophageal atresia repair.

Esophageal atresia (EA) repair has always been a source of immense professional gratification for the pediatric surgeon. In many ways, this anomaly defines the entire profession. Due to its rarity, there is an increased risk of inadvertent events occurring during correction. This article describes some of the error traps that may occur in attempting esophageal reconstruction and how they may be avoided.

Thoracoscopic posterior tracheopexy during primary esophageal atresia repair: a new approach to prevent tracheomalacia complications.

BACKGROUND: Esophageal atresia (EA) is usually accompanied by some form of tracheomalacia (TM). During the early phases in life, excessive dynamic collapse of the trachea can cause a wide spectrum of symptoms ranging from mild complaints to apparent life-threatening events (ALTE's) or brief resolved unexplained events (BRUE's). Therapeutic strategies for severe TM include aortopexy to lift the anterior weakened cartilaginous rings or posterior tracheopexy of the floppy membranous tracheal intrusion. In this study, we describe the development of a new approach in which the posterior tracheopexy is performed directly during the primary thoracoscopic correction of EA. METHODS: In 2017, all nine consecutive EA patients with trachea-esophageal fistula underwent a rigid tracheo-bronchoscopy (RTB) evaluation during induction of anesthesia prior to the thoracoscopic EA repair. A floppy posterior membrane was diagnosed in four patients. During the subsequent thoracoscopic procedure, the posterior membranous trachea was fixed to the anterior longitudinal spinal ligament with non-absorbable sutures. Then, the anastomosis was made between the two esophageal pouches. RESULTS: On preoperative RTB, two patients had a severe (70-90%) mid-tracheal collapse of the pars membranacea and two patients had a moderate (33-40%) mid-tracheal collapse. Thoracoscopic posterior tracheopexy with two or three sutures was possible in all four patients, prior to the formation of the esophageal anastomosis. Median time per suture was 6 min (range 4-12 min). All operative procedures were uneventful. A median follow-up of 6 months (range 4-9 months) revealed that all patients showed further recovery without any TM symptoms or ALTE/BRUE. CONCLUSIONS: This is the first report that introduces a new approach to thoracoscopic posterior tracheopexy during primary EA repair. We believe that this technique can prevent the potentially deleterious sequelae of mild to severe TM that may complicate the lives of EA patients. Also, a second, sometimes complex surgical procedure can be prevented as the posterior tracheopexy is performed during the primary thoracoscopic EA correction. LEVEL OF EVIDENCE: IV.

The incidence of associated abnormalities in patients with sacrococcygeal teratoma.

BACKGROUND: Gross genetic causes for SCT are unknown; however, it might be associated with other abnormalities. We assessed the incidence of associated abnormalities in a large national cohort of neonates with SCT and aimed to identify predictive risk factors. PROCEDURE: The medical records were reviewed of 235 consecutive neonates with SCT treated at the six pediatric surgical centers in the Netherlands from 1970 to 2010. Potential risk factors for associated abnormalities analyzed included sex, gestational age, tumor-volume/histology and Altman-classification. RESULTS: In 76 patients (32.3%) at least one associated abnormality was diagnosed, with hydronephrosis as the most common (16.2%) and hip dysplasia in 4.3%. Multiple abnormalities were documented for 21 (9.0%). Prematurity and Altman type IV SCT were associated with an increased risk of any associated abnormality. No association between increased tumor-volume and hydronephrosis or hip dysplasia was found. Patients with type IV Altman SCT had a fourfold risk of suffering from hydronephrosis compared to Altman type I SCT. CONCLUSIONS: SCT was associated with other abnormalities in one-third of children. Some were tumor-related while others were related to prematurity or occurred sporadically. In contrast to clinically obvious anomalies, hip dysplasia or hydronephrosis might be latently present with more subtle clinical presentation. We therefore suggest renal- and hip-ultrasound in all patients, certainly those with Altman type IV SCT. LEVEL OF EVIDENCE RATING: Level II (retrospective study).

Esophageal Atresia and Upper Airway Pathology.

Esophageal atresia is an anomaly with frequently occurring sequelae requiring lifelong management and follow-up. Because of the complex issues that can be encountered, patients with esophageal atresia preferably should be managed in centers of expertise that have the ability to deal with all types of anomalies and sequelae and can perform rigorous lifelong follow-up. Tracheomalacia is an often-occurring concurrent anomaly that may cause acute life-threatening events and may warrant immediate management. In the past, major thoracotomies were necessary to carry out the aortopexy. Nowadays, aortopexy and posterior tracheopexy can both be performed thoracoscopically with quick recovery.

Effect of general anesthesia on neonatal aEEG-A cohort study of patients with non-cardiac congenital anomalies.

INTRODUCTION: The aim of the current study was to determine the effect of general anesthesia on neonatal brain activity using amplitude-integrated EEG (aEEG). METHODS: A prospective cohort study of neonates (January 2013-December 2015), who underwent major neonatal surgery for non-cardiac congenital anomalies. Anesthesia was administered at the discretion of the anesthetist. aEEG monitoring was started six hours preoperatively until 24 hours after surgery. Analysis of classes of aEEG background patterns, ranging from continuous normal voltage to flat trace in six classes, and quantitative EEG-measures, using spontaneous activity transients (SATs) and interSATintervals (ISI), was performed. RESULTS: In total, 111 neonates were included (36 preterm/75 full-term), age at time of surgery was (median (range) 2 (0-32) days. During anesthesia depression of brain activity was seen, with background patterns ranging from flat trace to discontinuous normal voltage. In most patients brain activity was two background pattern classes lower during anesthesia. After cessation of anesthesia, recovery to preoperative brain activity occurred within 24 hours in 86% of the preterm and 96% of the term infants. Gestational age and the dose of sevoflurane were significantly associated with SAT-rate (F(2,68) = 9.288, p < 0.001) and ISI- durations during surgery (F(3,71) = 12.96, p < 0.001). Background pattern and quantitative EEG-values were not associated with brain lesions (χ2(4) = 2.086, ns). CONCLUSION: aEEG shows a variable reduction of brain activity in response to anesthesia in neonates with noncardiac congenital anomalies, with fast recovery after cessation of anesthesia. This reduction is related to gestational age and the dose of sevoflurane. The aEEG offers the opportunity to monitor the depth of anesthesia in the neonate.

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study.

INTRODUCTION: A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood. METHODS: All pediatric patients who underwent a surgical procedure for type I-IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation. Patients with type V CM were excluded from the present analysis. Symptoms, surgical details, short-term (<30 days) and long-term (>30 days) complications were studied retrospectively. RESULTS: Between January 1989 and December 2014, 91 pediatric patients underwent surgery for CM at a median age of 2.1 years (0.0-17.7 years). All patients underwent resection of the extrahepatic biliary tree with restoration of the continuity via Roux-en-Y hepaticojejunostomy. Twelve patients (12%) were operated laparoscopically. Short-term complications, mainly biliary leakage and cholangitis, occurred in 20 patients (22%), without significant correlations with weight or age at surgery or surgical approach. Long-term postoperative complications were mainly cholangitis (13%) and anastomotic stricture (4%). Eight patients (9%) required radiological intervention or additional surgery. Surgery before 1 year of age (OR 9.3) and laparoscopic surgery (OR 4.4) were associated with more postoperative long-term complications. We did not observe biliary malignancies during treatment or follow-up. CONCLUSION: Surgery for CM carries a significant short- and long-term morbidity. Given the low incidence, we would suggest that (laparoscopic) hepatobiliary surgery for CM should be performed in specialized pediatric surgical centers with a wide experience in laparoscopy and hepatobiliary surgery.