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Objectives: Laryngeal Cleft (LC) is an anatomical defect that can cause swallowing difficulties and subsequent recurrent respiratory symptoms. LC can be treated surgically by performing suture repair or by Injection Laryngoplasty (IL). The indications and efficacy of IL are debated among pediatric otolaryngologists. The aim of this survey study was to review the international perspective on IL for LC., Methods: An online survey was conducted to assess international opinions on the indications and efficacy of IL for LC patients. An online survey was sent to 250 pediatric otolaryngologists worldwide. The survey included questions on the management of LC, the physicians' experience with IL, and their use of IL., Results: Sixty two (25 %) pediatric otolaryngologists, from 47 medical centers, completed the survey. Of the respondents, 38 (62 %) perform IL. The most reported indication was as a diagnostic tool. For the respondents who did not perform IL, the reasons most reported were that the effect is temporary and that there is a need for a second surgery after IL., Conclusion: According to this survey, the perspectives on the use of IL for LC differ among pediatric otolaryngologists, and there is variation in peri-and postoperative standard of care. The respondents' opinions on IL were partly dependent on the intended outcome of its use, i.e., as a permanent treatment or for other indications., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier B.V.)

Purpose: Laryngeal cleft (LC) is an anatomical defect of the larynx, where there is a gap (or cleft) between the arytenoids. Milder types can be treated with injection laryngoplasty (IL), involving injection with a filler, resulting in a decreased depth of the cleft and thereby reducing tracheal penetration or aspiration. The effect, however, is temporary. The aim of this study was to investigate the possible indications and the efficacy of IL for LC., Methods: Patients who underwent IL for LC between March 2018 and June 2023 were retrospectively evaluated. The following parameters were studied: incidence of LC symptoms and objective swallowing evaluations before and after IL, the duration of possible symptom improvement, complications, and the number of subsequent suture repairs., Results: Eighty-five patients were included. Before IL, 81 (96 %) patients had symptoms of aspiration during feeding, compared to 41 (54 %) patients after IL (p ≤ 0.001). In 42 (49 %) patients, temporary symptom relief occurred, in 22 (26 %) patients symptoms persisted, in 16 (19 %) patients symptoms decreased permanently. Mild complications such as cough and desaturations in the direct postoperative period occurred., Conclusion: This study shows a statistically significant decrease in the number of parents/caretakers reporting swallowing symptoms after injection laryngoplasty, and a decrease in the average percentage of parents/caretakers reporting various other symptoms. Based on our results, injection laryngoplasty can be recommended as a diagnostic tool in the treatment of laryngeal cleft. Furthermore, it can be used as bridge therapy (i.e. until patients outgrow their symptoms, or until suture repair)., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier Inc.)

Background: Although ICG-FA may be valuable in assessing anastomotic perfusion, reliable data on its use in pediatric gastrointestinal surgery is lacking. This systematic review analyzes whether ICG is useful for intestinal perfusion assessment in pediatric gastrointestinal surgery and safe to use in neonates., Methods: Systematic searches of PubMed, EMBASE & MEDLINE and CENTRAL were performed (last conducted December 6, 2021). The main inclusion criteria were (1) use of ICG for intestinal perfusion assessment and (2) use of ICG in young infants. Exclusion criteria were lack of an English or Dutch full-text and MINORS quality score <60%. Data was presented in overview tables. The usefulness in pediatric gastrointestinal surgery was assessed by surgical outcome. Safety of ICG in neonates was assessed by complication or adverse event occurrence., Results: Regarding intestinal perfusion assessment, four studies were included, reporting 45 patients (median age 1.5 years). ICG was considered useful for anastomotic blood flow evaluation and intraoperative determination of resection length. Regarding ICG safety in neonates, eight studies were included, reporting 46 infants (median age 24.9 days), of which 18 neonates. All but one studies reported the absence of complications or adverse events. Two studies reported subcutaneous dye retention, which fully disappeared within two weeks., Conclusion: Although the number of available studies is small, ICG might be useful for intraoperative intestinal perfusion assessment, perhaps even more than conventional clinical assessment. Furthermore, its safety profile looks promising in neonates. Larger prospective studies are necessary to confirm these assumptions and seem warranted given the safety profile., Levels of Evidence: Since this is a systematic review, a Level of Evidence for clinical studies cannot be determined for this manuscript., Competing Interests: Declaration of Competing Interest One of the authors is a representative at Stryker Endoscopy., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

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.

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.

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., (Copyright © 2021. Published by Elsevier Inc.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 van Tuyll van Serooskerken, Tytgat, Verweij, Bittermann, Coenraad, Arets, van der Zee and Lindeboom.)

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., Competing Interests: None declared., (Thieme. All rights reserved.)

Purpose: Assessing quality of life (QoL) after esophageal replacement (ER) for long gap esophageal atresia (LGEA)., Methods: All patients after ER for LGEA with gastric pull-up (GPU n = 9) or jejunum interposition (JI n = 14) at the University Medical Center Groningen and Utrecht (1985-2007) were included. QoL was assessed with 1) gastrointestinal-related QoL using the Gastrointestinal Quality of Life Index (GIQLI)), 2) general QoL (Child Health questionnaire CHF87-BREF (children)/World Health Organization questionnaire WHOQOL-BREF (adults)), and 3) health-related QoL (HRQoL) (TNO AZL TACQoL/TAAQoL). Association of morbidity (heartburn, dysphagia, dyspnea on exertion, recurrent cough) and (HR)QoL was evaluated., Results: Six patients after GPU (75%) and eight patients after JI (57%) responded to the questionnaires (mean age 15.7, SD 5.9, 12 male, two female). Mean gastrointestinal, general and health-related QoL total scores of the patients were comparable to healthy controls. However, young adults reported a worse physical functioning (p = 0.02) but better social functioning compared to peers (p = 0.01). Morbidity was not associated with significant differences in (HR)QoL., Conclusions: With the current validated QoL most patients after ER with GPU and JI for LGEA have normal generic and disease specific QoL scores. Postoperative morbidity does not seem to influence (HR)QoL., Type of Study: Prognosis Study., Level of Evidence: III., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

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.

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.

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., (Copyright © 2019. Published by Elsevier Inc.)

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

A 14-year-old girl with anorexia nervosa was referred to our paediatric hospital. She had a five-day history of severe abdominal pain. On abdominal sonography and MRI a duodenal wall hematoma was seen, correlating anatomically to abdominal bruises found on physical examination. The girl admitted to self-injury related to her eating disorder.