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Background Visual examination is crucial for assessing pectus carinatum (PC) severity and treatment results. This cross-sectional study evaluates the inter- and intraobserver agreement of PC deformities before and after treatment. Methods Observers examined medical photographs of patients before and after treatment. Primary outcome was inter- and intraobserver agreement on esthetic results after treatment. Secondary outcomes included inter- and intraobserver agreement on severity and symmetry before treatment, differences in esthetic results after Ravitch surgery and dynamic compression bracing (DCS-bracing), and the impact of scars, age and treatment duration on esthetic results. Results Medical photographs of 201 patients (aged 4-18) were evaluated by five surgeons and five peers. Surgeons and peers demonstrated inadequate (κ<.61) interobserver agreement on esthetic results (κ=.26, κ=.22), severity of PC (κ=.43, κ=.38) and symmetry (κ=.37, surgeons only). Agreement between surgeons and peers on esthetic results (κ=.37) and severity before treatment (κ=.54) was similarly inadequate. Surgeons and peers demonstrated inadequate intraobserver agreement on esthetic results (κ=.49, κ=.34), severity of PC (κ=.54, κ=.48) and symmetry (κ=.60, surgeons only). Deformities treated with Ravitch surgery were perceived as more severe but yielded better results. Peers, unlike surgeons, viewed scars as negatively impacting results. No relationship was found between results after treatment and treatment duration (p=.682, p=.062) or age ( p=.205, p=.527). Conclusions Subjective assessment of PC severity and esthetic results is inconsistent. 3D-scanning could help standardize treatment completion and aid patients and surgeons in determining treatment completion. The psychosocial effects of scars should be addressed when discussing treatment options., Competing Interests: The authors declare that they have no conflict of interest., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Background: Patients with pectus carinatum have lower quality of life and self-esteem than their peers. We assessed the impact of dynamic compression system bracing on quality of life in patients with pectus carinatum., Methods: We conducted a prospective cohort study on patients aged 10-21 years. We assessed quality of life using the Child Health Questionnaire-87, the State-Trait Anxiety Inventory-6, the World Health Organization Quality of Life-BREF, the 36-Item Short Form Survey, and the Single-Step Questionnaire adapted for pectus carinatum., Results: Between March 2013 and March 2016, 225 patients treated with dynamic compression system bracing were included. Patients showed improvements across the overall scores of the 36-Item Short Form Survey (Δ7.7 (2.9-12.4)), Single-Step Questionnaire (Δ4.1 (2.0-6.3)) and three out of four World Health Organization Quality of Life-BREF domains (physical health (Δ8.7 (3.7-13.7)), psychological health (Δ11.8 (6.1-17.5)), environment (Δ5.7 (0.2-11.3))). No changes across the Child Health Questionnaire-87 overall score were observed (Δ5.5 (-0.5-11.5)). Most improvement occurred within six to twelve months after treatment initiation, stabilizing thereafter. Anxiety scores on the State-Trait Anxiety Inventory-6 did not improve (Δ0.5 (-0.1-1.2)). Scores on physical complaints, pain, psychological health and self-esteem/self-image improved across all questionnaires. In contrast to the successfully treated group, the unsuccessfully treated group showed no improvement on any of the questionnaires. Most patients (87.2 %) would choose bracing again, 94.9 % of patients were satisfied with the treatment., Conclusions: Dynamic compression system bracing improves quality of life, reduces physical complaints and pain and boosts psychological health and self-esteem in patients with pectus carinatum., Competing Interests: Conflicts of interest Sjoerd A. de Beer, contracted by the company Atricure, works as a proctor for cryotherapy in pectus patients. The other authors have no relevant financial or non-financial interests to disclose., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Long-term results and factors affecting outcomes of vacuum bell therapy for pectus excavatum are relatively unknown., Methods: We conducted a retrospective study on patients (<18y) treated with vacuum bell therapy between May 2008 and October 2021. Primary outcome was treatment success; secondary outcomes were analysis of daily time spent on treatment, treatment duration, complications, long-term follow-up, treatment for patients awaiting a Nuss procedure, treatment for female patients, and factors affecting outcomes., Results: Of 259 patients treated with vacuum bell therapy, 18.9% (n = 49/259) were still being treated, 17.4% (n = 45/259) were lost to follow-up and 63.7% (n = 165/259) completed treatment, with a 52.1% (n = 86/165) success rate. Median follow-up was 64.0 months (interquartile range 48.0-87.0). More time spent daily on vacuum bell therapy, total treatment duration, and overnight use led to a higher success rate (P = 0.002, P < 0.001, P < 0.001 resp.). Complications (22.8%, n = 59/259) were minor, recurrence occurred in 2.3% (n = 2/86) of patients. Of the patients treated while awaiting a Nuss procedure, 26.7% (n = 4/15) no longer required the Nuss procedure. Breast growth made 39.3% (n = 11/28) of female patients quit treatment. Deeper deformities (P = 0.02, P = 0.009), flexible chest wall (P = 0.007) and symptomatic pectus excavatum (P = 0.02) resulted in lower success rates., Conclusions: Vacuum bell therapy is successful in up to 52.1% of patients. Overnight vacuum bell use and treatment while awaiting a Nuss procedure should be encouraged. Older patients with a stiff chest wall can be successfully treated with prolonged treatment. For female patients watchful waiting or early treatment, to prevent challenges during breast growth, is preferred., Level of Evidence: Level II., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background:  Nuss procedure for pectus excavatum is a minimally invasive, but painful procedure. Recently, intercostal nerve cryoablation has been introduced as a pain management technique., Materials and Methods:  In this cohort study, we compared the efficacy of multimodal pain management strategies in children undergoing a Nuss procedure. The effectiveness of intercostal nerve cryoablation combined with patient-controlled systemic opioid analgesia (PCA) was compared with continuous epidural analgesia (CEA) combined with PCA. The study was conducted between January 2019 and July 2022. Primary outcome was length of stay (LOS), and secondary outcomes were operation room time, postoperative pain, opioid consumption, and gabapentin use., Results:  Sixty-six consecutive patients were included, 33 patients in each group. The cryoablation group exhibited lower Numeric Rating Scale (NRS) pain scores on postoperative day 1 and 2 ( p  = 0.002, p  = 0.001) and a shorter LOS (3 vs. 6 days ( p  < 0.001). Cryoablation resulted in less patients requiring opioids at discharge (30.3 vs. 97.0%; p  < 0.001) and 1 week after surgery (6.1 vs. 45.4%; p  < 0.001)). In the CEA group, gabapentin use was more prevalent (78.8 vs. 18.2%; p  < 0.001) and the operation room time was shorter (119.4 vs. 135.0 minutes; p  < .010). No neuropathic pain was reported., Conclusions:  Intercostal nerve cryoablation is a superior analgesic method compared with CEA, with reduced LOS, opioid use, and NRS pain scores. The prophylactic use of gabapentin is redundant., Competing Interests: S.A.B. works as a proctor for cryotherapy in pectus patients contracted by the company Atricure., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Background: Pectus carinatum is a pediatric condition that can be treated by dynamic compression system (DCS) bracing or surgery. Several publications on DCS bracing or surgery are available; however, they do not compare both treatments., Methods: Over a 10-year period, 738 patients with pectus carinatum were treated at the Amsterdam Pectus Center (Amsterdam, The Netherlands). This study describes this 10-year experience and the results of both treatments., Results: Of the 631 patients who underwent DCS bracing treatment, 553 finished treatment, and 78 patients are still under treatment. A total of 73.8% (n = 408) of these patients finished treatment successfully, 13.6% (n = 75) experienced treatment failure, and 12.7% (n = 70) were lost to follow-up. The success rate decreased with an increasing pressure of initial compression (84.2%-67.3%). Marfan syndrome and Poland syndrome were associated with unfavorable results. Ravitch surgery was performed in 105 patients, with a success rate of 92.4%. Complications occurred in 32.4% of patients, and 6.7% of patients had complications for which surgery was needed. No relationship was found between osteotomy or sternal fixation and outcomes or complications. The Abramson procedure was performed in 2 patients., Conclusions: DCS bracing should be the treatment of choice in patients with pectus carinatum because of its noninvasiveness, good results, and lower complication rate compared with surgery. Besides pressure of initial correction, motivation is an important factor influencing outcomes, and compliance remains a major challenge in treating pectus carinatum using DCS bracing. Bracing patients before their growth spurt should be discouraged. Patients with a higher pressure of initial compression (>8.0-8.5 psi) and Marfan syndrome or Poland syndrome have poorer outcomes. In those patients, surgery may be considered., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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/m 2 vs 19.5 kg/m 2 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., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.)

Background: Pectus carinatum (PC) is a congenital chest wall deformity. In childhood, it is increasingly treated with dynamic compression therapy. Factors for success for dynamic brace therapy are relatively unknown., Methods: Between 2013 and 2020, 740 patients treated with the Dynamic Compression System (DCS), were studied. This included the effect of age, gender, pectus height, symmetry and pectus rigidity on treatment time and symptoms with linear multiple regression analyses., Results: Carinatum height and high pressure of initial correction at the start of treatment were associated with a prolonged duration of treatment. For each cm increase in carinatum height, the total treatment duration increased with 1.9 months (p-value= 0.002, 95% CI: 0.70-3.13). An initial correction pressure of ≥7.6 pounds per square inch (psi), increased the treatment duration with 3.5 months (p-value 0.006, 95% CI: 1.04-6.01) compared to an initial correction pressure of ≤5.0 psi. A high initial pressure of correction of ≥7.6 psi increased the odds of having somatic symptoms with 1.19 (p-value= 0.012, 95% CI: 1.04-1.45) and psychosocial symptoms with 1.13 (p-value= 0.04, 95% CI: 1.01-1.27) compared to a low initial pressure of correction of ≤5.0 psi. An initial pressure of correction of 5.1-7.5 psi increased the odds of having somatic symptoms with 1.14 (p-value 0.046, 95% CI: 1.00-1.29) compared to an initial pressure of correction of ≤5.0 psi. Patients with asymmetric chests were more likely to abandon therapy CONCLUSIONS: High carinatum height and high initial pressure of correction are associated with prolonged bracing treatment and a higher failure rate., Level of Evidence: III., (Copyright © 2022. Published by Elsevier Inc.)

Background: Reliable assays to measure direct oral anticoagulant (DOAC) levels and their activity in critical situations are needed. Drug levels alone are not representative of the effect of DOACs on an individual's coagulation. We developed a technique that provides direct assessment of the global effect of rivaroxaban on the individual's coagulation in addition to plasma concentrations., Methods: DOAC concentrations were determined in fifty patients using rivaroxaban, with the new assay, Xross-CAT. The effect of rivaroxaban on coagulation (activity) was measured with thrombin generation (TG) in platelet poor plasma using 5 pM tissue factor on the same device. The levels were validated with the Biophen DiXal assay. The prothrombin time (PT) and dilute Russell viper venom time (dRVVT) were performed to estimate the effect on coagulation., Results: The variability of Xross-CAT was below 12%. Xross-CAT correlates well with Biophen DiXaI (r s  = 0.885). The bias, determined by Bland-Altman analysis, was 4.9% and the Passing-Bablok equation was y = 1.1x - 2.1. The correlation of plasma levels with TG was moderate (ETP r s  = -0.548; Peak r s  = -0.559), as for the PT (r s  = 0.739) and the dRVVT (r s  = 0.692)., Conclusions: Xross-CAT shows a good correlation with Biophen DiXaI that was previously confirmed to accurately assess rivaroxaban levels. Bleeding and thrombotic complications are not necessarily associated with drug levels and could be influenced by concomitant risk factors. The main benefit of Xross-CAT is that it can be performed simultaneously with thrombin generation, providing an overview of the global anticoagulation status of a patient in relation to circulating DOAC levels., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Introduction  Although physical exercise is protective against cardiovascular disease, it can also provoke sudden cardiac death (exercise paradox). Epidemiological studies suggest that systemic hypoxia at high altitude is a risk factor for venous thromboembolism. Forthcoming, this study investigated the effect of repeated exercise at high altitude on blood coagulation, platelet function, and fibrinolysis. Methods  Six trained male volunteers were recruited. Participants ascended from sea level to 3,375 m altitude. They performed four exercise tests at 65 to 80% of their heart-rate reserve during 2 hours: one time at sea level and three times on consecutive days at 3,375 m altitude. Thrombin generation (TG) was measured in whole blood (WB) and platelet-rich and platelet-poor plasma. Coagulation factor levels were measured. Platelet activation was measured as αIIbβ3 activation and P-selectin expression. Fibrinolysis was studied using a clot-lysis assay. Results  Normoxic exercise increased plasma peak TG through increased factor VIII (FVIII), and increased von Willebrand factor (VWF) and active VWF levels. Platelet granule release potential was slightly decreased. After repetitive hypoxic exercise, the increase in (active) VWF tapered, and there was no more distinct exercise-related increase in peak. Platelet aggregation potential and platelet-dependent TG decreased at high altitude. There were no effects on fibrinolysis upon exercise and/or hypoxia. Conclusion  Strenuous exercise induces a procoagulant state that is mediated by the endothelium, by increasing VWF and secondarily raising FVIII levels. After repetitive exercise, the amplitude of the endothelial response to exercise diminishes. A hypoxic environment appears to further attenuate the procoagulant changes by decreasing platelet activation and platelet-dependent TG.

Pectus carinatum is a congenital chest-wall deformity with a prevalence of 0.3-0.7%. This chest-wall deformity can lead to functional, cosmetic and psychosocial problems. For many years, the only available treatment was an invasive surgical procedure. A less-invasive treatment, namely external compression of the sternum with a brace, showed disappointing results due to discomfort, a high percentage of skin complications and low compliance. The introduction of the dynamic compression brace has meant that the pressure of sternal compression can be measured and adjusted, which has improved comfort and compliance and resulted in an increased success rate of 92% and a decrease in the percentage of skin complications to just 8%. Despite the fact that wearing a compression brace for a longer period of time is not an easy undertaking for an adolescent, the use of the dynamic compression brace results in a high success rate, and is a safe and an efficient treatment.

Introduction: Calibrated automated thrombinography (CAT) is a sensitive method to assess coagulation. Dabigatran inhibits both free thrombin and the α 2 macroglobulin (α 2 M)-thrombin complex, which results in an erroneously increased peak and endogenous thrombin potential (ETP) without affecting lag time and time-to-peak. The aim of this study was to elucidate the artefacts in CAT when dabigatran is present., Materials and Methods: Thrombin generation (TG) was measured in vitro by using CAT in the presence or absence of 6 μM idarucizumab in plasma spiked with dabigatran. Additionally, ex vivo measurements were performed in plasmas of 63 patients using dabigatran in the presence and absence of idarucizumab., Results: The in vitro experiments confirmed that the ETP, peak and velocity index were artificially increased. This was mainly due to the inhibition of the calibrator by dabigatran and partly due to CAT algorithms. The calibration artefact could be resolved by adding idarucizumab to the calibrator well. However, the second, mathematical artefact remains when dabigatran is present in the TG well. These findings were corroborated by ex vivo experiments i.e. the lag time and time-to-peak were significantly reduced in patients upon addition of idarucizumab, but the ETP and peak were not significantly affected. The velocity index did change significantly, since this is a combination of time-dependent factors and the peak., Conclusions: The peak, ETP and velocity index do not represent the anticoagulant effect of dabigatran on TG measured with CAT. The lag time and time-to-peak, however, do reflect the effect of dabigatran., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background  Bleeding is a feared adverse event during anticoagulant treatment. In patients on vitamin K antagonists, most bleedings occur with the international normalized ratio (INR) in the therapeutic range. Currently, identification of high-risk patients via laboratory methods is not reliable. In this systematic review, we assessed the ability of calibrated automated thrombin generation (CAT-TG) to predict bleeding in patients on anticoagulant treatment. Methods  A systematic search was executed in three databases: Medline, Embase, and Cochrane. Results  Seven studies were included; two were of good methodological quality. One study showed that patients on warfarin with INRs in range (2-3) admitted for hemorrhage ( n  = 28), had lower CAT-TG levels (endogenous thrombin potential [ETP]: 333 ± 89 nM/min) than patients on warfarin admitted for other reasons (ETP: 436 ± 207 nM/min; p  < 0.001). Another study found no difference in ETP or peak levels between bleeding and nonbleeding patients in PPP or PRP. When measured in whole blood, both levels were significantly lower in patients with bleeding compared with nonbleeding patients (median [interquartile range, IQR] ETP: 182.5 [157.2-2,847 nM/min] vs. median [IQR] ETP: 256.2 [194.9-344.2 nM/min]; p  < 0.001) and median [IQR] peak: 23.9 [19.6-41.8 nM] vs. median [IQR] peak: 39.1 [24.9-53.2 nM]; p  < 0.05). From the remaining studies, four suggested that CAT-TG is more sensitive in detecting hemostatic abnormalities than INR and one article found ETP and INR to be equally useful. However, insufficient data were provided to validate these conclusions. Conclusion  Studies investigating the direct association between decreased CAT-TG values and hemorrhagic events are rare. Therefore, the clinical consequences of low CAT-TG values remain to be further investigated.

Background: Children born with esophageal atresia require an anastomosis between the proximal and distal esophagus. When this distance is too wide (long gap esophageal atresia, LGEA) esophageal replacement strategies have to be deployed. The aim of this study was to assess long-term respiratory morbidity and lung function after esophageal replacement with either stomach (gastric pull-up, GPU) or jejunum (jejunal interposition, JI) for LGEA., Methods: Retrospective cohort study. Patients operated with GPU and JI for LGEA (1985-2007) underwent a semi-structured interview and lung function testing (LFT)., Results: Seven GPU-patients and eight JI-patients were included. Median age was 12years. One patient per group could not perform LFT. Respiratory symptoms were reported by 13/15 patients (7/7 GPU-patients vs 6/8 JI-patients). All LFT items were lower than reference values; 6/13 patients showed restriction and 6/13 obstruction. All six GPU-patients had abnormal TLC and/or FEV1/FVC vs 3/7 after JI. Restriction was noted in 4/6 GPU-patients vs 2/7 JI-patients., Conclusion: After esophageal replacement for LGEA many children have impaired lung function and respiratory symptoms are common. Lung volumes seem decreased after GPU compared to JI. This may be caused by the intrathoracic stomach which may limit normal lung growth. Respiratory follow-up in adult life is important after esophageal replacement., Level of Evidence: III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Until recently, vitamin K antagonists (VKAs) were the mainstay of oral anticoagulant treatment with bleeding as the most prevalent adverse effect. One to four percent of patients experience major bleeding episodes, while clinically relevant bleeding occurs in up to 20%. At this moment no laboratory assays are available to identify patients at risk for bleeding. With this study we aimed to investigate whether thrombin generation tests might identify a bleeding risk in patients taking VKAs. This prospective cohort study included 129 patients taking VKAs for more than three months. Calibrated automated thrombinography (CAT) was performed in whole blood, platelet rich and platelet poor plasma. Hematocrit, hemoglobin concentrations and the International Normalized Ratio (INR) were defined and coagulation factor levels were measured. Forty clinically relevant bleeding episodes were registered in 26 patients during follow-up. No differences were found in plasma CAT parameters or INR values. Bleeding was not associated with age, sex, hematocrit, hemoglobin levels or coagulation factor levels. In whole blood a significantly lower endogenous thrombin potential (ETP) and peak were found in patients with bleeding (median ETP: 182.5 versus 256.2 nM.min, p = 0.002; peak: 23.9 versus 39.1 nM, p = 0.029). Additionally, the area under the receiver operating curve (AUC ROC) was significantly associated with bleeding (ETP: 0.700, p = 0.002; peak: 0.642, p = 0.029). HAS-BLED scores were also significantly higher in bleeding patients (3 versus 2, p = 0.003), with an AUC ROC 0.682 (p = 0.004). In conclusion, bleeding in patients taking VKAs is associated with a decreased whole blood ETP and peak as well as with an increased HAS-BLED score.

Aims: Therapeutic windows for residual platelet reactivity in patients with coronary artery disease on P2Y12 inhibitors were proposed in a consensus document. We aimed to explore the level of agreement between windows for different platelet function tests (PFTs) used to classify patients in low, optimal, and high on-treatment platelet reactivity categories, and to identify variables contributing to the level of agreement., Methods and Results: In this explorative clinical study, the VerifyNow P2Y12, Multiplate adenosine diphosphate (ADP), and light transmission aggregometry (LTA) 20 μmol/L ADP were performed simultaneously in 145 consecutive vulnerable patients. Measurements were performed within 6 months of percutaneous intervention. Patients were considered vulnerable if they had ≥2 risk factors for bleeding or ischaemic events. Window-agreement between PFT pairs was slight to moderate. Multiplate-VerifyNow agreed in 72 patients (50%), κ = 0.41; VerifyNow-LTA agreed in 76 patients (52%), κ = 0.36; and LTA-Multiplate agreed in 64 patients (44%), κ = 0.20. Several variables including the type of P2Y12 inhibitor, aspirin, haemoglobin level, platelet count, age, and previous stroke significantly influenced agreement between PFTs., Conclusions: Our results suggest that the PFTs, with accompanying therapeutic windows, are not interchangeable when determining the response to antiplatelet therapy in vulnerable coronary artery disease patients on P2Y12 inhibitors. Hence, the type of PFT can directly affect the treatment strategy, which may be especially relevant for patients with multiple factors influencing individual PFTs and thereby test agreement., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For Permissions, please email: journals.permissions@oup.com.)

Introduction: In 2012, we started the KLANKbord-study. A quality of life (QoL) study that follows patients with an anorectal malformation (ARM) or Hirschsprung disease (HD) and their parents from diagnosis till the age of 18 years. We hypothesized that the diagnosis of ARM or HD initially has a negative influence on QoL and anxiety levels of parents, but that this influence will diminish over time. The aim of this study is to see whether QoL and anxiety levels of parents change within the first year after the diagnosis., Methods: Parents of all children born with ARM or HD, were eligible for this study. Within 3 months after the diagnosis ARM or HD, parents received a set of validated QoL questionnaires (measurement 1). Measurement 2 was 12 months after the first questionnaire., Main Results: During measurement 1 mothers (n = 20) scored significantly higher on the social (p value, 0.01; 95% confidence interval [CI], 0.3946-3.1528) and environmental domain (p value, 0.01; 95% CI, 0.4449-2.2851) of the World Health Organization Quality of Life-BREF (WHOQOL-BREF) compared with the known reference values. Fathers (n = 19) scored significantly higher on the physical (p value, 0.01; 95% CI, 0.2964-1.8072), psychological (p value, 0.001; 95% CI, 0.7697-2.4757), and environmental domain (p value, 0.003; 95% CI, 0.5586-2.4214) than the reference values. Comparison of all domains of the WHOQOL-BREF for mothers and fathers between measurements did not show a significant difference. Anxiety levels of mothers were lower during measurement 2 compared with measurement 1. Anxiety levels of fathers were higher during measurement 2 compared with measurement 1. These differences are not significant. Anxiety levels of mothers were significantly higher than anxiety levels of fathers during measurement 1 (p value, 0.002; 95% CI, 0.808-2,956). During measurement 2 this difference in anxiety of mothers versus fathers did not exist (p value, 0.373; 95% CI, -1.157 to 2.922)., Conclusion: A negative influence on the QoL of parents having a child with ARM or HD, compared with the reference population was not seen in this population. QoL did not change significantly during the first year for both fathers and mothers. Anxiety levels of mothers did decline during this first year. The number of parents included in this study is still small, which might influence our results. Therefore, we will continue the KLANKbord-study indefinitely., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: When restoration of the anatomical continuity in case of long gap esophageal atresia (LGEA) is not feasible, esophageal replacement surgery becomes mandatory. The aim of this paper is to critically compare the experience of two tertiary referral centers in The Netherlands performing either gastric pull-up (GPU) or jejunal interposition (JI)., Methods: Retrospective chart review of all the patients with LGEA who underwent GPU in the University Medical Center Groningen and JI in the University Medical Center Utrecht. Main endpoints were short term morbidity, mortality and long term functional outcome (digestive functioning and growth). Descriptive analyses conducted using Mann-Whitney U test for continuous variables and Fisher's exact test for categorical variables., Results: Nine children underwent GPU and 15 JI. Median age (years) at last follow up was fourteen (GPU) and eight (JI). One patient died, 10 years after JI. No grafts were lost. Perioperative anastomotic complications were reported more often after JI (73% vs. 22%, p=0.03). However reintervention rate was the same in both groups (33%). Among long term outcomes, functional obstruction was not registered after GPU, while it was recorded in 46% after JI (p=0.02). No other significant differences were found apart from some tendencies concerning full oral nutrition and gastroesophageal reflux (GPU>JI)., Conclusion: Comparative data from this study reveal no mortality but significant morbidity in both groups. No graft was lost. Although not statistically different as a result of small patient numbers, clinically important differences regarding gastrointestinal system were noted. Growth should be monitored closely in both groups., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: To study long-term (10-15 years) efficacy of antireflux surgery (ARS) in a prospectively followed cohort of pediatric patients with gastroesophageal reflux disease, using 24-hour pH monitoring and reflux-specific questionnaires., Background: Studies on short-term outcome of ARS in pediatric patients with gastroesophageal reflux disease have shown good to excellent results; however, long-term follow-up studies are scarce, retrospective, and have not used objective measurements., Methods: Between 1993 and 1998, a cohort of 57 pediatric patients (ages 1 month to 18 years; 46% with neurological impairment) underwent laparoscopic anterior partial fundoplication (Thal). Preoperatively and postoperatively (at 3-4 months and at 1-5 and 10-15 years), reflux-specific questionnaires were filled out, and 24-hour pH monitoring was performed., Results: At 3 to 4 months, at 1 to 5 years, and at 10 to 15 years after ARS, 81%, 80%, and 73% of patients, respectively, were completely free of reflux symptoms. Disease-free survival analysis, however, demonstrated that only 57% of patients were symptom free at 10 to 15 years after ARS. Total acid exposure time significantly decreased from 13.4% before ARS to 0.7% (P < 0.001) at 3 to 4 months after ARS; however, at 3 to 4 months after ARS, pH monitoring was still pathological in 18% of patients. At 10 to 15 years after ARS, the number of patients with pathological reflux had even significantly increased to 43% (P = 0.008). No significant differences were found comparing neurologically impaired and normally developed patients., Conclusions: As gastroesophageal reflux persists or recurs in 43% of children 10 to 15 years after laparoscopic Thal fundoplication, it is crucial to implement routine long-term follow-up after ARS in pediatric patients with gastroesophageal reflux disease.

Aim: The aim of this study was to investigate the difference in anxiety and quality of life (QOL) between mothers and fathers of children with anorectal malformations (ARM) or Hirschsprung disease (HD). In addition, anxiety of parents of newborns was compared with parents of children in the age-group of 10 to 13 years., Methods: Parents of 44 patients with ARM or HD completed the World Health Organization Quality of Life (WHOQOL)-BREF (26 items) and the Spielberger State-Trait Anxiety Inventory (STAI-trait 10 items, STAI-state 6 items). They were divided into two groups according to their children's age (0-4 years and 10-13 years)., Results: Overall mothers scored significantly worse than fathers on state anxiety (p = 0.005). Trait anxiety was not significantly different between mothers and fathers. The psychological domain of the WHOQOL-BREF was significantly different between parents (p = 0.016), with mothers scoring worse. Dividing the group in newborns and school going children mothers of newborns showed significantly more state anxiety compared with fathers of newborns (p = 0.016). In the group of older children, both anxiety and QOL were not significantly different between fathers and mothers. Comparing mothers of newborns with mothers of older children, the first group scored higher on state anxiety, but this difference was not significant (p = 0.138; 95% confidence interval, 0.535-3.717)., Conclusions: Mothers of newborns have a higher level of anxiety than fathers of newborns with ARM or HD. When children with ARM or HD become older, this difference in anxiety between parents is not significant anymore. This may be explained by coping strategies., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim: There is still no consensus about the optimal surgical approach for esophageal replacement in the case of long-gap esophageal atresia (LGEA) or extensive corrosive strictures. The aim of this article was to perform a meta-analysis comparing the most widely used techniques for esophageal replacement in children: jejunal interposition (JI), colon interposition (CI), and gastric pull-up (GPU)., Methods: Review of the English-language literature published in the past 5 years about esophageal replacement in children was done. The focus was on postoperative survival rate, morbidity (gastrointestinal complications such as anastomotic stenosis/leakage and respiratory complications such as pneumothorax, pneumonia, and atelectasis), and long-term follow-up when available. Among long-term gastrointestinal outcomes were dysphagia, reflux, and dumping; among long-term respiratory outcomes were recurrent pneumonia and recurrent aspiration leading to chronic lung disease. Data were computed by Comprehensive Meta-Analysis software (Version 2.2.064)., Main Results: A total of 15 studies (4 comparative retrospective, 8 retrospective, and 3 prospective) including 470 patients (264 LGEA) were identified; 344 (73%) patients underwent CI, 99 (21%) GPU, and 27 (6%) JI. Among these 15 studies, 9 provided data about long-term follow-up., Conclusion: Proper prospective comparative studies are lacking. GPU and CI appear comparable regarding postoperative mortality, anastomotic complications, and graft loss. On the long-term, GPU seems to be associated with a higher respiratory morbidity but fewer gastrointestinal complications than CI. Based on this article only two series provide data about JI, and they show highly divergent results. JI appears to be a valid replacement technique when performed by experienced centers; however larger numbers are needed to assess the outcomes of this procedure., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Thoracoscopic repair of esophageal atresia is considered to be one of the more advanced pediatric surgical procedures, and it undoubtedly has a learning curve. This is a single-center study that was designed to determine the learning curve of thoracoscopic repair of esophageal atresia., Methods: The study involved comparison of the first and second five-year outcomes of thoracoscopic esophageal atresia repair., Results: The demographics of the two groups were comparable. There was a remarkable reduction of postoperative leakage or stenosis, and recurrence of fistulae, in spite of the fact that nowadays the procedure is mainly performed by young staff members and fellows., Conclusions: There is a considerable learning curve for thoracoscopic repair of esophageal atresia. Centers with the ambition to start up a program for thoracoscopic repair of esophageal atresia should do so with the guidance of experienced centers.