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Introduction:  For parents of chronically ill children, the experiences of caregiving are challenged by increased demands and restrictions imposed by their child's disease. Therefore, this study aims to investigate the quality of life (QoL) in parents of children born with esophageal atresia (EA) and to explore associated factors.Materials and Methods:  Parents of children (2-17 years) with EA recruited from two German pediatric hospitals participated in this cross-sectional study about QoL in EA. Data on QoL, sociodemographic, and clinical characteristics were collected from parents and children. Parental QoL was assessed using the Short-Form 8 questionnaire, containing eight dimensions aggregated to a mental and physical health summary score which was compared with German representative population norms.Results:  Forty-nine families (47 mothers and 40 fathers) participated in the study. Compared with German population norms, both mothers and fathers showed significantly lower mental component score (MCS) but no differences in physical component score (PCS). Within the study sample, parents of younger children (2-7 years), severe EA, or high school/kindergarten absence had lower MCSs compared with those with older, less severe, and less absent children. Parental female gender was associated with lower MCS as well as lower family income.Conclusion:  Parents of children with EA reported lower mental health compared with the general population, especially mothers, and parents of young children, with severe EA, and a frequent school/kindergarten absence. This shows that parents may experience substantial emotional burden and highlights the need for psychosocial support for EA parents, especially in the first years. [ABSTRACT FROM AUTHOR]

Introduction:  Negative pressure wound therapy (NPWT) is a novel tool to reduce surgical site infections (SSIs). Although SSIs are a common source of morbidity in infants undergoing laparotomy, the cost of the available NPWT devices has restricted its use to adult high-risk patients. We developed a low-cost method of NPWT in infants and analyzed its impact on the incidence of SSIs in infant patients.Materials and Methods:  A consecutive series of infants (age ≤ 12 months) who underwent a clean-contaminated, contaminated, or dirty abdominal procedure via laparotomy from 08/2015 to 12/2016 were included. The choice of the applied dressing, either NPWT or standard surgical dressing (SSD), was made at the surgeon's discretion. SSIs were documented prospectively. The Horan definition and the Clavien-Dindo classification were used. The cost of material was calculated for both groups.Results:  Ninety-three consecutive patients were included (65 SSD and 28 NPWT). SSI occurred in 10 patients in the SSD group, Grade I in 7 and Grade II in 3 (Clavien-Dindo classification). No SSI occurred in patients with NPWT (p < 0.05). The cost of an SSD was less than 1 €, and the cost of a NPWT was less than 10 €.Conclusion:  The routine use of this modified vacuum wound dressing may be an efficient and affordable technique to decrease SSIs in infants who underwent contaminated abdominal operations. [ABSTRACT FROM AUTHOR]

Previous research has confirmed that patients with choledochal cyst have an elevated risk of cholangiocarcinoma and gallbladder carcinoma. Current data suggest a risk of malignancy of 6 to 30% in adults with choledochal cyst. Malignancy has also occasionally been identified in children and adolescents. Multiple factors, including the age of the patient, cyst type, histological findings, and localization, have an impact on the prognosis. Information on long-term outcomes after cyst excision is limited. However, recent data suggest a lifelong elevated risk of up to 4% of cancer development following operation. This paper presents a review of the literature on cancer in patients with choledochal cyst before and after excision. A postoperative follow-up concept that consists of annual controls of CA19-9 and abdominal ultrasound is introduced. [ABSTRACT FROM AUTHOR]

Aim:  Despite advances of outcomes of esophageal atresia (EA), knowledge on patients' health-related quality of life (HRQoL) is sparse. Due to the heterogeneity of EA, larger cohorts need to be investigated to ensure reliability of data. Aim was to determine generic HRQoL after EA repair in a Swedish-German cohort.Patients and Methods:  Ethical approval was obtained. A total of 192 patients (2-18 years; 134 Swedish; 58 German) were included. Clinical data were reviewed. EA was classified in "severe" and "mild/moderate." Pediatric Quality of Life Inventory 4.0 Generic Core Scale (PedsQL 4.0) was used in appropriate versions (2-7 years; 8-18 years; self- [SR] and proxy report [PR]) to determine generic HRQoL.Results:  Swedish and German samples were clinically and demographically comparable. HRQoL was lower in "severe EA" versus "mild/moderate" (2-18 years; total score; PR 85.6 vs. 73.6; p < 0.001) and Gross A versus Gross C type EA (2-7 years; total score; PR 61.0 vs. 79.3; p = 0.035). Total HRQoL was higher in the Swedish versus German sample (2-18 years; total score; PR 82.3 vs. 72.7; p = 0.002). HRQoL was impaired in the German sample versus healthy population (2-18 years; total score; PR 72.7 vs. 82.7; p = 0.001). In German patients (8-18 years), HRQoL was higher in SR versus PR (80.7 vs. 74.7; p = 0.044). Patients' age and presence of VACTERL association or isolated anorectal malformations did not affect HRQoL. Various differences were detected regarding different dimensions of PedsQL 4.0.Conclusion:  In this first international study, we found several differences in perception of generic HRQoL. HRQoL appears to be determined by the type of EA and severity rather than patients' age or the presence of typical associated malformations. Country-specific differences may be culturally dependent, but further investigations are suggested. A condition-specific instrument validated for EA may provide additional insights. [ABSTRACT FROM AUTHOR]

Aim:  Surgical expertise and advances in technical equipment and perioperative management have led to enormous progress in survival and morbidity of patients with esophageal atresia (EA) in the last decades. We aimed to analyze the available literature on surgical outcome of EA for the past 80 years.Materials and Methods:  A PubMed literature search was conducted for the years 1944 to 2017 using the keywords "esophageal/oesophageal atresia," "outcome," "experience," "management," and "follow-up/follow up." Reports on long-gap EA only, non-English articles, case reports, and reviews without original patient data were excluded. We focused on mortality and rates of recurrent fistula, leakage, and stricture.Results:  Literature search identified 747 articles, 118 manuscripts met the inclusion criteria. The first open end-to-end anastomosis and fistula ligation was reported in 1941. Thoracoscopic fistula ligation and primary anastomosis was performed first in 2000. Reported mortality rate decreased from 100% before 1941 to 54% in 1950 to 1959, 28% in 1970 to 1979, 16% in 1990 to 1999, and 9% nowadays. Rates of recurrent fistula varied over time between 4 and 9%. Leakage rate remained stable between 11 and 16%. However, stricture rate increased from 25 to 38%.Conclusion:  Including a full range of articles reflecting the heterogeneity of EA, mortality rate significantly decreased during the course of 80 years. Along with the decrease in mortality, there is a shift to the importance of major postoperative complications and long-term morbidity regardless of surgical technique. [ABSTRACT FROM AUTHOR]

Objective:  Recurrent esophageal strictures (ESs) following esophageal atresia repair or caustic ingestion represent a common clinical problem. Recently, Mitomycin C was reported to improve the outcome of patients by reducing the number of endoscopic dilatations. However, other groups failed to exhibit a beneficial effect. We report on our experience with topic Mitomycin C application following endoscopic dilatation for recurrent ES.Methods:  Retrospective chart review of patients with ES treated at the Hannover Medical School (Location A) and the University of Leipzig (Location B) between 2009 and 2015. A Mitomycin C-soaked cotton swab was endoscopically placed at the area of stricture in all subjects. Successful treatment was defined as resolution of stricture after Mitomycin C therapy with not more than three dilatations thereafter. Our results were compared with published outcomes of alternative studies that involved 10 or more patients.Results:  A total of 11 children received Mitomycin C concurrently with endoscopic dilatations. Seven children (64%) had gross type C esophageal atresia, two patients (18%) gross type A esophageal atresia, and two children (18%) caustic injury.After a median follow-up of 34 months (range, 14-75 months) and a median number of 3 ± 2.5 dilatations with Mitomycin C application per patient (range, 1-9), 6 of 11 patients (55%) achieved a resolution of their strictures. Five patients (45%) did not respond to Mitomycin C therapy, of which two needed esophageal redo-surgery.Conclusion:  We failed to confirm the high success rates of Mitomycin C treatment for recurrent ESs. Given the fact that there is limited data to prove the beneficial effect of Mitomycin C treatment, pediatric surgeons should carefully consider whether the advantages of this therapy outweigh the necessity of life-long endoscopic follow-ups. Further randomized controlled studies are recommended. [ABSTRACT FROM AUTHOR]

Video-assisted thoracoscopic surgery (VATS) has gained broad acceptance among pediatric surgeons. Today, VATS can be regarded as a routine approach for various conditions in neonates and infants. However, there is a lack of information concerning the complications of thoracoscopic pulmonary surgery in neonates and infants. We aimed to review the available data. A systematic review of the literature was performed using PubMed. All publications reporting on VATS for pulmonary procedures in neonates and infants up to the age of 1 year were included. Articles were reviewed in detail for occurrence of perioperative complications and their treatment. Nine case series were eligible for analysis. Entities treated included lung sequestration, congenital pulmonary airway malformation, congenital lobar emphysema, and bronchogenic cyst. Case series reported on a total number of 135 patients. Complications occurred in 14 patients (10.4%). The major complication was an accidental transection of the middle lobar bronchus that concluded in a later resection of a prior unaffected lung lobe. Other relevant complications reported were bleeding in four patients (3.0%) and persistent air leak in four cases (3.0%). Infectious complications were reported in four patients (3.0%). Additionally, iatrogenic phrenic nerve paralysis occurred in one patient. There were no deaths related to the VATS technique. In 11 cases (8.1%), conversion to thoracotomy had been necessary. General recommendations on the treatment of complications could not be derived due to small patient numbers and lack of details of the complications reported. Reports on major complications of pulmonary VATS in neonates and infants below 1 year of age are scarce. As severe complications such as accidental bronchus transection have been described, feasibility of neonatal/infant VATS cannot be unequivocally confirmed. No literature-based recommendation can be given on the treatment of complications. [ABSTRACT FROM AUTHOR]

Background:  Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) represents the most common developmental malformation of the upper digestive tract. It is classified into six subtypes according to the classification of Vogt, depending on anatomical variation of this malformation. Around 50% of the patients with EA/TEF present additional anomalies, which often influence, next to the EA/TEF subtype, the overall prognosis of EA/TEF newborns. Here, we investigated the association of the different EA/TEF subtypes with co-occurring congenital anomalies in EA/TEF patients and demonstrate their implications for postnatal diagnostic workup.Materials and Methods:  We investigated 333 patients of a large German multicenter study born between 1980 and 2012. After evaluation of all available clinical records, 235 patients were included in our analysis. We compared our results with existing data.Results:  The highest risk for co-occurring anomalies was seen in patients with most common Vogt 3b (p = 0.024), especially for additional gastrointestinal anomalies (p = 0.04). Co-occurring anomalies of the skin were significantly more common in patients with subtype Vogt 2 (p = 0.024). A significant correlation was observed for an impaired neurodevelopmental outcome and EA/TEF Vogt 3a (p = 0.041). Patients with EA/TEF showed a higher risk to present with any additional congenital anomaly compared with the general population (p < 0.001).Conclusion:  Our results warrant thorough clinical workup for gastrointestinal anomalies especially in patients with Vogt 3b. Moreover, it might be necessary to focus on a thorough aftercare for neurocognitive development in patients with Vogt 3a. The here presented observations need to be confirmed by future studies. [ABSTRACT FROM AUTHOR]

Neonates rely on their innate immune system. Resident tissue macrophages are considered to be initiators and regulators of the innate immune response and thus, appear to be especially important to neonates. We hypothesized that the phenotype and function of neonatal tissue macrophages differ from their adult counterparts. Peritoneal macrophages from neonatal (<24 h) and adult (6 weeks old) C57BL/6J mice were isolated and analyzed by high-content chipcytometry. After stimulation for 6 h with LPS (0, 1, 10, 100 ng/mL), macrophage transcriptome was analyzed by microarray and cytokine release was measured using multiparametric bead assays. Antigen presenting capacity was compared by T-cell stimulation assays. We observed that neonatal murine peritoneal macrophages are characterized by selective lack of expression of F4/80, MHC class II, and costimulatory molecules (CD80, CD86). Furthermore, we found differences in the transcriptome between neonatal and adult macrophages, unstimulated and after LPS stimulation. Although neonatal macrophages showed a significantly increased secretion of proinflammatory cytokines upon LPS stimulation, their potential to induce T-cell proliferation was significantly reduced. In conclusion, we observed a distinct phenotype of the neonatal macrophage population. The specific functions of this macrophage population could help to understand the excessive inflammatory reactions observed in the very young. [ABSTRACT FROM AUTHOR]