The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations.

BACKGROUND: The natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) is not fully understood, and the management of the newborn with an asymptomatic lesion is a controversial issue. We aimed to study the natural history and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and to investigate if any prognostic factors in the pre- and/or postnatal- period may predict the need for surgery. MATERIAL AND METHODS: A retrospective review study was conducted of children prenatally diagnosed with CPAM and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal period was collected and analysed. RESULTS: Sixty- six patients with prenatally observed lung lesions were entered in the study, with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively with a median follow-up of 4 years. Nineteen percent developed symptoms after the neonatal period. Children with a presence of mediastinal shift on postnatal imaging (p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery. CONCLUSION: Prenatal regression is common among prenatally diagnosed CPAM/BPS and the majority of children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. Future analysis with a longer follow-up might give new insights in order to identify children at risk of developing symptoms. LEVEL OF EVIDENCE: III.

Educational level and socioeconomic status in patients born with congenital diaphragmatic hernia: A population-based study.

BACKGROUND: Neurodevelopmental dysfunction is one of the most disabling outcomes for congenital diaphragmatic hernia (CDH) survivors and may have a long lasting impact in adult life. AIM: To evaluate to which extent being born with CDH has an impact on the educational level and socioeconomic status as a proxy for neurocognitive development. MATERIAL AND METHODS: Nationwide, population-based prospective study of newborn children in Sweden from 1982 to 2015. School grades, highest educational level and income were assessed through Swedish public registries. Children above 15 years of age with CDH were compared with randomly selected controls. RESULTS: A significantly higher number of cases (17% vs 10%) did achieve neither a school nor a university degree. Among those who achieved a degree there was no difference in the highest level of education. The qualification points in elementary school did not differ, but in high school female cases had significantly lower qualification points than female controls. There were no differences in individual disposable income between cases and controls. However, males had higher income compared to females. Prematurity and a long hospital stay had a negative impact on educational level. CONCLUSIONS: A higher proportion of children born with CDH compared to controls do not achieve a school degree. Among those who achieved a degree, the school achievements and educational level were similar to controls. Prematurity and a long hospital stay are risk factors for not achieving an educational degree. TYPE OF STUDY: Prognosis study (high-quality prospective cohort study with 99% of patients followed to the study end point). LEVEL OF EVIDENCE: Level I. I for a prognosis study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point.

Pathogenic copy number variants are detected in a subset of patients with gastrointestinal malformations.

BACKGROUND: Gastrointestinal atresias and urological defects are main causes of pediatric surgery in infants. As copy number variants (CNVs) have been shown to be involved in the development of congenital malformations, the aim of our study was to investigate the presence of CNVs in patients with gastrointestinal and urological malformations as well as the possibility of tissue-specific mosaicism for CNVs in the cohort. METHODS: We have collected tissue and/or blood samples from 25 patients with anorectal malformations, esophageal atresia, or hydronephrosis, and screened for pathogenic CNVs using array comparative genomic hybridization (array-CGH). RESULTS: We detected pathogenic aberrations in 2/25 patients (8%) and report a novel possible susceptibility region for esophageal atresia on 15q26.3. CNV analysis in different tissues from the same patients did not reveal evidence of tissue-specific mosaicism. CONCLUSION: Our study shows that it is important to perform clinical genetic investigations, including CNV analysis, in patients with congenital gastrointestinal malformations since this leads to improved information to families as well as an increased understanding of the pathogenesis.

Maternal Risk Factors and Perinatal Characteristics in Congenital Diaphragmatic Hernia: A Nationwide Population-Based Study.

BACKGROUND: The etiology of congenital diaphragmatic hernia (CDH) remains poorly understood. We hypothesize that environmental factors play an important role in the development of CDH. AIM: The objective of this study was to investigate associated maternal risk factors in pregnancies with CDH. MATERIAL AND METHODS: The study was a nationwide, population-based prospective case-control study consisting of a cohort of newborn children entered into the records of pregnant women receiving antenatal care in Sweden, registered in the Medical Birth Registry during the period from January 1, 1982 to December 31, 2015. The study outcome CDH and the different exposures were assessed through linkage to the Swedish National Patient Registry for both cases and mothers. RESULTS: A total of 972 cases of CDH were registered into one of the national registries in Sweden between 1982 and 2015. The incidence of neonates with CDH in Sweden from 1982 to 2015 was 3/10,000 live births. The mortality rate during the study period was 31%. Maternal age, ethnicity, parity, exposure to tobacco, BMI, IVF, previous history of spontaneous abortion or intrauterine fetal demise, and coexisting chronic diseases (urinary tract infection, chronic renal disease, pregestational diabetes, epilepsy, asthma, ulcerative colitis, inflammatory bowel disease, or systemic lupus erythematous) were not associated with an increased risk of CDH in the fetus. There was a significant association between maternal hypertension and the risk of the child being affected by CDH (OR 3.32, 95% CI 1.41-7.79, p = 0.01). No association was found between preeclampsia and CDH. CONCLUSIONS: Pregestational hypertension is associated with an increased risk of giving birth to a baby with CDH, but no association was observed in pregnancies developing preeclampsia and the occurrence of CDH.

Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries.

BACKGROUND: Several surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year. METHODS: Retrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life. RESULTS: We included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043). CONCLUSION: DPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Closer location of the tracheoesophageal fistula to the carina in newborns with esophageal atresia and VACTERL association.

BACKGROUND: VACTERL is a complicated syndrome with an unknown etiology where many studies have failed to identify the cause. In esophageal atresia (EA) roughly 10%-23% also have concurrent anomalies that align with VACTERL disorder. The aim of this study is to investigate if there is a difference regarding localization of the tracheoesophageal fistula between patients with VACTERL and non-VACTERL patients. METHODS: Retrospective chart review of newborn operated for esophageal atresia between 2006 and 2016 at our Institution was performed. Children with a C-type fistula according to Gross and reliable preoperative tracheoesophageal fistula to carina distance measurement at rigid tracheoscopy were included in the study. RESULTS: A total of 90 patients were included in the study. Fifteen of those were diagnosed with VACTERL. Before and after adjusting for weight and gestational week patients with VACTERL had significantly shorter carina to fistula distance at perioperative rigid tracheoscopy (p = <0.001 nonadjusted, p = 0.016 adjusted). CONCLUSION: Patients with VACTERL born with EA type C had shorter carina to fistula distance as shown at perioperative rigid tracheoscopy. The significantly shorter distance may not only present surgical difficulties but may also suggest a structural or molecular difference in the development of the esophageal atresia between the two groups. LEVEL OF EVIDENCE: III.

Partial gastric pull-up in the treatment of patients with long-gap esophageal atresia.

BACKGROUND: This study was to analyze outcomes of long-gap esophageal atresia (LGEA) treated with partial gastric pull-up (PGP) into the thorax. METHODS: The medical records of all children who had undergone PGP for LGEA from 1999 to 2012 were reviewed. Preoperative data, initial postoperative course, complications, time to full oral nutrition, follow-up diagnostics and nutritional status were assessed. RESULTS: Nine children who had undergone PGP were followed up for a mean period of 6.2 ± 3.1 years. Their median gestational age was 37 ± 2 weeks, and mean birth weight 2462 ± 658 g. Eight children were primarily treated with a gastrostomy, their mean age at PGP was 11.4 ± 10.9 weeks and mean weight was 4484 ± 1966 g. Their mean operation time was 199 ± 51 minutes. Leakage was an early postoperative complication in three children, one of whom had a consecutive stricture resection. Late complications were stenosis (n=7) and gastro-esophageal reflux (n=5). The general status of the children was judged as "good" or "very good" on the last presentation. The median percentile of the body-mass-index was 25. Gastroscopy at 3.7 ± 3.2 years after the operation revealed a grade I esophagitis in two children. There was no death in this group of children. CONCLUSIONS: Because of its high complication rate, partial gastric pull-up cannot be recommended as an alternative for the treatment of LGEA at present. A final judgment could be made on the basis of a comparative study.

[Broad injury panorama in children who swallowed a battery]. / Brett skadepanorama hos barn som svalt ett batteri.

Most ingested batteries in children pass through the gastrointestinal canal without problems. The risk for complications is at its highest if the battery gets stuck in the esophagus or if the child is less than 6 years of age or the size of the battery exceeds 15 mm. Thorough database searches only identified a limited number of articles, all were considered to provide low-grade evidence.

Use of Surgisis for abdominal wall reconstruction in children with abdominal wall defects.

AIM: Abdominal wall defects in children can present a challenge to the pediatric surgeon. Despite the development of new materials and modifications of surgical technique, no single approach has been established. The purpose of this study was to evaluate the authors experience using porcine small intestine submucosa for abdominal closure. PATIENTS AND METHODS: A retrospective review of all patients with abdominal wall defects who underwent reconstruction with Surgisis at the authors' institution from 2004 to 2011 was performed. Patient demographics, cause of defect, recurrence, rate of infection, and length of follow-up were reviewed. RESULTS: A total of 24 patients were identified as having Surgisis implanted for abdominal wall defects. The most common etiology of abdominal defect was omphalocele. All patients went on to heal wounds. Four patients developed postoperative seroma formation and another two had wound infection. Two recurrences in the form of incisional hernia were observed. Both resolved spontaneously without intervention during the follow-up. The median follow-up period was 5 years (range 2-9 y). No significant predictors of complications were identified. CONCLUSIONS: Surgisis is an effective adjunct in the repair of abdominal wall defects in children. Complication rates remain low. In addition, recurrence may disappear spontaneously as the patch absorbed and replaced by scar tissues. Further studies are warranted.

Neonatal short bowel syndrome: 'rectal feeding' in order to stimulate intestinal growth.

UNLABELLED: Short bowel syndrome (SBS) is characterized by a state of malabsorption following extensive resection of the small bowel, resulting in insufficient nutritive supply requiring artificial nutrition with long-term parenteral nutrition. Here we present an illustrative case report of a premature infant born with gastroschisis and SBS, who was treated with enteral refeeding via rectum. The infant developed during the period of rectal feeding with jejunostomy loses bowel lengthening and could be fed orally within a few months after birth. Rectal feeding with ostomy loses could stimulate bowel growth and adaptation in neonatal SBS. CONCLUSION: The purpose of this report is to describe an illustrative case of short bowel syndrome due to gastroschisis and to share a novel technique of rectal feeding to stimulate bowel growth and adaptation.