Esophageal Interventions in Infants Born with Esophageal Atresia: A Comprehensive Analysis of a National Database

2021 ◽  
Author(s):  
Tatjana T. König ◽  
Emilio Gianicolo ◽  
Luisa Frankenbach ◽  
Eva Wittenmeier ◽  
Oliver J. Muensterer
Keyword(s):  
Esophageal Atresia ◽  
Primary Anastomosis ◽  
Treatment Strategies ◽  
Primary Treatment ◽  
National Database ◽  
First Year ◽  
Rare Malformation ◽  
Low Incidence ◽  
First Year Of Life ◽  
And Training

Abstract Introduction Esophageal atresia (EA) is a rare malformation that often requires a series of procedures, including surgical primary anastomosis, staged repair, and endoscopic procedures. Actual numbers and trends in interventions and variety in treatment strategies remain unclear. Materials and Methods Data from the German federal bureau of statistics containing all EA-related inpatient procedures encoded from 2005 until 2018 were analyzed for children during the first year of life. The sum of esophageal anastomoses and replacements was used to calculate an estimate of incidence of EA. Results Over 14 years, 12,627,888 inpatient cases were recorded in infants in Germany. The mean incidence of EA was 1 per 4,217 live births. On average, 163.3 (95% confidence interval [CI]: 150.8–176.1) esophageal anastomoses, 11.2 (95% CI: 8.7–13.7) esophageal lengthening procedures, and 6.7 (95% CI: 5.42–8.00) esophageal replacements were recorded annually. Overall, 187.8 (95% CI: 147.1–200.4) endoluminal treatments (ballon dilatation, bougienage, stent placement, or injection) were performed per 100 anastomoses. Over the years, bougienage was increasingly replaced by ballon dilatation as primary treatment. Boys had a significantly higher number of esophageal procedures than girls, but the incidence of endoscopic treatments in relation to anastomoses was the same for both genders. Conclusion The low incidence of EA in relation to a relatively large number of units treating those patients in Germany may pose challenges for maintaining competency and training of all specialists involved. The number of esophagoscopic treatments for esophageal stricture per anastomosis is lower than previously estimated.

DOZ047.22: FEED-EASY: feeding disorders in children with esophageal atresia study

2019 ◽  
Vol 32 (Supplement_1) ◽  
Author(s):  
A Pham ◽  
E Dugelay ◽  
A Bonnard ◽  
T Gelas ◽  
V Rousseau ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Esophageal Atresia ◽  
Anastomotic Stricture ◽  
Primary Anastomosis ◽  
Feeding Disorders ◽  
First Year ◽  
Aversive Events ◽  
French Study ◽  
First Year Of Life

Abstract Introduction With advances in surgical and neonatal care, survival of patients with esophageal atresia (EA) has improved over time. While a number of conditions associated with EA may have an impact on feeding development (delayed primary anastomosis, anastomotic leaks, recurrent tracheoesophageal fistula, anastomotic stricture, gastroesophageal reflux, esophageal dysmotility, etc.) and although children with EA experience a number of oral aversive events in their first year of life, feeding disorders (FD) are poorly described and frequently unrecognized. The primary aim of this study was to describe FD in children born with EA, with a standardized scale. The secondary aim was to describe conditions associated with FD. Methods FEED-EASY is a multicentric French study. Parents of children born with EA between 2013 and 2016 in one of the 22 participating centers were asked to participate and received the French version of the standardized and reproductive ‘Montreal Children's Hospital Feeding Scale (MCH-FS)’. Results One hundred and forty-five children were included; 61 (42%) had FD according to the MCH-FS. These children were characterized by disinterest in food, oral hypersensitivity, difficulty in touching some textures and food avoidance, with an influence in quality of life. Nineteen (13%) were tube-fed between 1 and 4 years of age. Birth weight and chronic respiratory difficulties were associated with FD in children with EA. Anastomotic stricture (present in 31% of the included children) was not associated with FD. Conclusions FD is frequent and unrecognized in children with EA, and can influence growth and quality of life. MCH-FS allows pediatricians to identify FD in children with EA within a couple of minutes.

Outcome of esophageal atresia in Germany

2020 ◽  
Author(s):  
A Schmedding ◽  
B Wittekindt ◽  
R Schloesser ◽  
M Hutter ◽  
U Rolle
Keyword(s):  
Mortality Rate ◽  
Esophageal Atresia ◽  
First Year ◽  
Insurance Company ◽  
Associated Anomalies ◽  
Clinical Registry ◽  
Major Health ◽  
First Year Of Life ◽  
Overall Mortality

Abstract The aim of this study was to evaluate the outcome of esophageal atresia in Germany in a retrospective observational study of a large cohort. Data from the major health insurance company in Germany, which covers approximately 30% of German patients, were analyzed. All patients born and registered between 2009 and 2013 with a diagnosis of esophageal atresia at first admission to the hospital were included. Mortality was analyzed during the first year of life. We identified 287 patients with esophageal atresia, including 253 with and 34 without tracheoesophageal fistula. Associated anomalies were found in 53.7% of the patients; the most frequent were cardiac anomalies (41.8%), anomalies of the urinary tract (17.4%), and atresia of the colon, rectum, and anus (9.4%). Forty-one patients (14.3%) had a birth weight <1500 g. Seventeen patients (5.9%) died before surgery. Gastrostomy was performed during the index admission in 70 patients (25.9%). The reconstruction of the esophageal passage was performed in 247 patients (93.9%). Forty-eight percent of the patients who underwent an operation required dilatation. The mortality rate in the patients who underwent an operation was 10.4%. These results from Germany correspond to the international results that have been reported. The number of dilatations was in the middle of the range of those reported in the literature; the overall mortality rate was in the upper portion of the range of the international rates. Efforts should be made to establish a clinical registry to measure and improve the quality of care for this and other rare conditions.

Neurodevelopmental outcome in infants with esophageal atresia: risk factors in the first year of life

2020 ◽  
Author(s):  
Bevilacqua Francesca ◽  
Ragni Benedetta ◽  
Conforti Andrea ◽  
Braguglia Annabella ◽  
Gentile Simonetta ◽  
...  
Keyword(s):  
Risk Factors ◽  
Birth Weight ◽  
Cognitive Development ◽  
Low Birth Weight ◽  
Multiple Regression ◽  
Esophageal Atresia ◽  
Gestational Age ◽  
Motor Development ◽  
First Year ◽  
First Year Of Life

Summary Data on neurodevelopmental outcomes of infants born with esophageal atresia (EA) are still scarce and controversial. The aims of our study were to evaluate motor and cognitive development during the first year of life, in patients operated on of EA and to investigate potential risk factors for motor and cognitive development both at 6 and 12 months. This is an observational prospective longitudinal study in a selected cohort of type C and D EA infants enrolled in our follow-up program from 2009 to 2017. In order to exclude possible confounding factors, the following exclusion criteria were applied: (i) gestational age ≤ 32 weeks and/or birth weight ≤ 1500 g; (ii) genetic syndrome or chromosomal anomaly known to be associated with neurodevelopmental delay; (iii) neurologic disease; (iv) esophageal gap ≥three vertebral bodies. Patients were evaluated at 6 and 12 months of life (corrected age for infants with a gestational age of 32–37 weeks) with the Bayley Scales of Infant and Toddler Development—3rd Edition. In our selected cohort of EA infants, 82 were evaluated at 6 months and 59 were reevaluated at 12 months. Both Motor and Cognitive average scores were within the norm at both time points. However, we report increased number of infants with motor delay with time: 14% at 6 months and 24% at 12 months. Multiple regression analysis for Motor scores at 6 [F(4,74) = 4.363, P = 0.003] and 12 months [F(6,50) = 2.634, P = 0.027] identified (i) low birth weight, (ii) longer hospital stay and (iii) weight < fifth percentile at 1 year as risk factors. Interestingly, average Cognitive scores also increased with time from 85.2% at 6 months and 96.6% at 12 months. Multiple regression models explaining variance of Cognitive scores at 6 [F(4, 73) = 2.458, P = 0.053] and 12 months [F(6, 49) = 1.232, P = 0.306] were nonsignificant. Our selected cohort of EA patients shows, on the average, Motor and Cognitive scores within the norm both at 6 and 12 months. Nevertheless, the percentage of infants with Motor scores below the average increases regardless gestational age. None of clinical and sociodemographic variables taken into consideration was able to predict cognitive development both at 6 and 12 months whereas risk factors for Motor development change during the first year of life. Healthcare providers should pay particular attention to patients with low birth weight, longer hospital stays and weight under fifth percentile at 1 year. Future studies should include long-term outcomes to reveal possible catch up in motor development and/or possible findings in Cognitive scores.

STUDIES IN SICKLE CELL ANEMIA

PEDIATRICS ◽  
1954 ◽  
Vol 14 (3) ◽  
pp. 209-214
Author(s):  
ROLAND B. SCOTT ◽  
LELABELLE C. FREEMAN ◽  
ANGELLA D. FERGUSON
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Age Groups ◽  
Test Group ◽  
Progressive Increase ◽  
First Year ◽  
Older Children ◽  
Low Incidence ◽  
First Year Of Life

This survey of 1100 Negro children in various age categories was undertaken to determine the effect of age upon the appearance of the sickling phenomenon from infancy throughout childhood. The general incidence of sickling in 1100 Negro children including sickle cell anemia and sickle cell trait was 7.4%. The data on the incidence of the asymptomatic sickling trait and of sickle cell anemia are summarized by age and sex in Tables I and II. We encountered 22 cases of sickle anemia, seven of which were previously undiagnosed and unknown. Sixteen cases of sickle cell anemia in males and six in females were encountered in the total test group, comprising 651 males and 449 females. This investigation disclosed 60 subjects bearing the asymptomatic sickling trait. There were 40 and 20 instances of asymptomatic sickling observed in 635 males and 443 females, respectively. When the sexes were divided into two age categories (1 month through 4 years and 5 years through 16 years), there was an actual decrease in the incidence of sickling in the girls and an increase in the sickling phenomenon in the boys. We have no explanation for this finding. The overall incidence of the sickling trait for both sexes in all age groups represents no significant deviation from a 1:1 ratio. The data available from this study failed to disclose a definite progressive increase in the incidence of sickling in the age groups studied. Quantitatively the general transition from the low incidence of sickling in the newborn (3.4%) to the higher occurrence in older children (7.5%) apparently takes place during the first year of life. Additional studies of both a qualitative and quantitative nature and involving a detailed age breakdown during the first year of life would probably elucidate this period of transition.

Primary Immunodeficiency Disorders in Infancy

1999 ◽  
Vol 18 (1) ◽  
pp. 13-22 ◽  
Author(s):  
Barbara Pappas
Keyword(s):  
Primary Immunodeficiency ◽  
Defense Mechanisms ◽  
Clinical Presentation ◽  
Treatment Strategies ◽  
Basic Knowledge ◽  
First Year ◽  
Primary Immunodeficiency Disorders ◽  
Specific Disorders ◽  
First Year Of Life ◽  
Prompt Diagnosis

Certain primary immunodeficiency disorders may become evident during the first year of life. These disorders are characterized by recurrent infections. Prompt diagnosis is necessary, for infants may suffer significant morbidity and mortality. A basic knowledge of host defense mechanisms, including the natural and humoral systems, is essential to the understanding of immunodeficiency disorders.This article discusses the clinical presentation, evaluation, and treatment strategies available to infants with primary immunodeficiency disorders. Five specific disorders are discussed in detail, and specific treatments are reviewed.

scholarly journals Congenital Heart Disease and Its Impact on the Development of Anastomotic Strictures after Reconstruction of Esophageal Atresia

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Pernilla Stenström ◽  
Martin Salö ◽  
Magnus Anderberg ◽  
Einar Arnbjörnsson
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Esophageal Atresia ◽  
Congenital Heart ◽  
First Year ◽  
Retrospective Case ◽  
Anastomotic Strictures ◽  
First Year Of Life ◽  
Severe Congenital Heart Disease

Background. The aim was to explore if severe congenital heart disease (CHD) influenced the need for dilatation of anastomotic strictures (AS) after the repair of esophageal atresia (EA). Methods. A retrospective case-control study was conducted examining AS in children with EA and Gross type C. The spectra of CHD and cardiac interventions were reviewed. The frequency of dilatations of AS during the first year following EA reconstruction was compared between children with and without severe CHD requiring cardiac surgery during their first year of life. Endoscopic signs of stricture were an indication for dilatation. Results. Included in the follow-up for AS were 94 patients who had EA reconstructions, of whom 10 (11%) children had severe CHD requiring surgery during the first year including 19 different cardiac interventions. In total, 38 patients needed dilatation of esophageal AS, distributed as six (60%) with severe CHD and 32 (38%) without severe CHD (p=0.31). Conclusion. Severe CHD was present in 11% of children with EA. Esophageal AS developed in 60% children with concomitant CHD, but although high, it did not reach statistical difference from children without CHD (38%).

scholarly journals Suprapatellar bursitis in a newborn

2021 ◽  
Vol 10 (2) ◽  
pp. 199-202
Author(s):  
IIya O. Erenkov ◽  
Ivan S. Denisov ◽  
Svetlana A. Zarubina
Keyword(s):  
Knee Joint ◽  
Blood Test ◽  
White Cell Count ◽  
Treatment Strategies ◽  
Rare Condition ◽  
Well Being ◽  
Patient Treatment ◽  
First Year ◽  
First Year Of Life

Introduction. One of the most frequent pathologies in childhood is bursitis of the knee joint. Such conditions may occur in infectious pathologies, traumatic disorders, overuse syndromes, and other various pathologies. Diagnostic and treatment strategies depend on the cause of bursitis development. Particular attention should be paid to bursitis cases in newborns since the frequency of such pathologies is casuistic and clinical guidelines for treating patients in this age group are absent. Materials and methods. A 26-day-old newborn arrived for consultation with complaints of knee joint pain and movement restrictions for four days. From five to 21 days of life, she has had in-patient treatment at another clinic because of pneumonia and a urinary tract infection. Intravenous and intramuscular injections of antibacterial drugs were prescribed for two weeks. The condition of the newborn was satisfactory upon consultation in our clinic. Her physical examination showed swelling and muscle tightness on the distal part of the thigh. The range of motion of the shinbone was restricted. Her blood test showed that her white cell count was unchanged. According to an X-ray examination of the knee, no pathology was detected. According to the US-examination, exudative suprapatellar bursitis was detected. The nonsteroidal anti-inflammatory drug, Nimesulid, was prescribed for seven days. Results. Daily remote monitoring of the patient's well-being during treatment was performed. Movement restrictions reduced the swelling on day 3 of treatment. A follow-up blood test on day 4 of treatment showed no pathology. According to the follow-up US-examination after six days of drug therapy, no signs of bursitis were detected. Conclusion. Inflammation of the suprapatellar bursa is a rare condition in newborns. In isolated cases of bursitis in children during the first year of life, a bacterial presence was noted, with the predominance of staphylococcal and streptococcal floras. However, in our opinion, there is a possibility of developing aseptic forms of bursitis in infants. In these cases, it may be prudent to refrain from prescribing courses of antibiotic therapy.

scholarly journals Anastomotic Strictures after Esophageal Atresia Repair: Timing of Dilatation during the First Two Postoperative Years

2018 ◽  
Vol 04 (02) ◽  
pp. e62-e65 ◽  
Author(s):  
Martin Salö ◽  
Pernilla Stenström ◽  
Magnus Anderberg ◽  
Einar Arnbjörnsson
Keyword(s):  
Esophageal Atresia ◽  
Pediatric Surgery ◽  
First Year ◽  
Esophageal Dilatation ◽  
X Ray ◽  
Anastomotic Strictures ◽  
Tertiary Center ◽  
Type C ◽  
First Year Of Life ◽  
Time Frames

Background We determined time frames for dilatation of anastomotic strictures (ASs) occurring during the first 2 years after esophageal atresia (EA) repair. Methods A retrospective study was conducted on children with EA (Gross type C) who underwent direct repair between January 2008 and March 2015 at a single tertiary center of pediatric surgery. Endoscopic signs of stricture were indications for dilatation because the endoscopy provides more reliable information than X-ray imagining methods. Results Among our cohort of 49 children with EA, 19 (39%) required at least one esophageal dilatation. All children required initial dilatation within the first year of life and none was older than 1 year during initial dilatation (p < 0.01). A median of three dilatations (range: 1–13) took place per patient, with 87% performed during the first postoperative year. The timing of initial dilatation in the first year (< 6 months, 14/19 [74%] vs. 6–12 months, 5/19 [26%]) was predictive of the need for dilatation beyond the first year (9/14 [64%] vs. 0/5 [0%]; p = 0.03) but not of more numerous dilatations (median, 3 vs. 1; p = 0.07). Conclusion The need for dilatation within 6 months postoperatively predicts the need for dilatation after 1 year, but it does not indicate the number of dilatations that will be needed.

DOZ047.30: First year risk factors of mortality and morbidity in esophageal atresia with tracheo-esophageal fistula: data from a population-based national register

2019 ◽  
Vol 32 (Supplement_1) ◽  
Author(s):  
R Sfeir ◽  
V Rousseau ◽  
A Bonnard ◽  
T Gelas ◽  
L Michaud ◽  
...  
Keyword(s):  
Birth Weight ◽  
Prenatal Diagnosis ◽  
Esophageal Atresia ◽  
Population Based ◽  
National Database ◽  
First Year ◽  
Mortality And Morbidity ◽  
Type Iii ◽  
Length Of Hospitalization ◽  
Associated Abnormalities

Abstract Objectives and Study The aim of our study is to assess the risk factor of mortality and morbidity in a large population-based registry in a population of type III/C esophageal atresia (EA). Methods Under the umbrella of the national plan for rare disease, a population based register was set up in 2008 recording the data of all the live newborns with EA in France. Based on the registry data, survival and morbidity at 1 year were studied. Morbidity was approached by calculating the rate of full oral autonomy and the hospital length of stay during the first year. Multivariate analysis was performed via multinomial logistic regression to evaluate independent predictors of overall survival and morbidity items among all significant category variables identified on univariate analysis. Results A total of 1008 patients with type III EA were extracted from the national database born from January first 2008 till 31 December 2014. Mean birth weight was 2610 g (2060 to 3075). Right lateral thoracotomy was used in 93% of cases and primary anastomosis was possible in 95% of cases. Associated abnormalities were present in 53% of patients, VACTERL in 19%, and CHARGE syndrome in 3%. Mortality at 3 months was at 5% and at one year at 6% and was correlated to prenatal diagnosis (odd ratio (OR):2.96 (1.08 to 8.08)), low birth weight (OR: 0.52 (0.38 to 0.72)), and heart defect (OR: 6.09 (1.96 to 18.89)). Length of hospitalization was correlated to birth weight (OR: 0.83 (0.61–1.13)), the difficulty of the anastomosis (OR: 1.59 (0.71–3.55)) and associated abnormalities (OR: 1.93(0.65–5.68)), the prenatal diagnosis is correlated only to the rate of full oral autonomy and not with the length of hospitalization. Conclusions Surgical procedure or difficulties did not seem to affect survival in this group of patients. In addition to the continuous need to improve care of low weight neonates, our results clearly identify that efforts should be focused on the small group of EA with prenatal diagnosis and/or severe cardiac malformation to reduce even more mortality and morbidity in EA patients.

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