Edited by: Francesco Morini, Bambino Gesù Ospedale Pediatrico (IRCCS), Italy
Reviewed by: Hiromu Miyake, Shizuoka Children's Hospital, Japan; Florian Friedmacher, The Royal London Hospital, United Kingdom
This article was submitted to Pediatric Surgery, a section of the journal Frontiers in Pediatrics
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The study was registered at
Even after successful primary repair, patients born with EA often experience and learn to live with a broad spectrum of physical limitations (
In order to investigate the role of esophageal atresia, symptoms during physical activity, motor skills, and sports participation, we evaluated a cohort of esophageal atresia patients during the annual meeting of the national patient support group using a validated motor skills test and correlated these findings to the information given by the caregivers in a brief questionnaire.
A playful, voluntary extracurricular activity for children was established at the annual meeting of the German national patient support group for patients with esophageal atresia
The
Distribution of locomotor test scores of children with EA compared to the general population and control group. The distribution of test results for the general population is the same for every test item. PP = performance percentile.
Participants balance backwards on three bars with decreasing width (6, 4.5, and 3 cm wide, each 3 m long), counting the steps completed backwards without touching the ground. There are two attempts on each bar.
To test motor coordination under time constraint, participants jump sideways back and forth from two adjacent squares (50 × 50 cm each) for 15 s as many times as possible. Jumps completed without touching the lines are counted and scored.
Strength is tested by standing long jump. Participants stand behind a line and, without taking an extra step, jump as far as they can. Distance between the starting line and the back heel is measured perpendicular to the line.
Participants stand on a step, toes close to the edge with straight knees, and bend forwards. The distance between the fingertips and the edge is measured.
Parents received a link to an online-questionnaire on their child's medical history, physical activity, and symptoms during workout to answer at home (see Appendix for complete questionnaire). The test results and data from the questionnaire were used to give an individual activity recommendation for each participant. Questions 5, 14, 15, and 16 (marked with a *) were adapted from the standardized German Health Survey for Children and Adolescents (
EA participants received their test scores for each item. The test scores of the
Participants and Test scores according to
4, m* | 92 | −3,64 | 10 | −5,43 | 11,81 | −3,8 | 3 | 3 | 2 | 1 |
4, m | 104 | −1,06 | 15 | −1,59 | 13,87 | −1,25 | 2 | 4 | 1 | 4 |
4, m | 110 | 0,01 | 18,4 | 0,01 | 15,21 | −0,17 | 3 | 0 | 5 | 3 |
5, m | 105 | −2,22 | 16 | −2,04 | 14,51 | −0,89 | 3 | 1 | 3 | 1 |
6, m | 112 | −2,01 | 16,7 | −2,67 | 13,31 | −2,24 | 4 | 1 | 1 | 1 |
7, f | 120 | −1,25 | 18,4 | −2,26 | 12,78 | −2,14 | 1 | 1 | 1 | 1 |
8, m | 128 | −1,04 | 24,5 | −1,2 | 14,95 | −0,74 | 1 | 1 | 1 | 1 |
9, f | 128 | −1,75 | 21,6 | −2,58 | 13,18 | −2,13 | 1 | 2 | 5 | 2 |
9, m | 130 | −1,54 | 22,7 | −2,52 | 13,43 | −2,14 | 1 | 1 | 1 | 1 |
10, f | 146 | 0,41 | 27 | −1,64 | 12,67 | −3,12 | 5 | 1 | 5 | 4 |
11, f | 143 | −0,82 | 35 | −0,82 | 17,12 | −0,35 | 4 | 4 | 3 | 2 |
12, f | 151 | −0,89 | 56,5 | 0,98 | 24,78 | 1,65 | 1 | 3 | 2 | 1 |
4, m | 95,8 | −2,83 | 14,6 | −1,81 | 15,9 | 0,34 | 3 | 4 | 5 | 2 |
4, m | 104,5 | −0,95 | 15,1 | −1,53 | 13,8 | −1,64 | 5 | 4 | 5 | 4 |
4, m | 110,4 | 0,32 | 18,5 | 0,05 | 15,2 | −0,15 | 3 | 4 | 5 | 5 |
5, m | 106,2 | −1,98 | 16,8 | −1,62 | 14,9 | −0,36 | 2 | 4 | 5 | 3 |
6, m | 113,1 | −1,81 | 17,6 | −2,18 | 13,8 | −1,63 | 2 | 2 | 2 | 2 |
7, f | 120,1 | −1,23 | 20,1 | −1,58 | 13,9 | −1,26 | 1 | 2 | 2 | 2 |
8, m | 128,9 | −0,89 | 23,3 | −1,58 | 14,0 | −1,45 | 2 | 4 | 4 | 5 |
9, f | 128,8 | −1,63 | 22,9 | −2,17 | 13,8 | −1,67 | 1 | 1 | 1 | 1 |
9, m | 129,4 | −1,63 | 22,6 | −2,55 | 13,5 | −2,06 | 5 | 2 | 2 | 2 |
10, f | 147,9 | 0,41 | 32,1 | −0,64 | 14,7 | −1,3 | 2 | 1 | 1 | 5 |
11, f | 143,4 | −1,09 | 35,5 | −0,74 | 17,3 | −0,26 | 1 | 2 | 5 | 3 |
12, f | 152,5 | −0,68 | 57,8 | 1,09 | 24,9 | 1,67 | 1 | 1 | 1 | 1 |
A total of 17 children with EA were evaluated for locomotor skills. The median age was 7 (range 3–12) years (9 boys, 8 girls). Three patients hat to be excluded due to incomplete data, the motor test results of two 3-year-old children could not be evaluated because of missing reference values in that age group, but the data of the questionnaires was included. For one participant, parents didn't complete the questionnaire (Figure
Inclusion Flowchart.
Mean locomotor skill test scores in the EA cohort were below the average of three for all tested items (Figure
The children showed low bodyweight and -height according to German growth charts. The mean z-score was −1.81 (SD 1.59) for weight, −1.32 (SD 1.06) for height and −1.44 (SD 1.47) for body-mass-index (BMI, Table
There was no difference in the test scores of younger children (<8 years,
According to the parents, 86% of subjects enjoyed engaging in physical activity, and 93% participated in regular physical education programs in kindergarten or school. However, caregivers subjectively reported a deficit in locomotor skills compared to class mates: children are “most of the time” or “always” able to keep up with class mates in 12% regarding speed, and 8% regarding both strength and endurance. Eighty-six percent participated in sports outside school in a sports club at least once a week, but only 29% were also physically active in their free time without organized sports programs.
Comorbidities identified in the study cohort were prematurity (54%), birth weight <1,500 g (23%), CHD (46%), developmental delay (38%), skeletal deformity (28%), and anorectal malformation (15%, Figure
Mean locomotor test results of EA patients with and without associated comorbidity (
Subjective symptoms of respiratory distress (RD) and gastroesophageal reflux disease (GERD) were the most common complaints and caused the greatest limitation to physical activity and athletic performance according to the parents (RD 46%, GERD 31%). Even though the differences are not significant, there is a clear trend toward better overall motor test scores at the absence of tracheomalazia and broncho-pulmonary obstruction (“asthma-like symptoms,” Figure
Mean locomotor test results of EA patients according to perceived symptoms during physical activity and at rest (
None of our patients complained of musculoskeletal pain during exercise. Only one patient never complained about any exercise-associated discomfort at all. None of the caregivers reported complaints during jumping on the trampoline (Table
Frequency of symptoms during various activities according to parent.
Jumping on trampoline | 99% | 0% |
Run fast | 62% | 38% |
Great exertion of strength | 69% | 31% |
Upside-down position | 76% | 23% |
Riding bicycles | 84% | 15% |
Jogging | 76% | 23% |
Physical activity, sports, and play are a major health promotor and essential for normal emotional, cognitive, and psychosocial development of children and adolescents (
Promotion of physical activity is particularly important for children with pre-existing health conditions. Children with chronic disease generally show a decreased exercise capacity that might be due to physical inactivity caused by prolonged hospitalization, recurrent infection and growth retardation during early childhood (
Our data showed a mean motor test score substantially below average for all tested items. Significantly lower mean scores were recorded in the standing long jump, which is generally considered a measure of strength. In young children, motor coordination may also be tested in this complex task. Consistent with our measurements, parents described a lack of muscular strength in comparison to classmates. The mean score for sideways jumps, testing for coordination pressed for time, was significantly lower in EA patients compared to the general population. Interestingly, balance and flexibility were decreased as well, even though both of these skills do not depend on either exercise capacity or muscular strength and, at least theoretically, should therefore be less compromised in children with EA. A general lack of physical activity might contribute to deficits in these fields of motor skills. Our findings corroborate the study by Harmsen et al. which also describes compromised balance skills in children with EA at the ages of 5 and 8 years. In this group, improved skills could be shown at the age of 8 years, if the children engaged in physical activity during the observation period (
Growth retardation is a common finding in patients with EA and has also been seen in our patients. All children had a negative z-score for height and all but one a negative z-score for weight. According to parents, a low body weight and –height compared to class mates influence the motor performance, especially in terms of muscular strength. However, the control group got an average mean result in spite of their low body weight and –height. Generally, being smaller and having a lower body weight than peers makes it hard to compete in physical education or organized sports. Growth retardation has also been described as a cause for low self-esteem if children are teased by peers (
In our data, there was a trend toward better motor test results in absence of comorbidity, except for patients with CHD without reaching statistical significance. CHD, however, was not further classified. There were two children with EA and anal atresia. Both of them had the lowest possible motor mean score (1.00), which was significantly lower than the mean score of the rest of the EA group in spite of the small number (Figure
In our study, most common complaints during exercise were respiratory symptoms and gastroesophageal reflux disease, which occurred mainly during vigorous activity like running or great exertion of strength. Chronic lung disease, GERD and growth retardation have also been described as main chronic morbidities in children with EA by other authors (
Respiratory issues, such as tracheomalazia, irreversible non-allergic airway obstruction and pulmonary restriction, caused by recurrent infection and microaspiration are common in patients with EA (
Symptoms of GERD occur to some extend in almost all patients with EA (
Symptoms of GERD account for most upper gastrointestinal symptoms in healthy athletes and worsen with the intensity of physical activity and during postprandial activity. Especially during high intensity workout [maximal oxygen uptake (VO2 max) >90%], the number and duration of reflux episodes increase by decreased esophageal motility, decreased pressure of the lower esophageal sphincter (
In contrast to our expectations, none of the parents reported any symptoms during jumping on the trampoline (Table
Our study has some limitations. For one, there were only 12 patients who qualified for final analysis of motor results because data on the others were incomplete. The data for the control group was not collected in our own hospital, but was obtained from the source data of the
Children after EA repair have decreased physical fitness and impaired locomotor function compared to a control group and the general population on a standardized test. Physical discomfort is frequent during great exertion and athletic performance is mainly limited by symptoms of RD and GERD. These symptoms cause major subjective discomfort to the children and may lead to avoidance of physical activity in general. Aerobic training improves symptoms RD and GERD in other patients and might be helpful in patients with EA. Therefore, physicians should encourage caregivers to have their children engage in physical activity after optimizing training methods, dietary patterns, and environmental factors. Training balance, flexibility, motor coordination is mostly independent of symptoms of RD and GERD and should be encouraged as early as possible, since motor skills at a young age determine physical activity in later life. Endurance and muscular strength should be continuously promoted at a comfortable, but still challenging, level of exertion to improve fun and commitment and ensure an active and healthy life style for children and adults with EA. The effectiveness of such interventions should be assessed in future prospective studies.
The raw data of this study are available upon request from the lead author (TK). Reference values and test protocols for
The ethics board of the state of Rhineland-Palatinate was consulted and approved of the study, exempting it from formal review due to exclusively anonymized data and minimal risk to the participants. All caregivers received information on the study and signed an informed consent.
The study was planned, conducted, and managed by the first author (TK) with support of the second author (OM). Both authors were directly involved in the drafting, writing, and editing of this manuscript.
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.
The authors would like to thank the KEKS e.V. office for their support with the application process of the patients. Furthermore, we thank Dr. med. Karsten Laabs, Dr. med. Konstantin Folkert, and Marco Strobel for performance assessment during the study.
body mass index
body mass index- standard deviation score
congenital heart disease
Deutscher Motorik Test
esophageal atresia
forced exercise capacity in one second
gastroesophageal reflux disease
“Patienten- und Selbsthilfeorganisation für Kinder & Erwachsene mit kranker Speiseröhre“ national German support group for patients with esophageal atresia
Kinderturntest Plus
performance percentile
respiratory distress
standard deviation
maximal oxygen uptake
World Health Organization.