Background
Methods
Ethics
Setting
Study participants
Children with DREA
Comparison group; children with Gross type C who underwent primary anastomosis.
Data collection
Clinical data
Parent characteristics
Generic HRQOL
Condition-specific HRQOL
Data analysis
Results
Study participants
Congenital and parent-proxy characteristics
Children aged 2–7 years: n(%) | Children aged 8–18 years: n(%) | |||||||||
---|---|---|---|---|---|---|---|---|---|---|
Child charateristics | ntot | Delayed reconstruction | ntot | Primary anastomosis | p-value | ntot | Delayed reconstruction | ntot | Primary anastomosis | p-value |
Child gender male | 8 | 5 (62.5) | 42 | 27 (64.3) | 1.0 | 22 | 10 (45.5) | 64 | 34 (53.1) | 0.62 |
Gestational age weeks, median (range) | 8 | 36 (26–37) | 42 | 37 (31–41) | 0.096 | 22 | 36 (24–40) | 62 | 38 (28–43) | 0.002 |
Prematurely born (< 37 gestational weeks at birth) | 8 | 5 (62.5) | 42 | 19 (45.2) | 0.46 | 22 | 13 (59.1) | 62 | 17 (27.4) | 0.010 |
Birth weight grams, median (range) | 8 | 2202 (590- 2855) | 41 | 2653 (1614–4260) | 0.014 | 22 | 2312 (525–3225) | 62 | 2720 (1070–3390) | 0.030 |
Low birth weight (< 2500 g at birth) | 8 | 7 (87.5) | 41 | 21 (51.2) | 0.12 | 22 | 13 (59.1) | 62 | 18 (29.5) | 0.020 |
Gross type esophageal atresia | 8 | A = 3 (37.5) B = 1 (12.5) C = 4 (50.0) | 42 | C = 42 (100) | – | 22 | A = 9( 40.9) B = 7( 31.8) C = 6 (27.3) | 64 | C = 64 (100) | – |
Associated anomaliesa | 8 | 8 (100) | 42 | 24 (57.1) | 0.039 | 22 | 12 (54.5) | 64 | 39 (60.9) | 0.62 |
Cardiovascular | 8 | 5 (62.5) | 42 | 9 (21.4) | 0.030 | 22 | 5 (22.7) | 64 | 22 (34.4) | 0.43 |
Anorectal | 8 | 2 (25.0) | 42 | 6 (14.3) | 0.60 | 22 | 5 (22.7) | 64 | 4 (6.3) | 0.044 |
Urogenital | 8 | 4 (50.0) | 42 | 4 (9.5) | 0.016 | 22 | 7 (31.8) | 64 | 8 (12.5) | 0.053 |
VACTERL associationb | 8 | 3 (37.5) | 42 | 6 (14.3) | 0.14 | 22 | 3 (13.6) | 64 | 11 (17.2) | 1.0 |
Verified genetic disorder | 8 | 1 (12.5) | 42 | 3(7.1) | 0.51 | 22 | 4 (18.2) | 64 | 7 (10.9) | 0.46 |
Reason for delayed reconstruction | ||||||||||
Initital gap length, median cm (range) | 3 | 4 (2–7) | ||||||||
Initital gap length, median vertebral bodies (range) | 3 | 4 (3.5–4) | 19 | 4 (2–6)c | ||||||
Degree of prematurity, median (range) | 2 | 29 (26–31) | 3 | 29 (24–30) | ||||||
Characteristics of parent acting as proxy | ||||||||||
Parent respondent mother | 8 | 5 (62.5) | 42 | 37 (88.1) | 0.11 | 22 | 18 (81.8) | 63 | 56 (88.9) | 0.46 |
Parental age median (range | 8 | 40 (35–47) | 42 | 37 (26–46) | 0.09 | 22 | 46 (33–58) | 63 | 44 (33–69) | 0.26 |
Cohabitant partner | 7 | 6 (85.7) | 42 | 38 (90.5) | 0.55 | 22 | 21 (91.5) | 63 | 50 (79.4) | 0.10 |
University/College education | 8 | 4 (50.0) | 42 | 23 (54.8) | 1.0 | 22 | 13 (59.1) | 63 | 31 (49.2) | 0.47 |
Initial surgical treatment
Postoperative morbidity
Early postoperative complications
Children aged 2–7 years: n(%) | Children aged 8–18 years: n(%) | |||||||||
---|---|---|---|---|---|---|---|---|---|---|
ntot | Delayed reconstruction | ntot | Primary anastomosis, Gross type C | p-value | ntot | Delayed reconstruction | ntot | Primary anastomosis, Gross type C | p-value | |
Revisional surgery after repair of EA eg due to anastomotic leakage or recurrent fistula | 8 | 1 (12.5) | 42 | 3 (7.1) | 0.51 | 22 | 4 (18.2) | 62 | 7 (10.9) | 0.46 |
Anastomotic leakage | 8 | 1 (12.5) | 42 | 2 (4.8) | 0.41 | 22 | 8 (36.4) | 62 | 8 (12.9) | 0.026 |
Sepsis verified through blood culture | 8 | 4 (50.0) | 42 | 8 (19.0) | 0.082 | 22 | 6 (27.3) | 62 | 6 (9.7) | 0.071 |
Wound infection | 8 | 1 (12.5) | 42 | 1 (2.4) | 0.30 | 22 | 1 (4.5) | 62 | 2 (3.2) | 1.0 |
Pneumothorax treated with drainage | 8 | 3 (37.5) | 42 | 2 (4.8) | 0.024 | 22 | 3 (13.6) | 62 | 11 (17.7) | 0.75 |
Esophageal dilatation before hospital discharge | 5 | 2 (20.0) | 42 | 1 (2.4) | 0.20 | 20 | 2 (10.0) | 60 | 8 (13.3) | 1.0 |
Days before discharge from tertiary pediatric surgical care, median (range) | 8 | 230(69–1235) | 42 | 31 (4–231) | < 0.0001 | 22 | 188 (62–364) | 59 | 35 (19–464) | < 0.0001 |
Symptoms and treatment at follow-up
HRQOL
Generic HRQOL
Condition-specific HRQOL
Factors associated with lower HRQOL scores
Physical functioning | Emotional functioning | Social functioning | ||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Symptoms the past four weeks | n, yes | Median(range) | n, no | Median(range) | p-value | n, yes | Median(range) | n, no | Median(range) | p-value | n, yes | Median(range) | n, no | Median(range) | p-value | |
Generic health-related quality of life | ||||||||||||||||
Child-Report | Swallowing difficulties | 5 | 59.4 (40.6–100) | 15 | 93.8 (65.6–100) | 0.21 | 5 | 70.0 (60.0–100) | 15 | 90.0 (65.0–100) | 0.37 | 5 | 70.0 (50.0–100) | 15 | 100 (50.0–100) | 0.16 |
Heartburn | 5 | 87.5 (40.6–93.8) | 15 | 100 (56.3–100) | 0.054 | 5 | 85.0 (65.0–100) | 15 | 95.0 (60.0–100) | 0.35 | 5 | 95.0 (50.0–100) | 15 | 100 (50.0–100) | 0.26 | |
Cough | 7 | 87.5 (40.6–100) | 13 | 100 (65.6–100) | 0.046 | 7 | 85.0(60.0–100) | 13 | 95.0 (65.0–100) | 0.29 | 7 | 95.0 (50.0–100) | 13 | 100(50.0–100) | 0.21 | |
Airway infections | 5 | 84.4 (40.6–93.8) | 15 | 100 (59.4–100) | 0.025 | 5 | 70.0 (60.0–100) | 15 | 95.0 (65.0–100) | 0.075 | 5 | 70.0 (50.0–100) | 15 | 100 (55.0–100) | 0.020 | |
Parent-report | Swallowing difficulties | 5 | 56.3 (56.3–100) | 17 | 98.4 (31.3–100) | 0.091 | 5 | 80.0 (55.0–100) | 17 | 95.0 (30.0–100) | 0.35 | 5 | 67.5 (60.0–100) | 17 | 95.0 (20.0–100) | 0.36 |
Heartburn | 6 | 57.8 (31.3–87.5) | 16 | 100 (56.3–100) | 0.003 | 6 | 70.0 (30.0–100) | 16 | 95.0 (55.0–100) | 0.041 | 6 | 67.5 (20.0–100) | 15 | 95.0 (60.0–100) | 0.084 | |
Cough | 8 | 57.8 (31.3–87.5) | 13 | 100 (65.6–100) | < 0,001 | 9 | 80.0 (30.0–100) | 13 | 95.0 (70.0–100) | 0.026 | 8 | 67.5 (20.0100) | 13 | 100 (70.0–100) | 0.036 | |
Airway infections | 6 | 57.8 (31.2–100) | 15 | 100 (56.3–100) | 0.017 | 6 | 57.5 (30.0–100) | 16 | 95.0 (70.0–100) | 0.096 | 6 | 60.0 (20.0–100) | 15 | 95.0 (70.0–100) | 0.009 |
School functioning | Total PedsQL 4.0 scores | ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|
Symptoms the past four weeks | n, yes | Median(range) | n, no | Median(range) | p-value | n, yes | Median(range) | n, no | Median(range) | p-value | |
Generic health-related quality of life | |||||||||||
Child-Report | Swallowing difficulties | 5 | 60.0 (55.0–100) | 11 | 90.0 (40.0–100) | 0.40 | 5 | 60.9 (52.2–100) | 15 | 87.0 (63.0–100) | 0.19 |
Heartburn | 5 | 60.0 (55.0–65.0) | 15 | 90.0 (40.0–100) | 0.027 | 5 | 84.8 (52.2–87.0) | 15 | 94.6 (61.0–100) | 0.044 | |
Cough | 7 | 60.0 (55.0–100) | 13 | 94.6 (63.0–100) | 0.077 | 7 | 84.8 (52.2–100) | 13 | 93.5 (78.3–100) | 0.080 | |
Airway infections | 5 | 55.0 (40.0–65.0) | 15 | 90.0 (40.0–100) | 0.006 | 5 | 63.0 (52.2–87.0) | 15 | 94.6 (60.0–100) | 0.014 | |
Parent-report | Swallowing difficulties | 5 | 60.0 (55.0–100) | 17 | 55.0 (15.0–100) | 0.61 | 5 | 69.6 (56.5–100) | 17 | 85.9 (27.2–100) | 0.19 |
Heartburn | 6 | 55.0 (15.0–75.0) | 16 | 87.5 (35.0–100) | 0.009 | 6 | 64.1 (27.2–85.9) | 16 | 91.8 (56.5–100) | 0.009 | |
Cough | 9 | 60.0 15.0–100) | 13 | 90.0 (35.0–100) | 0.017 | 9 | 69.6 (27.2–100) | 13 | 93.5 (78.3–100) | 0.007 | |
Airway infections | 6 | 45.0 (15.0–60.0) | 16 | 87.5 (50.0–100) | 0.001 | 6 | 57.6 (27.2–85.9) | 16 | 91.8 (69.6–100) | 0.004 |
Eating | Social relationships | Body perception | ||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Symptoms the past four weeks | n, yes | Median(range) | n, no | Median(range) | p-value | n, yes | Median(range) | n, no | Median(range) | p-value | n, yes | Median(range) | n, no | Median(range) | p-value | |
Condition-specific health-related quality of life | ||||||||||||||||
Child report | Swallowing difficulties | 5 | 43.8 (17.9–59.4) | 14 | 81.3 (53.1–100) | 0.002 | 5 | 66.1 (57.1–85.7) | 14 | 91.1 (42.9–100) | 0.048 | 5 | 90.0 (75.0–95.0) | 14 | 95.0 (80.0–100) | 0.13 |
Heartburn | 5 | 75.0 (17.9–84.4) | 15 | 78.1 (43.8–100) | 0.29 | 5 | 73.2 (57.1–89.3) | 15 | 89.3 (32.1–100) | 0.27 | 5 | 85.0 (75.0–100) | 15 | 95.0 (60.0–100) | 0.30 | |
Cough | 7 | 59.4 (17.9–84.4) | 13 | 81.3 (43.8–100) | 0.080 | 6 | 73.2 (57.1–89.3) | 13 | 92.8 (32.1–100) | 0.13 | 7 | 95.0 (75.0–100) | 13 | 90.0 (60.0–100) | 0.54 | |
Airway infections | 5 | 59.4 (17.9–84.4) | 15 | 78.1 (40.6–100) | 0.26 | 5 | 66.1 (42.9–89.3) | 15 | 89.3 (32.1–100) | 0.11 | 5 | 95.0 (80.0–100) | 15 | 90.0 (60.0–100) | 0.86 | |
Parent-report | Swallowing difficulties | 5 | 50.0 (30.0–60.3) | 16 | 87.5 (50.0–100) | 0.002 | 5 | 74.1 (53.6–89.3) | 16 | 96.4 (42.9–100) | 0.15 | 5 | 80.0 (50.0–95.0) | 16 | 90.0 (65.0–100) | 0.51 |
Heartburn | 6 | 51.6 (30.0–96.9) | 16 | 84.4 (50.0–100) | 0.024 | 5 | 62.2 (42.9–71.4) | 16 | 94.6 (46.4–100) | 0.020 | 5 | 65.0 (50.0–80.0) | 16 | 92.5 (55.0–100) | 0.006 | |
Cough | 9 | 53.1 (30.0–96.9) | 13 | 90.6 (50.0–100) | 0.004 | 9 | 71.4 (42.9–89.3) | 13 | 100 (46.4–100) | 0.039 | 9 | 72.5 (50.0–95.0) | 13 | 90.0 (55.0–100) | 0.030 | |
Airway infections | 6 | 56.3 (30.0–96.9) | 16 | 84.4 (40.6–100) | 0.10 | 5 | 71.1 (46.4–89.3) | 16 | 94.6 (42.9–100) | 0.12 | 5 | 75.0 (65.0–95.0) | 16 | 90.0 (50.0–100) | 0.19 |
Health & well-being | Total EA-QOL | ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|
Symptoms the past four weeks | n, yes | Median(range) | n, no | Median(range) | p-value | n, yes | Median(range) | n, no | Median(range) | p-value | |
Condition-specific health-related quality of life | |||||||||||
Child report | Swallowing difficulties | 5 | 50.0 (43.8–87.5) | 14 | 90.6 (68.8–100) | 0.031 | 5 | 64.6 (45.3–78.1) | 14 | 88.5 (62.5–100) | 0.0046 |
Heartburn | 5 | 68.8 (50.0–93.8) | 15 | 87,5 (43.8–100) | 0.13 | 5 | 79.2 (45.3–90.6) | 15 | 85.4 (61.5–100) | 0.15 | |
Cough | 7 | 68.8 (43.8–93.8) | 13 | 87.5 (75.0–100) | 0.037 | 7 | 78.1 (45.3–90.6) | 13 | 86.5 (61.5–100) | 0.052 | |
Airway
infections | 5 | 69.8 (43.8–93.8) | 15 | 87.5 (50.0–100) | 0.046 | 5 | 64.6 (45.3–90.6) | 15 | 85.4 (54.2–100) | 0.097 | |
Parent-report | Swallowing difficulties | 5 | 56.3 (41.7–81.3) | 16 | 87.5 (62.5–100) | 0.011 | 5 | 64.6 (47.9–74.0) | 16 | 90.6 (55.2–100) | 0.013 |
Heartburn | 5 | 62.5 (41.7–81.3) | 16 | 87.5 (56.3–100) | 0.007 | 5 | 55.4 (47.9–80.1) | 16 | 90.1 (64.6–100) | 0.006 | |
Cough | 9 | 65.6 (41.7–81.3) | 13 | 87.5 (75.0–100) | 0.001 | 9 | 69.0 (47.9–80.2) | 13 | 92.7 (64.6–100) | 0.006 | |
Airway infections | 5 | 62.5 (41.7–87.5) | 16 | 84.4 (50.0–100) | 0.072 | 5 | 69.8 (55.4–80.2) | 16 | 90.1 (47.9–100) | 0.090 |
Spearman’s rho | ||||||
---|---|---|---|---|---|---|
Generic health− related quality of life | Gestational weeks at birth | Birth weight grams | Number of associated anomaliesa | Days to reconstruction | Days to discharge from tertiary pediatric surgical ward | Number of esophageal dilatations |
Children aged 2–7 years (parent− reports, n = 8) | ||||||
Physical functioning | − 0.38 | − 0.61 | − 0.83* | − 0.68 | − 0.76* | 0.074 |
Emotional functioning | − 0.61 | − 0.74* | − 0.80* | − 0.62 | − 0.68 | 0.11 |
Social functioning | − 0.15 | − 0.27 | − 0.72 | − 0.52 | − 0.78* | 0.12 |
School functioning | − 0.15 | − 0.31 | − 0.72 | − 0.46 | − 0.71 | 0.055 |
Total PedsQL 4.0 scores | − 0.34 | − 0.60 | − 0.80* | − 0.62 | − 0.79* | − 0.024 |
Children aged 8–18 years (child− reports, n = 20/parent− reports, n = 22) | ||||||
Physical functioning | 0.04/− 0.06 | 0.13/− 0.03 | − 0.40/− 0.36 | 0.18/0.23 | − 0.043/ − 0.007 | 0.042/0.37 |
Emotional functioning | − 0.12/0.15 | 0.20 /0.29 | − 0.052/− 0.25 | 0.14/− 0.46 | 0.063/− 0.16 | 0.053/0.33 |
Social functioning | 0.04/0.023 | 0.23/0.28 | − 0.15/− 0.05 | 0.21/0.04 | − 0.02/0.041 | − 0.12/0.14 |
School functioning | − 0.04/− 0.003 | 0.008/0.05 | − 0.25/− 0.29 | 0.051/− 0.08 | − 0.15/− 0.11 | 0.11/0.27 |
Total PedsQL 4.0 scores | − 0.07/0.04 | 0.03 /0.11 | − 0.37/− 0.37 | 0.14/0.06 | − 0.14/− 0.12 | 0.082/0.26 |
Condition− specific health− related quality of lifE | ||||||
Children aged 2–7 years (parent− reports, n = 8) | ||||||
Eating | − 0.67 | − 0.72 | − 0.81* | − 0.64 | − 0.71 | − 0.074 |
Physical health & treatment | − 0.39 | − 0.62 | − 0.80* | − 0.68 | − 0.74* | 0.024 |
Social isolation & stress | 0.38 | 0.10 | − 0.40 | − 0.30 | − 0.65 | − 0.077 |
Total EA− QOL | − 0.34 | − 0.54 | − 0.78* | − 0.57 | − 0.83* | − 0.073 |
Children aged 8–18 years (child− reports, n = 20/parent− reports, n = 22) | ||||||
Eating | 0.10/0.10 | 0.23/0.17 | − 0.10/− 0.12 | − 0.018/− 0.12 | − 0.17/− 0.24 | − 0.001/0.10 |
Social relationships | 0.10/0.13 | 0.12/0.028 | − 0.02/0.28 | 0.19/0.11 | − 0.10/− 0.04 | 0/0.02 |
Body perception | 0.21/0.15 | 0.25/0.037 | − 0.1/0.24 | 0.07/0.35 | − 0.18/0.08 | − 0.096/0.03 |
Health & well− being | 0.10/− 0.01 | 0.02 /− 0.03 | − 0.53*/− 0.35 | − 0.08/− 0.08 | − 0.37/− 0.28 | 0.21/0.37 |
Total EA− QOL | 0.13/0.07 | 0.24/0.09 | − 0.14/0.003 | 0.13/0.04 | − 0.12/− 0.14 | 0.07/0.17 |
Child-parent agreement regarding the child’s HRQOL
Intraclass correlation coefficients average measures | |
---|---|
Generic health-related quality of life (PedsQL 4.0) | |
Physical functioning | 0.80 |
Emotional functioning | 0.68 |
Social functioning | 0.61 |
School functioning | 0.87 |
Total PedsQL 4.0 scores | 0.76 |
Conditions-specific health-related quality of life (EA-QOL) | |
Eating | 0.88 |
Social relationships | 0.81 |
Body perception | 0.74 |
Health & well-being | 0.88 |
Total EA-QOL scores | 0.84 |