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To evaluate the outcome of the operated children of esophageal atresia (EA) focusing on their early and late morbidity and mortality and quality of life (QoL) of survivors.
A cross-sectional follow-up with retrospective analysis of available medical and surgical records of children who underwent repair for EA.
The medical records of the children who underwent repair for EA during the period from 2000 to 2011 at the Christian Medical College Hospital, Vellore, were collected retrospectively. Patients with parents were invited to visit the hospital for follow-up and nutritional status, digestive and respiratory symptoms, status of associated anomalies and QoL assessment of children done. QoL assessment was done using the PedsQL™ 4.0 generic core scales questionnaire comprising 4 scale scores: physical, emotional, social functioning, and school functioning. Mean scores are calculated based on a 5-point response scale for each item and transformed to a 0-100 scale with a higher score representing better QoL.
Statistical Package for Social Sciences (SPSS) version 16 using Chi-square or Fisher's exact test.
Of 79 patients operated during the said period, there were 10 deaths and a total of 69 (87%) children survived. Of the 66 patients available for follow-up, we interviewed 30 parents and children while for the remaining 36 children, out-patients charts were reviewed retrospectively. Mean follow-up duration was 3.56 years. The height and weight for age measurement showed 47% and 56% of children respectively as below the 5th percentile. Main problems faced by operated EA children were of the respiratory (26%) and gastroesophageal (36%) tracts. In spite of the mentioned problems faced, the overall QoL of this group appeared good. In 23 of 30 patients, who answered PedsQL™, more than 70% had scores >85 out of 100 in QoL scoring.
While survivals of the children born with EA have improved, these children still face nutritional, respiratory, and gastroesophageal problems during their early childhood. In spite of this, the overall QoL of this patient group appears good.
With advancements in the fields of neonatal surgical techniques and materials, neonatal intensive care, anesthesia, ventilatory and nutritional support and antibiotics, the survival of babies with this anomaly has reached up to 90% over recent decades, in specialized centers. The outcome of EA in a resource constrained country with heterogeneous large population needs to be ascertained. Earlier studies, done mostly in developed countries, have assessed morbidity and quality of life (QoL) of adult survivors of EA. The studies on pediatric age group are few.[5,6,7]
The aims of our study are to evaluate the morbidity and QoL of operated children of EA, along with their demographic and socioeconomic profile and to assess the effect of long-term complications on QoL of these children. It also provides a comprehensive database of this group of patients, which can be utilized for optimal follow-up care and further research in this field.
This study is a cross-sectional follow-up with retrospective analysis of available medical and surgical records of children who underwent repair for EA with or without tracheoesophageal fistula (EA ± TEF) during from 2000 to 2011 at the Christian Medical College and Hospital, Vellore, Tamil Nadu, India. The study also includes those children who were referred to our institute for the definitive surgical procedure after primary diversion or failed surgical attempts elsewhere.
The study has been done over a period of 2 years (2011-2012) involving total 79 children.
The birth-related details of the children were collected retrospectively from surgical and medical records. This included demographic data, the age of gestation, type of delivery, type of EA, associated anomalies, surgical details, and postoperative record.
Patients’ contact addresses and phone numbers were collected from medical records, and they were invited to visit the hospital for follow-up. On this visit, they were assessed for nutritional status, digestive and respiratory symptoms, status of associated anomalies and QoL. The data of patients who were not available for this follow-up visit was obtained from medical records of their last follow-up visit.
Nutritional status and growth were assessed by collecting data on weight and height and comparing the same with Christian Medical College Hospital's standard weight-for-age and height-for-age percentile chart, respectively.
Gastroesophageal symptoms included dysphagia, food bolus impaction, duration of meals, abdominal pain, symptoms of gastroesophageal reflux (regurgitation, heartburn). Dysphagia, gastroesophageal reflux, and anastomotic strictures were also assessed by barium swallow study.
Respiratory symptoms included a recurrent cough, wheeze, asthma, shortness of breath, and respiratory infections.
Associated anomalies status – children were assessed for the clinical status of associated anomalies, when present.
QoL assessment was done by the PedsQL™ 4.0 generic core scales questionnaire.[8,9,10] PedsQL™, a 23-item measurement model for health-related QoL in children aged 2-18 years, was applied in child self-report (from age 5 years and above) and parent proxy-report (from age 2 years and above) versions to assess physical, emotional, social, and school functioning. The questionnaire provides information on the physical, emotional, social, and school functioning of the child for the previous 4 weeks. Mean scores are calculated based on a 5-point response scale for each item and transformed to a 0-100 scale with a higher score representing better QoL. The PedsQL™ gives 3 summary scores: A total scale score, a physical health summary score, and a psychosocial health summary score. There are 4 scale scores: Physical functioning, emotional functioning, social functioning, and school functioning. The total score is comprised the average of all items in the questionnaire. The psychosocial summary score is comprised the average of the items in the emotional, social, and school functioning scales. The physical health summary score is comprised the average of items in the physical functioning scale and is the same score as the physical functioning score.
The PedsQL questionnaire was completed during follow-up visit and via telephonic interview by the parent/and or child after appropriate introduction for completion of the questionnaire was given by investigator. As the guidelines for administration of the PedsQL recommend, children, and parents completed the questionnaire independently. In addition, as recommended, children between 5 and 7 years of age completed the questionnaire with the assistance of investigator.
Family demographic details including socioeconomic status and education of parents were also recorded.
Data entry was done using Microsoft Excel. Data analysis was done using Statistical Package for Social Sciences (SPSS) version 16 (SPSS for Windows, Version 16.0. Chicago, SPSS Inc).
Aim and purpose of the study were informed to the parents and children and their informed consents were obtained. The data collection was done after obtaining approval from the Unit Heads of Department of Paediatric Surgery and Medical Records Department, Christian Medical College and Hospital, Vellore. Participants’ identity and all the data were kept confidential. The study was approved by Institutional Review Board for publication.
A total of 79 patients were operated for EA with or without TEF from the year 2000 to 2011. The male:female ratio was 41:38. Mean birth weight (based on 45 available records) was 2.31 kg (range 1.22-3.60 kg). Gestational age data were available for 53 patients with 12 patients being born prematurely. There were 64 (81%) babies with Type C, 12 (15%) with Type A, one with Type B, and two with Type E EA. The data for associated anomalies at birth were available for 71 patients [Table 1]. 40/71 (56%) babies had at least one significant associated anomaly with cardiovascular anomalies being the most common.
There were 21 (26.6%) patients, who were detected to have pneumonia during preoperation or early postoperation period. A total of 10/79 patients (12.65%) died, of which only one patient had Type A EA while rest all had Type C EA [Table 2].
A total of 69 out of 79 patients (87%) in our study group survived. 36 of the surviving patients were male. The mean birth weight (based on 38 available records) was 2.38 kg (range 1.22-3.60 kg). Gestational age data were available for 44 patients with 8 patients in survivors’ group were born prematurely. Fifty-one of the 69 patients underwent primary repair, 13 patients underwent esophageal replacement with stomach as conduit (12 as retrosternal gastric pull up and one by reverse gastric tube transposition), 5 went through cervical esophagostomy and feeding gastrostomy (definitive procedures yet to be done), and one had re-do repair after unsuccessful primary repair elsewhere. Twelve patients had anti-reflux surgery later, in form of fundoplication. 10 children had anastomotic site stricture which required dilatations, 2 of this category had required resection while one underwent gastrostomy.
The socioeconomic background was assessed by parental education and their monthly income and shown in Table 4.
We could contact 30 out of the 69 (43.5%) surviving children and their parents in person with follow-up assessment done by face-to-face interview in 24 cases and by telephonic/internet conversations in 6. For rest of the patients, medical records of their last outpatient department visit were assessed and analyzed, excluding the three patients who never came back for a follow-up. A total of 66 patients were available for follow-up.
Sixty-six patients were followed up after surgery with the youngest patient being 1-month-old and the oldest being 12 years. The mean age at follow-up was 3.85 years (standard deviation 3.08 years) while mean follow-up duration was 3.56 years (standard deviation 2.84 years). The difference in age at follow-up and follow-up duration is due to referred patients of older age in the study.
Weight and height data at follow-up were available for 38 (58%) and 35 (53%) patients, respectively. On putting weight and height data on Standard Growth Percentile chart (Christian Medical College and Hospital), majority of patients were within the 50th percentile of standard [Figure 1]. Overall, in our study, population majority of children were found to be undernourished at follow-up.
Respiratory symptoms were present in 26% (17/66) of patients.
Main respiratory problems during follow-up were recurrent cough and cold (12/66, 18%), recurrent pneumonia (10/66, 15%), wheeze (4/66, 6%), and asthma (4/66, 6%).
Gastroesophageal morbidity after repair of EA was recorded in 24/66 (36%) patients. Main gastroesophageal symptoms included dysphagia that was found in 6/66 (9%) patients and gastroesophageal reflux in 6/66 (9%) while 12 patients had already underwent antireflux (fundoplication) surgery in past before this follow-up. Anastomotic site stricture was recorded in 10/66 (15%) patients, 8 of whom were treated before this follow-up using dilatation and surgical measures when indicated. There were 6 children who suffered food bolus impaction at least once, and they had to undergo removal under esthesia.
One infant presented with failure to thrive with gastroenteritis.
No patient had major thoracic deformity. Parental concern about scar was not apparent in our study. Parents of 5/30 patients noticed asymmetry of the chest.
Two of the children underwent cardiac surgery during the follow-up period, one in our center (Atrial septal defect device closure) and other in another center (details not known) at 5 years and 4 months of age, respectively.
Age appropriate PedsQL™ 4.0 generic core scales questionnaire for QoL assessment was done for 23 patients.
Figure 2 shows mean QoL scores. For further comparison, QoL scores have been divided in three categories of <50, 50-85 and more than 85. For statistical analysis, we combined the parent and child scores. In our study group, majority of the QoL scores are in the highest category of >85 as shown by the chart [Figure 3].
To assess the influence of various factors such as gender, gestational age, types of EA, associated anomalies and parental socioeconomic status on QoL, the QoL scores are compared, and statistical significance tested using Chi-square or Fisher's exact test. Significance was set at P < 0.05. Presented data show combined parent-proxy and child self-assessment. In our study, it was found that the impact of the above-mentioned factors on QoL scores was not statistically significant [Table 5].
Sixty-six patients, who had come at least once for follow-up, were assessed for early and late morbidity and/or QoL assessment in this study.
The distribution of different types of EA was broadly in line with the known distribution pattern, with EA and distal TEF being most common.
We found no significant sex preponderance in incidences or survival of patients with EA. Associated anomalies were found in 40/71 (56%) patients, with cardiovascular anomalies being most common (45%). Stoll et al. recorded 46.5% associated anomalies in their cohort of 99 patients of EA from France with cardiovascular anomalies being majority (24%). van Heurn et al. published the comparison between Asian and European populations and found that the percentage of patients with at least one associated anomaly was 50% and 74%, respectively. From Indian study by Tandon et al., associated congenital anomalies were found in 52/127 (41%) patients, the most common being the cardiac anomalies followed by the gastrointestinal anomalies. In our study, VACTERL association found in 7/71 cases (9.85%). Spitz found similar 10% association in his 40 years study.
Survival in our study was 87% which is an improvement from the studies done in the past in India by Krishna et al., Sharma et al. and more recently by Tandon et al. Associated cardiac anomalies, pneumonia, low birth weight, and prematurity were major contributing factors in mortality.
Our study was a comprehensive cross-sectional and retrospective assessment of the nutritional status, digestive and respiratory morbidity and QoL of operated children with EA with or without TEF. Only a few studies in past have addressed such comprehensive mid-term outcome in children.[18,19]
In our study, mean follow-up duration was 3.56 years with mean age of follow-up being 3.85 years, thus giving early insight into the health status of operated children of EA.
In our cohort of children, majority were under 50th percentile of standard growth chart (87% in weight for age and 69% in height for age category). Findings are similar to study by Little et al. in which almost half of children showed growth <25th percentile in early follow-up (<5 years).
Respiratory symptoms were present in 26% (17/66) of patients. Main respiratory morbidities were recurrent cough and cold, wheeze, recurrent pneumonia, and bronchial asthma. The literature shows respiratory symptoms from 10% to 61% in various studies by authors Bouman et al. (10%), Somppi et al. (18%), Chetcuti et al. (24%), Little et al. (29%), Koivusalo et al. (32%), Ure et al. (60%), and Legrand et al. (61%).[18,19,20,21,22,23,24]
Legrand et al. found only 39% of children who were without respiratory symptoms in their study group with others having chronic cough, barky cough, and dyspnea on exertion as main symptoms. Lilja and Wester noted frequent respiratory symptoms such as shortness of breath, frequent coughing, respiratory infections, and impaired exercise capacity in all age groups in their study of long-term outcome. Taylor et al. reported asthma, bronchitis, pneumonia, and persistent cough as respiratory morbidity in their 32% patients. Little et al. reported respiratory infections in 29% of their study group during first 5 years of follow-up. Koivusalo et al. reported respiratory symptoms occurring significantly more often in EA patients as compared to controls with the presentation being shortness of breath, frequent coughing, frequent respiratory tract infections, and copious mucus secretion in airways. Similar findings were noted in other studies.
Frequent respiratory symptoms in operated EA warrants a careful long-term monitoring and appropriate management in these group of children. As pulmonary function studies were not done in our study due to young age of patients, the extent of respiratory morbidity in EA as found here may be an underestimate.
Overall gastroesophageal morbidity after repair of EA was recorded in 24/66 (36%) patients in the study population. Main presentations were dysphagia, gastroesophageal reflux, and anastomotic stricture. Past studies showed gastroesophageal symptoms in 10-81% patients as by Bouman et al. (10%), Ure et al. (22%), Somppi et al. (29%), Chetcuti et al. (46%), Koivusalo et al. (39%), Little et al. (48%), and Legrand et al. (81%).[18,19,20,21,22,23,24]
Legrand et al. reported that only 19% of their study group of operated EA Type III children were free of digestive symptoms and dysphagia and gastroesophageal reflux were most common symptoms in symptomatic patient. Little et al. found dysphagia and gastroesophageal reflux disease (GERD) in 45% and 48%, respectively in their study. This finding of significant gastroesophageal morbidity emphasizes the importance of regular and proactive follow-up and interventions, when required, in operated patients of EA.
The QoL scores were found to be overall good in our study group. The QoL scores for factors such as gestational age, gender, associated anomalies; parental socioeconomic status and type of EA (as well esophageal replacement) were compared within the group. None of them were found to significantly influence the QoL scores based on statistical analysis. A larger participation in such assessment by parents and children and longer follow-up is needed to confirm this observation. Legrand et al. used the same tool to assess QoL in assessing the long-term outcome of children with EA Type III and found significant lower QoL than in healthy children. Bouman et al. found that operated EA children were at increased risk of learning, emotional, and behavioral problems compared to healthy children. Somppi et al. recorded impaired QoL due to respiratory infection, dyspnea, night cough, and dysphagia in one-third of their study patients.
Majority of other studies have reported good QoL in operated cases of EA. A recent study by Dingemann et al. evaluated long-term health-related QoL after complex and/or complicated EA in adults and children registered in a German patient support group and found excellent health-related Qol in children. Chetcuti et al. studied the adult survivors of EA and reported normal QoL in almost all patients. Ure et al. conducted study 58 patients comprising fifty patients of primary anastomosis and 8 patients with colonic interposition. They reported excellent QoL in primary anastomosis patients and acceptable QoL in colonic transposition patients. All children with Type A EA underwent replacement using stomach as conduit in our study. QoL score compared between Type A and Type C EA children and no statistical difference could be found among these groups [Table 5].
Table 6 enlists a summary of follow-up studies and findings on QoL.
The survival of operated children of EA with/without TEF has improved significantly. Associated cardiac anomalies, pneumonia, low birth weight, and prematurity were major contributing factors in mortality. Operated EA children show poor growth at least during early years of their life. Main problems faced by operated EA children are respiratory and gastroesophageal namely recurrent cough and cold, pneumonia, reactive airway, dysphagia, and GERD. In spite of this, the overall QoL of this patient group appears good, although a larger study with longer follow-up is needed to confirm this. Factors such as gender, gestational age, associated anomalies, type of EA, esophageal replacement, and parental socioeconomic background do not appear to significantly affect the QoL.
In view of problems faced by significant number of children in this group, a regular life-long follow-up protocol is needed to monitor their overall health. Such follow-up should cover nutritional, respiratory, and developmental assessment and also carry out other necessary evaluation depending upon problems associated with individual child. Further, parental education regarding care of operated EA children cannot be overemphasized. Parents should ensure that the child takes small frequent meals, takes smaller bites, and chews slowly and thoroughly. The child should be in an upright position while eating and for a while afterward. Parents should also be made aware regarding the risks of respiratory infections and choking, the measures to prevent them and should be encouraged to seek early medical help in such conditions. Hence, a regular follow-up and proper parental care are the important factors in improving the QoL of an operated EA child.
There are no conflicts of interest.