Author Affiliations: Pediatric Surgical Center of Amsterdam, Emma Children[[rsquo]]s Hospital AMC, and VU Medical Center (Drs Deurloo, Ekkelkamp, and Aronson), and Department of Medical Psychology, Academic Medical Center (Drs Hartman and Sprangers), Amsterdam, the Netherlands.
Long-term quality of life (QOL) in adults after correction of esophageal atresia (EA) is comparable with that of healthy adults.
Outcome study with a consecutive sample and follow-up after 16 years or longer.
Pediatric surgical center (academic center) in a tertiary hospital.
Patients and Methods
A questionnaire was sent to 119 consecutive surviving adults who were treated for EA between 1947 and 1986. The questionnaire was to assess sociodemographic characteristics and generic and disease-specific QOL and contained 3 open-ended questions about the consequences of the disease in daily life. Clinical characteristics were collected from patient case notes.
Ninety-seven (82%) of the 119 questionnaires were completed and returned. When comparing the generic QOL of patients who had EA with that of healthy subjects, we found no differences in overall physical and mental health. The presence of concomitant congenital anomalies did not influence generic QOL. A third of the patients reported that EA had had negative effects on their daily lives. Gastrointestinal symptoms such as dysphagia were most often mentioned (23%).
In general, after EA correction, patients perceive their generic and disease-specific QOL to be good. The presence of concomitant congenital anomalies did not influence generic QOL. However, a third of patients reported that the disease had had negative consequences.
Over the last 50 years, the survival rate of patients with esophageal atresia (EA) has increased from 0% to 90% or 95%. There is a growing interest in the long-term results following correction of EA. Up to now, most studies have focused on the functional outcome and on dysphagia and gastroesophageal reflux in particular.1- 4
Little is known about the influence of EA and its long-term sequelae on the quality of life (QOL) of the surviving patients. Although several studies mention QOL after correction of EA, most of these studies focus on the number of medical problems and on psychological development and call this quality of life.5- 9 Only 2 studies have used standardized and validated QOL questionnaires. In 1 of these studies, the QOL is examined together with the functional results from 8 patients after colon interposition for long-gap EA.7 The other study, from the same research group, examined the QOL in 58 patients more than 20 years after correction of EA.8 These results showed “acceptable” QOL after colon interposition and “excellent” QOL after primary repair of EA.
The first aim of our study was to investigate the generic QOL after correction of EA in a large adult population and compare it with the generic QOL of a healthy population.
The second aim was to investigate whether the type of EA is related to QOL. Patients with long-gap atresia (type A and B atresia) have usually undergone multiple surgical procedures and experience more postoperative problems. We therefore hypothesized that these patients have a poorer QOL than patients with other types of atresia.
The third aim was to investigate whether the presence of additional congenital anomalies influences the QOL. We hypothesized that patients with additional congenital anomalies have a poorer QOL than patients with none.
The fourth aim was to explore the QOL of patients with EA in greater detail by asking open-ended questions about the influence of EA on their daily life and to relate these responses to those given to the standardized questionnaires.
We included all patients older than 16 years. Between 1947 and 1986, 227 patients were treated for EA at the Pediatric Surgical Center of Amsterdam (the Netherlands). At the start of our study, there were 134 surviving patients. We used medical records, operative reports, and office notes to assess the medical history of each patient. Patients whose addresses could not be traced (n = 11) or who were not able to self-report their QOL because of mental retardation (n = 4) were excluded. After getting approval from the medical ethical committee, we sent questionnaires with stamped return envelopes to the remaining 119 patients.
Sociodemographic factors were assessed using structured questions on age; sex; marital status (married/living with partner, unmarried/divorced/living alone, living with others); number of children and their health (no children, children healthy, children not healthy); education (primary school, high school, higher education, university); and occupation (paid employment, unpaid job, student, unemployed). Clinical characteristics were collected from patient office notes. Long-gap atresia was defined as type A or B atresia, and non–long-gap atresia was defined as type C, D, or E atresia (Gross classifications10). Concomitant congenital anomalies were divided into anorectal, cardiac, renal, limb, or other malformations (VACTERL association).
We used a generic questionnaire to measure physical and mental functioning: the Medical Outcome Study 36-Item Short-Form Health Survey (SF-36),11 employing a 4-week time frame. The SF-36 measures 8 components of health, including physical functioning (Cronbach α in this study = .90), physical role functioning (α = .88), emotional role functioning (α = .85), bodily pain (α = .87), general health (α = .80), vitality (α = .88), social functioning (α = .73), and mental health (α = .81). We used the validated Dutch translation of the SF-36. The scores of the 8 domains were linearly transformed into scales ranging from 0 (worst health) to 100 (best health). The 8 domains were aggregated to a Physical Component Summary scale and a Mental Component Summary scale.
Positive affect was measured with the Positive Affect Scale.12 Patients were asked if 8 items were applicable to them. There are 4 categories of answers, ranging from “never” to “often.” The scores of these items were added up and linearly transformed into a scale ranging from 0 (worst) to 100 (best) (α = .86).
The Gastrointestinal Quality of Life Index (GIQLI)13 employs a 2-week time frame and contains 36 questions on various symptoms of gastrointestinal disease. Each question has 5 response categories, with 0 being the most negative option and 4 being the most positive option. There are 4 domains: physical well-being (α = .70), gastrointestinal symptoms (α = .84), social well-being (α = .52), and emotional well-being (α = .60). The responses to the questions were scored numerically, with a range from 0 (worst health) to 144 (best health). Because of the low α (<.60), we did not use the scales for social well-being and emotional well-being in further analysis.
The European Organisation for Research and Treatment of Cancer (Brussels, Belgium) developed an Esophageal Cancer Module questionnaire (EORTC-OES24) for patients with esophageal cancer.14 It uses a 1-week time frame. We used 19 of the 24 questions because some were specifically designed for patients being treated for cancer. It consists of 5 single-symptom items and 5 scales: dysphagia (α = .30), deglutition (α = .10), eating (α = .48), indigestion (α = .71), and pain (α = .50). The scores of the domains were linearly transformed into scales ranging from 0 to 100. For the symptom scales and items, a high score is equivalent to more severe or multiple symptoms; in the functional scales, a high score is equivalent to better functioning. Because of the low α (<.60), we did not use the dysphagia, deglutition, eating, and pain scales in further analysis.
The Illness Cognition Questionnaire (ICQ) was developed to assess 3 illness cognitions that reflect different ways of re-evaluating the inherently aversive character of a chronic condition.15 It contains 3 scales: helplessness (α = .88), acceptation (α = .62), and disease benefits (α = .89). There are 4 response options, ranging from “disagree” to “strongly agree.” Item scores are added up to a numerical score for the 3 scales.
The final 3 questions were open-ended. First, we asked whether there was anything that the patients wanted to do but could not do because of the treatment they had undergone for EA. Second, we asked whether it had had negative consequences on their life, and third, we asked whether it had had positive consequences on their life.
Differences in age, sex, type of atresia, and concomitant congenital anomalies between responders and nonresponders were tested by means of the χ2 test (discrete factors) or independent t test (continuous factors). To detect even small differences between responders and nonresponders, the level of significance was set at P<.10.
The means of the SF-36 scores of the groups examined were compared with reference values of comparable age groups from a Dutch validation study (n = 607)16 using independent t tests. To examine the magnitude of statistically significant differences, effect sizes were calculated. The effect size is the difference between the mean scores of each group divided by the standard deviation. Following Cohen’s method,17 effect sizes (d) of 0.20, 0.50, and 0.80 were considered to be small, medium, and large, respectively. For reasons of multiple testing, the level of significance was set at P<.01.
For the SF-36, GIQLI, EORTC-OES24, Positive Affect Scale, and ICQ, the outcomes of patients with and without long-gap atresia and patients with and without concomitant congenital anomalies were compared by means of independent t tests. Effect sizes were calculated for statistically significant differences (P<.01).
We first analyzed responses to the open-ended questions qualitatively by categorizing the responses into positive and negative answers. We also analyzed the specific answers that were given. For the SF-36, GIQLI, EORTC-OES24, Positive Affect Scale, and ICQ, the outcomes of patients with positive and negative answers were compared by means of independent t tests. Effect sizes were calculated for statistically significant differences (P<.01).
Analysis was performed using SPSS (Statistical Package for the Social Sciences) version 10.0.1 for Windows (SPSS Inc, Chicago, Ill).
Ninety-seven of the 119 questionnaires were completed and returned (a response rate of 82%). The sociodemographic and clinical characteristics of the responders are presented in Table 1.
Women responded more often (91%) than men (75%; P = .02). There was no significant difference in age, type of atresia, or presence of concomitant congenital anomalies between responders and nonresponders.
The results of the SF-36 questionnaire and the reference values are presented in Table 2. Patients with EA scored significantly lower than healthy subjects on the general health scale (d = 0.29) and the vitality scale (d = 1.71).
Only 4 patients had long-gap atresia, which made a comparison between patients with and without long-gap atresia impossible.
Table 3 outlines the results from patients both with and without concomitant congenital anomalies. Patients with concomitant congenital anomalies scored significantly lower on the indigestion scale (EORTC-OES24, d = 0.47). We found no significant differences among the various subgroups of concomitant congenital anomalies (eg, anorectal or cardiac malformations; data not shown).
In response to the question, “Is there anything that you would like to do that you cannot do because of the treatment you have undergone for EA?” 8 patients (8%) responded in the affirmative. Four of these reported they were not able to play certain sports or lift heavy objects. Two felt limited because of eating difficulties. One had a cosmetic problem with the operation scar, and 1 was limited in her daily activities because of a severe tracheal stenosis. The patients who indicated that they felt limited due to EA treatment had a significantly lower score on overall physical functioning (SF-36, d = 0.73), physical well-being (GIQLI, d = 1.50), the total score of the GIQLI (d = 0.92), and acceptation (ICQ, d = 0.60). They had a significantly higher score on helplessness (ICQ, d = 0.50; data not shown).
In response to the question, “Have you experienced negative consequences of EA in your daily life?” 32 patients (33%) responded in the affirmative. Gastrointestinal symptoms such as dysphagia were mentioned most often (22/32, 69%). Some patients complained of getting tired easily and having less stamina than other people (6/32, 19%). Other patients mentioned their scars (3/32, 9%). One patient even wrote, “Too many to write down.” The patients who indicated that they experienced negative consequences from EA had a significantly lower score on the overall physical functioning section (SF-36, d = 0.50), physical well-being (GIQLI, d = 0.75), gastrointestinal symptoms (GIQLI, d = 0.63), and the total score of the GIQLI (d = 0.75; data not shown).
In response to the question, “Have you experienced positive consequences of EA in your daily life?” 14 patients (14%) responded in the affirmative. Most of these patients felt grateful to be alive and had a positive outlook on life (7/14, 50%). One patient is working as a nurse in pediatric surgery and felt that her experience is very useful in her work. The patients who indicated that they experienced positive consequences had a significantly lower score on the domains of physical role (SF-36, d = 0.50) and acceptation (ICQ, d = 0.67). They had a significantly higher score on the domain disease benefits (ICQ, d = 1.00; data not shown).
This study measured the QOL following correction of EA in a group of 97 patients (16 to 48 years old). Until recently, little was known about the QOL in patients who had EA: only 2 studies have used standardized and validated QOL questionnaires.7,8 These studies measured QOL using a visual analogue scale, the Spitzer index,18 and an early 32-item version of the GIQLI.13 In this study, we measured QOL by means of several validated generic and disease-specific QOL questionnaires.
Information about the QOL after correction of EA can be used as information for parents of newborns with EA about the long-term consequences of the malformation for their children. It may also indicate which subgroups of these children have impaired QOL and may need extra medical or nonmedical treatment.
When comparing the generic QOL of patients who had EA with that of healthy subjects, we found no differences in the overall physical and mental health. However, patients did report worse general health and less vitality than healthy subjects. These differences may result from the fact that approximately a quarter of patients still have gastrointestinal problems, eg, dysphagia. Most patients were either employed and had finished high school (77%) or were in school full-time (31%). Marital and family status did not differ from that of the general Dutch population.19
Patients with concomitant congenital anomalies (35%) reported a lower QOL, but only in the domain indigestion (EORTC-OES24). This difference has a large effect size. It is not clear how the presence of a concomitant congenital anomaly influences the symptom of indigestion. We did expect to find a lower QOL in patients with concomitant congenital anomalies, but not specifically in this domain. It is possible that patients with associated congenital anomalies are more focused on their symptoms and therefore report indigestion more often.
The results of the exploratory questions showed that only a small number of patients felt limited because of their EA (8% of the patients indicated that they were not able to do whatever they wanted). However, 34% indicated that they experienced negative consequences of the EA in their daily lives such as gastrointestinal symptoms. As expected, patients who reported negative consequences in the exploratory questions perceived their QOL to be significantly impaired in several areas. Patients who reported positive consequences in the exploratory questions scored significantly higher on the domain disease benefits of the ICQ. Unexpectedly, these patients did report a lower score on the domain acceptation of the ICQ. We expected that patients who reported positive consequences would have a higher score on the domain disease benefits, as well as on the domain acceptation of the ICQ.
The number of nonresponders (22/119, 18%) is relatively low. In this type of study, a response rate of 82% is regarded as fairly good. There was no significant difference in age, type of atresia, or presence of concomitant congenital anomalies between responders and nonresponders. Unfortunately, nothing is known about their current state of health. It is possible that their nonresponse resulted from their poor QOL or dissatisfaction with their medical treatment in the past. Of course, it could also be possible that their QOL is so good that they did not bother to fill out lengthy forms.
Unfortunately, the number of patients with long-gap EA was too small to make a comparison between patients with and without long-gap EA. Larger numbers are needed to be able to study the QOL in patients with long-gap EA.
In conclusion, at long-term follow-up after correction of EA, patients perceived their QOL to be good. Compared with a healthy Dutch reference group, patients did have a lower score on the domains of general health (with a small effect size) and vitality (with a large effect size). The presence of concomitant congenital anomalies did not seem to influence the QOL. Patients who reported experiencing negative consequences perceived their QOL to be significantly impaired in several areas. Parents of newborns with EA can be reassured that the long-term outlook for their children with regard to their QOL is good.
Correspondence: Daniel C. Aronson, MD, PhD, Pediatric Surgical Center of Amsterdam, Emma Children’s Hospital AMC, PO Box 22660, 1100 DD Amsterdam, the Netherlands (firstname.lastname@example.org).
Accepted for Publication: December 7, 2004.
Previous Presentation: Part of this work was presented as a poster at the annual meeting of the International Society for Quality of Life Research; November 12-15, 2003; Prague, the Czech Republic.
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