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Quality of life more than 20 years after repair of esophageal atresia
Quality of Life More Than 20 Years After Repair of Esophageal Atresia By
B.M.
Ure,
E. Slany,
E.i?
Eypasch,
K. Weiler,
Cologne,
Purpose:To examine the quality geal atresia, follow-up studies surviving patients (81.7%).
of life after repair were performed
of esophain 58 of 71
Methods: Fifty patients with primary anastomosis and all eight surviving patients with colon interposition were seen. The mean age was 25.3 years (range, 20 to 311. Symptoms were evaluated by a standardized interview. Quality of life assessment was performed using a visual analogue scale (0 to 100 points), the Spitzer Index (5 dimensions, 10 points), and the Gastrointestinal Quality of Life Index (GIQLI, 5 dimensions, 128 points). /?esukAfter primary anastomosis the estimated meal capacrty was unrestricted in 46 patients (92%), but numerous symptoms such as recidivating cough (60%), hold up (48%), and short breath (30%) were reported. All symptoms except cough were seen more frequently in patients with colon interposition, and all of these patrents suffered from periods of short breath. Quality of life scores were higher in patients with primary anastomosis compared with colon interposition. The difference in the visual analogue scale score did not reach statistical significance, but the mean Spitzer Index was
T
HE LITERATURE on the long-term outcome of esophageal atresia has been devoted mainly to endpoints such as symptoms, esophageal function, and swallowing ability.rm5 Little is known about the real impact of gastrointestinal and pulmonary symptoms on the activities of daily living, and to our knowledge the quality of life after repair of esophageal atresia has not been investigated with validated instruments. Therefore, the aim of the present study was to determine the long-term quality of life in patients who underwent primary anastomosis or colon interposition for esophageal atresia.
MATERIALS
AND
METHODS
From 1963 to 1971 146 children were operated on for esophageal atresia m the Department of Pediatric Surgery of the Children’s Hospital of Cologne.6 Primary anastomosis was performed in 137 (93.8%) children; 63 (46%) of these survived. The mean age at primary anastomosis was 1.7 days (range, 0 to 6 days), one patient with atresia type II according to VogtT underwent delayed anastomosis at the age of 5 months. In nine children (6.2%) colon interposition was used for long gap atresia: eight of these (88.9%) survived. The mean age at colon interposttion was 15.1 months (range, 5 to 32 months), and all patients underwent retrostemal reconstruction. The tranverse colon was mterposed isoperistalttcally in three children and antiperistalttcally m one. .hurna/
ofPedfatr/c
Surgery,
Vol33,
No 3 (March),
1998: p&? 51 I-515
H. Troidl,
and
A.M.
Hokchneider
Germany
9.7 compared with 8.8 after colon interposition (P < .05). The GIQLI after primary anastomosis was similar to that rn healthy controls and was significantly lower in patients with colon interposition. This was because of specific symptoms, which scored 49.3 after colon interposition compared with 61.7 after primary anastomsis (P < .05) and to 54.8 (SD 5) in healthy controls (P< .05). Physical and social functions, emotions, and inconvenience of a medical treatment scored similar in patients with primary anastomosis, colon interposition, and healthyvolunteers. Cor~ck~sio~s; The long-term quality of life after primary anastomosis was excellent. Patients with colon interposition suffer more frequently from various gastrointestrnal and respiratory symptoms, but they lead an otherwise normal life. J Pediatr Surg 33:511-515. Copyright o 7998 by W.EL Saunders Company. INDEX WORDS: Esophageal atresia, interposition, long-term follow-up.
quality
of
life,
colon
The right colon with termmal ileum was interposed isoperistalttcally in one and antiperistaltically in three children. Fifty-eight patients (81.7%) underwent follow-up; one had been excluded because of mental retardation. Ftfty patients with primary anastomsis (79.4%) and all patients with colon interpositton were seen (Table 1). Twelve patients after primary anastomosis could not be traced and were lost to follow-up. The mean age at follow-up was 25.3 years (range. 20 to 31 years). One patient after primary anastomosis and two patients after colon interpositton were mentally retarded. They were able to communicate, and the parents helped with the interviews. The results of functional assessments that were performed in patients with colon interposition are published elsewhere,8 and the present analysts focused on symptoms and the quahty of life.
Assessme& of Symptoms The interview was performed by two intervtewers: one pedtatnc surgeon and one research fellow. A standardized questionnaire included 41 items and focused on gastrointestinal and pulmonary symptoms. The eating habits, swallowing ability, and long-term side effects of the
From the Department of Pedzatnc Surgery, The Chcldren’s Hospctal of Cologne, and the Department of Surge?, University of Cologne, Cologne, Gemany. Address reprint requests to Benno M. Ure, MD, PhD, Universcty Childrens Hospital “ Wdhelrnina, ” PO Box 18009, 3501 CA Utrecht, The Netherlands. Copyright 0 1998 by WB. Saunders Company 0022.3468/98/3303-0022$03.00/O 511
URE ET AL
512
Table
1. Characteristics After
Male/female Birth weight
In) (mean)
Assocrated malformations lmperforate anus Duodenal Cardial
of 58 Patients Esophageal
Who Underwent Atresia
Follow-Up
PMlW/ Anastomosrs bl = 50)
COlOll lnterposhx3
22128 2,666 g
711 3,081 g
4
-
(n)
atresia 2
Renal Skeletal Type of atresia
according
to Vogt7 fn)
Tw 1 Type II Type llla
-
?
-
-
48
2 1 5
1
Follow-up data Age (mean) Body weight Hight (mean)
(mean)
circumference
(mean)
26.3 yr
24 yr
64 kg 168cm 91 cm
63 kg 174cm 90 cm
operation were evaluated according to the criteria published by DeMeester et al9 The assessment of eating habits included the number of meals per day, the estimated meal capacity. and the reqmrements of liquids with meals. Patients graduated their complaints such as sensattons of hold up, pain or choking with swallowmg. nocturnal regurgitatrons, or others by Indicating whether they suffered from each specific symptom (1) all of the time, (2) most of the time, (3) some of the ttme, or (4) never. In addition, patients underwent a standardized physical examination. Finally, the patients together with both interviewers scored the result of the operation within three categories according to Ure et allo: (A) very good result/no complaints, (B) moderate result/ symptoms that the patient is willmg to tolerate. (C) poor result/therapy indicated.
Assessment of the Quality
Statistical Methods The biomedical data package PC-90 (BMDP Statistical Software, Cork, Ireland) was used. Univariate analysis was carried out by the Wilcoxon rank sum test and the x2 test with Yates correction as appropriate. A P value of less than .05 was considered stgmficant.
1
-
-
Type lllb Type lllc
1
2 13
Other
Thoracal
h = 8)
questions was scored from 0 to 4 points. The index was calculated by adding all points and the maximum of 128 points theoretically reflected unimpatred quality of life. For comparison we used the GIQLI of a series of 150 healthy individuals who underwent investigatron by Eypasch et al.14,1s
of Life
The quality of life measures were self report measures completed in private and not in the presence of the interviewers. Three instruments were used. The global quality of life was determined by a visual analogue sca1e.n Patients were asked to graduate their actual global quality of life on a loo-mm line anchored by two extremes: “very poor/my life is extremely unpleasant” and ‘*optimal/my life is normal.” The level of quality of life was scaled from zero to 100. The Spitzer Indexlz was used for the assessment of five items: (1) activity/involvement in own occupation, (2) activities of daily living, (3) health, (4) support of family and friends, and (5) outlook on life/future. Each item was assessed for the period “during the last week” and was scored from 0 to 2. The total range of scores of the Spitzer Index was 0 to 10. We used the version 4/1991 of the Gastrointestinal Quality of Life Index (GIQLI) which was established by Eypasch et all3 and includes 32 items. A Germant3,14 and an English versionts of the index is available, and the questions comprise the accepted dimensions of quality of life: symptoms (17 items), physical functions (seven items), emotions (five items). and social functions (two items). The inconvenience of a medical treatment is added. Symptoms include core symptoms such as pain, bloating, or restricted eating and diseasespecific items such as regurgitation, dysphagia, or others. Each of the 32
RESULTS
After primary anastomosis, 40 patients (80%) were working full time, seven (14%) were students, and three [6%) were unemployed. Seventeen (34%) of these patients were married and 13 (26%) had children. After colon interposition, five patients (62%) were working full time; two of these were married and had children. One patient was a student, and two were working in an institution for handicaped. Symptoms The estimated meal capacity9 was unrestricted in 46 (92%) of 50 patients after primary anastomosis. Twentyfour patients (48%) suffered at least “some of the time” from hold up, 12 (24%) from diarrhea, and 11 (22%) from heartburn (Fig 1). Other gastrointestinal symptoms were identified in less than 20% of the patients each. Respiratory symptoms were found more frequently; 30 patients (60%) reported on recidivating cough and frequent periods of bronchitis, and 15 (30%) were short of breath at least some of the time. After colon interposition, all gastrointestinal symptoms were documented more frequently (Fig 2). Five patients (62.5%) suffered from hold up and another five from diarrhea. All patients with colon interposition felt to be short of breath, five of these “all of the time.” The meal capacity was unrestricted in three patients (37.5%) with colon interposition. Qua&
of Life
The global quality of life score, the Spitzer Index, and GIQLI were higher in patients primary anastomosis compared with those with colon interposition (Table 2). The difference in the global quality of life score assessed by the visual analogue scale did not reach statistical significance. However, after primary anastomosis the mean Spitzer Index was 9.7 compared with 8.8 after colon interposition (P < .05). The GIQLI after primary anastomosis was similar to healthy controls, but there was a significant reduction in the GIQLI in patients with colon interposition compared with primary anastomosis (P =C.OOOl)and compared with healthy controls (P -C .05, Table 2).
QUALITY
OF LIFE AFTER
ESOPHAGEAL
ATRESIA
513
Primary
Anastomosis n=50
Cough Hold up Short breath Diarrhoea Heartburn Early satiety Bloating Regurgitation Bad breath Vomiting Pain Nausea 0
Fig I. Symptoms at follow-up of 50 patients after primary anastomosis. Patients indicated for each symptom whether they suffered from it “all of the time,” “most of the time,” “some of the time,” or “never.”
1 a
all
ik!@z@
some
The breakdown of the dimensions of the GIQLI showed significant differences for symptoms (Fig 3). Symptoms scored 49.3 (SD 10) after colon interposition compared with 61.7 (SD 4.7) after primary anastomosis (P < .OOOl) and compared with 54.8 (SD 5) in healthy controls (P < -05). No significant differences were found for physical functions, social functions, emotions, and inconvenience of a medical treatment between patients with primary anastomosis, colon interposition, and healthy controls. The result of the operation was classified as “poor.’ in
Colon
20 Number
10
of
the of
30 of Patients
time the
@@
most
D
never
tjrne
40
of
the
50
time
one patient because of a persistent stenosis of the esophagus. Subsequent bouginage was performed in another department. All other patients had an “optimal” result or were tolerating their symptoms without further therapy (Table 2). DISCUSSION
Many investigators have drawn attention to the longterm outcome of repair of esophageal atresia, and the majority agree that specific symptoms are mainly gastrointestinal and respiratory. 2,3.‘6Symptoms improve with
Interposition n=8
Short
breath Hold up Diarrhoea Bloating Early satiety Heartburn Regurgitation Bad breath Nausea Cough Pain Vomiting 0
2 Number a
all of
the
time
&%#
some
of the
time
4 of Patients m
most
m
never
6
of the
8
tlrne
Fig 2. Symptoms at follow-up of eight patients after colon interposition. Patients indicated for each symptom whether they suffered from it “all of the time,” “most of the time,” “some of the time,” or “never.”
WE
514
Table 2. Quality
of Life After
Primary Anastomos!s (n = 501 Mean (SD)
Method of Evaluation
Visual
Analogue
CObl lnterpositmn In = 81 Mean (SD)
Quality
80.1 (18.6) 9.7 (0.8)
Healthy controls
h = 1501 Mean (SD)
67.5 (18) 8.8 (2.1)*
-
92.2 (26,5H
107.6 (18.7)
36 (72%) 13 (26%)
3 (37.5%) 5 (62.5%)
1(2%1
-
-
of Life
Index GiQL113-15 (O-128 points) Classification
Atresia
Scale”
(O-100 points) Spitter lndexq2 (O-10 points) Gastrointestinal
Esophageal
111.5 (8)
of the outcome10
(n) A. Very good B. Moderate c. Poor
*P< .05 versus primary anastomosis. tP < .OOOl versus primary anastomosis
and P < .05 versus
healthy
controls.
time in patients with primary anastomosis and in patients with esophageal replacement.1-3,5,17 In the present series 60% of the patients after prima.ry anastomosis suffered from respiratory symptoms, mainly attacks of cough, frequent bronchitis, and short breath. This corresponds well with the report of Chetcuti and Phelan3 who found persisting respiratory symptoms in over half of 164 patients over 1.5 years of age. Annual bouts of bronchitis were documented in 41% and recurrent wheeze in 40%. The most frequent gastrointestinal symptom after primary anastomosis in the present series was hold up, which was reported by 48% of the patients; 22% had gastroesophageal reflux symptoms such as heartburn or regurgitation. Chetcuti and Phela$ found Gastromtestinal
Quality
of Life index
(mean)
70
Fig 3. Gastrointestinal Quality of Life Index (GIQLII version 4/1991 in 58 patients after esophageal atresia: symptoms (17 items), physical functions (seven items), emotions (five items), social functions (two items), inconvenience of a medical treatment (one item). Each of the 32 items is scaled 0 to 4 points, and the score is calculated by adding all points. The maximum of 128 points reflects unimpaired quality of life. The difference in symptoms of patients with colon interposition compared with patients with primary anastomosis and 150 healthy volunteers was significant (P < ,051.
ET AL
daily reflux symptoms in approximately 25% of their patients more than 15 years after repair. The investigators stated that in the majority of their older patients, gastrointestinal and respiratory symptoms were not considered major. This corresponds with the findings in the present analysis. Only a minority of the patients reported to suffer from specific symptoms “most of the time” or “all of the time.” However, the impact of the reported symptoms on the activities of daily living and the quality of life remained unclear. We were able to show that patients with primary anastomosis had an unimpaired quality of life. The global quality of life scored 80 of 100 points, and it is well known that normal healthy individuals give similar average scores.l* The Spitzer Index was optimal and the GIQLI was similar to that in 150 healthy volunteers reported on by Eypasch et al.14.1sThis indicates that neither gastrointestinal nor respiratory symptoms reported after primary anastomosis had a relevant impact on the quality of life and may therefore not be considered major. In children with long gap atresia, the optimal method of esophagea1 reconstruction remains controversial. Because of the lack of precise definition of the term long gap, because of differences in patient characteristics, follow-up periods, and methods in different series, a comparison of long-term results remains questionable. In the present series of patients with colon interposition, all gastrointestinal symptoms were found more frequently compared with patients who have primary anastomosis, and all of these patients suffered from periods of short breath. The Spitzer Index and the GIQLI were significantly lower compared with patients who have primav anastomosis. In addition, the GIQLI was lower compared with healthy volunteers. However, the impairement in the GIQLI was exclusively caused by specific symptoms, which had no impact on physical functions, emotions, and social functions. The long-term quality of life in the present series of patients with colon interposition was acceptable. Besides suffering from specific symptoms these patients lead an otherwise normal life. Gastric transposition has been established as the method of choice for long gap atresia.19,*o However, series investigated for the long-term effectszoJ1 have shorter follow-up periods compared with series with colon interposition. Of 17 patients with gastric transposition investigated by Davenport et a121 29% were entirely asymptomatic with swallowing. Two frequently had symptoms during swallowing, four suffered from diarrhea, two from postprandial weakness, four from breathlessness, and all except one had an impaired lung function after a mean of 9 years. This compares with the symptomatic outcome in the present series of patients
QUALITY
OF LIFE AFTER
ESOPHAGEAL
ATRESIA
515
with colon interposition, but the quality of life after gastric transposition has not yet been investigated. The instruments used in the present analysis had been set up with adult patients suffering from diseases including cancer,11s1z-15 The GIQLI was validated using a large series of patients with gastrointestinal disorders including diseases of the esophagus, small and large bowel, and liver.14 These patients were able to compare their state of disease with a previously healthy condition. On the contrary, patients with congenital malformations may acquire coping mechanisms for their handicap. This may explain that in the present analysis patients after primary anastomosis scored even better than healthy control subjects. However, many other variables could contribute
to this finding, and a way to bypass this problem would be the use of so-called “generic” questionnaires such as the SF 36.zz Profiles of different patient groups have been published recently and would allow comparison in future studies. The survival rate in the present series was 43%, which is similar to other reports of the period from 1960 to 1970.2,4.19,23 Today, because of advances in perinatal and neonatal care, more than 95% of the children with esophageal atresia survive, 19.~*including those with associated malformations or VACTERL association. For these patients the favorable results of the present analysis do not count and the long-term quality of life remains to be reinvestigated.
REFERENCES 1. Ahmed A. Spitz L: The outcome of colotnc replacement of the oesophagus in clnldren. Prog Pedati Surg 19:37-54.1986 2. Chetcuti P, Phelan PD: Gastrointestmal morbldlty and growth after repair of oesophageal atresia and trachea-oesophageal fistula. Arch Dis Child 68:163-166, 1993 3. Chetcuti P, Phelan PD: Respiratory morbidity after repair of oesophageal atresia and trachea-oesophageal fismla. Arch Dls Child 68:167-170,1993 4. Lindahl H, Rintala k Long-term comphcauons in cases of isolated esophageal atresia treated wtth esophageal anastomosis. J Pedlatr Surg 30:1222-1223, 1995 5. Raffensperger JG, Luck SR, Reynolds M, et al: Intestinal bypass of the esophagus. J Pedia@ Surg 31:38-47, 1996 6 Slepen HJ: bsophagusatresie unter besonderer Benickslchtigung der Nahttechnik. Thesis. University of Cologne, 1974 7. Vogt EC: Congenital esophageal atresia. Am J Roentgen01 22:463465,
1929
8. Ure BM, Slany E. Eypasch ER et al: Long-term functional results and quality of life after colon interposition for long gap oesophageal atresla. Eu .I Pediatr Surg 5:206-210, 1995 9. DeMeester T, Johansson K-E, France I, et al: Indications, surgical technique. and long-term functIona results of colon interposition or bypass. Ann Surg 208:460-473. 1988 10. Ure BM, Troidl H, Spangenberger W, et al: Symptoms more than one year after laparoscopic cholecystectomy. Br J Surg 82:267-270. 1995 11. Selby PJ, Chapman JAW, Etazadi-Amoli J, et al: The development of a method for assessing the quality of life of cancer patients. Br J Cancer 50:13-22, 1984 12. Spitter WO, Dobson AJ, Hall J. et al: Measuring the quality of hfe in cancer patients. A concise QL-index for use by physicians. J Chron Dis 34:585-597, 1981
13. Eypasch E, Troldl H, Wood-Dauphin&e S. et al: Quality of life and gastrointestinal surgev-A clinimetric approach to developing an instrument for Its measurement. Theor Surg 5:3-10, 1990 14. Eypasch E, Wood-Dauphin&e S. Williams JI. et al: Der Gastrointestinale Lebensqualittitsindex (GLQI): Ein klmlmetischer Index zur Befindlichkeitsmessung m der gastroenterologischen Chirurgie. Chug 64:264-274,
1993
1.5. Eypasch E, Wood-Dauphinke S, Williams JL, et al: Gastrointestinal quahty of life Index. Development. validation and application of a new mstrument. Br J Surg 82:216-222, 1995 16. Zaccaria A. Felici F, Turchetta A. et al: Physical fitness testing m children operated on for tracheoesophageal fistula. J Pediatr Surg 30:1334-1337, 1995 17. Lehner M: Esophageal atresia and quality of hfe. 2 Kinderchir 45:209,
1990
18. Glatzer W. Zapf W Lebensqualitgt in der Bundesrepublik. New York, Campus. 1984 19, Spitz L: Gastric transposition via the mediastinal route for infants with long-gap esophageal atresla J Pediatr Surg 19.149-54, 1984 20. Spitz L Esophageal atresia: Past> present, and future. J Pedlatr Surg 31:19-25, 1996 21 Davenport M, Hosie GP, Tasker RC. et al: Long-term effects of gastric transposition in children: A physiological study. J Pediatr Surg 31:588-593,
1996
22, Ware JE. Kosinski
M. Bayliss MS, et al: Comparison of methods for the scoring and statistical analysis of SF-36 health profile and summary measures: Summary of results from the medical outcomes study. Med Care 33:AS264-AS279,1995 23. Ein SH, Shandling B: Pure esophageal atresia: A 50 year review. J Pediatr Surg 29:1208-1211, 1994
B.M.
Ure,
E. Slany,
E.i?
Eypasch,
K. Weiler,
Cologne,
Purpose:To examine the quality geal atresia, follow-up studies surviving patients (81.7%).
of life after repair were performed
of esophain 58 of 71
Methods: Fifty patients with primary anastomosis and all eight surviving patients with colon interposition were seen. The mean age was 25.3 years (range, 20 to 311. Symptoms were evaluated by a standardized interview. Quality of life assessment was performed using a visual analogue scale (0 to 100 points), the Spitzer Index (5 dimensions, 10 points), and the Gastrointestinal Quality of Life Index (GIQLI, 5 dimensions, 128 points). /?esukAfter primary anastomosis the estimated meal capacrty was unrestricted in 46 patients (92%), but numerous symptoms such as recidivating cough (60%), hold up (48%), and short breath (30%) were reported. All symptoms except cough were seen more frequently in patients with colon interposition, and all of these patrents suffered from periods of short breath. Quality of life scores were higher in patients with primary anastomosis compared with colon interposition. The difference in the visual analogue scale score did not reach statistical significance, but the mean Spitzer Index was
T
HE LITERATURE on the long-term outcome of esophageal atresia has been devoted mainly to endpoints such as symptoms, esophageal function, and swallowing ability.rm5 Little is known about the real impact of gastrointestinal and pulmonary symptoms on the activities of daily living, and to our knowledge the quality of life after repair of esophageal atresia has not been investigated with validated instruments. Therefore, the aim of the present study was to determine the long-term quality of life in patients who underwent primary anastomosis or colon interposition for esophageal atresia.
MATERIALS
AND
METHODS
From 1963 to 1971 146 children were operated on for esophageal atresia m the Department of Pediatric Surgery of the Children’s Hospital of Cologne.6 Primary anastomosis was performed in 137 (93.8%) children; 63 (46%) of these survived. The mean age at primary anastomosis was 1.7 days (range, 0 to 6 days), one patient with atresia type II according to VogtT underwent delayed anastomosis at the age of 5 months. In nine children (6.2%) colon interposition was used for long gap atresia: eight of these (88.9%) survived. The mean age at colon interposttion was 15.1 months (range, 5 to 32 months), and all patients underwent retrostemal reconstruction. The tranverse colon was mterposed isoperistalttcally in three children and antiperistalttcally m one. .hurna/
ofPedfatr/c
Surgery,
Vol33,
No 3 (March),
1998: p&? 51 I-515
H. Troidl,
and
A.M.
Hokchneider
Germany
9.7 compared with 8.8 after colon interposition (P < .05). The GIQLI after primary anastomosis was similar to that rn healthy controls and was significantly lower in patients with colon interposition. This was because of specific symptoms, which scored 49.3 after colon interposition compared with 61.7 after primary anastomsis (P < .05) and to 54.8 (SD 5) in healthy controls (P< .05). Physical and social functions, emotions, and inconvenience of a medical treatment scored similar in patients with primary anastomosis, colon interposition, and healthyvolunteers. Cor~ck~sio~s; The long-term quality of life after primary anastomosis was excellent. Patients with colon interposition suffer more frequently from various gastrointestrnal and respiratory symptoms, but they lead an otherwise normal life. J Pediatr Surg 33:511-515. Copyright o 7998 by W.EL Saunders Company. INDEX WORDS: Esophageal atresia, interposition, long-term follow-up.
quality
of
life,
colon
The right colon with termmal ileum was interposed isoperistalttcally in one and antiperistaltically in three children. Fifty-eight patients (81.7%) underwent follow-up; one had been excluded because of mental retardation. Ftfty patients with primary anastomsis (79.4%) and all patients with colon interpositton were seen (Table 1). Twelve patients after primary anastomosis could not be traced and were lost to follow-up. The mean age at follow-up was 25.3 years (range. 20 to 31 years). One patient after primary anastomosis and two patients after colon interpositton were mentally retarded. They were able to communicate, and the parents helped with the interviews. The results of functional assessments that were performed in patients with colon interposition are published elsewhere,8 and the present analysts focused on symptoms and the quahty of life.
Assessme& of Symptoms The interview was performed by two intervtewers: one pedtatnc surgeon and one research fellow. A standardized questionnaire included 41 items and focused on gastrointestinal and pulmonary symptoms. The eating habits, swallowing ability, and long-term side effects of the
From the Department of Pedzatnc Surgery, The Chcldren’s Hospctal of Cologne, and the Department of Surge?, University of Cologne, Cologne, Gemany. Address reprint requests to Benno M. Ure, MD, PhD, Universcty Childrens Hospital “ Wdhelrnina, ” PO Box 18009, 3501 CA Utrecht, The Netherlands. Copyright 0 1998 by WB. Saunders Company 0022.3468/98/3303-0022$03.00/O 511
URE ET AL
512
Table
1. Characteristics After
Male/female Birth weight
In) (mean)
Assocrated malformations lmperforate anus Duodenal Cardial
of 58 Patients Esophageal
Who Underwent Atresia
Follow-Up
PMlW/ Anastomosrs bl = 50)
COlOll lnterposhx3
22128 2,666 g
711 3,081 g
4
-
(n)
atresia 2
Renal Skeletal Type of atresia
according
to Vogt7 fn)
Tw 1 Type II Type llla
-
?
-
-
48
2 1 5
1
Follow-up data Age (mean) Body weight Hight (mean)
(mean)
circumference
(mean)
26.3 yr
24 yr
64 kg 168cm 91 cm
63 kg 174cm 90 cm
operation were evaluated according to the criteria published by DeMeester et al9 The assessment of eating habits included the number of meals per day, the estimated meal capacity. and the reqmrements of liquids with meals. Patients graduated their complaints such as sensattons of hold up, pain or choking with swallowmg. nocturnal regurgitatrons, or others by Indicating whether they suffered from each specific symptom (1) all of the time, (2) most of the time, (3) some of the ttme, or (4) never. In addition, patients underwent a standardized physical examination. Finally, the patients together with both interviewers scored the result of the operation within three categories according to Ure et allo: (A) very good result/no complaints, (B) moderate result/ symptoms that the patient is willmg to tolerate. (C) poor result/therapy indicated.
Assessment of the Quality
Statistical Methods The biomedical data package PC-90 (BMDP Statistical Software, Cork, Ireland) was used. Univariate analysis was carried out by the Wilcoxon rank sum test and the x2 test with Yates correction as appropriate. A P value of less than .05 was considered stgmficant.
1
-
-
Type lllb Type lllc
1
2 13
Other
Thoracal
h = 8)
questions was scored from 0 to 4 points. The index was calculated by adding all points and the maximum of 128 points theoretically reflected unimpatred quality of life. For comparison we used the GIQLI of a series of 150 healthy individuals who underwent investigatron by Eypasch et al.14,1s
of Life
The quality of life measures were self report measures completed in private and not in the presence of the interviewers. Three instruments were used. The global quality of life was determined by a visual analogue sca1e.n Patients were asked to graduate their actual global quality of life on a loo-mm line anchored by two extremes: “very poor/my life is extremely unpleasant” and ‘*optimal/my life is normal.” The level of quality of life was scaled from zero to 100. The Spitzer Indexlz was used for the assessment of five items: (1) activity/involvement in own occupation, (2) activities of daily living, (3) health, (4) support of family and friends, and (5) outlook on life/future. Each item was assessed for the period “during the last week” and was scored from 0 to 2. The total range of scores of the Spitzer Index was 0 to 10. We used the version 4/1991 of the Gastrointestinal Quality of Life Index (GIQLI) which was established by Eypasch et all3 and includes 32 items. A Germant3,14 and an English versionts of the index is available, and the questions comprise the accepted dimensions of quality of life: symptoms (17 items), physical functions (seven items), emotions (five items). and social functions (two items). The inconvenience of a medical treatment is added. Symptoms include core symptoms such as pain, bloating, or restricted eating and diseasespecific items such as regurgitation, dysphagia, or others. Each of the 32
RESULTS
After primary anastomosis, 40 patients (80%) were working full time, seven (14%) were students, and three [6%) were unemployed. Seventeen (34%) of these patients were married and 13 (26%) had children. After colon interposition, five patients (62%) were working full time; two of these were married and had children. One patient was a student, and two were working in an institution for handicaped. Symptoms The estimated meal capacity9 was unrestricted in 46 (92%) of 50 patients after primary anastomosis. Twentyfour patients (48%) suffered at least “some of the time” from hold up, 12 (24%) from diarrhea, and 11 (22%) from heartburn (Fig 1). Other gastrointestinal symptoms were identified in less than 20% of the patients each. Respiratory symptoms were found more frequently; 30 patients (60%) reported on recidivating cough and frequent periods of bronchitis, and 15 (30%) were short of breath at least some of the time. After colon interposition, all gastrointestinal symptoms were documented more frequently (Fig 2). Five patients (62.5%) suffered from hold up and another five from diarrhea. All patients with colon interposition felt to be short of breath, five of these “all of the time.” The meal capacity was unrestricted in three patients (37.5%) with colon interposition. Qua&
of Life
The global quality of life score, the Spitzer Index, and GIQLI were higher in patients primary anastomosis compared with those with colon interposition (Table 2). The difference in the global quality of life score assessed by the visual analogue scale did not reach statistical significance. However, after primary anastomosis the mean Spitzer Index was 9.7 compared with 8.8 after colon interposition (P < .05). The GIQLI after primary anastomosis was similar to healthy controls, but there was a significant reduction in the GIQLI in patients with colon interposition compared with primary anastomosis (P =C.OOOl)and compared with healthy controls (P -C .05, Table 2).
QUALITY
OF LIFE AFTER
ESOPHAGEAL
ATRESIA
513
Primary
Anastomosis n=50
Cough Hold up Short breath Diarrhoea Heartburn Early satiety Bloating Regurgitation Bad breath Vomiting Pain Nausea 0
Fig I. Symptoms at follow-up of 50 patients after primary anastomosis. Patients indicated for each symptom whether they suffered from it “all of the time,” “most of the time,” “some of the time,” or “never.”
1 a
all
ik!@z@
some
The breakdown of the dimensions of the GIQLI showed significant differences for symptoms (Fig 3). Symptoms scored 49.3 (SD 10) after colon interposition compared with 61.7 (SD 4.7) after primary anastomosis (P < .OOOl) and compared with 54.8 (SD 5) in healthy controls (P < -05). No significant differences were found for physical functions, social functions, emotions, and inconvenience of a medical treatment between patients with primary anastomosis, colon interposition, and healthy controls. The result of the operation was classified as “poor.’ in
Colon
20 Number
10
of
the of
30 of Patients
time the
@@
most
D
never
tjrne
40
of
the
50
time
one patient because of a persistent stenosis of the esophagus. Subsequent bouginage was performed in another department. All other patients had an “optimal” result or were tolerating their symptoms without further therapy (Table 2). DISCUSSION
Many investigators have drawn attention to the longterm outcome of repair of esophageal atresia, and the majority agree that specific symptoms are mainly gastrointestinal and respiratory. 2,3.‘6Symptoms improve with
Interposition n=8
Short
breath Hold up Diarrhoea Bloating Early satiety Heartburn Regurgitation Bad breath Nausea Cough Pain Vomiting 0
2 Number a
all of
the
time
&%#
some
of the
time
4 of Patients m
most
m
never
6
of the
8
tlrne
Fig 2. Symptoms at follow-up of eight patients after colon interposition. Patients indicated for each symptom whether they suffered from it “all of the time,” “most of the time,” “some of the time,” or “never.”
WE
514
Table 2. Quality
of Life After
Primary Anastomos!s (n = 501 Mean (SD)
Method of Evaluation
Visual
Analogue
CObl lnterpositmn In = 81 Mean (SD)
Quality
80.1 (18.6) 9.7 (0.8)
Healthy controls
h = 1501 Mean (SD)
67.5 (18) 8.8 (2.1)*
-
92.2 (26,5H
107.6 (18.7)
36 (72%) 13 (26%)
3 (37.5%) 5 (62.5%)
1(2%1
-
-
of Life
Index GiQL113-15 (O-128 points) Classification
Atresia
Scale”
(O-100 points) Spitter lndexq2 (O-10 points) Gastrointestinal
Esophageal
111.5 (8)
of the outcome10
(n) A. Very good B. Moderate c. Poor
*P< .05 versus primary anastomosis. tP < .OOOl versus primary anastomosis
and P < .05 versus
healthy
controls.
time in patients with primary anastomosis and in patients with esophageal replacement.1-3,5,17 In the present series 60% of the patients after prima.ry anastomosis suffered from respiratory symptoms, mainly attacks of cough, frequent bronchitis, and short breath. This corresponds well with the report of Chetcuti and Phelan3 who found persisting respiratory symptoms in over half of 164 patients over 1.5 years of age. Annual bouts of bronchitis were documented in 41% and recurrent wheeze in 40%. The most frequent gastrointestinal symptom after primary anastomosis in the present series was hold up, which was reported by 48% of the patients; 22% had gastroesophageal reflux symptoms such as heartburn or regurgitation. Chetcuti and Phela$ found Gastromtestinal
Quality
of Life index
(mean)
70
Fig 3. Gastrointestinal Quality of Life Index (GIQLII version 4/1991 in 58 patients after esophageal atresia: symptoms (17 items), physical functions (seven items), emotions (five items), social functions (two items), inconvenience of a medical treatment (one item). Each of the 32 items is scaled 0 to 4 points, and the score is calculated by adding all points. The maximum of 128 points reflects unimpaired quality of life. The difference in symptoms of patients with colon interposition compared with patients with primary anastomosis and 150 healthy volunteers was significant (P < ,051.
ET AL
daily reflux symptoms in approximately 25% of their patients more than 15 years after repair. The investigators stated that in the majority of their older patients, gastrointestinal and respiratory symptoms were not considered major. This corresponds with the findings in the present analysis. Only a minority of the patients reported to suffer from specific symptoms “most of the time” or “all of the time.” However, the impact of the reported symptoms on the activities of daily living and the quality of life remained unclear. We were able to show that patients with primary anastomosis had an unimpaired quality of life. The global quality of life scored 80 of 100 points, and it is well known that normal healthy individuals give similar average scores.l* The Spitzer Index was optimal and the GIQLI was similar to that in 150 healthy volunteers reported on by Eypasch et al.14.1sThis indicates that neither gastrointestinal nor respiratory symptoms reported after primary anastomosis had a relevant impact on the quality of life and may therefore not be considered major. In children with long gap atresia, the optimal method of esophagea1 reconstruction remains controversial. Because of the lack of precise definition of the term long gap, because of differences in patient characteristics, follow-up periods, and methods in different series, a comparison of long-term results remains questionable. In the present series of patients with colon interposition, all gastrointestinal symptoms were found more frequently compared with patients who have primary anastomosis, and all of these patients suffered from periods of short breath. The Spitzer Index and the GIQLI were significantly lower compared with patients who have primav anastomosis. In addition, the GIQLI was lower compared with healthy volunteers. However, the impairement in the GIQLI was exclusively caused by specific symptoms, which had no impact on physical functions, emotions, and social functions. The long-term quality of life in the present series of patients with colon interposition was acceptable. Besides suffering from specific symptoms these patients lead an otherwise normal life. Gastric transposition has been established as the method of choice for long gap atresia.19,*o However, series investigated for the long-term effectszoJ1 have shorter follow-up periods compared with series with colon interposition. Of 17 patients with gastric transposition investigated by Davenport et a121 29% were entirely asymptomatic with swallowing. Two frequently had symptoms during swallowing, four suffered from diarrhea, two from postprandial weakness, four from breathlessness, and all except one had an impaired lung function after a mean of 9 years. This compares with the symptomatic outcome in the present series of patients
QUALITY
OF LIFE AFTER
ESOPHAGEAL
ATRESIA
515
with colon interposition, but the quality of life after gastric transposition has not yet been investigated. The instruments used in the present analysis had been set up with adult patients suffering from diseases including cancer,11s1z-15 The GIQLI was validated using a large series of patients with gastrointestinal disorders including diseases of the esophagus, small and large bowel, and liver.14 These patients were able to compare their state of disease with a previously healthy condition. On the contrary, patients with congenital malformations may acquire coping mechanisms for their handicap. This may explain that in the present analysis patients after primary anastomosis scored even better than healthy control subjects. However, many other variables could contribute
to this finding, and a way to bypass this problem would be the use of so-called “generic” questionnaires such as the SF 36.zz Profiles of different patient groups have been published recently and would allow comparison in future studies. The survival rate in the present series was 43%, which is similar to other reports of the period from 1960 to 1970.2,4.19,23 Today, because of advances in perinatal and neonatal care, more than 95% of the children with esophageal atresia survive, 19.~*including those with associated malformations or VACTERL association. For these patients the favorable results of the present analysis do not count and the long-term quality of life remains to be reinvestigated.
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1929
8. Ure BM, Slany E. Eypasch ER et al: Long-term functional results and quality of life after colon interposition for long gap oesophageal atresla. Eu .I Pediatr Surg 5:206-210, 1995 9. DeMeester T, Johansson K-E, France I, et al: Indications, surgical technique. and long-term functIona results of colon interposition or bypass. Ann Surg 208:460-473. 1988 10. Ure BM, Troidl H, Spangenberger W, et al: Symptoms more than one year after laparoscopic cholecystectomy. Br J Surg 82:267-270. 1995 11. Selby PJ, Chapman JAW, Etazadi-Amoli J, et al: The development of a method for assessing the quality of life of cancer patients. Br J Cancer 50:13-22, 1984 12. Spitter WO, Dobson AJ, Hall J. et al: Measuring the quality of hfe in cancer patients. A concise QL-index for use by physicians. J Chron Dis 34:585-597, 1981
13. Eypasch E, Troldl H, Wood-Dauphin&e S. et al: Quality of life and gastrointestinal surgev-A clinimetric approach to developing an instrument for Its measurement. Theor Surg 5:3-10, 1990 14. Eypasch E, Wood-Dauphin&e S. Williams JI. et al: Der Gastrointestinale Lebensqualittitsindex (GLQI): Ein klmlmetischer Index zur Befindlichkeitsmessung m der gastroenterologischen Chirurgie. Chug 64:264-274,
1993
1.5. Eypasch E, Wood-Dauphinke S, Williams JL, et al: Gastrointestinal quahty of life Index. Development. validation and application of a new mstrument. Br J Surg 82:216-222, 1995 16. Zaccaria A. Felici F, Turchetta A. et al: Physical fitness testing m children operated on for tracheoesophageal fistula. J Pediatr Surg 30:1334-1337, 1995 17. Lehner M: Esophageal atresia and quality of hfe. 2 Kinderchir 45:209,
1990
18. Glatzer W. Zapf W Lebensqualitgt in der Bundesrepublik. New York, Campus. 1984 19, Spitz L: Gastric transposition via the mediastinal route for infants with long-gap esophageal atresla J Pediatr Surg 19.149-54, 1984 20. Spitz L Esophageal atresia: Past> present, and future. J Pedlatr Surg 31:19-25, 1996 21 Davenport M, Hosie GP, Tasker RC. et al: Long-term effects of gastric transposition in children: A physiological study. J Pediatr Surg 31:588-593,
1996
22, Ware JE. Kosinski
M. Bayliss MS, et al: Comparison of methods for the scoring and statistical analysis of SF-36 health profile and summary measures: Summary of results from the medical outcomes study. Med Care 33:AS264-AS279,1995 23. Ein SH, Shandling B: Pure esophageal atresia: A 50 year review. J Pediatr Surg 29:1208-1211, 1994