bs_bs_banner

doi:10.1111/jpc.12206

ORIGINAL ARTICLE

Early developmental outcome following surgery for

oesophageal atresia
Karen Walker,1,3,4 Robert Halliday,1 Nadia Badawi,1,3 Jan Stewart1 and Andrew JA Holland2,3
1

Grace Centre for Newborn Care and 2Douglas Cohen Department of Paediatric Surgery, The Childrens Hospital at Westmead, Westmead, 3Discipline of
Paediatrics and Child Health, Sydney Medical School, and 4Sydney Nursing School, The University of Sydney, Sydney, New South Wales, Australia

Aim: To compare the developmental outcome of infants with oesophageal atresia with or without trachea-oesophageal stula (OA/TOF) who
underwent surgery in early infancy with healthy control infants in New South Wales, Australia.
Methods: Infants diagnosed with OA/TOF requiring surgical intervention were enrolled prospectively between 1 August 2006 and the 31
December 2008. Healthy control infants were enrolled in the same time period. The children underwent a developmental assessment at 1 year
of age (corrected) using the Bayley Scales of Infant and Toddler Development (Version III).
Results: Of 34 infants with OA/TOF that were enrolled, 31 had developmental assessments. The majority (75%) were term infants (37 weeks
gestation) with a mean birth weight of 2717 g. Fourteen infants (44%) had an associated birth defect and one infant with multiple associated
anomalies subsequently died. Developmental assessments were also performed on 62 control infants matched for gestational age. Infants with
OA/TOF had a mean score signicantly lower on the expressive language subscale (P < 0.05) compared with the control infants.
Conclusions: This study found a lower than expected developmental score for infants following surgery for OA/TOF in the expressive language
subscale compared with the healthy control infants. These ndings support concerns over the potential impact of OA/TOF and its effects on
development. Further studies, including continuing developmental review to determine whether these differences persist and their functional
importance, should be performed.
Key words:

infant; oesophageal atresia; outcome; survival; trachea-oesophageal stula.

What is already known on this topic

What this paper adds

1 Survival of infants with oesophageal atresia (OA) is high.

2 Infants with OA have long-term surgical morbidity.
3 Neurodevelopmental morbidity associated with OA and its
repair appears unclear.

1 Infants with OA are at risk of developmental delay.

2 Early outcomes show that the majority of delay is mild.
3 Evidence that infants with OA should be enrolled in multidisciplinary follow-up clinics.

Introduction
Oesophageal atresia (OA) is a congenital developmental malformation characterised by the absence of the normal continuity of
the oesophagus.1 An abnormal communication between the
oesophagus and the trachea is found in 8588% of infants.2 The
first reported case of OA was in 1670 by Durston and in 1697
Gibson described the first case of OA with a fistula.3,4 OA occurs
between one in 25004500 live births,5 with an increased incidence in twins.6 While survival of infants with OA continues to
improve, there are recognised long-term morbidities, both
physical and psychological.79
Infants with OA have long-term ongoing physical and nutritional difficulties that may affect their development. Problems
Correspondence: Dr Karen Walker, Grace Centre for Newborn Care, The
Childrens Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145,
Australia. Fax: +61 2 9845 1923; email: karen.walker@health.nsw.gov.au
Conict of interest: None declared.
Accepted for publication 30 November 2012.

include abnormal oesophageal motility, tracheomalacia and

gastro-oesophageal reflux. These may have a major impact on
growth and nutritional status and may also impact on quality
of life.5,7,9
Faugli et al. reported that at a median age of 13 months, early
mental health disorders were identified using specific diagnostic
criteria in 31% of 39 infants, 27 boys and 12 girls, with OA
treated in Norway between 1999 and 2002.10 Although only a
relatively small group, these data may have resulted from
parental anxieties. A later study by the same author using standardised questionnaires found normal mental health and psychosocial functioning in 21 adolescents born with OA compared
with 36 of their peers without OA.11 Other reports have been
less encouraging, suggesting a need for detailed prospective
evaluation of these infants compared with age-matched controls.12 Despite these concerns, in New South Wales (NSW),
infants with OA have not routinely been enrolled in neonatal
developmental follow-up clinics.
In this study, we aimed to describe both the current survival
and developmental outcome of a prospectively enrolled cohort

Journal of Paediatrics and Child Health 49 (2013) 467470

2013 The Authors
Journal of Paediatrics and Child Health 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

467

Outcomes of infants with OA

K Walker et al.

of infants in NSW with OA/trachea-oesophageal fistula (TOF)

compared with an age-matched control group.

Materials and Methods

Patients and data collection
Data were sourced from the Development after Infant Surgery
(DAISy) study. Infants enrolled in this population-based study
from 1 August 2006 to 31 December 2008 were eligible for
inclusion. The DAISy database represents a prospectively collected database from five participating tertiary centres in NSW.
Three of these units perform major neonatal surgery defined as
the opening of a body cavity.13 Criteria for inclusion in the DAISy
database were either major cardiac or non-cardiac surgery
within the first 90 days of life, with healthy controls enrolled
from co-located maternity units. There were 34 infants enrolled
with a diagnosis of OA/TOF. Of these, one infant was excluded
with a chromosomal anomaly that affected development; one
infant died and one was extremely preterm and unable to be
matched with a healthy control. The remaining 31 infants had a
developmental assessment at 1 year of age. A majority14 had a
diagnosis of OA/TOF; one just had a TOF and three had pure OA.
The control infants were children enrolled contemporaneously
in the DAISy study over the period of the TOF/OA children and
were matched two-for-one by gestational age.
The general approach of our institution to the operative management of neonates with OA has been for primary extrapleural repair via a postero-lateral thoracotomy, conducted
within 2448 h of birth, with gastrostomy reserved for those
infants with long-gap OA. Dilatations would not be routinely
performed although patients would be treated with ranitidine
post-operatively.

Developmental Assessment
Infants were assessed at a corrected age of 1 year using The
Bayley Scales of Infant and Toddler Development, Version III
(BSITD-III).15 The assessment consisted of five scales: Cognition,
Receptive Language, Expressive Language, Fine Motor and
Gross Motor. This standardised test of infant development is age
normed for each subscale to have a mean score of 10 with a
standard deviation of 3. Mild developmental delay was considered from >-2 SD to -1 SD, moderate delay >-3 to -2 SD and
severe delay a score of -3 SD below the mean.16
This assessment has been well validated as developmentally
appropriate. The previous version has been used widely in Australia and in our preliminary work.1719 Each infant was assessed
by two Bayley trained assessors, one of whom was blinded to
the infants study group. Parents were asked not to identify their
childs surgical status to the assessor.
The mean scores for Infants with OA/TOF on the five subscales
of the BSITD-III were then compared with the mean scores for
the control infants and a P < 0.05 was considered statistically
significant. Statistical analysis was performed using SPSS statistical software version 19 (SPSS Inc., Chicago, IL, USA).20 Normal
probability plots were calculated to determine normality of the
data and Students t-test was used to determine the significance
of differences between the means for the subscales.
468

Table 1

Associated anomalies

Associated
anomaly

Type

Cardiac

ASD
VSD
PDA/PFO
Congenital malformation of aorta
Renal agenesis
Ectopic kidney
Syndactyly
Limb anomalies
Congenital malformation of ribs
Duodenal atresia
Other congenital malformation of intestine
Congenital tracheomalacia
VACTERL
Bicornuate uterus
Cleft lip and palate

4
2
3
1
2
1
1
1
1
2
1
3
2
2
1

Renal
Vertebral

Gastrointestinal
Others

Infants may have more than one anomaly. ASD, atrial septal defect;
VSD, ventricular septal defect; PFO, patent foramen ovale; PDA, patent
ductus arteriosus.

Results
There was no significant difference between the gestational age
of the infants with OA/TOF (37.6 weeks) and the control infants
(38.1 weeks) or between their birth weights (2718 g, SD
717 g). Eight of the infants with OA/TOF were preterm,
defined as delivery at less than 37 weeks of gestation. The
majority of the babies were female (62.5%) and singleton pregnancies (94%). There was one reported death prior to 1 year of
age within the cohort, a preterm infant with VACTERL association (vertebral anomalies, anorectal malformations, cardiovascular anomalies, TOF, OA, renal anomalies, limb defects and
multiple other anomalies). The median length of stay was 19
days (range 8134 days). Fourteen infants (44%) had associated
congenital anomalies (Table 1).
Infants with OA/TOF scored significantly lower on one of the
subscales of the BSITD-III. They scored lower on the expressive
language subscale compared with the healthy control infants
(P < 0.05). However, in all subscales, there was a small difference in the mean with the infants with OA/TOF scoring lower
than the control infants (Tables 2 and 3).

Discussion
The traditional focus for neonatal research has been on survival,
physical outcomes and long-term medical issues. There are a
plethora of studies reporting these outcomes of infants
with OA/TOF, with many concentrating on survival and the
long-term medical outcomes, including the prevalence of
oesophagitis and late malignancy.7,9,21 Others document surgical
technique22 and specific types of atresia such as long-gap OA.23,24
Several studies have examined long-term physical and psychosocial outcomes.11,25 Few studies, however, document the early

Journal of Paediatrics and Child Health 49 (2013) 467470

2013 The Authors
Journal of Paediatrics and Child Health 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

K Walker et al.

Table 2

Outcomes of infants with OA

Summary of developmental assessment

Bayley-III domain

Group

Mean

P value

Cognition

Controls 62
OA/TOF 31
Controls 62
OA/TOF 31
Controls 62
OA/TOF 31
Controls 62
OA/TOF 31
Controls 62
OA/TOF 30

11.69
11.00
10.94
10.23
10.06
9.03
10.05
9.16
9.27
8.37

NS

Receptive language
Expressive language
Fine motor
Gross motor

NS
P < 0.05
NS
NS

One infant was in a plaster cast and gross motor was not assessed. NS,
not signicant; OA, oesophageal atresia; TOF, trachea-oesophageal
stula.

Table 3

Categorisation of delay

Bayley-III domain

Delay

OA/TOF (n = 31)

Control (n = 62)

Cognition

Mild
Moderate
Severe
Total n (%)
Mild
Moderate
Severe
Total
Mild
Moderate
Severe
Total
Mild
Moderate
Severe
Total
Mild
Moderate
Severe
Total

1
0
0
1 (3%)
7
0
0
7 (23%)
5
1
0
6 (19%)
4
0
0
4 (13%)
8
3
1
12/(39%)

2
0
0
2 (3%)
11
1
0
12 (18%)
8
0
0
8 (13%)
5
0
0
5 (8%)
12
1
0
13 (21%)

Receptive language

Expressive language

Fine motor

Gross motor

One infant was in a plaster cast and gross motor was not assessed. OA,
oesophageal atresia; TOF, trachea-oesophageal stula.

developmental outcome of these infants. With improved survival, we are now moving the critical lens to neurodevelopmental and educational outcomes of these children in comparison
with a normal cohort.
This report appears to be one of the first prospective studies to
compare the developmental outcomes at 1 year of age of infants
with OA/TOF who underwent early major surgery with a cohort
of healthy controls using the standardised norms of the BSITDIII. We found that the children with OA/TOF displayed evidence
of a statistically significant difference in the mean scores on the

expressive language subtest (at 12 months corrected) when

compared with the control infants, although the mean scores
were lower in all subscales.
Delay in gross motor has been recognised in a study by Gischler et al. who found impaired growth and psychomotor developmental delay up to 2 years of age in infants with congenital
diaphragmatic hernia and OA.12 Although the mean scores in
gross motor in our study were not statistically significant compared with the control infants, we did find that 39% of the
infants with OA/TOF had evidence of delay in gross motor skills
compared with the control infants (21%), with the majority of
delay classified as mild. Despite the lack of statistical significance, perhaps a reflection of our relatively small numbers, this
finding remains of great importance in the application of early
intervention services, with early intervention preferred to
ensure optimal outcome for these children.
Identifying delay at the earliest possibility enables early intervention that may ultimately improve the outcome for the child,
their families and the community as a whole.26 Early identification of children at risk of poor neurodevelopmental outcome
and the institution of timely intervention, such as physiotherapy, speech and occupational therapy, should improve the
outcomes for these infants and families, thus both improving
their health and reducing the burden on society. The importance of early childhood intervention has recently been recognised with strong government support for an Early Childhood
Development strategy.27
While we believe that these data support the need for detailed
neurodevelopmental assessment, this study was limited by the
relatively small sample size of the OA/TOF cohort. Furthermore,
we have to date performed only one developmental assessment
at the relatively early age of 12 months. These children are
currently being reassessed at 3 years of age to ascertain fully
their developmental progress. Although the infants with
OA/TOF performed significantly worse than control infants on
the expressive language sub-scales of the BSITD at 12 months of
age, they still had a mean score within the average range for the
assessment. Furthermore, for the majority of infants who had a
delay, reassuringly this was minor.
The finding of delay in infants who have had major surgery is
receiving increasing interest in the surgical literature,12 with the
recommendation that infants who have undergone major
surgery be enrolled in developmental follow-up clinics.14,28
Nearly half of the children in this study had associated anomalies including VACTERL. This has also been reported in other
studies1 and would also suggest the need for a high level of
surveillance for associated anomalies at the time of birth.
Further prospective studies from several centres, using a
standardised assessment protocol with detailed documentation
of additional surgical interventions, including the requirement
for anaesthesia and oesophageal dilatations, would provide
additional evidence of the need for this group of patients to be
offered additional developmental support post-discharge.

Acknowledgements
We thank our neonatal and paediatric surgical colleagues for
permission to enrol their patients in this study.

Journal of Paediatrics and Child Health 49 (2013) 467470

2013 The Authors
Journal of Paediatrics and Child Health 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

469

Outcomes of infants with OA

K Walker et al.

Funded by March of Dimes Birth Defects Foundation, Project

Grant #12-FY06-232.

References
1 Holland AJA, Ron O, Pierro A et al. Surgical outcomes of esophageal
atresia without stula for 24 years at a single institution. J. Pediatr.
Surg. 2009; 44: 192832.
2 Holland A, Fitzgerald DA. Oesophageal atresia and
tracheo-oesophageal stula: current management strategies and
complications. Paediatr. Respir. Rev. 2010; 11: 1006.
3 Durston W. A narrative of a monstrous birth in Plymouth. October 22
1670: together with the anatomical observations taken thereupon.
Philos. Trans. R. Soc. 16701671; 5: 2098.
4 Gibson T. The Anatomy of Humane Bodies Epitomized. London,
England: Awnsham and Churchill, 1697.
5 Spitz L. Esophageal atresia lessons I have learned in a 40-year
experience. J. Pediatr. Surg. 2006; 41: 163540.
6 Orford JGM, Beasley S, Shi E, Myers N, Cass D. Oesophageal atresia in
twins. Pediatr. Surg. Int. 2000; 16: 5415.
7 Rintala RJ, Sistonen S, Pakarinen MP. Outcome of esophageal atresia
beyond childhood. Semin. Pediatr. Surg. 2009; 18: 506.
8 Little DC, Rescorla FJ, Grosfeld JL, West KW, Scherer LR, Engum SA.
Long-term analysis of children with esophageal atresia and
tracheoesophageal stula. J. Pediatr. Surg. 2003; 38: 8526.
9 Kovesi T, Rubin S. Long-term complications of congenital esophageal
atresia and/or tracheoesophageal stula. Chest 2004; 126: 91525.
10 Faugli A, Emblem R, Bjornland K, Diseth TH. Mental health in infants
with esophageal atresia. Infant Ment. Health J. 2009; 30: 4056.
11 Faugli A, Bjornland K, Emblem R, Novik TS, Diseth TH. Mental health
and psychosocial functioning in adolescents with esophageal atresia.
J. Pediatr. Surg. 2009; 44: 72937.
12 Gischler SJ, Mazer P, Duivenvoorden HJ et al. Interdisciplinary
structural follow-up of surgical newborns: a prospective evaluation.
J. Pediatr. Surg. 2009; 44: 13829.
13 Badawi N, Adelson P, Roberts C, Spence K, Laing S, Cass D. Neonatal
surgery in New South Wales what is performed where? J. Pediatr.
Surg. 2003; 38: 102531.
14 Walker K, Holland AJA, Winlaw D, Sherwood M, Badawi N.
Neurodevelopmental outcomes and surgery in neonates. J. Paediatr.
Child Health 2006; 42: 74951.

470

15 Bayley N. Bayley Scales of Infant and Toddler Development, 3rd edn.

San Antonio: The Psychological Corporation, 2006.
16 Walker K, Badawi N, Halliday R et al. Early developmental outcomes
following major noncardiac and cardiac surgery in term infants: a
population-based study. J. Pediatr. 2012; 161: 74852.
17 Spittle A, Ferretti C, Anderson PJ et al. Improving the outcome of
infants born at <30 weeks gestation a randomized controlled trial of
preventative care at home. BMC Pediatr. 2009; 9: 73.
18 Sun J, Mohay H, OCallaghan M. A comparison of executive function in
very preterm and term infants at 8 months corrected age. Early Hum.
Dev. 2009; 85: 22530.
19 Cheong JLY, Hunt RW, Anderson PJ et al. Head growth in preterm
infants: correlation with magnetic resonance imaging and
neurodevelopmental outcome. Pediatrics 2008; 121: e153440.
20 SPSS Inc. SPSS Base 19.0 for Windows Users Guide. Chicago, IL: SPSS,
2010.
21 Choudhury SR, Ashcraft KW, Sharp RJ, Murphy JP, Snyder CL, Sigalet
DL. Survival of patients with esophageal atresia: inuence of birth
weight, cardiac anomaly, and late respiratory complications. J.
Pediatr. Surg. 1999; 34: 703.
22 MacKinlay GA. Esophageal atresia surgery in the 21st century. Semin.
Pediatr. Surg. 2009; 18: 202.
23 Foker JE, Kendall Krosch TC, Catton K, Munro F, Khan KM. Long-gap
esophageal atresia treated by growth induction: the biological
potential and early follow-up results. Semin. Pediatr. Surg. 2009; 18:
239.
24 Al-Shanafey S, Harvey J. Long gap oesophageal atresia: an Australian
experience. J. Pediatr. Surg. 2008; 43: 597601.
25 Bouman NH, Koot HM, Hazebroek FW. Long-term physical,
psychological, and social functioning of children with esophageal
atresia. J. Pediatr. Surg. 1999; 34: 399404.
26 Anderson LM, Shinn C, Fullilove MT et al. The effectiveness of early
childhood development programs. A systematic review. Am. J. Prev.
Med. 2003; 24: 3246.
27 Halfon N, Russ S, Oberklaid F, Bertrand J, Eisenstadt N. An
international comparison of early childhood initiatives: from services
to systems. J. Dev. Behav. Pediatr. 2009; 30: 4713.
28 Laing SWK, Ungerer J, Badawi N, Spence K. Early development of
children with major birth defects requiring newborn surgery.
J. Paediatr. Child Health 2011; 47: 1407.

Journal of Paediatrics and Child Health 49 (2013) 467470

2013 The Authors
Journal of Paediatrics and Child Health 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

Copyright of Journal of Paediatrics & Child Health is the property of Wiley-Blackwell and its
content may not be copied or emailed to multiple sites or posted to a listserv without the
copyright holder's express written permission. However, users may print, download, or email
articles for individual use.