Paul D. Losty 1, Rebecca Thursfield2
- Department Of Paediatric Surgery, Alder Hey Children’s Hospital NHS
Foundation Trust , School Of Health And Life Science , University of
Liverpool, UK
- Department Of Paediatric Respiratory Medicine , Alder Hey Children’s
Hospital NHS Foundation Trust , Liverpool , UK
Tracheoesophageal fistula (TOF) are caused by the failed fusion of the
tracheoesophageal ridges around the fourth week of embryonic
development. Closely linked with maldevelopment of the foregut it is
commonly associated with oesophageal atresia (OA). Congenital
oesophageal atresia, with or without TOF, occurs in approximately 1 in
3500 live births. The lesion may be suspected antenatally in a minority
of cases (25%) associated with polyhydramnios though most index cases
are diagnosed in the new-born period. Many babies present within the
first days of life with inability to tolerate feeds and diagnosis is
linked to the inability to secure passage of a nasogastric tube into the
infant’s stomach. Surgical repair is promptly scheduled after diagnosis
with closure of the fistula tract and anastomosis of the oesophagus.
Newborns with a ‘ long gap ‘ in the separated oesophageal segments
cannot readily have primary repair due to the risk of ‘ anastomotic
breakdown ‘ if the oesophagus is brought together by the surgeon under
high suture tension. Here , ‘ delayed primary repair ‘ with a period of
gastrostomy feeding and “sham” oral feeds with a cervical
oesophagostomy ensures the child can acquire key oral motor skills for
later definitive repair. If delayed repair of the oesophagus is not
feasible after some 8 weeks, oesophageal replacement should be
undertaken with varied options available including gastric
transposition, a colon graft, or jejunal interposition .
No matter what surgical technique(s) are deployed even with primary
repair of the oesophagus where anatomical correction is achieved,
significant functional abnormality(s) remain thereafter for patients.
There are a multitude of factors to consider which are herein discussed
in the paper by Koumbourlis, Belessis et al in this issue of Pediatric
Pulmonology. These functional abnormalities can cause life-long
morbidity and impact patients and families in many different ways. Some
morbidities are very specific, such as food bolus obstruction at the
site of the oesophageal anastomosis. Others lead to much more vague
troublesome symptoms notably chesty cough, breathlessness, vomiting,
poor appetite, epigastric pain, chest infections and failure to thrive.
If these patients are seen by health care professionals without adequate
experience of TOF - OA, symptoms here may not be recognised such that
accurate diagnosis and planned management can be delayed. The problems
are often multifactorial and for optimal health care these children
should ideally be managed in specialist clinics staffed by
multidisciplinary teams including, paediatric surgeons, respiratory
physicians, dieticians, physiotherapists, speech and language therapists
(SALT), ENT surgeons, medical gastroenterology and psychologists. Access
to such key specialists appears to be highly variable amongst centres;
one study found that two thirds of patients developed respiratory
symptoms and most were seen by appropriate specialists , whilst another
single centre paper highlighted that only half of all patients with
respiratory or gastrointestinal problems were seen by the appropriate
specialist . These two articles clearly highlight wide clinical practice
variation amongst such centres and a lack of consistency in MDT care.
Delay in accurate diagnosis of respiratory sequale of OA-TOF can lead to
poor quality of life (QoL), development of oral feeding aversion and
marked failure to thrive with poor caloric intake and increased energy
expenditure linked with tachypnoea and bronchiectasis through recurrent
infections.
For parents of children with TOF-OA, medical problems are combined with
the task of managing day to day issues faced in “normal” life. There
is increased parental anxiety at milestone points such as weaning to
infant diets and starting school, added to numerous hospital clinic
visits and other surgical operations for some of our more vulnerable
patients. For a birth defect that is characterised with such morbidity
surprisingly few robust multicentre data exist including care pathway
guidelines. As Koumbourlis, Belessis et al discuss , most current data
emerge from single centre experience and here we agree there is a
crucial need for larger scale collaborative studies. This manuscript is
therefore timely in Pediatric Pulmonology as a ‘work in progress’
consensus on the utility of diagnostic studies and therapeutics
addressing respiratory complications of OA-TOF patients.
The article is a good starting point aimed at developing better outcome
data for OA-TOF patients and the authors should be congratulated for
this work. The GRADE methodology was rejected by the study authors due
to the lack of good quality evidence currently available. Instead, the
RAND appropriateness method (RAM) was adopted by authors. The resultant
consensus recommendations are thorough and cover all aspects of
respiratory management of patients with OA-TOF. The study authors make
37 recommendations on various diagnostic techniques and 25 key points on
respiratory interventions including management of tracheobronchomalacia,
antibiotic treatment, chest physiotherapy and therapy of
gastro-oesophageal reflux disease. The authors also describe the need
for multidisciplinary (MDT) follow-up. After care follow-up of every
child with OA-TOF in a specialist centre is essential ideally with a
multidisciplinary team to better detect long term morbidity(s) with
prompt better treatments and outcomes.
Advances in neonatal surgery with better intensive care over the past
decade(s) are such that excellent survival (> 97%) is the
norm expected. Outcomes in the modern era of care must therefore focus
on QoL and long-term health. In order to set better outcomes for OA-TOF
children tools such as core outcomes measures (CoS) are required. Such
metrics are currently lacking and collaborative development with health
care professionals and parent support groups notably TOFS UK and EATS is
important .
Large scale multi-centre collaborative national and international data
should be developed. Longitudinal follow up data and registries would
allow for better understanding of the nature and extent of the problems
faced by OA -OF patients and families and more tailored evidence based
management plans.
This current manuscript by Koumbourlis, Belessis et al in Pediatric
Pulmonology is a step forward in the right direction towards consensus
based management of respiratory health in OA-TOF children. We now need
to work together as health care professionals to establish core
outcomes, collate national and international data (with registries) and
ensure these special children receive the best care possible for a long
and healthy life.
1. P, Losty. Esophageal Atresia and Tracheo-Esophageal fistula
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Rickhams neonatal Surgery. London : Springer Publishers, 2018, 23, pp.
541-563.
2. Porcaro F, Valfre L, Rotondi Aufiero L, Angelis P, Villani A,
Bagolan P, Bottero S and Cutrera R. Respiratory problems in children
with esophageal atresia and tracheoesophageal fistula. 1, 2017, ItalianJournal of Paediatrics , Vol. 43, p. 77.
3. DeBoer E, Prager J, Ruiz A, Jenson E, Deterding R,
Friedlander JA, Sodon J. Multidisciplinary care of children with
repaired esophageal atresia and tracheoesophageal fistula. 6, 2016,Pediatric Pulmonology, Vol. 51, pp. 576-81.
4. Koumbourlis A, Belessis Y, Cataletto M, Cutera R, DeBoer E,
Kazachkov M, Laberge S, popler J, Porcaro F, Kovesi T. Care
Recommendations for the Respiratory Complications of Esophageal
Atresia-Tracheoesophageal Fistula: The International Network of
Esophageal Atresia, Respiratory Complications Working Group. 2020,Pediatric Pulmonology .
5. TOFS. [Online] https://www.tofs.org.uk/home.aspx.
6. EAT. Federation of Esophageal atresia and trache-eosophageal
fistula support groups. [Online] https://www.we-are-eat.org/.