EA Conference October 2-3, 2014 Rotterdam

4 October 2014

The 3rd International Conference on Esophageal Atresia was held in Rotterdam, The Netherlands, on October 2 and 3, 2014.

This meeting put together multidisciplinary teams and family support groups dedicated to the care of EA patients all over the world.

Here are the storify reviews of these two days:




Abstracts of the different presentations during the conference


Angelo Barbato, Dept. of Women’s and Children’s Health (SDB)-University of Padova/General Hospital (09.00 October 2)

Fiberoptic bronchoscopy (FB) is nowadays considered the “gold standard” procedure for identifying trachea­ bronchomalacia (TBM) in preschool children. It requires conscious sedation or deep sedation so that the child can ventilate spontaneously during the procedure, providing a record of any variations in the shape of the trachea during inhalations and deep exhalations.
Modem pediatric video fiberoptic bronchoscopes are capable of generating good-quality images, which can be saved to enable a deferred accurate examination of the cross section of the trachea and the severity of any TBM.
If FB is unavailable, then other procedures (dynamic volumetric MDCT scan, virtual bronchoscopy, cine-MRI, bronchography, fluoroscopy) can be used to assess patients with TBM, albeit with their limitations relating to the age of the patient and the amount of radiation involved.
As they evolve, CT scanning and MRI technologies will probably play an even larger part in the imaging of the pediatric airways and surrounding structures.
Angio-CT or angio-MRI are necessary to study patients who have TBM associated with vascular compression.
A stricter diagnostic criterion than the 50% reduction in the cross section of the trachea should be adopted to prevent the overdiagnosis of TBM in children normally presenting a high physiological compliance of the tracheal and bronchial structures.


Pierluigi Ciel (09.25 October 2)

Teaching Points
Central airways assessment is routinely performed with bronchoscopy, which requires sedation and does not allow exact airway measurements. Although cine-CT is often used as a non-invasive alternative, its use is limited by radiation. MRI, as a radiation-free technique, is an attractive alternative for CT. We aim to give a comprehensive overview of MRI capabilities to assess central airways disease in pediatric and adult patients in order to assist radiologists in “imaging genUy and wisely.” Table of Contents/Outline
We will provide a practical approach to assess pediatric and adult central airways disease with MRI. We will describe and illustrate: 1) advantages of MRI over CT/bronchoscopy; 2) 20 vs. 30 MRI; 3) static vs. dynamic MRI; 4) spirometry-gated dynamic MRI; and 5) contrast enhanced MRI of the central airways.
MRI has the advantage of providing 20, 30, static and dynamic evaluation of central airways collapsibility without radiation exposure. Specific breathing maneuvers can be performed even in the pediatric population in reproducible fashion by using a MRl-rompatible spirometer. MRI can supply functional information, which cannot be obtained by bronchoscopy/CT. By providing a practical set of MRI protocols for daily practice, this exhibit will facilitate the ability of radiologists to wisely select MRI over CT for specific airway indications.


R. Jennings  (09.40 October 2)

Tracheobronchomalacia (TBM) is commonly associated with developmental syndromes, including a high incidence in esophageal atresia patients. TBM has been described as a deficiency in the structural integrity of the tracheal rings, resulting in dynamic airway collapse. The airway collapse is felt to cause decreased air movement and impaired clearance of secretions. Consequences can include increased work of breathing, increased respiratory infections, hypoxic episodes, and death spells.

We review some of the clinical presentations of TBM, and the pathophysiology of TBM. We have identified the regions and locations of narrowing of the airway, and developed a classification system for TBM which appears to have some merit.

We then review the anatomy of TBM including surrounding structures and their contribution to both static and dynamic airway narrowing. The anatomical relationships of the aorta, innominate artery, pulmonary arteries and other structures are somewhat variable., and significant.

We then review the theory behind treatment of the various forms of TBM. We review some therapeutic options and their results, and provide new options for some types of TBM.

Johan C. de Jongste (10.05 October 2)

The aims of treating children with tracheomalacia is to reduce the symptoms that are due to dynamic airway collapse, improve mucociliary clearance, reduce the risk of airway infections and thereby improve the long-term prognosis. There is a very weak relation between the severity of malacia symptoms, observed abnormalities at bronchoscopy and lung function impairment.For this purpose, a number of strategies are possible: surgical, physiotherapy, antibiotic, mucolytic and bronchodilator treatment. Surgery is presently only relevant for children with severe, life threatening episodes of airway obstruction and this is mainly the case in infancy. Physiotherapy is directed against airway collapse, and to promote sputum evacuation. The positive expiratory pressure (PEP) mask is widely used for this purpose, but evidence of a beneficial effect is limited, and comes from studies in cystic fibrosis. Low-threshold prescription of antibiotic courses and maintenance treatment with low- beta-2-agonists in malacia, both in infants and older children. There are no data on the effect of inhaled corticosteroids in malacia. One should keep in mind that symptoms in children with malacia may be due to comorbidity, including reflux
and aspiration, ENT infections, or asthma and atopy. This may give rise to other therapies. Establishing an asthma diagnosis in a child with malacia is a challenge, and involves careful medical and family history taking, allergy testing, lung function and exhaled nitric oxide measurements and a closely supervised therapeutic trial with an inhaled steroid during 2-3 months. The symptoms of malacia commonly improve over many years, while the anatomy and lung function remain unchanged. Although it may seem unlikely that any drug treatment will substantially influence the prognosis of malacia, careful long-term follow-up studies and evaluation of current treatment approaches are needed.


Dr Mark A Chilvers, MB ChB, BSC, MD, MRCPCH Pediatric Respirologist and CF Clinic Director
BC Childrens Hospital, Vancouver, British Columbia, Canada (11.00 October 2)

Airway clearance involves the interplay between cough, anatomical barriers, aerodynamic changes and cellular mechanisms. The lower respiratory tract is protected by local mucociliary clearance mechanisms that involve the integration of ciliated epithelium, mucus and periciliary fluid. Mucus acts as a physical and chemical barrier onto which particles and organisms adhere. Cilia lining the respiratory tract beat in a regular co- Disruption of the interplay between ciliated epithelium, periciliary fluid and mucus may be primary, from diseases such as cystic fibrosis, primary ciliary dyskinesia and airway abnormalities resulting in tracheomalacia, or secondary due to a variety of insults including environmental pollutants, toxins, viral and bacterial infections. This may result in abnormal ciliary function due to impaired ciliary beat pattern, beat frequency or a combination of both. This failure leads to a build up of mucus within the airways and subsequent airway blockage, infection and airway damage. Overlime this may cause permanent damage and result in bronchiectasis.
Strategies to assist airway clearance range from cough or huffing manoeuvres to positive pressure physiotherapy techniques to assist in collateral ventilation and open up collapsed airways. Mucolytics, such as pulmozyme may be of use to assist mucus breakdown allowing easier clearance by the ciliated epithelium. Ciliary beating may be facilitated by alteration of the periciliary fluid layer with agents such as hypertonic saline or mannitol.
In summary, mucociliary clearance is essential for lung health. If impaired or defective strategies need to be employed to assist and support airway clearance within the lung to prevent permanent damage.


LJ van der Giessen PhD, pediatric physiotherapist, Erasmus MC Sophia Children’s Hospital, Rotterdam, The Netherlands
(11.25 October 2)

Tracheomalacia (TM) is one of the few causes of irreversible airways obstruction in children, with symptoms varying from recurrent wheeze, exercise intolerance, cough, and recurrent lower airways infections to severe dyspnea and respiratory insufficiency. (1) The incidence in the general population is unknown. (1) In patients with a oesophageal atresia with a distal tracheo-50%) but also in other forms (type II, Ill and IV). It remains a major surgical challenge. Options for esophageal reconstruction include the use of native esophagus -which is the preferred strategy- or esophageal replacement with stomach, colon, or small intestine. Besides surgical difficulties, digestive and nutritional problems appear much more frequent than in other forms of EA and are responsible for a high morbidity. These problems result of the combination of several factors: EA per se (gastric and esophageal dysmotility, GERO), anatomical form (microgastria), and surgery (lack of oral feeding due to delayed anastomosis, anastomotic tension, esophageal stenosis, GERO, consequences of esophageal replacement).
Several factors contribute to the physiopathology of GERO in long gap EA. Excessive tension at the esophageal anastomosis is associated with a higher incidence of GERO. Increased GERO associated with anastomotic tension could be related to a decrease in lower sphincter tone and shortening of the intra-abdominal esophageal segment or deformity of the cardioesophageal junction. Abnormal esophageal motility reduces esophageal clearance. Esogastric dysmotility is more severe in long gap EA while there is no relationship between severity of esogastric dysmotility and symptoms. In a study combining pH-impedancemetry, manometry and gastric emptying data to evaluate mechanisms underlying GER events and gastroesophageal function in EA Type C (no study in long gap EA are available), TLESR was shown to be the main mechanism underlying GER episodes in EA patients both shortly after primary anastomosis and in adulthood.
Slow gastric emptying has been also reported and could be congenital or the consequence of vagal nerve injury that may occur during dissection of the distal esophageal segment. However relationship between delayed gastric emptying and symptoms of GER remain controversial, since in most of the studies no correlation was seen between gastric emptying and severity of esophageal motor abnormalities or GER episodes. Abnormal gastric myoelectrical activity as demonstrated with electrogastrography is a marker of disturbed neuromuscular function that plays a conflicting, probably minor role in the pathogenesis of GERO. Antral hypomotility has also been reported in EA patients using gastric manometry. The aggravating role of gastrostomy is controversial. Comparing 2 populations of EA patients with or without gastrostomy, no difference in GER frequency was found at pH-metry, nor in fundoplication rate, suggesting that gastrostomy did not play any role in the GER pathophysiology in these patients. In a recent study combining long gap
and non-long gap EA patients, gastrostomy was a significant predictor for having a fundoplication. This could be because severe GERO with vomiting often necessitates a gastrostomy, gastrostomy can worsen GERO, or that long gap EA patients often require gastrostomy and long gap is associated with severe GERO.
Compared to non-long gap population, the need of repeated dilations (up to 50%), fundoplication (often performed very early in life) (20 to 44 %), as well as gastrostomy (near 100%), and malnutrition (33%) are much higher in long gap EA. Microgastria when severe often represents an additional challenging problem for gastrostomy feeding (feeding intolerance, leakage around the stomy, dumping syndrome) and for fundoplication.
Although there are no randomized studies comparing esophageal end to end anastomosis to gastro/coloplasty, and the published series are small and retrospective, it has been suggested a higher risk of recurrent esophageal stenosis in end to end anastomosis, similar rate of GERO but more undemutrition specially in the long term in gastro/coloplasty.
The long gap population is a small but high-risk group for prolonged digestive and nutritional problems. These patients require a prolonged multidisciplinary follow-up and should be referred to reference centers.


Rachel R. Rosen (14.45 October 2)

Abstract: Respiratory symptoms are common in children with esophageal atresia yet one of the oveooked reasons for these symptoms is oropharyngeal dysfunction and aspiration. Without an understanding of the causes for oropharyngeal dysfunction and aspiration, children with EA will be incorrecUy diagnosed with gastroesophageal reflux and managed incorrectly with continue respiratory morbidity. It is the goal of this presentation to (1) understand the causes for oropharyngeal dysfunction and respiratory symptoms in children with EA; (2) to pursue the correct diagnostic work-up for patients with feeding difficulties and aerodigestive symptoms; (3) understand the rationale behind different feeding strategies for EA patients at risk for aspiration; and (4) synthesize the non-EA literature on aspiration and how it relates to patients with EA.


Daniel von Allmen; Cincinnati Children’s Hospital Medical Center (15.10 0ctober 2)

Surgical procedures to repair esophageal atresia and tracheoesophageal fistula are associated with frequent complications that require secondary repairs. The operative procedures span a wide spectrum of interventions ranging from endoscopic treatment of recurrent TEF to significant open operations on the esophagus and/or trachea including bypass or replacement of the esophagus. In this presentation the options for treatment will be reviewed along with the experience from the Children’s Hospital of Cincinnati Aerodigestive and Esophageal Center.


Maria Ramsay, Ph.D. (09.20 October 3)

To feed or eat, we need an adequate oral sensory-motor pattern to propel the liquid or food from the lips to the pharynx. A coordinated pharyngeal and esophageal peristalsis then propels the food to the stomach. In the feeding process, this part is the second phase. However, the feeding process actually starts well before sucking and swallowing takes place, and difficulties in the first phase has implications for the second. In my talk Iwill focus on the first phase, which includes the physiology of feeding (appetite, odour and taste) and mother-child relationships, as well as the interrelationship of the
two feeding phases.

Newborn infants feed in the context of mother-child relationship. Infants cry and mothers respond by holding and feeding. Both infants and mothers are wired for this life-sustaining act in the context of bonding and maternal expectation of being the primary feeder. This expectation gets interrupted when an infant is born with EA-TEF, and thus, the second phase of the feeding process is not optimal. Depending on the severity and complexity associated with of EA-TEF, this interruption may be for several months. In addition, at times the first phase is also affected independently from EA-TEF by the
infanrs level of appetite and sensitivities to food odour and taste, as well as by maternal reaction to feeding difficulties.

Thus, timely intervention to aid the recovery of the feeding process and to support mothers and families in their attempt at re-establishing their primary role should be part of multidisciplinary care of this fragile population.


Manon Spaander, Gastroenterologist EMC, Rotterdam (10.45 October 3)

After repair of esophageal atresia (EA) in newborns, gastroesophageal reflux (GER) is reported during follow-up. The high incidence of GER and extended survival among individuals with repaired EA, raises concerns about the possibility of an increased risk of Barretfs esophagus and esophageal cancer.
Results presented
Overview of esophageal cancer cases in literature .
Preliminary results of screening in esophageal atresia patient in the Rotterdam cohort. Implications for screening protocols in these patients.


A.O. Koch, gastroenterologist (11.05 October 3)

Endoscopic resection of early esophageal neoplasia is a feasible and organ preserving treatment modality and has become the treatment of choice in the western world, Japan and large parts of Asia. Resected lesions offer accurate histopathological assessment and provide a precise prediction of the chances of lymph node metastasis. Excellent survival rates as well as preservation of quality of life can be expected if endoscopic resections are carried out following strict oncological rules in patient selection.


Benjamin D. Solomon, MD (13.45 October 3)

Although esophageal atresia (EA) is not a rare congenital anomaly, the overall causes of the condition remain incompletely understood, and genetic explanations are available in the minority of affected patients. EA may occur as an isolated anomaly, and is typically sporadic in these instances, but frequently co-occurs with other major anomalies, such as in VACTERUVATER association, or as part of a less-


Henneke IJsselstijn MD PhD, Dept. of Pediatric Surgery and Intensive Care, Erasmus MC – Sophia Children’s Hospital,
Rotterdam, The Netherlands (14.25 October 3)

Compared to several decades ago surgical and pediatric intensive care treatment modalities have improved resulting in more surviving esophageal atresia (EA) patients nowadays. Therefore, it becomes even more important to evaluate long­ term morbidity and quality of life of the surviving child and of its family. A structured multidisciplinary follow-up program is important to improve care and to perform adequate transition into adulthood on an individual basis. By evaluation of data obtained in a standardized way – i.e. by using structured assessment moments and standardized instruments – follow-up programs can be adapted to provide appropriate care and interventions can be initialed at an early stage. Moreover, risk factors for adverse outcome may be identified.
During the presentation the balance between patient care and outcome research will be discussed and results of EA patients who join our follow-up program in children with anatomical congenital anomalies will be shown as an example. This multidisciplinary follow-up program, which was initialed 15 years ago, provided unique data on somatic and
developmental outcome and formed the basis for randomized intervention studies aiming at improvement of long-term


C.P. van de Ven (16.10 0ctober 3)

Since the introduction of thoracoscopic repair for esophageal atresia 15 years ago, there is still debate on the “gold standard” of esophageal repair. In the article of Laberge et al. a critical analysis is done of the pros and cons of open versus thoracoscopic repair. They conclude that hard evidence favoring one or the other lacks and Tovars conclusion in their paper of 2011 prevails hat the gold standard remains a good anastomosis with survival, limited sequelae and good
quality of life3 8 Referring to the Balliol collaboration in 2009, proposing a systematic way of introducing new surgical technique should contain certain successive steps. Starting from the initial idea where a few innovators explore
possibilities of a new concept, the second stage of exploration and development focuses on refinement of the technique and conducting a comparative retrospective series to compare the new technique with the open approach 2
With an increasing number of “converts”, evaluation should be performed in a prospective trial and the final assessment could comprise a long term evaluation.
The impression exists that in case of esophageal repair we appear to stagnate in the second step. Adoption of the new technique by only 6% of delegates to the 2012 Eupsa-Baps conference, in a survey by Zani et all. illustrates the fact that this technique still is not widely adopted’. Spreading the technique, not by experimenting but by teaching sessions in the
lab and mentoring in real practice should be performed carefully with minimal risk to the patient.
Although randomization would be the best solution suggestions of a multicenter, comparative study in a registry fashion seems more feasible.

1. Van der Zee DC, Bax NMA. Thoracoscopic treatment of esophageal atresia with distal fistula and of tracheomalacia.
Seminars in Pediatric Surgery 2007;16:224-230
2. McCulloch P, Altman DG, Campbell WB, Flum DR, Glasziou P, Marshall JC, Nicholl J, for the Balliol Collaboration. No surgical innovation without evaluation: the IDEAL recommendations. Lancet 2009;374:1105-12
3. Tovar JA, Fragoso AC. CutTent controversies in the surgical treatment of esophageal atresia. Scand J Surg. 2011;100(4):273-8.
4. Oomen MWN. Systematic review of the literature: comparison of open and minimal access surgery (thoracoscopic repair) of esophageal atresia with tracho-esophageal fistula. Front lines of thoracic surgery. 2012;309-18.
5. Borruto FA, lmpellizzeri P, Montalto AS, ANtonuccio P, Santacaterina E, Scalfari G, Arena F, Romeo C.
Thoracoscopy versus Thoracotomy for esophageal atresia and tracheoesophageal fistula repair: Review of the literature and meta-analysis. Eur J pediatr. surg. 2012;415-19
6. Dingemann C, Ure B. Minamally invasive repair of esophageal atresia: An update. Eur J pediatr surg 2013;23:198- 203.
7. Zani A, Eaton S, HOllwarth M, Puri P, Tovar J, Fasching G, Bagolan P, Lukac M, Wljnen R, Kuebler J, Cechetto G, Rintala R, Pierro A. lntemational survey on the management of esophageal atresia. Eur J pediatr surg. 2014; 24: 3-8.
8. Laberge JM, Blair GK. Thoracotomy for repair of esophageal atresia: not as bad as they want you to think! Diseases of the Esophagus. 2013;26;3657


AGraham Slater-Chair, EAT (and a Board member of TOFS, the English EA support group) JoAnne Fruithof – Vice-Chair, EAT (and a Board Member of VOKS, the Dutch EA support group) (16.30 October 3)

EAT (the Federation of Esophageal Atresia and Tracheo-asophageal fistula support groups e.V.) is an international federation of support groups which support both the families of those suffering from esophageal atresia and patient survivors of the condition.
In July and August 2014, EAT conducted an internet-based international survey of parents/carers and EA patients which generated very neariy 1000 responses. This survey – which was available in five languages (English, Dutch, French, German and Italian) – comprised forty questions which, besides capturing each patient’s nationality, date of birth, gender, height and weight, and initial condition, also covered a number of topics ranging from number of surgeries, follow­
uplafter- relationships.
Our initial analysis of the survey results shows that the vast majority of the respondents were satisfied or indeed very satisfied with the initial surgery. Similariy most respondents are happy with their after- As the survey is so recent, we have so far only completed a limited analysis but we are pleased to have the opportunity
to present our current findings at this congress; we intend to carry out further analysis and publish our findings in more detail at a later date.

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