Born Unable to Swallow European Reference Networks

European Reference Network

European Reference Networks (ERNs) are virtual networks involving healthcare providers across Europe. They aim to tackle complex or rare diseases and conditions that require highly specialised treatment and a concentration of knowledge and resources.

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To review a patient’s diagnosis and treatment, ERN coordinators will convene ‘virtual’ advisory boards of medical specialists across different disciplines, using a dedicated IT platform and telemedicine tools. The knowledge will travel rather than the patients or doctors. This will facilitate equal access to expert advice and best treatment options. It will increase patient’s chances for better, more effective and more efficient treatment.

The first ERNs were launched in March 2017, involving more than 900 highly-specialized healthcare units from over 300 hospitals in 26 Member States. A total number of 24 ERNs are working on a range of thematic issues including bone disorders, rare cancer, and craniofacial disorders.

The European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) was officially established in March 2017 and is one of 24 European Reference Networks co-funded by the European Union (Health Programme). ERNICA involves teams from 20 European hospitals from 10 Member States. Our network covers rare inherited and congenital digestive disorders, including gastrointestinal disorders, which have in common early manifestation in life and the need for multidisciplinary care and long term follow-up. For these complex and rare diseases, pooling the available resources at European level is a necessity to spur advancements in care and treatment.


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Type A: Oesophageal atresia without tracheoesophageal fistula (8%):
With this type, a large part of the oesophagus, so-called long-gap, is missing and there is no connection to the trachea. This distance has to be bridged: depending on the distance to be bridged, the surgical technique is determined.

Type B: Oesophageal atresia with a proximal tracheoesophageal fistula (1%):
In this case, not only is part of the oesophagus missing, but there is also a connection (fistula) between the upper (proximal) part and the trachea. Saliva or food goes via the fistula to the lungs, instead of to the stomach. The lower part of the oesophagus ends blindly. The atresia is often long-gap.

Type C: Oesophageal atresia with a distal tracheoesophageal fistula (85%):
This is the most common type. There is a connection between the lower (distal) part of the oesophagus and the windpipe, which means a direct connection between the stomach and the airways. The upper half of the oesophagus ends blindly. During breathing the child suffers from the accumulation and overflow of saliva from the closed “pouch”. In addition, the lungs can be irritated from gastric fluid flowing through the connection with the windpipe in the lower part of the oesophagus.

Type D: Oesophageal atresia with both a proximal and a distal tracheoesophageal fistula (1%):
Here we find a combination of both types B and C. This type has a double fistula connection: the upper and lower oesophageal stumps both end into the trachea. Through these two connections, both saliva and stomach contents can go to the lungs.

Type E: Tracheoesophageal fistula without atresia (H-fistula) (4%):
With this type, the oesophagus has normally developed but there is a connection between the oesophagus and the windpipe. This type is usually only noticed a few days or even weeks after birth because there is a passage to the stomach. This allows a child to drink normally. However, food and saliva may enter the lungs, causing the child to choke or having trouble breathing.

Type F: Oesophageal stenosis (1%):
There is no connection between the oesophagus and the trachea, but the oesophagus is constricted. The narrowing can be so severe that nutrition and/or saliva cannot pass through to the stomach.

II   Achalasia
A sphincter, which is a kind of ’valve’, controls the passage of food from the oesophagus to the stomach. It is a bundle of muscle fibres that opens up when food goes from the oesophagus to the stomach. Immediately after that, the sphincter closes to prevent the backward flow of gastric juices into the oesophagus. In the case of achalasia, this lower oesophagus muscle does not work properly: it does not relax sufficiently, so that food remains in the oesophagus after swallowing. This is because the nerves that control this sphincter are affected. Another characteristic of achalasia is the lack of the normal rhythmic contractions of the oesophagus (wave movements) that propel the food from top to bottom into the oesophagus. Probably this is also due to an impairment of the nerves.
Achalasia can occur at any age but usually starts between 20 and 40 years. It can remain unnoticed for a long time and gradually increase over the years.

Other Pages dealing with:


Intestinal diseases

Gastroenterological diseases

Intestinal failure

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Centers of expertise

The ERNICA network involves 20 teams from 10 European countries (Belgium, Denmark, Finland, France, United Kingdom, Italy, Norway, Sweden, The Netherlands and Germany). The multidisciplinary teams of each center are involved in the different diagnostic areas covered in ERNICA.


ERN Centers

With the establishment of the ERNICA network in March 2017, 20 hospitals were officially approved as ERNICA member.