Oesophageal Atresia in Children: Causes, Symptoms, and Treatment Options

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What is Oesophageal Atresia?

Oesophageal atresia occurs when the oesophagus doesn’t develop normally in utero. The upper part of the oesophagus (gullet) is a blind-ending pouch. The lower part is commonly connected to the trachea (windpipe).

Babies with oesophageal atresia are born without being able to swallow saliva or milk. These can spill into the airways and lungs, causing difficulty breathing and lung infections. Without treatment, oesophageal atresia is a fatal condition.

The goal of surgery for oesophageal atresia is to disconnect the oesophagus from the trachea, and to join the two ends of the oesophagus back together to create oesophageal continuity.

How Are The Words Pronounced?

Oesophagus ee-SOF-ah-gus

Oesophageal ee-SOF-ah-jeel

OA/TOF Oh-A toff

Trachea Tra-KEY-ah, or TRAY-key-ah

Polyhydramnios Polly-high-dram-knee-oss

Thoracotomy THOR-ah-cot-oh-me

Thoracoscopy THOR-ah-koss-copy

Replogle Rep-LOW-gill

Tracheomalacia TRAY-key-oh-mal-aish-ee-ah

Scoliosis SKOL-ee-oh-sis

Is there More than One Type of Oesophageal Atresia?

Yes – there are several types. Some are much rarer than others.

The most common type is oesophageal atresia, with a connection between the lower part of the oesophagus and the trachea. This connection is called a tracheo-oesophageal fistula (TOF), and this type is often called ‘OA distal TOF’. OA distal TOF accounts for about 85% of all OA/TOF children.

The next most common type is ‘pure’ oesophageal atresia (6-8%). In this type, there is no connection to the trachea anywhere, but the two ends of the oesophagus tend to be a long way apart from each other. This is one of the ‘long-gap’ OAs.

Some children who look like they have pure oesophageal atresia also have a connection to the trachea in the upper part of the oesophagus – ‘OA proximal TOF’ (about 2%). Like pure OA, this is a long-gap oesophageal atresia.

Just as you can have OA without a TOF, you can also have a TOF without an OA. This type is commonly called a ‘H fistula’, as there is a connection between oesophagus and trachea. This type is often diagnosed later, since these babies can feed normally from birth. The spillover can lead to choking episodes and repeated chest infections – this is how this group is commonly diagnosed.

Finally, you can have connections to the trachea at both the top end and the bottom end of the oesophagus – ‘OA double TOF’. This is the rarest of the commonly seen variants (1-2%).

How is Oesophageal Atresia Diagnosed?

Babies with oesophageal atresia cannot swallow in utero. Because of that the stomach might appear very small, or difficult to find (‘absent’) on antenatal scanning. Because they cannot swallow, the amniotic fluid increases around the baby. Sometimes, the swollen upper oesophagus can also be seen on scanning. Not all babies with OA are diagnosed antenatally, and not all babies suspected of having OA antenatally will actually turn out to have the condition.

After babies are born, OA can be suspected if babies are very frothy or cannot swallow. The simplest test is to try and pass a nasogastric tube at the cotside. If the baby has OA, the tube will pass easily for about 10cm before stopping in the base of the upper pouch.

Does Oesophageal Atresia occur with Other Anomalies?

Yes – but not in all babies. Many babies will only have oesophageal atresia, with no other anomalies.

There is a cluster of other conditions that can occur in babies with OA. They are common enough that we look for them routinely and have a name to help us remember them. That name is VACTERL – each letter stands for something:

V vertebral (spine) anomalies: extra ribs, missing backbone elements
A ano-rectal anomalies: issues with the lower part of the bowel and anus
C cardiac anomalies: abnormalities of the structure of the heart
T tracheo-
E esophageal (because it’s American and they spell differently)
R renal (kidney) anomalies: absent or deformed kidney, poor drainage
L limb anomalies: missing or deformed thumb, absent forearm bones

All babies will have a careful examination, and ultrasound scans of their kidneys, heart, and spine as a routine part of their workup before any operations.

OA is more common in babies with trisomies. Trisomies occur when there is an extra copy of one of the chromosomes in every cell. The most common trisomy is trisomy 21 – Down (or Down’s) Syndrome, but there are others. Most are detectable antenatally.

OA can also occur in a number of other syndromes. While rare, these combinations occurs often enough that it’s usually not the first time your child’s treating team will have seen them.

What Happens After Oesophageal Atresia is Diagnosed?

The Neonatal team will call us, the Paediatric Surgeons. If OA was suspected in utero you will already have met both a Neonatologist and a Paediatric Surgeon antenatally, who will have gone through some of this already with you.

Your baby will be moved to the Neonatal Intensive Care Unit. They will have an intravenous line and be started on medications. Ideally, we will keep your baby breathing on their own rather than anaesthetise and intubate them. To control secretions, your baby will have a small suction tube (a Replogle tube) placed into the upper oesophagus. This will be suctioned and flushed every 10 minutes or so to clear secretions that may otherwise spill over into the airway.

A number of tests will be done to determine the type of OA your baby has, and if there are any associated anomalies. An x-ray of the baby will be done; and ultrasound scans of the heart, kidneys, and spine.

Normal baby

How is Oesophageal Atresia Repaired?

It depends on the type of OA, the size of the baby, and how sick or well they are.

Repair of most OAs is done through the right side of the chest – either with a large incision around the side of the chest spreading the ribs (thoracotomy) or with thoracoscopy (keyhole surgery). The surgeon will find the lower oesophagus and follow it back up to its connection to the trachea. This will then be divided and the trachea closed. The surgeon will then find the upper pouch, and free both ends to bring them closer together before joining them. The surgeon might pass a nasogastric tube through the join into the stomach, and/or a chest drain alongside the repair to allow drainage of any leakage of fluid.

Proximal fistulas, and H-TOFs, are often divided using an incision in the right side of the neck.

In babies with long-gap OA it’s usually not possible to join the two ends immediately. In those cases the surgeon will usually perform a gastrostomy as the first operation, to allow feeding while the baby grows and plans are made for subsequent repair of the OA. See our Gastrostomy page for further information.

Post-operatively, babies are continued on antibiotics and pain relief. We check the integrity of the join by having the baby swallow x-ray dye to confirm there are no leaks, before allowing babies to feed. This is done a few days to a week after the operation.

What Are the Risks of the Operation?

Oesophageal atresia repair is one of the more complex Paediatric Surgical operations. Successful repair of OA is less than 100 years old, and there are a number of risks. Without an operation though, the condition is fatal.

Anaesthetic Risks: Airway and breathing issues are rare.
Airway Injury: In rare cases there is injury to the trachea. If recognised immediately it can be repaired but the child will need to stay intubated for a while after the operation.
Infection: There is much care and attention required after surgery. 
Bleeding: This operation is close to major blood vessels in the chest. The risk of major bleeding is small. 
Leaking: The joined oesophagus can leak, spilling saliva into the chest.  
Bleeding, Bruising, and Swelling: These are common minor side effects of surgery and typically resolve without issue. 
Inability to join: In some cases the two ends of the oesophagus are too far apart, or the baby is too unwell, for a join to succeed. In those situations the fistula will be divided and a gastrostomy formed. The eventual oesophageal join will be delayed by weeks to a few months.

Pain Relief After Surgery

Your child will receive good pain relief during the operation, and afterwards. This is a major operation, and so strong pain relief will be needed. This pain relief is so strong that it can sometimes interfere with baby’s breathing. If that is the case the breathing tube will remain for some time after the operation.

As days pass from the surgery, pain levels will fall and the strength of the pain relief can be decreased.

What to Expect After Surgery

Once the surgery is complete, your child will be taken directly back to the Neonatal Intensive Care Unit. You will be able to join them shortly after. It is quite common for children to remain intubated for a day or two post-operatively. Feeds can start via the nasogastric tube, even with the baby intubated. Feeds will be stopped before extubation. 

Some days after surgery an x-ray dye test will be done to check the integrity of the join. If it’s fine the baby can be allowed to suckle. Chest drains are removed after a normal x-ray dye test.

Long-Term Effects of Oesophageal Atresia

Oesophageal atresia can have many longer-term effects on your child:

Reflux: All babies have reflux, but it is often more severe and can last for longer in children with oesophageal atresia.
Swallowing and feeding difficulties: Reflux can make babies not want to eat. In addition, babies who have had a Replogle tube for a long time may also develop an aversion to feeding. Narrowing at the join in the oesophagus can further make it difficult to feed.
Oral aversion: the most severe form of feeding and swallowing difficulty, where children don’t want to feed. These children often require a gastrostomy.
Dysmotility: another contributor to poor feeding. The oesophagus doesn’t function normally, further affecting swallowing.
Stricture: this is a narrowing at the join in the oesophagus. Acid reflux can make this stricture worse. Children sometimes need to have this stricture dilated under general anaesthetic
‘Stickies’: this is where food becomes stuck at the join in the oesophagus, either because of narrowing (a stricture) or because of dysmotility. Stickies sometimes clear with drinking but sometimes need an operation to remove the obstructing food particles.
Aspiration: if feeding is impaired, then the normal mechanisms stopping food or liquids going down the wrong way can also be impaired. This can lead to some swallowed contents getting into the airways.
Recurrent fistula: if the tracheo-oesophageal fistula re-forms, this can also lead to aspiration.
Tracheomalacia: the trachea in babies with oesophageal atresia is floppier than normal and can collapse. This in turn can lead to noisy breathing or recurrent infections.
Acute life-threatening events (ALTEs): in severe cases, tracheomalacia or aspiration can cause babies to stop breathing and become blue. It’s wise for parents to learn CPR techniques for their baby before leaving hospital. Some ALTEs occur so frequently further operations are needed to decrease the risk of them occurring.
Chest wall deformity / scoliosis: OA can occur with vertebral deformities, and operations for OA can lead to fused ribs after surgery. This can lead to a bend in the spine (scoliosis) or asymmetry of the chest wall as the child grows.
Poor growth: In the past poor growth of children with OA was common. We now are much more determined to not let that happen.
Oesophageal cancer: It is now known adults who had oesophageal atresia have a slightly higher risk of cancer of the oesophagus. This risk does not appear in childhood and it is possibly due to the long-term effects of acid reflux. Ongoing surveillance of the oesophagus in adult life is recommended.

Who Looks After Oesophageal Atresia Children Long-term?

Because the list of longer-term effects is so large, all children with OA in Queensland are now followed by the Interdisciplinary Aerodigestive Clinic at Queensland Children’s Hospital. This clinic has:

Paediatric Surgeons
Paediatric Gastroenterologists
Paediatric Respiratory Physicians
Paediatric ENT Surgeons
Dietitians
Speech and Language Therapists
Physiotherapists

The clinic is designed as a single place where families can see all the above specialties in one visit to the hospital, rather than having to attend multiple visits. The Aerodigestive Clinic works with your local GP and Paediatrician to deliver the best care for your child.

FAQs About Oesophageal Atresia Surgery in Children

How long does the surgery take?

The operation takes several hours, depending on its complexity and the type of OA/TOF your child has.

Is OA surgery safe for children?

OA surgery is complex and can be risky but the alternative is to leave the child with oesophageal atresia. Untreated, this condition is universally fatal.

Will my child be in pain after surgery?

Yes – but the neonatal and paediatric surgical teams will make sure your child has adequate pain relief.

How long will it take for my child to recover?

Recovery depends on the complexity of the surgery. Many children are able to be fed within a day or two of surgery. Oral feeding may not start for a week or so after the operation.

Recovery to discharge from hospital may take several weeks in routine cases, but more complex cases may be in hospital for 3-6 months. More complex cases usually require more than one operation over a series of months.