Medicines for Oesophageal atresia and tracheo-oesophageal fistula

What it is

Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are rare conditions, present from birth, affecting the way the oesophagus (the food pipe, which carries food from the mouth to the stomach) has formed. In oesophageal atresia, the oesophagus has not developed properly and is not a continuous tube — instead, it ends in a pouch (a blind end), so it does not connect all the way down to the stomach, meaning food and saliva cannot pass to the stomach normally. In tracheo-oesophageal fistula, there is an abnormal connection (fistula) between the oesophagus and the trachea (the windpipe, which carries air to the lungs) — which should be separate tubes. These two conditions often occur together (in the most common form, the oesophagus ends in a pouch and there is also a connection between the lower part and the windpipe). Because the oesophagus does not connect to the stomach, and because of the abnormal connection to the windpipe, these conditions cause problems from birth and need treatment: a baby with OA/TOF cannot swallow saliva or feed normally, and there is a risk of saliva or feeds getting into the lungs (through the abnormal connection, or because they cannot pass to the stomach), which can cause choking, coughing, and breathing problems. The condition is often detected soon after birth, when the baby has excessive drooling or frothy saliva, coughing or choking, breathing difficulties, or difficulty feeding, or when a feeding tube cannot be passed to the stomach; it may sometimes be suspected before birth (for example if there was excess fluid around the baby in pregnancy). OA/TOF is treated with surgery, usually carried out soon after birth by paediatric surgeons, to close the abnormal connection to the windpipe and join the ends of the oesophagus so that it forms a continuous tube to the stomach. With modern surgery and neonatal care, outcomes are often good, and most children go on to eat and grow, though some may have ongoing issues (such as with swallowing, reflux, or the airway) that are managed over time. OA/TOF can sometimes occur alongside other conditions or as part of a wider pattern, so the baby is assessed for any associated features. The key messages are that OA/TOF is a rare condition present from birth where the food pipe does not form properly and may connect to the windpipe, that it is treated with surgery (usually soon after birth), and that outcomes are often good.

How it is treated

Oesophageal atresia and tracheo-oesophageal fistula are treated with surgery by paediatric surgeons, usually soon after birth, with neonatal care and follow-up; outcomes are often good, and the baby is assessed for any associated conditions. OA/TOF is often detected soon after birth — for example when a baby has excessive frothy saliva or drooling, coughing, choking, or breathing difficulties, or difficulty feeding, or when a feeding tube cannot be passed down to the stomach — and is confirmed with tests (such as X-rays). It may sometimes be suspected before birth (for example if there was excess fluid around the baby in pregnancy — polyhydramnios). Once identified, the baby is cared for by specialists (in a neonatal unit, with paediatric surgeons), and initial care focuses on keeping the baby safe and stable before surgery — for example, keeping the pouch clear of saliva (to protect the lungs), not feeding by mouth, and supporting the baby. The main treatment is surgery, usually carried out soon after birth (the timing depends on the baby’s condition and the specific anatomy): the operation aims to close the abnormal connection (fistula) between the oesophagus and the windpipe, and to join the two ends of the oesophagus together so that it forms a continuous tube from the mouth to the stomach, allowing the baby to feed. In most cases the ends can be joined; in some situations where the gap is large, a staged approach or other techniques may be needed. After surgery, the baby is cared for in the neonatal unit, with support as they recover and gradually establish feeding, and ongoing care and monitoring. Because OA/TOF can be associated with other conditions or be part of a wider pattern of features (affecting other systems), the baby is assessed for any associated features, which are managed as relevant. With modern surgery and neonatal care, outcomes are often good, and most children go on to eat and grow well; however, some children have ongoing issues that are managed over time — such as swallowing difficulties, narrowing of the joined oesophagus (which may need treatment to stretch it), reflux (which is common and managed), or airway or breathing issues — so follow-up and specialist care continue as needed, tailored to the child. Support for the family is an important part of care. Support organisations for OA/TOF provide information and community for families. The reassuring messages are that OA/TOF, although a rare and serious condition present from birth, is treatable with surgery (usually soon after birth), that outcomes are often good with modern surgery and neonatal care, and that any ongoing issues (such as swallowing, reflux, or airway problems) are managed over time with follow-up; so prompt recognition, specialist surgical treatment, and ongoing follow-up are the keys, along with assessing for any associated conditions.