Back Print

Tracheo-oesophageal Fistula and/or Oesophageal Atresia

Also known as: TOF/OA

Background

Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are rare conditions of the oesophagus (the pipe that food goes down from your mouth to the stomach) and/or trachea (the tube that air passes down from the mouth and nose to the lungs). They are congenital conditions, which means people are born with them and so it affects newborn babies.

Credits

Medical text written December 2005 by Mr B Jaffray. Last updated November 2014 by Mr Joseph Curry, Consultant Neonatal and Paediatric Surgeon, Great Ormond Street Hospital, London, UK.

What are the symptoms?

With OA, the oesophagus does not connect the mouth and stomach properly, but forms a closed off pouch that prevents food from reaching the stomach. If it is not corrected, this pouch can fill up with food and saliva, which can eventually overflow into the baby's trachea (windpipe), entering the lungs and causing choking. With TOF, usually the lower part of the oesophagus is connected to the windpipe. Without treatment, this causes air to pass from the windpipe to the food pipe and stomach. It can also allow stomach acid to pass into the lungs.

Most commonly, babies have both TOF and OA, though on rare occasions either TOF or OA can occur on their own.

VACTERL Association
Babies with TOF/OA may also have other health problems, particularly heart defects, imperforate anus (opening to the anus is missing or blocked) and problems with the kidneys, spine and limbs. There is a recognised association between a particular group of difficulties, which has been called the VACTERL Association.

What are the causes?

Unfortunately, the causes of TOA/OA are still unknown.

How is it diagnosed?

Doctors will suspect TOF/OA if babies froth at the mouth, splutter or choke after their first attempt at feeding, or have laboured breathing and/or blueness of the lips and fingertips. If OA is suspected, a tube will be passed down into the oesophagus from the mouth. When the baby has an OA, the tube will not pass beyond the end of the upper part of the oesophagus, and this finding will confirm the diagnosis. If the tube goes right down to the stomach then this rules out the diagnosis.

How is it treated?

Babies with TOF/OA require intensive care in a special baby unit. Surgery to correct the TOF/OA usually happens within days of the baby being born. Some children have to undergo additional surgery later on in life.

Inheritance patterns and prenatal diagnosis

Inheritance
None in the majority of cases.

Prenatal diagnosis
Maternal polyhydramnios (excessive quantity of amniotic fluid) may indicate the presence of oesophageal atresia especially if accompanied by a small or absent stomach in the developing fetus. This can be detected by prenatal ultrasound. All babies born to a mum who has polyhydramnios should have a tube passed into the oesophagus at birth to rule out OA.

Is there support?

TOFS (Tracheo-Oesophageal Fistula Support)

Tel: 0115 961 3092
Email: info@tofs.org.uk
www.tofs.org.uk

The Organisation is a Registered Charity in England and Wales No. 327735. It provides information and support to families of children born with Tracheo-Oesophageal Fistula (TOF), Oesophageal Atresia (OA) and associated conditions. The Organisation offers one-to-one support from parents and relatives with experience of caring for a child with TOF/OA, social events and activities, and an online community.

Group details last updated December 2014.