How common is Esophageal Atresia?
Esophageal Atresia and tracheoesophageal fistula are commonly referred to as EA/TEF (or TOF in countries outside of the US.) Basically, this is a condition within unknown cause and occurs around 4-6 weeks in pregnancy.
As a mom of a child with EA, I was desperately in search of numbers and facts. One of the most common questions that came up all the time was “How rare is it?” and “How many kids are born with it?”
Honestly there is very vague information regarding this, so I am going to give you a breakdown of the numbers.
How many babies are born with esophageal atresia each year?
The most common response to this question is that 1 in every 4,000 births have EA. Well, that doesn’t really give you the answer, does it?
To break this down, we are going to look at the number of births in 2021 in the United States. According to the CDC, they estimate that there were 3,659,289 born in the US in 2021.
Next, we will divide 3,659,289 by 4,000 and we get 914.8. The estimated number of babies born in the US with EA is approximately 915 babies for the year 2021.
3 million is a huge number, but when you see that out of the number approximately only 915 are born with EA/TEF it starts to make you realize just how rare this condition is.
Each year, only 0.03% of all babies born will have Esophageal Atresia and/or tracheoesophageal fistula.
We can take this math a little further and see how many children are estimated to have EA/TEF in the whole world. UNICEF estimates that 140 million children were born in 2021, so that means an estimated 35,000 children born in 2021 will have EA/TEF.
How many babies have each type of esophageal atresia?
We already know that EA/TEF is extremely rare. All EA/TEF cases are then further segmented into types.
There are 5 main types of EA/TEF, and some are much rarer than others. Here is a chart breaking down how the estimated 915 babies born with EA/TEF would probably be sorted by type.
(This is just an estimate based on percentages given by RareDiseases.org)
| Type of EA/TEF | Estimated Percentage | Estimated Number of Babies affected in 2021 |
| Type A | 8% | 73 babies |
| Type B | 2% | 18 babies |
| Type C | 85% | 778 babies |
| Type D | less than 1% | 9 babies |
| Type E | 4 % | 37 babies |
| 100% | 915 babies |
I’m breaking down these numbers not to scare you, but to better inform you about this birth defect and how often it occurs.
How rare is esophageal atresia?
I feel like we sometimes need to be able to compare numbers to truly understand them. I have compiled a list of the 10 most common birth defects in the US and how often they occur.
I am going to use the 2021 birth data of 3,659,289 to estimate the number of babies born with each defect.
| Birth Defect | Rate of Occurance | Estimated Number of Babies born with the birth defect in 2021 |
| 1. Congenital Heart Defects (CHD) | 1 in every 110 births | 33,266 |
| 2. Hypospadias (males only) | 1 in every 200 births | 18,296 |
| 3. Ventricular Septal Defects (heart) | 1 in every 240 births | 15,247 |
| 4. Clubfoot | 1 in every 593 births | 6,171 |
| 5. Down Syndrome | 1 in every 700 births | 5,228 |
| 6. Pulmonary valve atresia and stenosis (heart) | 1 in every 1,052 births | 3,478 |
| 7. Cleft lip with cleft palate | 1 in every 1,563 births | 2,341 |
| 8. Cleft palate | 1 in every 1,687 births | 2,169 |
| 9. Atrioventricular septal defect (heart) | 1 in every 1,859 births | 1,968 |
| 10. Limb defects | 1 in every 1,943 births | 1,883 |
All numbers given are purely estimates based on available data for 2021.
For more information related to EA/TEF, check out “Esophageal Atresia & Stuck Food (Tips & Tricks)“
