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Esophageal Atresia

National centralization of care has resulted in increased knowledge, quality, and patient safety, and Karolinska is now one of Europe's largest centers for children with esophageal atresia.

Esophageal atresia is a rare congenital malformation where there is no connection between the esophagus and the stomach. About 25 of 100,000 Swedish children are born with this condition annually.  

Babies with esophageal atresia usually have symptoms right after birth. They have a high amount of saliva in their mouths, have difficulty breathing, cough, and exhibit attacks of cyanosis (blue skin color). Symptoms often increase during nursing, and the diagnosis is verified by a simple pulmonary x-ray. 

The condition is usually detected right after birth when the baby tries to nurse, but in some cases, it is suspected already before birth. Esophageal atresia is suspected when the fetal stomach is invisible on an ultrasound and/or when a dilated upper esophageal pouch is found on an ultrasound. Children with esophageal atresia are commonly born prematurely, and polyhydramnios (an increased amount of amniotic fluid) in the mother is often present. When esophageal atresia is suspected, the mother is referred to our Center for Fetal Medicine (CFM) for a definitive prenatal diagnosis. Parents are offered prenatal counseling by our team, and the delivery is planned. Esophageal atresia is associated with an increased risk of other malformations, predominantly cardiac malformations and chromosomal defects that can be investigated before birth.  

Surgery is given as soon as possible following birth in the daytime and is performed by a specialized team. The procedure includes sewing together the ends of the esophagus. In uncomplicated esophageal atresia, the normal length of stay in the hospital is around two weeks. 
 
Since 2018, treatment and follow-up of children with esophageal atresia have been part of Sweden's national highly specialized pediatric surgery care and surgery is performed only at Karolinska and Skåne University Hospital. The centralization of care results in increased knowledge, quality, and patient safety, and Karolinska is now one of Europe's largest centers for children with esophageal atresia. Children with esophageal atresia are followed up regularly until the age of 15 by a multidisciplinary team consisting of pediatric surgeons, pediatric pulmonologists, dieticians, pediatric gastroenterologists, specialist nurses, and speech therapists when needed.