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Our techniques may sound complicated, but our work here is simple: use the most effective surgical approach available to help children born with an incomplete or blocked esophagus to eventually be able to eat solid foods.
Specialists within the EAT Program also care for children with:
VACTERL association (also called VATER syndrome) is a group of associated birth defects. These defects aren't always related, but they do tend to occur together. The name is an acronym, standing for:
V - Vertebral anomalies
A - Anorectal anomalies
C - Cardiovascular anomalies
TE - Tracheoesophageal fistula
R - Renal (Kidney) and/or radial anomalies
L - Limb defects
Because TE is one of the associated conditions (it's present in about 70 percent of VACTERL patients) many children with EA/TEF may also have some of the other conditions found in VACTERL patients.
CHARGE syndrome is a genetic condition, caused by a change (mutation) in a single gene. Originally the term CHARGE was an acronym, representing symptoms often associated with the condition: coloboma (defect) of the eye, heart defect, atresia of the choanae, retardation of growth and/or development, genital and/or urinary abnormalities and ear abnormalities and deafness. These features are no longer used in making a diagnosis of CHARGE syndrome, but the name has remained.
CHARGE is a complex condition that involves extensive medical and physical difficulties that can be different for each patient. Although EA/TEF is not a major criterion for diagnosis of CHARGE, about 10 percent of children with CHARGE also have EA/TEF.
Polyhydramnios is an abnormally large buildup of the fluid that surrounds the baby in the mother's uterus during pregnancy. Polyhydramnios doesn't cause EA/TEF, but a diagnosis of polyhydramnios in the mother may be the first sign of EA in a fetus. This is because a fetus with EA is unable to swallow amniotic fluid normally and therefore the fluid volume accumulates. This diagnosis is further supported if a “stomach bubble” is not seen in an ultrasound.
There are multiple surgical treatments available to treat cases of EA/TEF and long-gap EA. Boston Children's Hospital’s EAT Program uses the Foker process as the primary strategy for infants with long-gap EA who have not previously had surgery. We recommend the Foker process because, if successful, patients are more likely to be able to eat and swallow normally compared with patients who have received other procedures to correct long-gap EA, such as gastric pull-up, jejunal or colonic interposition. For complex cases having had prior attempts at repair, other alternatives to the Foker process may be appropriate. We recommend alternatives if we predict that the patient will require very extensive surgery and multiple procedures with many weeks or months in the intensive care unit, and a low chance of achieving continuity and being able to eat normally.
To perform a colonic interposition, surgeons move a section of colon from its normal location in the abdomen into the chest, and insert this section to bridge the esophageal gap, creating a replacement conduit. Fortunately, there is plenty of colon available in the body to use in this surgery, and colonic interposition is a standard but substantial procedure.
Food and fluids in a “colon esophagus” move by gravity to the stomach. Contractions of the muscular wall (a process called peristalsis) do not push food along as with a normal esophagus. The lower portion of an esophagus with esophageal atresia also does not move fluid and food along in a normal fashion. One problem that can occur in a colon conduit is progressive dilatation and elongation of the colon, which may lead to redundancy in the chest cavity and impaired transit of food to the stomach. This can result in aspiration (passage of food or liquids into the lungs). As the conduit becomes more distended, the chest cavity is filled with colon decreasing the volume available for the lungs.
Revision of the colon conduit may be required to straighten its course and remove the redundancy after a colonic interposition.
In jejunal interposition, surgeons use a section of the jejunum (the middle part of the small intestine) as a replacement for the missing section of esophagus. This reconstruction can be performed either by rotating the bowel on a segment of its blood supply, or as a “free graft,” where its blood vessels are divided and then joined to an artery and vein in close proximity to where the segment is needed in the chest. The jejunum is thin enough to be placed behind the trachea, mimicking the normal position of the esophagus. These replacements generally do not dilate or "kink" but are dependent upon the adequacy of the blood supply for success.
In a gastric pull-up procedure, the stomach is repositioned into the thoracic cavity and attached directly to the upper end of the esophagus. The stomach can be mobilized into the chest cavity because it has a very good blood supply, which can be brought with it into the chest. The stomach continues its normal function.
When the stomach is closer to the mouth, it increases the risk of acid reflux into the mouth and potentially the lungs, which can result in pneumonia. This reflux also can irritate the lining of the upper esophagus, which can lead to mild discomfort (esophagitis). Chronic injury to the esophageal lining can result in a pre-cancerous condition (Barrett's esophagus).
Placing the stomach higher in the chest can also cause crowding, getting in the way of the lungs and decreasing the amount of air for breathing. Gastric pull-ups also increase risk of chronic aspiration and recurrent pneumonia, which can injure the lungs. Gastric pull-ups have been linked to chronic anemia, chronic malnutrition and dumping syndrome (in which the patient experiences diarrhea and low blood sugar if food is eaten too fast and is rapidly transferred into the small bowel). If the Foker process is successful, these side effects are avoided because it does not require that the stomach be moved.
A gastric “tube” also has been used in the past for replacement of the esophagus. For this process, a segment of the stomach is used to create a tube that can be rotated into the chest. The problem with this approach is that children with EA often have small stomachs, so use of this method may decrease the size of the remaining stomach and therefore the amount of food they can eat.
(cervical esophagostomy). Creation of a “spit fistula” is a surgery sometimes performed before a gastric pull-up or interposition. This procedure entails the surgeon connecting the upper esophagus to the skin of the neck, allowing saliva to drain from the upper esophageal pouch through a small hole, called a stoma. This is typically a temporary intervention until the patient is old enough for an interposition or gastric pull-up.
A spit fistula presents a minimal risk of aspiration and allows the patient to chew (and taste) some solid food. It also allows infants to learn how to suck and swallow in early infancy before their esophagus is repaired or placed in continuity with a conduit of jejunum, colon or stomach. However, the ability to eat is severely limited and the drainage of saliva and chewed foods on to the neck can be messy and difficult to manage. In addition, when young patients receive spit fistulas their stomas may need to be dilated over the years as they grow.
Patients who receive the Foker process do not need a spit fistula as the repair is done soon after birth.
Frequently, patients who have had their EA/TEF treated at another facility will come to Boston Children's Hospital for follow-up care. The EAT Program has a state-of-the-art outpatient clinic that specializes in caring for patients experiencing complications with earlier EA/TEF repair.
Services include access to:
The EAT Program's outpatient clinic is fully equipped to treat patients with complications from common EA treatments, and it offers follow-up service designed for life-long evaluation of patients with any/all esophageal problems.
Bridging the Gap of EA/TEF: A family to family support and resource network
A Family-to-Family Support and Resource Network devoted to fostering an ongoing relationship between family members and medical professionals.
EA/TEF Family Support Connection
The support group was formed by and for families of children born with EA/TEF (esophageal atresia and tracheoesophageal fistula) and the medical professionals who treat them.
The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”