Browse THP Patient Cases

Five-Day-Old Female Patient Referred with Esophageal Atresia (Hendren Patient Case)

  • 5-d-old baby girl referred July 1963 with esophageal atresia.
  • X-ray study had shown a blindly ending upper esophagus; abdominal film showed no gas in the abdomen.
  • Stamm gastrostomy using a mushroom catheter was performed, bringing the tube out through a small stab wound.
  • Right chest was opened to confirm for certain that the distal esophagus was rudimentary (having seen a prior case where a small fistula had been present, with a good lower esophagus but without abdominal gas). (Modern imaging would obviate that need.)
  • Upper pouch was mobilized, during which a fistula was seen from the back of the upper pouch to the trachea, not seen on pre-op study.
  • Fistula was divided and closed.
  • After chest closure, infant was turned and a transverse incision in the lower neck just above the clavicle anterior to the sternocleidomastoid muscle and the jugular vein and carotid artery.
  • Previously freed upper esophagus was quickly opened and sewn to the wound to provide free drainage of saliva.
  • Baby thrived.

Fig. 1. Patient at Age 10 Mo Before Colon Esophagus Operation. Note excellent nutritional status. Upper esophagus stoma to drain saliva above left clavicle. Gastrostomy tube covered with ample tape to avoid its being pulled out.

  • At 10 mo, through a left 7th interspace thoracoabdominal incision, a colon esophagus was made using transverse colon on a left colic arterial pedicle (see video of operation).
  • Lower end of colon esophagus did not lie comfortably for anastomosis to the distal esophageal remnant and so it was anastomosed to the anterior wall of the stomach.
  • Postoperatively, when gastrostomy feedings were begun barium study showed free reflux up the colon conduit and less flow through the pylorus, although both vagi were spared intraoperatively.
  • At 2 weeks postop, laparotomy was performed.
  • Gastrostomy tube site was moved farther proximally in the upper stomach.
  • Generous Heineke-Mikulicz pyloroplasty was performed, incising 1 inch (2.54 cm) into the duodenum and 1.5 inches (3.81 cm) into the stomach, closing transversely. Pyloric tissues were indeed thicker than expected.
  • Colon esophagus remained somewhat dilated as if there were functional partial obstruction at its lower end.
  • Some gastrointestinal blood was observed on multiple occasions, never great in amount.
  • At 10 wk, reoperation was performed to accomplish three tasks:
    • (1) shorten the lower end of the colon esophagus;
    • (2) anastomose it to the original rudimentary lower esophagus, as once considered; and
    • (3) divide both vagus nerves to reduce gastric acidity.  

Fig. 2. Barium Swallow in 1968. Although caliber of colon was still somewhat dilated it emptied well, there was no aspiration, and patient was well nourished. 

  • Patient thrived; family moved to Oklahoma.
  • At age 3 yr, she was seen. She and her barium study were both fine.
  • At age 22 yr, patient was and healthy.
  • Unable to get pregnant, she underwent laparoscopy elsewhere; after lysis of fallopian tube adhesions she had two healthy sons.

Takeaways

Early Esophageal Atresia Managed by Closure of the Tracheoesophageal Fistula (Hendren Patient Case)

One of the early survivors among 12 babies with esophageal atresia was managed by closure of the tracheoesophageal fistula (TEF), gastrostomy, and exteriorization of the cervical esophagus to the left neck as a neonate. Then a subcutaneous presternal “esophagus” was made in stages.

n

  • 11-yr-old female patient born in 1944. Her first surgery was done by William Ladd, M.D., and Orvar Swenson, M.D. It included the above as an eonate plus repair of duodenal atresia. Upper presternal tube is a (skin-graft-lined) skin flap tube connected to the cervical esophagus. Lower half of the “esophagus” was a roux-en-y jejunal tube. Her swallowed food was manually massaged downward.
  • In these early cases, the stomach was bypassed. They grew poorly until the stomach was functionalized.
  • This child had 30 prior operations in her lifetime. I met her at age 16. A substernal colonic esophagus was constructed using left colon, antiperistaltically. The original subcutaneous tube was moved later.
  • At age 19 years, large secundum atrial septal defect was closed using a dacron patch during cardiopulmonary bypass. Substernal colon was carefully removed aside to access the heart.