U physician fights to give girl normal esophagus

by Mickie Barg

A University physician is using a procedure he himself developed to save the life of 11-month-old Italian baby Mannela Magaldi.
Born with esophageal atresia — a birth defect where a portion of her esophagus, the tube that connects the mouth and stomach, is missing — Manuela cannot swallow anything, including her own saliva.
She arrived at the University from Naples, Italy, with her parents, Flavia Montefusco and Ercole Magaldi, after a long search for a doctor who could give her a chance to eat like any other child.
Manuela and her parents are staying at the Ronald McDonald House near Stadium Village. A feeding tube sticks out of her stomach and tubing down her throat is attached to a suction machine. The machine sucks out her saliva, preventing her from choking.
A stalwart patient
For the most part, Manuela appears unfazed by all of the trappings associated with her affliction.
“She only cries when they change the suction tube,” Montefusco said. “But as soon as they’re done, she is smiling again.”
Montefusco discovered the birth defect during a routine prenatal ultrasound in Naples.
“The radiologist said, ‘Your child is perfect except for this one problem,'” she said. “He said ‘now you must search on the Internet for the best solution for your daughter.'”
Montefusco and Magaldi purchased a computer, hooked up the Internet and began their search.
In Europe, the name of the birth defect is spelled differently, and Montefusco only found doctors who bridge the gap in the esophagus with artificial tubing or a piece of the patient’s colon, or move the stomach up to the chest cavity.
“They said they would have to wait for her to stand on her own because the substitute won’t work as an esophagus does,” Montefusco said. “There would be no movement, only gravity, to force the food down to the stomach.”
With a change in spelling during one of her Web searches, Montefusco found University surgeon John Foker’s Web page and knew his procedure was the answer.
“I knew this was the solution for her,” she said.
Foker is the only surgeon to use a technique called true primary repair to make the esophagus grow together.
“We are the only place in the world that does this surgery,” Foker said. “That makes us better than world-class.”
Within two days of contacting Foker, Montefusco began another search for the money to pay for the surgery. Because the surgery is not available in Italy, the state health administration agreed to pay 80 percent of the cost.
Each parent then took a leave-of-absence from their jobs, left Manuela’s 14-year-old brother with family members and came to the University.
The family will stay at the Ronald McDonald House for three months while doctors perform each of the surgeries necessary to complete Manuela’s recovery.
During Manuela’s first surgery Oct. 12, Foker placed the esophagus back into its natural position. A previous surgery in Naples moved her esophagus from its original position upward so that her saliva could drain out through an opening in her neck.
“Manuela had a pouch hanging in her neck which wasn’t connected to anything,” Foker said. “Unless they did something, there would be trouble with stuff going into her lungs.”
The procedure
After giving this time to heal, Manuela will be ready for the next surgery scheduled for Nov. 6.
Foker will bring together the two ends of the esophagus through internal traction sutures. If the gap between the two ends is too long, sutures are attached to the ends of the esophagus and extend out through the skin. The sutures are pulled each day to bring the two ends together.
Foker was unsure which type of traction he would need to use with Manuela.
“We have quite a way to go to bring them together,” Foker said. “We will probably have to bring them out of the skin.”
Foker said it isn’t a single operation but no matter how little esophagus there is he will get the two ends together. He has been performing this surgery since 1983 but has only perfected the technique in the last six years.
“The esophagus really grows like mad because there is nothing much in the chest to stop it,” Foker said. “I stimulate the ends to grow.”
In order to keep the traction in place, Manuela will be sedated and kept on a ventilator for as long as 12 days. The final step is suturing the ends together, giving Manuela a normal esophagus.
In approximately 10 percent of children born with esophageal atresia, the condition is similar to Manuela’s. The upper and lower unconnected esophagus end in pouches. The gap between the ends can vary, and the connection of the esophagus to the trachea can vary, making each case extremely different from the other.
“There often is no predicting how the surgery will go ahead of time,” Foker said.
He said that depending on the patient it can be easy or hard. But there is no artificial tubing, transplants that could be rejected, or taking parts from other places in the body, he said.
“It’s all natural,” Foker said.
He has completed more than 90 esophageal atresia surgeries and most patients have been under one year old. The oldest has been one- and-a-half.
The child patient’s overall health affects the treatment plan, such as premature birth, other congenital disabilities, or a type of esophageal atresia that could be difficult to repair.
Luckily, Manuela is in very good health. Through the feeding tube in her stomach, her mother has been giving her a normal Italian diet, heavy in pasta.
“She is in extraordinary health,” Foker said. “I have never seen a baby quite like that — she is always smiling, no matter what.”
Montefusco said she has arranged for a doctor and anesthesiologist from Naples to come to Minnesota and watch Manuela’s surgery in order to learn the technique.
Foker said he would be happy to share his techniques with any doctor.
“This surgery makes an enormous difference to these children,” he said. “People have to start doing it.”
Great lengths
Foker said Manuela’s family is the first he has received from Europe.
“It isn’t a doctor sending the patients here,” he said. “Virtually all of them find me themselves, and they go through great lengths to find me.”
He said a Delaware woman read a syndicated article on his heart ventricle repair that included a few paragraphs on the esophageal atresia repair technique. She immediately contacted her sister in Ohio, whose daughter was born with the defect.
The child had the procedure but her doctor tried to prevent them from coming here for the surgery, Foker said.
As for Manuela, she has no idea of the gravity of her situation and the importance of the surgery. She prefers meeting people over playing with her toys. She constantly smiles, and when she hears her brother’s voice on the phone she blows him kisses and looks at the telephone to see him.
Montefusco said she is nervous about everything that her daughter is going through and not as optimistic as her husband.
“I am the optimist,” Magaldi said. “I am certain everything will turn out good.”
But Montefusco is confident that the surgery will go fine and nothing will go wrong — it is a mother’s feeling, she said.

Mickie Barg covers the Medical School and welcomes comments at [email protected]