His quick smile and bubbly personality tell nothing of Guy Robinson’s rough start in life. The only outward signs of his medical ordeal are the scars visible on his belly when he laughs and raises his arms to play. But even the scars understate the severity of his rare condition and how lucky he is to be alive and thriving.
Guy was born in October 2011, a week past his due date, after a normal pregnancy. His mother, Phylicia, counted 10 fingers and 10 toes and relaxed. But during his first night of life, Guy couldn’t take in any milk without choking and throwing up.
X-rays showed that Guy’s esophagus – the tube that normally carries food from the throat to the stomach – came to a dead end. It wasn’t connected to his stomach. Doctors broke difficult news to the brand new mom: Her baby had esophageal atresia – a serious birth defect – and needed to be transferred to CHKD immediately.
“I was devastated,” Phylicia said, “but I was relieved he wasn’t sent out of state. I knew all of the experts and instruments were at CHKD, and I knew he’d be in the best hands.”
That knowledge made it easier for Phylicia, who had to stay one more night in the hospital, to kiss Guy goodbye as the CHKD transport team whisked him away in CHKD’s mobile ICU.
At just over 7 pounds, Guy was the giant in CHKD’s neonatal intensive care unit (NICU), where many patients barely weigh more than a pound. CHKD’s NICU, the only unit of its kind in the region, is staffed and equipped for every eventuality after birth, offering quick access to physicians and surgeons covering every pediatric subspecialty for tiny preemies as well as for many full-term babies like Guy. In Guy’s case, he needed the attention of CHKD neonatologist Tom Bass and CHKD surgeon Frazier Frantz.
The first order of business was to find a way to feed Guy. Inserting a gastrostomy tube directly into his stomach would allow food to bypass Guy’s defect and allow doctors to get a good look at his digestive tract.
“In most cases of esophageal atresia, the esophagus is split into two parts,” says Dr. Frazier Frantz, who took care of Guy. “The upper part is a dead end and the lower part is either connected from the windpipe to the stomach or is just a short stump attached to the stomach.
“Normally, we would put in the feeding tube,” he continued, “and hope that the lower end of the esophagus would grow enough with time that we could attach it to the upper end.”
However, when the endoscopic camera gave Dr. Frantz a closer look, he realized that Guy didn’t have a lower esophagus at all.
Guy had a very rare form of esophageal atresia. “There is no universal fix when there is no lower esophagus,” says Dr. Frantz. “There are a lot of options. When you go to the operating room, you have to know them all and be prepared to use the best one for the situation.”
Dr. Frantz had to come up with some sort of substitute pathway between Guy’s upper esophagus and stomach so he could eventually eat and have the best chance for a good quality of life.
The plan was to create a gastric tube out of a section of stomach and sew the tube to the existing upper esophagus. However, Guy’s stomach was too small to make a tube.
Instead, Dr. Frantz ended up pulling half of Guy’s stomach up into his chest to bridge the unusually large gap. So half of his stomach sits in his chest, and the other half is in his abdomen. With this arrangement, Guy is able to eat normally. The part of the stomach in the chest functions as an esophagus and the lower half functions as a normal, if small, stomach.
“If you do this once or twice in your career, that’s a lot,” says Dr. Frantz. “That’s how rare it is to see. We repair esophageal atresia here all the time, which for us is probably six to ten cases a year. But this particular variant is really rare.”
“At first, he threw up whenever he changed positions,” says Phylicia. “But he was always so very happy. In the NICU, he would do anything to get a smile.”
Guy remained in the NICU for four months, and the feeding tube in his small intestine remained in as a precaution for a couple of months after he went home. But Phylicia says once he started eating, his appetite just kept growing.
“He eats everything he can,” she says.
Phylicia keeps a close watch to make sure all food is cut in small pieces, and he can’t eat acidic foods. She’s also very careful about crackers and chips with pointy edges. Other than that, he acts as if he was born to eat like any other child.
“The kid’s a miracle, honestly,” says Dr. Frantz. “There’s nothing that works as well as the esophagus does. We try to preserve the esophagus at all costs. But when you don’t have a complete one, you have to do what you can and hope for the best. Guy has exceeded all our expectations.”
Today, Phylicia brings Guy to see Dr. Frantz for follow-up care. “No one knows him better,” she says.
Which is why the family is extra pleased when Dr. Frantz reports on little Guy’s excellent progress. “He’s growing. He looks great. He has a good quality of life. And he has a family that loves him dearly,” says Dr. Frantz. “All signs are good.”
Dr. Frantz practices with CHKD Surgical Group’s Pediatric Surgery. Dr. Bass practices with Children’s Specialty Group, PLLC, at CHKD.
To learn more about CHKD’s surgical services, visit chkd.org/cssg.
This story was featured in the Spring 2013 issue of KidStuff, a publication of Children's Hospital of The King's Daughters.