Rare cyst requires delicate surgery
Tummy aches usually come and go, but 7-year-old Ariana Haigler’s didn’t go away.
Ariana’s parents, Julie and David Haigler of Newport News, agreed with the doctor at an urgent care center near their home that Ariana probably just had a bug. But her symptoms – including vomiting and constipation – got worse.
Then, a week later, the Haiglers noticed a new, more worrisome symptom: jaundice.
“The whites of her eyes and her skin were turning yellow,” Julie said. “That’s when I knew this wasn’t an ordinary stomach bug. I called and made an appointment with her regular pediatrician.”
When Dr. Lewis Curd of Newport News Pediatrics examined Ariana, he realized she was tender and swollen on the upper right side of her torso. “Pain in that area accompanied by jaundice can indicate problems with the liver or gallbladder,” he said. He sent her for an ultrasound and referred her to pediatric gastroenterologist Vita Goei at the CHKD Health Center at Oyster Point.
Ariana’s grandmother, Patricia Haigler, took her for the appointments because Julie had to work. But after diagnostic tests and the appointment with the GI specialist, it was Julie who got the call that night. “The doctor told me she had a cyst in her bile duct that needed to be corrected as soon as possible,” Julie said. “She told me to take Ariana to CHKD in Norfolk right away.”
Julie remembers how distraught she was during that trip. “I didn’t know anything about bile ducts, but I knew how sick Ariana was, and it scared me to death,” Julie said. “She couldn’t eat. She was yellow. She just wanted to lie down and sleep. I really thought I was going to lose my child.”
The medical team waiting to care for Ariana at CHKD included Dr. Goei and pediatric surgeon Frazier Frantz.
“Ariana was quite ill when she got here,” Dr. Frantz said. “Bile wasn’t draining properly from the liver to the small intestine because a cyst was blocking the duct. That made the bile back up into the liver. The top of the duct was swollen to about 10 times its normal size.
“Normally, bile flows like a river, but hers was just sitting there like a lake, causing infection and irritation,” he continued. “It was also irritating the tube that empties secretions from the pancreas, which caused pancreatitis, a very painful condition. Excess bile was being absorbed into her bloodstream causing jaundice.”
Ariana was so sick from vomiting and dehydration that her surgery was temporarily postponed to allow her to get stronger.
“We knew we had to operate,” Dr. Frantz said. “The question was when. It’s a very big operation. Doing it while tissues are inflamed and infected would be quite treacherous.”
In order for her body to produce less bile and pancreatic juice, Ariana’s doctors stopped feeding her regular food and began giving nutrition intravenously. “We gave her total parenteral nutrition, or TPN, so that she would get the nourishment she needed while her digestive system could calm down,” Dr. Goei said. TPN is a special mixture of glucose, protein, fat, vitamins and minerals that is given directly into the veins.
For two weeks, Ariana continued a regimen of antibiotics, TPN and IV fluids under the watchful eyes of Drs. Goei and Frantz. Julie stayed at her daughter’s bedside the entire time. “My whole life turned upside down,” said Julie, who has two other children. “For people who have never been through a serious illness with a child, I can tell you it is very, very stressful for a family, including the other children. It was tough.”
Julie often called her father for support. “He reminded me that 30 years ago, CHKD saved my life,” she said. “I was premature and spent two months in the NICU. He told me, ‘Ariana is in the best hands she can possibly be in. You are living proof that these people know what they’re doing.’ ”
Ariana’s condition – called a choledochal cyst – is rare enough that only a busy children’s hospital with experienced pediatric gastroenterologists and surgeons could have handled it. “It occurs in about one in every 13- to 15 thousand people,” Dr. Frantz said.
“Children like Ariana who are born with the condition usually show symptoms by six months of age,” he continued. “In those cases, the diagnosis is typically very clear cut. But cases like Ariana’s, when symptoms develop later in childhood, are more difficult to diagnose and treat. Ariana was lucky that Dr. Curd and Dr. Goei diagnosed it accurately as soon as they saw her and began treatment right away.”
Clearing the infection and inflammation was only the first hurdle. As soon as Ariana started feeling better, it was time for surgery.
“The first thing we do in the surgery is inject dye into the gallbladder to allow us to see exactly where the cyst starts near the liver and where it stops near the pancreas. It’s crucial to remove the whole cyst or the remaining tissue may lead to recurrence or become cancerous in the future,” Dr. Frantz said.
Even with all the advanced imaging tools available today, surgeons can never be 100 percent sure of what they’ll see with their own eyes in the operating room. That’s one reason why it is so important to have experienced pediatric surgeons operating on your child.
The dye showed that Ariana’s cyst, which was about the size of an orange, was in front of and adhering tightly to the blood vessels that supply the liver. “Cutting it out would risk interrupting the blood flow to the liver and damaging it,” Dr. Frantz explained. “So we had to peel most of it off in layers like an onion. It was painstaking. But after we did that, we could clearly see the rest of the tissue we needed to remove, which included the gallbladder and the rest of the bile duct.”
Bile is necessary to digest food, so Dr. Frantz then had to create a way for it to drain from the liver into the rest of the digestive system. To do that, he isolated a loop of small intestine – so food would no longer travel through it – and then sewed it to the remaining bile ducts adjacent to the liver. Now bile could drain from the liver directly into the small intestine.
Ariana spent about a week at the hospital recuperating from the surgery. She gradually transitioned to a regular diet. Just days after she got home, though, she started vomiting again. She returned to CHKD, where Dr. Frantz discovered that a piece of scar tissue already forming from the first surgery was obstructing her bowel. That meant a trip back to the OR – a much quicker one – and two more days in the hospital.
“After that second surgery, she got better really fast,” Julie says. “She was quiet for a day or two, but then she was right back playing with her friends.”
Dr. Frantz will continue to see Ariana for the next three or four years. At her most recent visit, the news was good. “She’s doing very well. The jaundice is gone and she has put on weight,” Dr. Frantz reported.
Today, the Haiglers are doubly grateful to CHKD, where two generations found the care they needed.
My mom always told me that CHKD had the best pediatricians,” Julie said. “I was really happy when CHKD opened the Health Center at Oyster Point.
“There is nothing like CHKD,” she added.
Dr. Frantz is with CHKD Surgical Group’s Pediatric Surgery practice. Dr. Curd is with CHKD Medical Group’s Newport News Pediatrics. Dr. Goei practices with Children’s Specialty Group PLLC at CHKD.
This story was featured in the third quarter 2010 issue of KidStuff, a publication of Children's Hospital of The King's Daughters. Click to read more patients' stories.