Riley's Riddle

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Ridgely Ingersoll

What causes on-again, off-again tummy pain?

Riley2When Riley Zell of Virginia Beach started having stomachaches during the summer of 2008, no one was sure what to make of it. “Some mornings, she woke up in severe pain,” says the 6-year-old’s mom, Cheryl. “She was so sick, she’d throw up, but within a few hours, she’d be up and running around.”

The family’s pediatrician, Robert Dowdy, asked Cheryl and Riley’s father, Pete, to record what Riley ate and when she had symptoms to see if they could detect a pattern. Maybe it was acid reflux, stress or something she ate. “We couldn’t pinpoint anything,” Cheryl said. “We eliminated lactose from her diet to see if that helped, then wheat. Nothing made any difference.”

The mystery continued through the fall, and the Zells grew increasingly worried and frustrated. In early 2009, Dr. Dowdy referred Riley to CHKD pediatric gastroenterologist Vita Goei, who immediately began a series of tests to pinpoint the cause of Riley’s pain.

“It was an incredibly difficult time for us,” Pete says. “While we were going through all this with Riley, my wife started to get really sick. She was weak and losing weight. I couldn’t believe what was happening to my family.”

Everything escalated in March, Pete says. “One day, while we were in the emergency room with Cheryl, Riley had a bout of really severe pain at school. I called Cheryl’s parents to come and stay with her while I took Riley to the emergency room at CHKD.”

That trip to CHKD revealed a surprising and important clue to the mystery of Riley’s stomachaches. An ultrasound of her abdominal area indicated that her left kidney was enlarged. Dr. Goei asked CHKD pediatric urologist Jyoti Upadhyay to take a look, and Dr. Upadhyay suspected right away what might be causing Riley’s oddly sporadic symptoms.

“An enlarged kidney plus severe abdominal pains and vomiting that occur intermittently and resolve on their own can indicate a syndrome called Dietl’s crisis,” Dr. Upadhyay explained.

“Normally, urine flows from the kidneys to the bladder through tubes called the ureters. If something blocks that flow, urine backs up in the kidney causing painful distension, nausea and vomiting. Dietl’s crisis is a telltale symptom of a blockage at the ureteral pelvic junction, or UPJ, the point where the kidneys and ureters connect.”

The attacks often occur when the bladder is full or after a child has consumed a lot of liquid. The blockages can also be aggravated by certain body positions and therefore relieved when a child stretches out. The fact that Riley’s attacks often occurred in the morning when her bladder was full and subsided after she vomited and rested in the bed for a bit fit the clinical pattern of a UPJ obstruction.

Most UPJ obstructions develop before a child is born; they’re often detected on prenatal ultrasounds. Others develop when scar tissue from an infection, previous surgery or kidney stones – none of which had ever affected Riley – cause a blockage. Obstructions may be minimal or so severe they require surgery.

Riley3Dr. Upadhyay arranged for Riley to have a nuclear medicine scan. This procedure would confirm whether Riley had an obstruction, and if so, how severe it was. This time, Cheryl was determined to accompany her daughter. “When the staff noticed how sick I was, they brought me a stretcher and insisted that I lie down while I waited for Riley’s test to be finished. I was amazed. They were so kind and so caring … to both of us. When Riley came back from the test, they pulled her bed right up next to mine.”

A day later, the Zells learned Riley did have a UPJ obstruction and that it was so severe her left kidney was barely functioning. She would definitely need surgery. “The surgery to correct a UPJ obstruction is called a pyeloplasty,” Dr. Upadhyay says. “We remove the obstruction and make a new connection between the kidney and ureter so the urine can reach the bladder.”

Dr. Upadhyay has performed more than 150 pyeloplasties and has done studies and published outcomes data on the effectiveness of various pyeloplasty techniques, all of which she uses herself.

“The traditional method of doing a pyeloplasty is through an open incision in the flank area,” she says. “In younger children, I prefer to do the procedure through a small incision in the lumbar area of the back, because it makes recuperation easier for them.”

Dr. Upadhyay also does minimally-invasive laparoscopic pyeloplasties, with and without the aid of a DaVinci robot. “The advantage to knowing many different ways to do a procedure is that you can choose the very best option for the patient,” she continues. “And that isn’t necessarily the latest development or trend.”

For instance, Dr. Upadhyay has done several pyeloplasty surgeries on CHKD patients using the DaVinci robot at Sentara Norfolk General, but she says she likes that option only for children who weigh more than 60 pounds. “The DaVinci gives us incredible magnification and precision, but it has three robotic arms and a scope, so smaller children’s torsos just can’t accommodate the larger-sized equipment currently available at Sentara.”

“Choosing between a laparoscopic or open procedure and a flank or lumbar incision depends on the nature and location of the obstruction and the position of the kidneys and ureters,” Dr. Upadhyay explains. “Riley had a large obstruction and her kidney was abnormally rotated. The best choice for her was a classic open pyeloplasty with a flank incision.”

There was just one problem. The procedure would require some recuperation time at home, and by this time, Cheryl had been hospitalized with a diagnosis of ulcerative colitis. To buy the family some time, Dr. Upadhyay did an interim procedure, installing a stent that did the work of Riley’s ureters. “From that moment forward, Riley never had another bout of pain,” says Cheryl.

By the end of April, Cheryl was feeling strong enough to schedule Riley’s surgery. “They were so wonderful to her,” Cheryl said of their surgical experience. “Dr. Upadhyay came to see her beforehand in the pre-surgery area and told her exactly what to expect. She made a little mark on Riley’s side where she was going to make an incision and then signed her name on Riley’s tummy. Riley loves Dr. Upadhyay.”

The surgery was a text-book success. After one night in the hospital, Riley went home.

“She bounced right back. After a few days, we had to hold her back,” Cheryl said.

Today, Riley is healthy, active and symptom free. Cheryl and Pete are extremely grateful for that and to the compassionate caregivers at CHKD who took such good care of them during an extremely stressful time.

“It was like a perfect storm of illness hit our family,” Pete remembers, “and CHKD was wonderful to us. Everyone went out of their way to help us again and again.”

“It takes very special people to work with children,” Cheryl adds. “Dr. Upadhyay treated Riley as if she were her own child. We never had any experience with CHKD before this, and the level of care was so different from your typical hospital. We were just amazed.”

Dr. Jyoti Upadhyay practices with CHKD’s urology surgical practice.

This story was featured in the first quarter 2010 issue of KidStuff, a publication of Children's Hospital of The King's Daughters.

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