Complex procedure offers relief for children with chronic pancreatitis

Acute recurrent pancreatitis or chronic pancreatitis can take an enormous toll on children, causing severe pain and repeated hospitalizations.

For some children, total pancreatectomy with islet autotransplantation (TPIAT) can offer a way out of this debilitating cycle. The highly complex procedure involves removing the diseased pancreas and transplanting the patient's own islet cells to help preserve endocrine function.

In 2024, Children's Hospital Los Angeles became the first center in Southern California-and one of just a few in the country-to perform TPIAT in a pediatric patient. The team has since provided the surgery for four patients.

TPIAT requires complex postoperative care from a multidisciplinary team spanning critical care, pain management, gastroenterology, endocrinology, hematology, and infectious diseases.

Priority one is to alleviate the pain. Priority two is to preserve as many pancreatic islets as possible."

Kambiz Etesami, MD, FACS, Chief of Abdominal Organ Transplantation at CHLA

Don't wait for pancreatitis to become advanced

At CHLA, TPIAT is just one part of a Pancreas Program that provides comprehensive care for children and adolescents with acute recurrent pancreatitis or chronic pancreatitis. That specialized care starts well before a child needs surgery.

"Children should be referred once they have had two episodes of pancreatitis or a first episode of pancreatitis where they already are showing chronic change," says Yuhua Zheng, MD, MS, Medical Director of the Pancreas Program at CHLA. "Every pancreatitis attack can cause more damage, and we want to evaluate children before too much tissue and function is lost."

At CHLA, that evaluation can include advanced pancreatic endoscopy-including endoscopic retrograde cholangiopancreatography (ERCP), endoscopic ultrasound, and endoscopic pancreatic function testing-as well as comprehensive genetic testing.

"Genetic mutations are a common cause of acute recurrent pancreatitis or chronic pancreatitis in children, but standard panels can be too limited," Dr. Zheng explains. "At CHLA we look at a much broader range of genes."

Pain severity doesn't always track with lipase

One common misconception about pancreatitis is that if pain is severe, lipase levels should be markedly elevated. But that is not always the case.

While lipase reflects inflammation, inflammation is only one source of pancreatic pain, Dr. Zheng notes. Ischemia, hypersensitivity, and micro-obstruction can also cause significant pain.

"When lipase levels come back lower, some physicians question whether the child is truly in that much pain," she says. "But in pancreatitis, the lipase doesn't always match the pain."

Earlier evaluation may help preserve islet function

For children who ultimately need TPIAT, timing matters. A pancreas that is already severely scarred may yield fewer viable islets for autotransplantation.

For example, a recent CHLA patient was experiencing daily abdominal pain but still had largely intact islet cells at the time of TPIAT. The team was able to isolate and transplant a large number of islets, and the patient was off insulin two months after surgery.

"That's the best outcome," Dr. Zheng says.

Even when TPIAT does not eliminate the need for insulin, preserving islet function can still reduce insulin requirements. But for children with debilitating pancreatitis, the primary goal of TPIAT is giving them relief from the pain.

"You can take a child who is essentially homebound in chronic pain and give them their life back," Dr. Etesami says. "That is something that very few interventions can do."