Children’s Hospital of Philadelphia’s Congenital Hyperinsulinism Center Celebrates 600 Surgeries

The Congenital Hyperinsulinism (HI) Center at Children’s Hospital of Philadelphia (CHOP), the nation’s largest and most active center of its kind, is celebrating the completion of 600 pancreatectomies, a surgical procedure that involves removing all or part of the pancreas. The procedure is used to treat some forms of congenital HI, a life-threatening genetic disorder in which the insulin cells of the pancreas, called beta cells, secrete too much insulin, leading to low blood sugar (hypoglycemia).

Established in 1998, CHOP’s HI Center treats children with congenital HI from all over the world. Some forms of the condition can be managed with medication, other forms require a near-total pancreatectomy to prevent low blood sugar levels, but children with a focal form of the disease can be cured after surgical removal of the lesion. The cure rate for focal hyperinsulinism at CHOP is 97%. The multidisciplinary team in CHOP’s HI Center works together to provide specialized, patient-centered care for children with congenital HI and their families.

“I am so proud of the entire team for this achievement of completing 600 surgeries,” said N. Scott Adzick, MD, Surgeon-in-Chief of CHOP. “From the inception of our HI Center, we’ve made meaningful strides in advancing care for children living with this rare disease. Looking ahead to the future, we will continue to be relentless in the pursuit of more answers and even better outcomes for all.”

Our Congenital HI Center is a very important part of the comprehensive care offered at CHOP. This accomplishment would not be possible without our vast team of experts, ranging from pediatric endocrinologists and surgeons to nurses, social workers, and dieticians, who all work to improve the care of children living with this rare disease.”

Congenital HI affects approximately 1 in 25,000 to 50,000 babies. The rarity of the condition means most children’s hospitals encounter only one or two cases a year. Given the severe complications that can occur when the brain does not get the sugar it needs, including seizures, brain damage, and even death, it is important that children with the condition receive medical care from an experienced treatment center. In its more than 20-year history, the Congenital HI Center at CHOP has evaluated and treated more than 1,600 patients, making it the largest program of its kind in the world.

“It is hard to imagine what the care of children with hyperinsulinism would be today without the contributions of our Center,” said Diva De León-Crutchlow, MD, MSCE, director of CHOP’s HI Center and Chief of the Division of Endocrinology and Diabetes. “I’m proud of what we’ve accomplished to get to this point and excited about what we hope to accomplish next.”

The 600th Surgery

When Eliza Hatfield was born on August 18, 2022 at her local hospital in Northern Virginia, doctors tested her blood sugar and discovered it was low. Eliza went to the Newborn Intensive Care Unit, where she spent six days before going home. Nine days later, Eliza’s pediatrician checked her blood sugar again, and although Eliza was exhibiting no symptoms at the time, her blood sugar was still very low. The family headed to the emergency room in Fairfax, VA, which officially began the Hatfields’ journey to their ultimate destination: CHOP.

Upon arrival in Philadelphia, the CHOP team began genetic testing. There are two main types of HI, both of which are genetically inherited: focal, where the overactive beta cells are clustered in one area, and diffuse, where the beta cells are distributed throughout the pancreas. The genetic tests revealed something astonishing: Eliza had a paternally-inherited mutation that resulted in focal HI, as well as maternally-inherited mutations that supported both a focal and diffuse diagnosis, making this the first incidence of such genetics recorded in history.

On September 30, Eliza had 25% of her pancreas removed and became CHOP’s 600th pancreatectomy. Following her surgery, Eliza’s glucose values stabilized, and after more than a month in CHOP’s Newborn/Infant Intensive Care Unit, she returned home to Virginia, where she is continuing to thrive. Read more about Eliza’s story here.