The Children's Hospital of Philadelphia
www.chop.edu
Unraveling the Mysteries of Hyperinsulinism - Outcomes: Focal Disease

Laura Ann Warner, NP: 50% of the beta cells, the insulin producing cells in the pancreas, are in the head of the pancreas, so what we have found is that half of our focal lesions are in the head of the pancreas.

Lori Prinz Halaby, NP: If the pathologist finds normal pancreatic tissue in the tail and the body it leads the surgeon to believe that the lesion is likely a focal lesion and probably in the head of the pancreas.

Laura Ann Warner, NP: The head of the pancreas is the toughest part to get to because it is stuck to the duodenum. So the head of the pancreas is embedded into the bowel.

N. Scott Adzick, MD: There's all this important traffic that goes through or around the pancreatic head. The portal vein is behind it. The superior mesenteric artery is behind it. The common bile duct passes through it. The arterial blood supply to the duodenum is shared, so it's a very delicate, very fine dissection. And the way things work, all the drainage of the pancreatic secretions and enzymes occurs from the tail toward the head into the duodenum. So if you take a focal lesion of the head out I'd have to take out the pancreatic duct in that portion. Then we've learned that it's important to save the body and the tail on the pancreas, bring up a loop of bowel, the medical term is Roux-en-Y Loop, so that those pancreatic secretions can drain into the intestine where they belong and so that we preserve the normal islet cells and that normal portion of the pancreas.

Lisa Mass, Parent: There were suspect areas that they could see in the tail of Caroline's pancreas, but nothing stood out like a light bulb.

Michael Mass, Parent: You're praying and praying and praying for focal, because if you have a focal issue then hopefully we can go in and find that focal piece and take out the offensive part of the pancreas and leave the rest of the pancreas and, in essence, you're cured.

N. Scott Adzick, MD: If there is not a visible or palpable hint as to where the focal lesion is, with preoperative PET scanning I know where to zero in. Focal lesions can be as small as two or three millimeters in size, so sometimes it can be tricky, even with the preoperative information, to find it. A focal lesion in the tail or the body or the neck of the pancreas is straightforward to excise. It may involve taking off the end of the pancreas along with the focal lesion, or just the focal lesion itself if it's not close to the main pancreatic duct, which is sort of the pipeline through which pancreatic secretions run, and we don't want to cause an injury to that during the operation.

N. Scott Adzick, MD in operating room: Yeah. Great. Thanks. Thanks again. Nope. We are done. Thanks. See you. Bye.

Michael Mass, Parent: We didn't know why we were getting an update mid-surgery, but I wouldn't have thought in my wildest dreams that it was done.

N. Scott Adzick, MD: It's a wonderful feeling to leave the operating room having done something that will have a huge impact on a child's life and on that family.

N. Scott Adzick, MD: Extraordinarily good news.

Lisa Mass, Parent: Okay.

N. Scott Adzick, MD: You ready for some good news?

Lisa Mass, Parent: I am ready for some good news. She had indeed had a focal lesion that was in the tail of her pancreas, which was the most unlikely place for it to be and it's the best place to have a lesion because it's not attached to anything else. I remember him saying that there was every reason to believe that Caroline would be cured.

N. Scott Adzick, MD: All right. Oh, a hug. I get a hug. Wow. That's a bear hug. Okay. All right.