The Children's Hospital of Philadelphia
www.chop.edu
Unraveling the Mysteries of Hyperinsulinism - Treatment: Surgery

Michael Mass, Parent: The thing I remember most about the day of surgery I think it's the first time we ever moved Caroline where she was awake during the move.

Lisa Mass, Parent: Well, we could see on the monitor that her heart rate was very high and that she was frightened. That was upsetting.

Michael Mass, Parent: We had been very well prepared and well briefed. Dr. Adzick had talked to us the day before. We felt like we understood what was going to be happening the day of the surgery.

N. Scott Adzick, MD: The surgical approach at the beginning is the same. A laparotomy is performed on a baby who is under general anesthesia by one of our expert pediatric anesthesiologists. A sideways wound is made above the level of the belly button and then the pancreas is exposed. The pancreas has a head, a neck, a body and a tail. I examine the pancreas using magnifying glasses that magnify things by a factor of four. And sometimes there are little visual hints as to where a focal lesion might be. I also palpate the pancreas because focal lesions tend to be a bit firmer than surrounding normal pancreatic tissue. If there are no abnormal findings on inspection or palpation I do three biopsies, one from the head, one from the body and one from the tail of the pancreas.

Charles A. Stanley, MD: It's important that the surgeon take biopsies to try to distinguish between whether it's diffuse disease or focal disease.

Eduardo Ruchelli, MD: These certainly can be, to some extent, delineated by PET scan, but it's only by the pathologic examination that we can recognize what portion of the pancreas is exactly abnormal that the surgeon will in turn be able to remove the abnormality.

Pierre Russo, MD: Where the arrow is, these are the islets. Okay, so this is normal and this is abnormal and this abnormal area, this child was hyperinsulinemic because he had these huge, abnormal islets.

Eduardo Ruchelli, MD: Because these cells are engaged in a continuous production of insulin they become larger. They are cells that are continuously working very hard, they never rest and as a result of that they tend to become larger.

N. Scott Adzick, MD: The interaction between the surgeon and the pathologist in the operating room is crucial, because one wants accurate information given to the pathologist. One wants the frozen section diagnosis to be prompt because the baby is anesthetized. And actually we have things arranged in the operating room such that there's a large screen onto which the pathologic sections can be projected from pathology an entire floor away, so that we can in real time discuss what the findings are.

Eduardo Ruchelli, MD: It's an instantaneous feedback that the surgeon receives from us.