Surgery for Hyperinsulinism (4 of 7)

By: The Children's Hospital of Philadelphia

Our daughter caroline was born on june 13th, at central baptist hospital in lexington, kentucky. She was pronounced healthy and we were discharged from the hospital and getting ready to go home when a very astute nurse noticed and thought that caroline seemed cold and a little bit lethargic. we were worried about the usual things that you worry about, you know, you want them to be healthy and you want them to have 10 fingers and 10 toes, but we had never heard about hyperinsulinism. We had been very well prepared and well briefed the day of the surgery. Dr.

Adzick had talked with us the day before. We felt like we understood what was going to be happening. a surgical approach at the beginning is the same with either focal disease or diffuse disease.

A laparotomy is performed in a baby who's under general anesthesia by one of our expert pediatric anesthesiologists. A sideways wound is made above the level of the belly button and 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 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. when you have a focal lesion, the rest of your pancreas is normal. When there's diffuse disease, all the cells, all the beta cells in the pancreas, are abnormal.

Surgery for Hyperinsulinism (4 of 7)

So there will be multiple biopsies taken just to be sure. it's only by pathological examination we can recognize what portion of the pancreas is exactly abnormal. And the surgeon will, in turn, be able to remove the abnormality. where the arrow is, these are the islets, ok.

So this is normal. And this is abnormal. 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 diagnoses 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. for babies with focal disease if there is not a visible or palpable hint as to where the focal lesion is, now 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 straight forward to excise and 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. Focal lesions in the head of the pancreas are more complex because 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 secretion and enzymes occurs from the tail toward the head and to the duodenum. So if you take a focal lesion of the head out and 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 of 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 we preserve the normal islet cells and that normal portion of the pancreas. our surgeon has done over 400 pancreatectomies so he has the most experience in the world. 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. You ready for some good news? i'm 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. approximately half of the children that require surgery have this focal form of hyperinsulinism. The other half have diffuse hyperinsulinism. those that have diffuse disease, we can reduce the amount of pancreas by surgery, but we can't cure it completely. if there are abnormal findings consistent with diffuse disease on each of the three biopsies, then a near total pancreatectomy is done, saving only a tiny nub in the pancreas between the duodenum and the common bile duct.

This hopefully will help in the management of the disease in terms of prevention of brain threatening hypoglycemia, but it's really not a cure. Some babies do quite well and don't need much in the way of additional medical therapy. Others can have recurrent hypoglycemia and need additional medical therapy and there's a third group that develops insulin- dependent diabetes.

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