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Gallstones in Children

Most of the time in children we don’t know why they get stones in their gallbladder. Gallstones in children are most commonly caused by a hemolytic problem. Cystic fibrosis is another classic association, and then there is children that already have liver disease. They may get stones. Children who have had a large portion of their small bowel resected, obesity, a family history and also parenteral nutrition is associated with the formation of gallstones. But it’s not very often that these stones in the gallbladder occlude the cystic common bile duct.

Generally speaking, the presentation can be just with occasional abdominal pain. They don’t have to have jaundice. It’s only if the stones migrate into the common bile duct that you get the full-blown picture of obstruction, cholangitis, fever, you may have abscesses in the liver and you may develop a secondary pancreatitis. Not very common in children. The tip-off on the lab tests, again, look for that alkaline phosphatase being very high. Remember, alkaline phosphatase comes from the bile canaliculi. If you’ve got an obstructive liver disease.

Treatment; you obviously want to exclude any of the contributing factors, and again we try the synthetic bile acids. But many of these children are going to need to have their gallbladder out on an elective basis. Well, what about cholecystitis? Now this of course is acute or chronic inflammation of the gallbladder. You don’t have to have stones. It’s just an acute or chronic inflammation of the gallbladder. Now when you do have stones in association with cholecystitis it means that the stone has got into the cystic duct and impacted in there and caused an infection upstream, if you like. Very often there is a super-infection with other bugs such as E. coli or enterococcus. Just as in adults, it tends to be more common in girls.

The clinical presentation of cholecystitis; usually these children look sick. They’ve got right upper quadrant pain, it’s a colicky pain. It might be referred to the scapula. They’ve got fever and they are often jaundiced. Again, look at that alkaline phosphatase and the bilirubin.

How about inflammation of the gallbladder without stones? This is actually a diagnosis that is often hard to remember to think about. Where you most often see it is in the context of another systemic infection, particularly enteric infections like salmonella, and shigella.

Cystic fibrosis. You normally think about this as being a disease involving the lungs. Interestingly, cirrhosis with portal hypertension can occur in cystic fibrosis, generally presenting in mid or older age children. Sometimes the liver disease is much more important than the lung disease in these children, and they may also present with portal hypertension and already developed cirrhosis.

Wilson’s disease. You might only see one in your life but you sure don’t want to miss it. This is a disease which is marked by defective copper metabolism. You wind up with far too much copper in your liver, your central nervous system, and also in the kidney. The liver and the central nervous system are the two that really present to you most often, in terms of clinical symptoms. Now on the lab tests, what are you going to see? The serum copper is low and the serum ceruloplasmin, which is the carrier protein, is low.

The presentation can be all over the map. It can present as acute fulminant hepatitis. If it does, the tip-off is that these children have acute hemolytic anemia in association with their fulminant liver failure. If you see that combination, think Wilson’s disease until proven otherwise. They may present as a chronic active hepatitis, cirrhosis, portal hypertension, variceal bleeding. They may be totally asymptomatic with just a moderately elevated transaminitis. Some of these children do present with the neurologic symptoms more than the liver symptoms and they can be very subtle. Changes in personality, school performance, behavior characteristics that the parents come in and say, "This just isn’t anything like the child I used to know." Be very aware of that. The Kayser-Fleischer rings, the brown pigment around the iris, is always talked about in the books. Very difficult to see and you usually are not going to see it until the mid-teenage years.

The treatment for Wilson’s disease depends on when your diagnose is, which is why early diagnosis is essential. If there is no cirrhosis you are going to treat these patients with chelating agents, like penicillamine. However, if fully developed cirrhosis has already occurred.