• Hemolytic disease (17%–29% of children with sickle cell disease)
• Total parenteral nutrition
• Prematurity
• Necrotizing enterocolitis
• Cystic fibrosis
• Obesity
• Pregnancy
• Oral contraceptives
• Down syndrome

• Bile is composed of five major components: water, bilirubin, cholesterol, pigments, and phospholipids and calcium salts. Stones are of two types: pigment and cholesterol stones. The formation of cholesterol stones is associated with sludge and cholesterol supersaturation. Other important factors include:
  • Gallbladder stasis
  • Excess lecithin
  • Increased biliary mucus secretion
  • Rapidity of nucleation time
• Total parenteral alimentation is associated with decrease in bile flow, stasis, sludge, and stone formation.
• The formation of gallstones secondary to ileal disease and resection has been reported in children and adults because of a decreased bile acid pool and cholesterol supersaturation of the bile.

• Cholelithiasis is relatively uncommon in childhood; however, gallstones have even been detected in utero.
• Pigment stones are more prevalent in prepubertal children, whereas cholesterol stones are predominant in adolescence and adulthood.
• The incidence of gallstones remains negligible in males throughout childhood and adolescence to adulthood.
• In females, the incidence is 0.27% during ages 6 to 19 years and increases to 2.7% between the ages of 18 and 29; gallstones predominate in females.
• Canadian Eskimos and native Africans have the lowest risk of cholelithiasis.
• Native Americans, Swedes, and Czechs have the highest risk.

• Silent gallstones present in infancy and pre-school-age children.
• The classic symptoms of right-upper-quadrant pain and vomiting exist in older children and adolescents.
• Younger children present with nonspecific symptoms, including obstructive jaundice.
• Fever is unusual in all age groups and often indicates the development of complications such as cholecystitis, choledocholithiasis, cholangitis, or gallbladder perforation, all of which are rare in children.
• Pancreatitis exists in 8% of patients with gallstones and is the most common complication. Pancreatitis is more common in obese adolescents who have undergone rapid weight reduction, as reported in the adult population.

• Most gallstones are incidental findings on abdominal ultrasound and are clinically silent.
• Biliary colic, pancreatitis, obstructive jaundice, cholangitis, or other complications should be excluded.
• Intolerance to fatty food rarely exists in children.

• Previous episodes of right-upper-quadrant abdominal pain
• Any risk factors for hemolysis
• History of necrotizing enterocolitis
• Total parenteral nutrition
• Diuretic use
• Short gut syndrome
• History of resection of the terminal ileum.

• The physical examination may be completely normal or may uncover the acute abdomen of pancreatitis.
• In adolescents, the Murphy sign (tenderness on palpation of the RUQ of the abdomen associated with inspiration) may be elicited.

• Blood testing is usually unrewarding.
• Occasionally, a leukocytosis, elevation in liver enzymes, or elevated amylase/lipase may be detected.
• Abdominal radiography may show the presence of a gallstone; 50% of these are radiopaque.
• Ultrasound is the diagnostic procedure of choice—noninvasive, sensitive, and specific.
• Endoscopic retrograde cholangiopancreatography (ERCP) is especially good for evaluation of choledocholithiasis and removal of common bile duct stones.

• In children with asymptomatic gallstones, observation is the best therapy.
• In infants, there is a chance for spontaneous stone dissolution, especially in cholelithiasis linked to total parenteral nutrition (TPN). In children who are dependent on TPN, such as patients with short-bowel syndrome, pseudo-obstruction, and inflammatory bowel disease, gallstones should be removed.
• Cholecystectomy remains the procedure of choice in children with symptoms or in the presence of silent gallstones.
• Laparoscopic cholecystectomy may decrease the length of the hospital stay and the extent of the required abdominal incision.
• Medical treatment for cholesterol gallstones in children may include chenodeoxycholic acid and ursodeoxycholic acid. This treatment is not recommended in the pediatric age group because of low success rate.
• Prevention of gallstone formation is linked to recognition of possible underlying risk factors and attempts to limit these risk factors (small enteral feeds in addition to TPN, early pancreatic enzyme supplements in patients with cystic fibrosis, using alternative forms of contraception in high-risk populations, and weight control in obese infants and children with known hemolytic disease).
• Pigment stone formation increases with age. Cholecystectomy, even for the asymptomatic patient, is warranted. In patients with sickle cell disease, the gallbladder should be removed once stones are identified. This will decrease the risk of cholecystitis and other complications, and will also help to differentiate between biliary colic and sickle cell abdominal pain crisis.
• Patients with a history of cholecystitis are at increased risk for further episodes (69% will have biliary colic within 2 years, and 6% will require cholecystectomy).

• Asymptomatic patients: Follow up every year; monitor for onset of symptoms.
• Symptomatic patients: Consider cholecystectomy.

Q: Does my child with cystic fibrosis have a greater problem with gallstones?
A: Yes, children with CF may have more frequent development of gallstones than will normal children. Reports of gallstones while on ursodeoxycholic acid therapy have also been noted.

Q: Why does my child with sickle cell disease have gallstones?
A: Because the process involves breakdown of hemoglobin, which is then derived into bilirubin, this process may accelerate the formation of gallstones.

Q: If my child has repeated attacks of abdominal pain and there are gallstones in the gallbladder, should he have surgery? What kind?
A: Yes; in older adolescents, laparascopic cholecystectomy is being recommended. For younger children or infants, open cholecystectomy is the preferred choice of treatment. There is no role for Actigall in their therapy.

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