Cholelithiasis
Cholelithiasis: Excerpt from The 5-Minute Pediatric Consult
Vera De Matos, MDDror Wasserman, MD (4th edition)
Cholelithiasis - BASICS
Cholelithiasis - description
Cholelithiasis is defined by the presence of cholesterol and/or pigment stones in the gallbladder. Rare in infancy and childhood, it is usually found incidentally on an ultrasound. Risk factors in children include obesity, sickle cell disease, cystic fibrosis (CF), Crohn disease, and long-term total parenteral nutrition (TPN).
Cholelithiasis - general prevention
Nutrition: Avoid carbohydrate- and triglyceride-rich and low-fiber diet.
Cholelithiasis - epidemiology
- Cholelithiasis is relatively uncommon in childhood and adolescence. However, gallstones occurring in utero have been described.
- Obesity accounts for up to 1/3 of the gallstones observed in all children and the majority of children with no underlying medical conditions. Obesity is estimated to increase the risk of gallstones in children by over 4-fold.
- Canadian Eskimos and Native Africans have the lowest risk of cholelithiasis.
- Native Americans, Swedes, and Czechs have the highest risk.
- Pigment stones are more prevalent in prepubertal children, whereas cholesterol stones are predominant in adolescence and adulthood.
Cholelithiasis - prevalence
- The prevalence of cholelithiasis in children and adolescents reported in the literature is approximately 0.1–0.6%.
- In obese children, the prevalence is 2%.
- In children with sickle cell disease, the prevalence is 17–29%.
- The prevalence of gallstones in North American and European adults is 10–20%.
Cholelithiasis - incidence
- In females, the incidence of cholelithiasis is 0.27% between the ages of 6 and 19 years increasing to 2.7% between the ages of 18 and 29.
- In males, the incidence of gallstones remains negligible throughout childhood and adolescence.
Cholelithiasis - risk factors
- Chronic overnutrition with carbohydrate and triglyceride-rich, low-fiber diet
- Obesity
- Positive family history
- Native American descent
- Female gender
- Pregnancy/Parity
- Chronic hemolysis (sickle cell disease, thalassemia, spherocytosis, malaria).
- Ineffective erythropoiesis (vitamin B12 and folate deficiencies)
- Liver cirrhosis
- TPN
- Severe Crohn disease of the ileum and/or ileal resection
- Anatomical abnormalities (biliary stricture, duodenal diverticulum)
- Medications (estrogens, octreotide, clofibrate, furosemide, cyclosporine, ceftriaxone)
Cholelithiasis - genetics
- Mutations have been identified in genes encoding the ABCB transporters for phosphatidylcholine (adenosine triphosphate-binding cassette, subfamily B), for bile salts (ABCB11), or for cholesterol 7α-hydroxylase (CYP7A1), the CCK-A receptor (CCKAR) and the CF gene (CFTR).
- ABCB4 is also known as MDR3 (multidrug–resistant 3 glycoprotein). MDR3 is a phospholipid translocator in the hepatocyte membrane, involved in biliary phosphatidylcholine excretion. MDR3 deficiency can cause severe neonatal liver disease, but mutations in MDR3 have also been associated with cholelithiasis, cholestasis of pregnancy, and biliary cirrhosis.
- Other gene polymorphisms are currently under investigation in humans.
Cholelithiasis - pathophysiology
- Bile is an aqueous solution of lipids, with bile salts, phospholipids, and cholesterol. Changes in the proportion of bile constituents, nucleation (aggregation of cholesterol crystals), changes in gallbladder motility, or infection can lead to stone formation.
- Stones are of 2 types: Pigment (30–72% in pediatric patients) and cholesterol stones
- Black pigment stones are associated with increased unconjugated bilirubin:
- Hemolytic diseases
- Abnormal erythropoiesis
- Enterohepatic circulation of unconjugated bilirubin:
- Ileal resection, Crohn disease
- CF
- Brown pigment stones are associated with infection.
- The solubility of cholesterol in bile depends on bile salts and phospholipid concentrations. Cholesterol stones are associated with:
- A decrease in bile salts pool
- Decreased bile acid synthesis
- Hypersecretion of cholesterol into the bile
- Gallbladder stasis (weight loss, pregnancy, long-term TPN)
- Increased biliary mucus secretion
- Medications: Furosemide, ceftriaxone, cyclosporine
Cholelithiasis - etiology
- Hemolytic disease (17–29% of children with sickle cell disease)
- TPN
- Prematurity
- Necrotizing enterocolitis
- Crohn disease of the ileum
- CF
- Obesity
- Pregnancy
- Oral contraceptives
- Down syndrome
Cholelithiasis - DIAGNOSIS
Cholelithiasis - signs & symptoms
- Silent gallstones present coincidentally in infancy and preschool-age children.
- Classic symptoms of right upper quadrant (RUQ) pain (Murphy sign) and vomiting exist only 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 rare complications in children:
- Cholecystitis
- Choledocholithiasis
- Cholangitis
- Gallbladder perforation:
- Pancreatitis develops 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.
Cholelithiasis - history
- 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.
- The history should always include questions concerning:
- Previous episodes of RUQ abdominal pain
- Any risk factors for hemolysis
- History of prematurity and necrotizing enterocolitis
- TPN
- Diuretic use
- Short gut syndrome/resection of the terminal ileum
Cholelithiasis - physical exam
- The physical examination may be completely normal or may uncover the acute abdomen of pancreatitis.
- Murphy sign (tenderness on palpation of the RUQ of the abdomen associated with inspiration) may be elicited in adolescents.
Cholelithiasis - tests
Cholelithiasis - lab
- Blood testing is usually unrewarding.
- Leukocytosis, elevation in liver enzymes, or elevated amylase/lipase may be detected.
Cholelithiasis - imaging
- Plain abdominal films are not the investigation of choice; however, up to 50% of gallstones are radio-opaque with conventional radiology.
- Ultrasound is the diagnostic procedure of choice: Noninvasive with high sensitivity and specificity
- Endoscopic retrograde cholangiopancreatography (ERCP) is used for evaluation of choledocholithiasis and removal of common bile duct stones.
Cholelithiasis - differencial diagnosis
- Acalculous gallbladder disease
- Hydrops of the gall bladder
- Common bile duct stones
- Congenital biliary anomalies
Cholelithiasis - TREATMENT
Cholelithiasis - general measures
- Primary prevention: High fiber intake, low saturated fatty acid and nuts, moderate physical activity. Children with asymptomatic gallstones should only be observed. During infancy, there is a chance for spontaneous stone dissolution, especially if cholelithiasis is linked to TPN.
- In children who are dependent on TPN, patients with short-bowel syndrome, pseudo-obstruction, inflammatory bowel disease, and with a hemoglobinopathy, gallstones should be removed.
- Laparoscopic cholecystectomy is the procedure of choice in children with symptoms or in the presence of silent gallstones in older children.
- Prevention of gallstone formation is done by treating underlying risk factors (small enteral feeds in addition to TPN, early pancreatic enzyme supplements in patients with CF, 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. Sickle cell patients should have the gallbladder removed when 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 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).
Cholelithiasis - medication
- Chenodeoxycholic acid has been replaced by ursodeoxycholic acid (UDCA), which is safer and more effective.
- UCDA was shown to improve gallbladder muscle contractility by decreasing muscle cell cholesterol content in the plasma membranes.
Cholelithiasis - surgery
- Indicated in symptomatic gall stones
- Laparoscopic cholecystectomy is the procedure of choice.
Lithotripsy using shock waves has not been approved for use in children.
Cholelithiasis - patient monitoring
- Asymptomatic patients: Follow up every year with ultrasound; monitor for onset of symptoms.
- Symptomatic patients: Consider cholecystectomy.
Cholelithiasis - bibliography
- Gertner M, Farmer DL. Laparoscopic cholecystectomy in a 16-day-old infant with chronic cholelithiasis. J Pediatr Surg. 2004;39:E17–E19.
- Guarino MP, Cong P, Cicala M, et al. Ursodeoxycholic acid improves muscle contractility and inflammation in symptomatic gallbladders with cholesterol gallstones. Gut. 2007;56(6):815–820.
Heubi JE, Lewis LG. Diseases of the gallbladder in infancy, childhood, and adolescence. In: Suchy F, ed. Liver Diseases in Children. Chicago, IL: Mosby–Year Book; 2001:343–362.- Irish MS, Pearl RH, Caty MG, et al. The approach to common abdominal diagnosis in infants and children. Pediatr Clin North Am. 1998;45:729–772.
- Kasirajan K, Obermeyer RJ, Kehris J, et al. Microinvasive laparoscopic cholecystectomy in pediatric patients. J Laparoendosc Adv Surg Tech A. 1998;8:131–135.
- Lobe TE. Cholelithiasis and cholecystitis in children. Semin Pediatr Surg. 2000;9:170–176.
- Lucena JF, Herrero JI, Quiroga J, et al. A multidrug resistance 3 gene mutation causing cholelithiasis, cholestasis of pregnancy, and adulthood biliary cirrhosis. Gastroenterology. 2003;124(4):1037–1042.
- Rosmorduc O, Hermelin B, Boelle PY, et al. ABCB4 gene mutation-associated cholelithiasis in adults. Gastroenterology. 2003;125(2):452–459.
- Stringer MD, Taylor DR, Soloway RD. Gallstone composition: Are children different? J Pediatr. 2003;142:435–440.
- Venneman NG, van Erpecum KJ. Gallstone disease: Primary and secondary prevention. Best Pract Res Clin Gastroenterol. 2006;20(6):1063–1073.
- Yusoff IF, Barkun JS, Barkun AN. Diagnosis and management of cholecystitis and cholangitis. Gastroenterol Clin North Am. 2003;32:1145–1168.
Cholelithiasis - CODES
Cholelithiasis - icd9
574.20 Cholelithiasis without obstruction
Cholelithiasis - FAQ
- Q: Does my child with CF 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 UDCA 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 pigmented 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, laparoscopic cholecystectomy is recommended. For younger children or infants, open cholecystectomy is the preferred choice of treatment.
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
More About Gallstones
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Medical Books Excerpts
- Colic
- "The 5-Minute Pediatric Consult" (2008)
- [ read ]
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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