Cholelithiasis in children presentationion

gall stones in children

1. Cholelithiasis in children D r .anjana

2. introduction Cholelithiasis and choledocholithiasis were considered to be uncommon in infants and children but have been increasingly diagnosed in recent years due to widespread use of ultrasonography . Cholelithiasis -Involves the presence of gallstones which are concretions that form in the biliary tract, usually in the gallbladder. Choledocholithiasis -Refers to the presence of 1 or more gallstones in the common bile duct (CBD)

3. epidemiology Little is known about the epidemiology of cholelithiasis in children Cholelithiasis is relatively rare in otherwise healthy children, occurring more commonly in patients with various predisposing disorders. Studies from Europe have shown an overall prevalence of gallstone disease of 0.13% to 0.2% in children. Studies have shown a bimodal distribution, with a small peak in infancy and a steadily rising incidence from early adolescence . Boys and girls are equally affected in early childhood, but as in adults, a clear female preponderance emerges during adolescence.

4. Acute acalculous cholecystitis - is uncommon in children and is usually caused by infection. Pathogens include streptococci (groups A and B), Gram-negative organisms(Salmonella and Leptospira ) Parasitic infestation with ( Ascaris or Giardia lamblia ) Calculous cholecystitis - may rarely follow -abdominal trauma or -burn injury or - is associated with systemic vasculitis ( periarteritis nodosa .)

5. Types of gallstones Black pigment Cholesterol Calcium carbonate Protein-dominant Brown pigment stones. In children, - >70% of gallstones are the pigment type, -15-20% are cholesterol stones, and -the rest a mixture of cholesterol, organic matrix, and calcium bilirubinate .

6. Black pigment gallstones- -mostly of calcium bilirubinate and glycoprotein matrix, -frequent complication of chronic hemolytic anemias Brown pigment stones - form mostly in infants as a result of biliary tract infection. - - Unconjugated bilirubin is the predominant component (by the high β- glucuronidase activity of infected bile. ) Cholesterol gallstones - composed purely of cholesterol or contain >50% cholesterol along with a mucin glycoprotein matrix and calcium bilirubinate . Calcium carbonate stones have also been described in children.

7. MDR3 deficiency -caused by ABCB4 mutations is a cholestatic syndrome related to impaired biliary phospholipid excretion. -It is associated with symptomatic and recurring cholelithiasis . -Patients may show intrahepatic lithiasis , sludge, or microlithiasis along the biliary tree. Sick premature infants - may also have gallstones; - their treatment is often complicated by such factors as - bowel resection & necrotizing enterocolitis , -prolonged parenteral nutrition without enteral feeding - cholestasis , -frequent blood transfusions, and use of diuretics.

8. Microliths Are gallstones smaller than 3 mm Can form within the intrahepatic and extrahepatic biliary tree May lead to biliary colic, cholecystitis , and pancreatitis Can persist after cholecystectomy . BILIARY SLUDGE - Is made up of precipitates of: -cholesterol monohydrate crystals -calcium bilirubinate -calcium phosphate , carbonate -calcium salts of fatty acids -Are embedded in biliary mucin

9. Pathophysiology If a gallstone obstructs the cystic duct: Acute cholecystitis distension of the gallbladder wall Necrosis and spillage of bile . If gallstones migrate from the gallbladder into the cystic duct and main biliary ductal system, further complications such as: Choledocholithiasis Biliary obstruction with or without cholangitis Gallstone pancreatitis

10. Cholesterol gallstone - seems to result from an excess of cholesterol in relation to the cholesterol-carrying capacity of micelles in bile. - Supersaturation of bile with cholesterol, lead to crystal and stone formation( decreased bile acid or from an increased cholesterol concentration in bile.) Other initiating factors -gallbladder stasis or -the presence in bile of abnormal mucoproteins or bile pigments that may serve as a nidus for cholesterol crystallization

11. Prolonged use of high-dose ceftriaxone , formation of calcium- ceftriaxone salt precipitates ( biliary pseudolithiasis ) Biliary sludge or cholelithiasis can be detected in >40% of children who are treated with ceftriaxone for at least 10 days.

12. Presentation Only 33-40% of children are asymptomatic, More than 50% of patients with gallstones have symptoms, 18% present with a complication such as pancreatitis, choledocholithiasis or acute calculous cholecystitis In symptomatic patients: -The most common symptom is recurrent abdominal pain, which is often colicky and localized to the right upper quadrant -Nausea and vomiting. may accompany pain

13. An older child may have intolerance for fatty foods. Acute cholecystitis is characterized by fever, pain in the right upper quadrant, and often a palpable mass. Jaundice occurs more commonly in children than adults. Pain may radiate to an area just below the right scapula. PHYSICAL EXAMINATION Murphy sign (expiratory arrest with palpation in the RUQ) is pathognomonic Right upper quadrant guarding and tenderness are present.

14. In the presence of nonspecific, intermittent abdominal pain in children with risk factors, gallstones must be considered as a possible cause. - Risk factors include: -Chronic hemolysis -Obesity - Ileal disease -Family history of childhood gallstones - Parenteral nutrition

15. DIFFERENTIAL DIAGNOSIS Biliary dyskinesia Biliary pseudolithiasis Choledochal cyst Cholestasis Neonatal Jaundice Pediatric Cholecystitis Pancreatitis and pancreatic pseudocyst

16. Workup Complete blood count Gamma- glutamyltransferase (GGT) Amylase Urinalysis Liver function tests IMAGING Ultrasonography is the study of choice in patients with uncomplicated cholelithiasis . Plain radiography, radionuclide scanning, and cholangiopancreatography -may be useful

17. ULTRASONOGRAPHY can be used to identify : -The location of the stone - Gallstones are usually mobile, single or multiple and characteristically cast an acoustic shadow.. -Gallbladder wall thickening -The presence of gallbladder sludge - appear echogenic on ultrasound , does not cast an acoustic shadow - Pericholecystic fluid A stone, as small as 1.5 mm, can be detected by ultrasonography . The sensitivity and specificity of ultrasonography exceeds 95% for gallbladder cholelithiasis , but 50%-75% for choledocholithiasis . In children 20% to 50% gallstones are radiopaque

18. ABDOMINAL PLAIN RADIOGRAPHY - is seldom useful, because gallstones, with the exception of calcium carbonate stones, are not radio – opaque. - Identifying small-bowel obstruction , or -Free air under the diaphragm. Cholescintigraphy , with technetium 99m labeled diisopropyl iminodiacetic acid (DISIDA), - the most accurate method of diagnosing acute cholecystitis . Nonvisulization of the gallbladder in an otherwise patent biliary system suggests acute cholecystitis . Assess gallbladder filling and bile excretion, particularly in response to cholecystokinin or a fatty meal

19. Magnetic resonance cholangiopancreatography (MRCP) is being used increasingly to investigate complicated GB disease. Endoscopic retrograde cholangiopancreatography (ERCP) offers the additional advantage of therapeutic intervention in common bile duct stones. -Delineate the anatomy of the extrahepatic and intrahepatic biliary tract -Identify the presence of ductal stones -Provide a therapeutic mode of removing a stone or decompressing the biliary tract

20. treatment Management of gallstones depends on the symptoms and the age of the patient. Symptomatic gallstones need cholecystectomy and same is true for complicated gallstones but there is no consensus about the management of asymptomatic gallstones in children. Children with gallstones should be divided into two groups. SYMPTOMATIC GALLSTONES- -Laparoscopic cholecystectomy is currently the criterion standard -It is safe and effective in children, with a low rate of postoperative complications.

21. It has the advantage of being - less invasive - lower morbidity and mortality and -shorter hospital stay over conventional open cholecystectomy Cholecystectomy is indicated for -symptomatic cholelithiasis , - asymptomatic choleli-thiasis persisting beyond 12 months and - radiopaque calculi. The most appropriate method of management of CBD stones seems to be laparoscopic cholecystectomy (LC) with intraoperative cholangiogram (IOC) followed by ERCP.

22. Asymptomatic children or children with nonspecific symptoms can undergo safe follow up. -These children will require observation into adulthood to determine their lifetime risk of developing symptoms. Children with sickle cell anemia, laparoscopic cholecystectomy is currently recommended for asymptomatic gallstones. Spontaneous resolution without specific treatment is most commonly observed in asymptomatic cholelithiasis . If surgery is deferred for any patient, parents should be counseled about signs and symptoms consistent with cholecystitis or obstruction of the common bile duct by a gallstone.

23. COMPLICATIONS. In cases associated with liver disease, severe obesity, or cystic fibrosis, the surgical risk of cholecystectomy may be substantial so risks and benefits of surgery need to be carefully considered. - Cholecystitis -Ascending cholangitis PREVENTION The followings may be effective in preventing the development of cholesterol stones. -Decrease in the consumption of fatty foods -Controlled weight reduction in patients with obesity

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