eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology
Caroli Disease: Treatment & Medication
Updated: Oct 1, 2009
- Overview
- Differential Diagnoses & Workup
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Treatment
Medical Care
Ursodeoxycholic acid can decrease the frequency of Caroli disease complications due to cholelithiasis. Broad-spectrum antibiotic coverage, including anaerobic coverage, is indicated in cases of cholangitis. Patients with cholestasis should receive fat soluble vitamin supplementation. Because patients with Caroli syndrome or Caroli disease are at an increased risk for cholangiocarcinoma, initial radiographic (ie, ultrasonography, MRI) and serologic (ie, CA19-9, CEA) screening should be performed.
Surgical Care
Surgical treatment may be necessary for recurrent or refractory cholangitis. Obstructing stones can be removed and bile flow can be maintained by means of a hepaticojejunostomy or external drainage. In cases of localized stasis, lobectomy can be curative and can also reduce the risk of cholangiocarcinoma. Liver transplantation may be indicated in severe cases of refractory or chronic cholangitis, liver failure, or malignant transformation.2
Medication
Ursodiol can promote the dissolution of intrahepatic stones and promote bile flow in Caroli disease or Caroli syndrome. Broad-spectrum antibiotics are used in the treatment of cholangitis associated with Caroli disease or Caroli syndrome.
Gallstone dissolution agents
These agents are used to prevent and possibly to dissolve gallbladder stones. They enhance bile salt–dependent biliary flow. They also act as choleretic agents and may prove to be a valuable addition to therapy in repeated and refractory cholangitis.
Ursodiol (Urso, Actigall)
Also called ursodeoxycholic acid. Naturally occurring bile used to dissolve radiolucent gallstones. Suppresses hepatic cholesterol synthesis, cholesterol secretion, and inhibits cholesterol intestinal absorption. Little inhibitory effect on synthesis and secretion into bile of endogenous bile acids and does not appear to affect phospholipid secretion into bile. Alters bile composition from supersaturated to unsaturated. Ursodiol-rich bile solubilizes cholesterol by increasing the concentration level at which cholesterol saturation occurs. Promotes bile flow in cholestatic conditions associated with patent extrahepatic biliary system.
Adult
300 mg PO bid
Pediatric
8-10 mg/kg/d PO divided q8-12h
Aluminum-containing antacids, cholestyramine, and colestipol may decrease absorption
Documented hypersensitivity; bile pigment or radiopaque stones; stones >20 mm diameter; obstruction of extrahepatic biliary tree
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Stone dissolution may take months, and stones may recur; caution in nonvisualized gall bladder and chronic liver disease; GI effects include nausea, vomiting, diarrhea, or constipation; dermatologic effects include rash; monitor hepatic enzymes
Antibiotic agents
Broad-spectrum antibiotics are used to treat cholangitis. Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.
Ampicillin and sulbactam (Unasyn)
Drug combination of beta-lactamase inhibitor with ampicillin. Used to treat infections involving skin, enteric flora, and anaerobes. Provides broad gram-positive and anaerobic coverage. Should be combined with an agent with gram-negative coverage, such as aminoglycoside.
Adult
1.5 (1 g ampicillin + 0.5 g sulbactam) to 3 g (2 g ampicillin + 1 g sulbactam) IV/IM q6-8h; not to exceed 4 g/d sulbactam or 8 g/d ampicillin
Pediatric
3 months to 12 years: 100-200 mg ampicillin/kg/d (150-300 mg Unasyn) IV divided q6h
>12 years: Administer as in adults; not to exceed 4 g/d sulbactam or 8 g/d ampicillin
Probenecid and disulfiram elevate ampicillin levels; allopurinol decreases ampicillin effects and has additive effects on ampicillin rash; may decrease effects of PO contraceptives
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in patients with renal impairment; potential cross-sensitivity to other beta-lactams
Gentamicin (Garamycin)
Used to treat cholangitis. Aminoglycoside antibiotic for gram-negative coverage. Used in combination with an agent against gram-positive organisms and one that covers anaerobes.
Adult
5-6 mg/kg/d IV divided q8h; must adjust dose and frequency to serum drug levels
Pediatric
Postnatal age <7 days:
<28 weeks gestational age: 2.5 mg/kg IV q24h
28-34 weeks gestational age: 2.5 mg/kg IV q18h
>34 weeks gestational age: 2.5 mg/kg IV q12h
Postnatal age >7 days:
1200-2000 grams: 2.5 mg/kg IV q12h
>2000 grams: 2.5 mg/kg IV q8h
Infants and children
<10 years: 2.5 mg/kg IV q8h
>10 years: Administer as in adults
Must adjust dose and frequency to serum drug levels
Coadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity; aminoglycosides enhance effects of neuromuscular blocking agents (prolonged respiratory depression may occur); coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying degrees may occur (monitor regularly)
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Narrow therapeutic index (not intended for long-term therapy); caution in renal failure (patient not receiving dialysis), myasthenia gravis, hypocalcemia, and conditions that depress neuromuscular transmission; adjust dose and frequency in renal insufficiency and renal impairment
More on Caroli Disease |
| Overview: Caroli Disease |
| Differential Diagnoses & Workup: Caroli Disease |
 Treatment & Medication: Caroli Disease |
| Follow-up: Caroli Disease |
| Multimedia: Caroli Disease |
| References |
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References
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Further Reading
Keywords
Caroli disease, Caroli's disease, congenital hepatic fibrosis, autosomal recessive polycystic kidney disease, ARPKD, autosomal dominant polycystic kidney disease, ADPKD, simple form Caroli disease, choledochal cyst type V, Caroli syndrome, Caroli's syndrome, ductal plate malformation, ductal-plate malformation, DPM, cholelithiasis, cholangitis, cholangiocarcinoma, sepsis, ascites, portal hypertension, hepatomegaly, splenomegaly, jaundice, treatment, diagnosis
