eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology
Congenital Hepatic Fibrosis: Treatment & Medication
Updated: Mar 31, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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Treatment
Medical Care
- Medical therapy is provided mainly in the presence of cholangitis. Results of the liver biopsy and culture determine medical therapy in congenital hepatic fibrosis (CHF).
- Portal hypertension with secondary esophageal varices also requires treatment.
- Some episodes of variceal bleeding may spontaneously resolve. However, persistent hemorrhage that lasts longer than 12 hours or requires blood transfusion warrants the consideration of medical therapy, surgical therapy, or both.
- Acute management includes intravenous fluid administration, nasogastric tube placement, and, once the patient is stable, an endoscopy.
- An initial pharmacologic approach with vasopressin, somatostatin, or other vasoconstricting medications is preferred in pediatrics. Each is discussed more thoroughly in the Medication section.
- In cases of uncontrolled hemorrhage, one may resort to other interventions, including endoscopic sclerotherapy or band ligation, transjugular intrahepatic portosystemic shunting, or surgical shunting.
Surgical Care
Portosystemic shunt surgery is the treatment of choice for these patients because the risk of postoperative hepatic encephalopathy is low. Patients also have a patent portal vein and preserved liver function. External or internal drainage may be required to resolve the refractory hepatobiliary infection.
- Sclerotherapy is indicated for the treatment of acute hemorrhage from esophageal varices and as a primary therapy for management of recurrent or chronic variceal bleeding. Prophylactic use of sclerotherapy is still controversial.
- Relative contraindications to the procedure include uncorrectable severe coagulopathy, fever, or compromise of respiratory status.
- Complications of sclerotherapy include ulcers, strictures, rebleeding, perforations, and bacteremia.
- A Sengstaken-Blakemore tube may be required in some patients to control massive life-threatening bleeding. However, its current use is very much limited to patients who fail to respond to endoscopic sclerotherapy and in whom band ligation is not possible.
- Endoscopic variceal ligation is an effective and safe method for early variceal obliteration in children. It is effective in controlling active bleeding and preventing recurrences. However, its benefit over sclerotherapy has not been uniformly established.
- Types of surgical shunt include nonselective total portosystemic shunts, nonselective partial portosystemic shunts that maintain some antegrade blood flow to the liver, and selective portosystemic shunts, which decompress the gastroesophageal junction and the spleen through the splenic vein to the left renal vein.
- Transjugular intrahepatic portosystemic shunts are considered for patients not amenable to sclerotherapy. It is particularly valuable in treating patients with refractory bleeding before liver transplantation.
- Early shunt surgery with splenorenal or portacaval shunting may be required if repeated endoscopic sclerotherapy fails to arrest the variceal bleeding. Select the type of shunt carefully so that renal or hepatic transplantation remains a future option, with minimal limitations and complications. Both the portacaval H-graft shunt and the distal splenorenal shunt are appropriate options.
- Liver transplantation is also considered in the management of CHF complicated by recurrent cholangitis or failure to respond to various medical and surgical therapeutic modalities resulting in progressive hepatic dysfunction.12
Consultations
At one stage of the clinical course of CHF, management and follow-up evaluation require consultations with other disciplines, medical and surgical.- Pediatric nephrologist - Required in most cases because of frequent association of CHF with ARPKD
- Pediatric surgeon - Required for biliary drainage procedure and wedge liver biopsy
- Invasive radiologist - Required for imaging studies, angiography, and splenic portography
- Vascular surgeon - Required for evaluation of the case with regard to type and timing of shunt surgery
- Transplant surgeon - Required for liver transplantation, renal transplantation, or both
Diet
Patients with CHF are usually placed on a regular diet.
Activity
The activity of children with CHF is not restricted, except in late stages of severe hepatic involvement with progressive bleeding varices, severe renal impairment, and shortly after liver or kidney transplantation.
Medication
No specific medical therapy is available for congenital hepatic fibrosis (CHF). The child's condition is usually stable, with liver enzyme levels within the reference range.
Antibiotics
Antibiotic therapy is indicated for acute and recurrent cholangitis and is based essentially on the results of culture.
Sulfamethoxazole and trimethoprim (Bactrim, Septra, Cotrim)
Reported to be an effective therapy in cholangitis that complicates CHF. Efficacy is attributed to high concentration in bile and hepatic parenchyma. Also has good in vitro activity against Enterobacteriaceae.
Adult
IV: 15-20 mg/kg/d (based on trimethoprim component) IV divided q12h
PO: 160/800 mg (ie, 1 double-strength tab) PO bid
Pediatric
8-20 mg/kg/d (based on the trimethoprim component) IV divided q12h for about 2 wk; followed by PO treatment for up to 3 mo
May increase PT when used with warfarin (perform coagulation tests and adjust dose accordingly); coadministration with dapsone may increase blood levels of both drugs; coadministration of diuretics increases incidence of thrombocytopenia purpura in elderly patients; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow depression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine
Documented hypersensitivity; megaloblastic anemia caused by folate deficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Do not use in pregnancy near term because of risk of kernicterus; discontinue at first appearance of rash or sign of adverse reaction; obtain CBC counts frequently; discontinue therapy if significant hematologic changes occur; goiter, diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses may cause bone marrow depression (if signs occur, give 5-15 mg/d leucovorin); caution in folate deficiency (eg, patients with chronic alcoholism, elderly patients, patients receiving anticonvulsant therapy, or patients with malabsorption syndrome); hemolysis may occur in G-6-PD deficiency; caution in renal or hepatic impairment (perform urinalyses and renal function tests during therapy); administer fluids to prevent crystalluria and stone formation
Choleretic agents
These agents enhance bile salt–dependent biliary flow. These may prove to be a valuable addition to therapy in repeated and refractory cholangitis.
Ursodiol (Actigall, Urso)
Also called ursodeoxycholic acid. Has been shown to promote bile flow in cholestatic conditions associated with a patent extrahepatic biliary system.
Adult
13-15 mg/kg/d PO divided bid/qid
Pediatric
10-20 mg/kg/d PO divided tid/qid
Antacids, charcoal, cholestyramine, and colestipol interfere with absorption
Documented hypersensitivity; extrahepatic biliary tree obstruction
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in chronic liver disease, peptic ulcer, or inflammatory bowel disease; GI effects (eg, nausea, vomiting, diarrhea, constipation); dermatologic effects (eg, rash); monitor hepatic enzymes
Vasoconstrictors
These agents are used in medical management of portal hypertension. They reduce portal pressure through vasoconstriction of the mesenteric arterioles and reduce inflow to the portal venous system and portosystemic collaterals.
Vasopressin (Pitressin)
Decreases portal pressure in portal hypertension through vasoconstriction of the splanchnic arterioles thus controlling hemorrhage. Coronary artery disease is a notable undesirable effect. May dispose patients with coronary artery disease to cardiac ischemia. This can be prevented with concurrent use of nitrates.
Has vasopressor and ADH activity. Increases water resorption at distal renal tubular epithelium (ADH effect) and promotes smooth muscle contraction throughout vascular bed of renal tubular epithelium.
Glypressin, triglycyl lysine vasopressin, can also be used in a dose of up to 2 mg IV q6h.
Adult
0.1-0.5 U/min IV and titrate dose prn
After bleeding stops, continue at same dose for 12 h and taper off over 24-48 h
Pediatric
Initial dose: 0.002-0.005 U/kg/min IV, titrate dose prn, not to exceed 0.01 U/kg/min
After bleeding stops, continue at same dose for 12 h and taper off over 24-48 h
Lithium, epinephrine, demeclocycline, heparin, and alcohol may decrease effects; chlorpropamide, urea, fludrocortisone, and carbamazepine may potentiate effects
Documented hypersensitivity; coronary artery disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in cardiovascular disease, seizure disorders, nitrogen retention, asthma, or migraine; excessive doses may result in hyponatremia
Somatostatin (Zecnil)
Diminishes blood flow to portal system because of vasoconstriction, thus decreasing variceal bleeding. Has similar effects as vasopressin but does not cause coronary vasoconstriction.
Adult
250 mcg IV bolus initially, followed by a 250 mcg/h IV
Pediatric
Not established, limited data suggest 15-25 mcg IV bolus initially, followed by 15-25 mcg/h IV for up to 48 h
Epinephrine, demeclocycline, and thyroid hormone supplementation may decrease effects
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May exacerbate or cause gall bladder disease; alters balance in counter-regulatory hormones and may cause hypothyroidism and cardiac conduction defects; monitor blood glucose and adjust infusion rate accordingly
Propranolol (Inderal)
Beta-blocker that lowers heart rate, myocardial contractility, cardiac output, and portal hypertension, thus reducing the risk of bleeding. Additionally, prevents increases in portal pressure (hepatic venous pressure gradient) during physical exertion.
Both propranolol and nadolol, beta-blockers, are effective in preventing first bleeding and reducing the mortality rate associated with bleeding.
Adult
20 mg PO bid initially; may titrate upward by increments of 10 mg/d q3-4d according to heart rate (heart rate should be decreased by about 25%, but not <55 bpm)
Pediatric
0.5-1 mg/kg/d PO divided q6-8h initially; may gradually titrate upward q4-7d to desired effect
Epinephrine, demeclocycline, and thyroid hormone supplementation may decrease effects
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May exacerbate or cause gall bladder disease; alters balance in counter-regulatory hormones and may cause hypothyroidism and cardiac conduction defects
More on Congenital Hepatic Fibrosis |
| Overview: Congenital Hepatic Fibrosis |
| Differential Diagnoses & Workup: Congenital Hepatic Fibrosis |
 Treatment & Medication: Congenital Hepatic Fibrosis |
| Follow-up: Congenital Hepatic Fibrosis |
| Multimedia: Congenital Hepatic Fibrosis |
| References |
| « Previous Page | Next Page » |
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Further Reading
Keywords
congenital hepatic fibrosis, CHF, biliary fibroadenomatosis, hepatic fibroangioadenomatosis, fibrous angio adenomatosis, ductal plate malformation, autosomal recessive polycystic kidney disease, ARPKD, portal hypertension, renal cystic disease, Caroli disease, autosomal dominant polycystic kidney disease, ADPKD, cholangitis, hematemesis, melena, cholestasis, hepatomegaly, cirrhosis, hypersplenism, nephromegaly
