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. Moreover, spontaneous extrahepatic portosystemic shunting has been reported in congenital hepatic fibrosis. [16]
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. 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. 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. [17]
Liver transplantation is also considered in the management of congenital hepatic fibrosis complicated by recurrent cholangitis or failure to respond to various medical and surgical therapeutic modalities resulting in progressive hepatic dysfunction. [18, 19]
Consultations
At one stage of the clinical course of congenital hepatic fibrosis, management and follow-up evaluation require consultations with other disciplines, medical and surgical.
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Pediatric nephrologist - Required in most cases because of frequent association of congenital hepatic fibrosis with autosomal recessive polycystic kidney disease (ARPKD)
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Pediatric surgeon - Required for biliary drainage procedure and wedge liver biopsy
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Invasive radiologist - Required for imaging studies, angiography, and splenic portography
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Vascular surgeon - Required for evaluation of the case with regard to type and timing of shunt surgery
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Transplant surgeon - Required for liver transplantation, renal transplantation, or both
Diet
Patients with congenital hepatic fibrosis are usually placed on a regular diet.
Activity
The activity of children with congenital hepatic fibrosis 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.
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Histopathology of liver biopsy in congenital hepatic fibrosis, which shows a widened portal tract with bands of fibrous tissue that separate areas of normal hepatic parenchyma. Note the multiple irregularly shaped narrow and elongated bile ducts and the absent lobular and portal inflammation.
