Congenital Dilatation of Intrahepatic and Common Bile Ducts with Congenital Hepatic Fibrosis By Yoshiyuki

Fujiwara,

Tetsunosuke

Ohizumi, Akita,

Congenital Corcos’

cystic

reviewed

dilatation

of the intrahepatic

the previously

recorded

Goro

Kakizaki,

and

Tetsuro

Fujiwara

Japan bile duct

cases and classified

CASE REPORT

is a rare

disease

this condition

entity.

into various

Caroli

and

forms.

*

A 1: yr-old girl was admitted to the Akita University Hospital, because of recurrent fever, abdominal distention, and hepatosplenomegaly. Laboratory examinations revealed a hemoglobin of I l.7g, WBC 15,100/mm3 with polymorphonuclear leukocytosis, and normal liver function tests. Urinalysis was normal except for positive vanillyl mandelic acid. X-ray’s revealed no evidence of esophageal varices. A liver scan showed a moderate hepatomegaly with some areas of decreased radioactivity. Selective celiac arteriogram showed small radiolucent round areas and ultrasonic echogram showed multiple cystic structures in the right lobe of the liver. Intravenous cholangiogram failed to visualize the biliary ducts. An intravenous pyelogram suggested ectatic change of renal tubuli. At exploratory laparotomy the liver and spleen were markedly enlarged and firm in consistency. The common bile duct showed a fusiform dilatation of a moderate degree. Cystic dilatation of intrahepatic bile ducts was confirmed by the operative cholangiography (Fig. I). Wedge specimen of the liver taken at operation revealed extensive fibrosis in portal areas, in which dilatated bile ducts and imflammatory reaction were noticed. No particular change was found in the hepatic parenchyma (Fig. 2). In order to prevent ascending cholangitis, cholecystectomy and resection of common bile duct associated with hepaticojejunostomy (Roux en Y) were performed. Following the operation, her temperature gradually came down and the patient was discharged 3 mo after the operation. The

Fig. 1. Operative cholangiogram. The intrahepatic bile ducts of the right lobe are dilated and show multiple cysticfeatures, whereos common bile duct show fusiform dilatation.

0 I976 by Grune

& Stratton.

JournalofPediotricSurgery,

Inc.

Vol.

11 ,No.2

(April), 1976

273

CASE REPORTS

274

Fig. 2. Biopsy specimen of the liver. Fibrosis and imflammatory area and extended into the parenchyma. Bile ducts are proliferated

essential feature of congenital dilatation of intrahepatic dilatation of intrahepatic bile ducts in various degree.

change are found in the portal and show marked dilatation.

bile duct is the segmental Dilated cystic structures

and saccular communicate

freely with bilary tree and therefore contain bile and sometimes bile calculi. This condition is often associated with hepatic fibrosis.’ The fibrosis is found in periportal and interlobular areas and as a consequence small portal veins are compressed and distorted, leading to juvenile portal hypertension. In such cases, hepatosplenomegaly and hematemesis are the only findings.2 Both clinical symptoms and biochemical data rarely indicate abnormal hepatic function. In fibrotic portal areas bile ducts are proliferated and dilatated in various degree. If dilatation and tortuosity are predominant and cause clinical symptoms of bile stasis, the term “Caroli’s disease” is used. Caroli’s disease has a serious prognosis due to bile stasis, calculi formation and complicating liver abscess with sepsis3 The associated congenital hepatic fibrosis is also a serious condition, because of portal hypertension and liver failure. The case presented is Caroli’s disease associated with congenital hepatic fibrosis. As it was found in the presented case, congenital dilatation of renal tubuli is frequently associated with this disease. In severe cases, kidneys show renal cysts or spongy pattern in medullary region. The cystic dilatation of both intra- and extrahepatic bile ducts are not infrequent but the surgical treatment should be directed only to the extrahepatic bile duct. We resected extrahepatic bile ducts and performed hepaticojejunostomy (Roux en Y) to prevent bile stasis and ascending infection. REFERENCES I. Caroli J, Corcos V: La dilatation congenitale des voies biliaires intrahepatiques. Rev Medicochir Mal Foie 39: l-24, 1964 2. Kerr DNS, Harrison CV, Sherlock S, et al: Congenital hepatic fibrosis. Quart J Med 30:91-117, 1961

3. Caroli J, Soupault R, Kossakowski J, et al: La dilatation polykystique congenitale des voies biliaires intrahepatiques. Essei de classification. Sem Hop Paris 34:4888495, 1958