Choledochal cyst and congenital hepatic fibrosis

Choledochal

Cyst and Congenital By G. Evans-Jones Chester,

l A case of choledochal cyst with congenital hepatic fibrosis is described. This association is known but insufficiently recognized, as is the association between Caroli disease and choledochal cyst. We urge that a specific search be made for these related disorders when patients present with manifestations of one of these disorders. 0 1999 by W.B. Saunders Company. INDEX WORDS: Choledochal brosis; Caroli disease.

cyst; congenital

hepatic

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C

HOLEDOCHAL cyst, polycystic liver disease, congenital hepatic fibrosis (CHF), and Caroli disease (congenital intrahepatic biliary dilatation) belong to a family of disorders termed fibropolycystic diseases of the liver.’ That choledochal cyst and CHF may coexist may not be fully appreciated, and we report a case of this association. CASE

REPORT

A 2-year-old boy was referred following the detection of hepatosplenomegaly at a routine medical examination. He was asymptomatic and there was no significant past or family history. On examination the only positive findings were a firm enlarged liver and moderate splenomegaly. Investigations showed the following relevant findings: hemoglobin 10.4 g/dL, white blood cell count 10.55 x lO’/L, platelets 320 x 109/L, serum bilirubin 11Mmol/L (normal, 5 to 17). alkaline phosphatase 762 IU/L (normal, 30 to 127). alanine transferase (ALT) 195 IU/L (normal, 35), and prothrombin time 13.6 seconds (control, 12.6). Abdominal ultrasound scan showed a cyst approximately 4 cm in diameter close to the gall bladder, suggestive of a choledochal cyst; both kidneys appeared normal. Barium meal showed medial displacement of the duodenal loop but no other abnormality. Computed axial tomography (CAT) showed a choledochal cyst embedded in the head of the pancreas with dilatation of the biliary tree proximal to the cyst, with no evidence of Caroli disease or liver parenchymal disease. At laparotomy a large choledochal cyst (type 1)2 was excised with the gallbladder, and a choledochojejunostomy was fashioned retrocolically to a Roux-en-Y loop. A liver biopsy was performed, which showed the appearance of CHF with bands of fibrous expansion of the portal tracts with an increased number of bile ducts but normal liver parenchyma. There was no evidence of biliary obstruction, cholangitis, or cirrhosis. Histology of the cyst and gallbladder showed mild acute and chronic intramural inflammation. Postoperative progress was uneventful. At 12 months of postoperative follow-up he was well and his liver was less enlarged and no splenomegaly was detectable. Liver function tests were normal. Two years after surgery he is asymptomatic with no hepatosplenomegaly, and both liver and renal function tests are normal. Ultrasonography is also normal. DISCUSSION

This boy’s presentation of asymptomatic hepatosplenomegaly; his biochemical liver dysfunction and

Hepatic Fibrosis

and f?. Cudmore England

liver biopsy histology are typical of CHF.3 There were no symptoms to suggest choledochal cyst at presentation. The association between Caroli disease and CHF is well described recently in 12 patients.3 Furthermore, the association between extrahepatic choledochal cyst (type 4) and Caroli disease has been well described.4 The best means of diagnosing Caroli disease is controversial-Hermansen et al recommend percutaneous transhepatic cholangiography’ but Lilly and Silverman recommend hepatobiliary scintiscanning and/or CAT.6 There is insufficient awareness of the significant association between choledochal cysts, Caroli disease, and CHF. For example, in a review of 16 cases of choledochal cysts, liver biopsy was not performed in any case or Caroli disease specifically looked for.’ We would suggest that children with choledochal cyst should have a liver biopsy to exclude CHF and that Caroli disease should be specifically excluded, either by preoperative cholangiography or liver scintiscanning and/or CAT scanning, or peroperative cholangiography. Only in this way will the true incidence of these associated hepatobiliary disorders be documented, and will individual patients receive optimal management. Long-term follow-up is required in this case because the natural history of CHF is variable. For example, the first alimentary bleed due to portal hypertension can occur as early as infancy or as late as 10 years of age. Similarly, in cases of choledochal cysts associated with Caroli disease complications such as recurrent cholangitis and, in a small number, hepatic carcinoma may occur. REFERENCES

1. Sherlock S: Cysts and congenital biliary abnormalities, in Sherlock S (ed): Diseases of the Liver and Biliary System. London, England, Blackwell, 1985, pp 429-441 2. Mowat AP: Disorders of the gallbladder and biliary tract, in Mowat AP (ed): Liver Disorders in Childhood. London, England, Butterworth, 1979, pp 331-353 3. Summerfield JA, Nagafuchi Y, Sherlock S, et al: Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol2:141-156, 1986

From the Department of Paediatrics. Countess of Chester Hospital, Chester, England. Address reprint requests to G. Evans-Jones, FRCP, Consultant Paediatrician, Department of Paediatrics. Countess of Chester Hospital, Liverpool Rd, Chester CH2 IBQ, England. 8 1990 by W.B. Saunders Company. 0022-3468/90/2512-0020$03.00/O

Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1259-1260

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4. Loubeau JM, Steichen FM: Dilatation of intrahepatic bile ducts in choledochal cysts. Arch Surg 111:1384-1390.1976 5. Hermansen MC, Starshak RJ, Werlin SL: Caroli disease: The diagnostic approach. J Pediatr 94:879-882,1977

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6. Lilly JR, Silverman A: Diagnosis of Caroli disease. J Pediatr 97:329, 1980 7. Kobayashi A, Ohbe Y: Choledochal cyst in infancy and childhood. Arch Dis Child 52:121-128, 1977