Caroli's disease

Abstract

In 1958, Caroli [1, 2] was the first to describe congenital dilatations of intrahepatic bile ducts concerning a segment, a sector, a lobe, or both halves of the liver. Sometimes the dilatations are associated with congenital hepatic fibrosis as described in 1954 by Grumbach [3], dilatations of biliary ductules (biliary angiomatosis), and parenchymal sclerosis. This hereditary autosomal recessive disease is associated in many cases with nephrospongiosis or Cacchi‐Ricci's disease [4]. Caroli's disease is quite rare. By 1982, a total of 99 cases had been reported in the world literature. Aside from these cases we have seen and operated on 10 patients [5, 6]. In Caroli's disease, stenosis and dilatations give rise initially to biliary stasis and, secondly, to intrahepatic biliary lithiasis and biliary infection. The normal course in Caroli's disease consists of suppurative cholangitis, septicemia with gram‐negative organisms, and intrahepatic and subphrenic abscesses. The disease must be recognized before these serious complications arise. The new hepatobiliary imaging procedures should now allow an early diagnosis and, therefore, a better therapeutic approach.

Choledochal Cyst: Case Report and Review of Literature

“…Reconstruction with Roux-en-Y hepaticojejunostomy may then be performed. In Type V (Caroli's disease), if the disease is confined to one lobe, hemihepatectomy may be performed with a Roux-en-Y jejunal anastomosis [20]. Recurrent infection and calculi are a persistent problem in patients with Caroli's disease and surgical cure is generally not possible particularly in bilateral disease.…”

Curative Partial Hepatectomy in Unilobar Caroli’s Syndrome - Report of Three Cases with Long-Term Follow-Up

“…The patient described by Caroli himself in 1958 had to undergo three surgical interventions before the diagnosis of a unilobar dilatation of the biliary ducts of the left lobe of the liver could be established [2]. Today, 37 years later, this course continues to be a typical one of many cases with Caroli's syndrome [5]. An explanation for the delay in diagnosing this syndrome is the discrete and unspecific nature of the complaints during the early stage of this dis ease.…”

Roux-En-Y Hepaticojejunostomy: An Evaluation Of Its Indications and Results in Benign and Malignant Biliary Tree Disease

“…We performed RYHJ for three Choledochal cyst, which is described as the treatment of choice for type I, IV and selected type V choledochal cyst [7][8][9] . The excision of the extrahepatic cyst is essential and the biliary continuity is best corrected with a RYHJ 5 .…”