Korean J Anesthesiol.  1979 Sep;12(3):285-290.

General Anesthesia for Carolis Disease

• Lee MH
• Choi YR
• Kang HG
• Kim TS
• Shin KI

Abstract

Caroli's disease is associated with the combination of congenital dilation of the intrahepatic bile ducts, polycystic disease of the kidney and congenital hepatic fibrosis. The disease entity was first reported by Caroli and associates(1958) and was extensively reviewed in a monograph by Caroli and Corcos (1964). The original first description of Carolis disease is characterized by congenital saccular dilation of the intrahepatic bile ducts, a high incidence of stone formation and cholangitis, absence of hepatic fibrosis and portal hypertension but associated with renal tubular ectasia or other forms of cystic disease of the kidneys, possibly of the pancreas. However the pure form, as described above, has been uncommon. We have experience with a case of general anesthesia for a 12 year old female patient who had Caroli's disease, which was confirmed preoperatively by computerized tomography in Kyung Hee Medical Center. As the Carolis disease may involve hypofunction of the liver and kidney, anesthetics with less action on the liver and kidney are-preferable. This patient was premedicated with atropine and meperidine. Anesthesia was induced with thiopental and succinylcholine for intubation, then maintained with N2O-O2 pancuronium and meperidine. The anesthetic and immediate postoperative course was uneventful, but the patient expired with expected cardiac arrest one week later.

MeSH Terms

Anesthesia
Anesthesia, General*
Anesthetics
Atropine
Bile Ducts, Intrahepatic
Caroli Disease*
Cholangitis
Dilatation, Pathologic
Female
Fibrosis
Heart Arrest
Humans
Hypertension, Portal
Incidence
Intubation
Kidney
Liver
Meperidine
Pancreas
Pancuronium
Succinylcholine
Thiopental
Anesthetics
Atropine
Meperidine
Pancuronium
Succinylcholine
Thiopental