Management of Congenital Choledochal Cyst with Intrahepatic
Involvement
Takuji Todani, Mitsuo Narusue, Yasuhiro Watanabe, Katsusuke
Tabuchi, Kunio Okajima
Ann Surg 187: 272-280, 1978
The clinical features and surgical treatment of 75 patients
with choledochal cysts with intrahepatic involvement (Type IV-A) were evaluated.
Seventeen were in this series and 58 were reviewed from the literature.
It was of interest that the incidence of Type IV-A choledochal cysts was
nearly 30%, considerably higher than had been previously assumed. This type
is commonly found in older children and young adults. Abdominal pain and
fever indicative of biliary tract infection are more frequently seen than
in patients with Type I, and a mass is not commonly palpable. Surgical treatment
of Type IV-A must achieve bile drainage from the intrahepatic cysts as well
as from the choledochal cyst. For this reason , as well as the prevention
of later development of cancer in the wall of the cyst, hepaticoenterostomy
at the porta hepatis with partial resection of the wall of the intrahepatic
cyst (or partial hepatectomy if possible) combined with excision of the
intrahepatic cyst is recommended as the procedure of choice for type IV-A
cysts.
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