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KMID : 0371319710130050027
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Journal of the Korean Surgical Society
1971 Volume.13 No. 5 p.27 ~ p.32
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Five Cases of Congenital Biliary Atresia
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Abstract
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It is well known that congenital biliary atresia is the most common surgical problems in the
biliary tract of the neonates.
The authors report 5 cases of congenital extrahepatic biliary atresia, 3 males and 2 females
which were proven by explolatory laparatomy or percutaneous liver biopsy among 7 case of cong
enital biliary atresia diagnosed clinically at surgical department at Severance Hospital College of
Medicine Yonsei University.
In this study, coexistent abnormalities of other organs were not found. Jaundice is the most pro
minent symptoml; it usually appears earlier days of life and steadily increases in severity with
little fluctuation. Meconium appears normal and stools are acholic or lightly colored and the urine
is dark. General condition development of these children remains strikingly good for several
months. On physical examination enlarged and indurated liver was noted and also splenomegaly
noted. Laboratory findings revealed anemia and persistent high level of bilirubin, especially direct
fraction of bilirubin and significantly high level of alkaline phosphatase in all cases. None of our
cases were surgically correctible which was confirmed by operative cholangiography and the liver
biopy revealed biliary cirrhosis. One of 5 cases was observed nearly one year after surgical explor
ation who was falen moribund state.
Even though fetal peritonitis and neonatal hepatitis are listed as etiologic factors, primary diso
rders of organogenesis is generally accepted as eiologic factor of congenital biliary atresia. Other
authors report that 10 to 20 percent of lesions are amen able to surgery but only about 5 percent
of cases could be done successful surgocal correction. Optimal time for operation starts after the
eighth to tenth week of life and ends to the third month of age, at which time parenchyma
tous liver damage sets in and progresses rapidly. Surgical treatment depends on the type of
malformation; cholecystoenterostomy and hepatoenterostomy. Considering
that there are few indications for bypass procedure, it is advisable liver transplanation for this
rare condition.
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