| Infantile Choledochal Cyst Presenting with Neonatal Cholestasis; Review of Anatomical and Clinical Aspect |
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Jae Won Jeong1, Jeong Kee Seo1, Kwi Won Park2, In Won Kim3 |
1Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
2Department of Surgery, College of Medicine, Seoul National University, Seoul, Korea
3Department of Radiology, College of Medicine, Seoul National University, Seoul, Korea |
| 신생아 담즙정체로 발현된 영아기 담관낭종: 해부학적, 엄상적 특성에 관한 고찰 |
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정재원1, 서정기1, 박귀원2, 김인원3 |
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 외과학교실
3서울대학교 의과대학 진단방사선과학교실 |
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| Abstract |
Purpose
: Extrahepatic biliary atresia, neonatal hepatitis, Allagille syndrome are most common primary causes of the neonatal cholestasis. Infantile choledochal cyst is another primary disease presenting with neonatal cholestasis but its incidence is so low that it has been reported rarely. Choledochal cyst in infancy has been known to have clinically and anatomically different characteristics from ones after infancy. Thus we are designed this trial for further evaluation and management of infantile choledochal cyst.
Methods
: We evaluated 21 cases of infantile choledochal cysts diagnosed with operative cholangiography and abdominal ultrasonography between 1979 and 1994. Clinical features, laboratory findings, associated diseases, surgical procedures, anatomical classification, complications and prognosis were reviwed.
Results
: Twenty-one(23%) patients with choledochal cysts in childhood were infantile type. Sixteen patients were female and five, male. Clinical manifestations at onset were jaundice(l00%), hepatomegaly(81%), acholic stool(57%), fever(33%) or abdominal mass(24%). The classic triad consisting of jaundice, abdominal pain and abdominal mass was not present in any patients. Laboratory findings revealed elevated level of conjugated bilirubin, SGOT, SGPT and serum alkaline phosphatase. Prothrombin time was delayed in 8 cases. In order to confirm the diagnosis, operative cholangiography was done in 19 cases, abdominal ultrasonography in 17 cases and abdominal CT or hepatobiliary scintigraphy in some cases. Anatomically, extrahepatic biliary atresia was combined in 5 cases(26%), distal CBD obstruction in 7 cases(37%). According to Todani classification, type I is 16, type IVa is 2. Surprisingly, one case was unclassified by previous classification system and had choledochal cyst limited in common hepatic duct. Combined anomalies were congenital heart diseases(2), imperforated anus(l), congenital hepatic fibrosis(l). The usually performed operative procedure was total cyst excision with Roux-en- Y choledochojejunostomy. Kasai operation was done when combined with extrahepatic biliary atresia. Mortality rate was 28%. Complications were cholangitis(43%), cirrhosis(24%), sepsis(l9%) and cyst rupture(5%). Among the 15 cases who survived, 12 cases had showed normal liver function within 1 month after operation.
Conclusion
: Infantile choledochal cyst presents with neonatal cholestasis and is occasionally associated with extrahepatic biliary atresia and distal common bile duct obstruction. If operative procedures is delayed, cholangitis and cirrhosis progress rapidly and prognosis is worse than one after infancy. But choledochal cyst not associated biliary atresia would be curable with early operation. Neonatal cholestasis should be precluded from choledochal cyst using the radiologic diagnostic tools including abdominal ultrasonography. |
| Key Words:
Infantile choledochal cyst, Neonatal cholestasis |
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