Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
권호 표지

A Case of Choledochal Cyst Complicated by Liver Cirrhosis on Pathology in a 20-Month-Old-Girl

수술 후 임상증상이 호전된 간경화를 합병한 2세 여아에서의 총수담관낭 1례

  • Bae, Sun Hwan (Department of Pediatrics, School of Medicine, Konkuk University) ;
  • Choi, Sung Yun (Department of Pediatrics, School of Medicine, Eul-Ji University) ;
  • Lee, Tae Seok (Department of General Surgery, School of Medicine, Eul-Ji University) ;
  • Lee, Ho Jeong (Department of Pathology, School of Medicine, Eul-Ji University)
  • 배선환 (건국대학교 의과대학 소아과학교실) ;
  • 최성윤 (을지의과대학교 소아과학교실) ;
  • 이태석 (을지의과대학교 일반외과학교실) ;
  • 이호정 (을지의과대학교 해부병리학교실)
  • Received : 2004.08.09
  • Accepted : 2004.09.23
  • Published : 2005.01.15
Download PDF
⟨ Previous Next ⟩

Abstract

Choledochal cyst is considered to be congenital anomalies of the biliary tract, characterized by varying degrees of cystic dilatation at various segments of the biliary tract. A 20-month-old girl was admitted to Eul-Ji general hospital because of abdominal distension. Physical examination revealed marked splenomegaly and hepatomegaly with nodular surface and hard consistency. Laboratory examination showed elevated transaminase level, alkaline phosphatase level and gamma glutamyltranspeptidase level without evidence of cholestasis. Diagnostic imaging study revealed choledochal cyst with Todani classification type 1. Cholecystectomy and Roux-en-Y choledochojejunostomy was performed, and wedge liver biopsy showed diffuse periportal fibrosis with cirrhotic change and ductular proliferation in the portal area. After operation, hepatosplenomegaly and abnormal laboratory examinations improved rapidly, and in 9 months, the liver and spleen became not palpable. We experienced a case of choledochal cyst complicated by liver cirrhosis on pathology in a 20 month-old girl, and removal of choledochal cyst improved clinical manifestations rapidly.

저자들은 20개월 유아에서 복통이나 담즙정체 등의 특이 증상이 동반되지 않으면서도 간, 비장종대를 동반하고 간 조직검사에서 간경화가 있었으나, 종괴제거 수술 후 간기능검사 뿐 아니라 간, 비장 종대가 급격하게 해결된 총수담관낭을 경험하였다. 총수담관낭을 조기에 발견하여 적절하게 제거하는 것이 간기능 검사의 호전 뿐 아니라 조직학적으로 입증된 간경화를 호전시키는 데에도 중요함을 시사한다고 하겠다.

Keywords

References

  1. Song JB, Hwang KK, Cha CI, Kim KT. Two cases of idiopathic dilatation of the common bile duct. J Korean Pediatr Soc 1977;20:136-43
  2. Lee JM, Sung IK, Kim JS, Oh CK. A case of choledochal cyst in a neonate. J Korean Pediatr Soc 1982;25:1068-72
  3. Kim JH, Kim SJ, Kim KC, Cheong BY, Park WJ. A case of congenital choledochal cyst. J Korean Pediatr Soc 1981; 24:584-8
  4. Lim SW. Choledochal cyst in children. J Korean Assoc Pediatr Surg 1999;5:121-5
  5. Balistreri WF, Bove KE, Ryckman FC. Biliary atresia and other disorders of the extrahepatic bile ducts. In : Suchy FJ. Sokol RJ, Balistreri WF, editors. Liver disease in children. 2nd ed. Philadelphia : Lippincott Williams & Wilkins, 2001: 253-74
  6. de Vries JS, de vries S, Aronson DC, Bosman DK, Rauws EA, Bosma A, et al. Choledochal cyst : Age of presentation, symptoms, and late complications related to Todani's classification. J Pediatr Surg 2002;37:1568-73 https://doi.org/10.1053/jpsu.2002.36186
  7. Lee SG, Kang YH, Han KC. Perforated choledochal cyst with bile peritonitis in an infant. J Korean Pediatr Soc 1982;25:854-8
  8. Shin HS, Nam KJ, Lee JH, Kim CS, Choi JC, Oh JY. Spontaneous rupture of choledochal cyst : case report. J Korean Radiol Soc 2002;47:519-22
  9. Choledochal cyst in Korea : A survey by the Korean association of pediatric surgeons. J Korean Assoc Pediatr Surgery 2003;9:45-51
  10. Davenport M, Howard ER. Surgical disorders of the liver and bile ducts. In : Kelly DA, editor. Diseases of the liver and biliary system in children. 1st ed. London : Blackwell Science, 1999:253-78
  11. Wanless IR, Nakashima E, Sherman M. Regression of human cirrhosis. Morphologic feature and the genesis of incomplete septal cirrhosis. Arch Pathol Lab Med 2000;124: 1599-607
  12. Shiratori Y, Imazeki F, Moriyama M, Yano M, Arakawa Y, Yokosuka O, et al. Histologic improvement of fibrosis in patients with hepatitis C who have sustained response to interferon therapy. Ann Intern Med 2000;132:517-24
  13. Jackson CC, Wu Y, Chenren S, Somme S, Chwals WJ, Liu DC. Bile decompression in children with histopathological evidence of pre-existing liver cirrhosis. Am Surg 2002;68: 816-9
  14. Varani S, Landini MP. Cytomegalovirus as a hepatotropic virus. Clin Lab 2002;48:39-44