Bile duct disease
I. Congenital Anomalies
1.Biliary atresia & hypoplasia
½Å»ý¾Æ¿¡¼ °¡Àå ÈçÇÑ ´ãµµ°è ÀÌ»óÀ̸ç Ãâ»ýÈÄ 1°³¿ùµ¿¾È severe obstructive jaundice¸¦
¾ß±âÇÑ´Ù.
Áø´ÜÀº ¼ö¼ú(surgical exploration with op cholangiography)À» ÅëÇØ È®ÁøÇÒ¼ö ÀÖÀ¸¸ç
´ë·« 10%°¡ Kasai op(hepatic portoenterostomy with Roux-en-Y
choledochojejunostomy)·Î Ä¡·áÇÒ¼ö ÀÖ´Ù.
¼º°øÀûÀ¸·Î biliary-enteric anastomosis¸¦ ½ÃÇà¹Þ¾Ò´Ù ÇÏ´õ¶óµµ ´ëºÎºÐÀÇ È¯ÀÚ´Â °á±¹
chronic cholangitis, extensive hepatic fibrosis, portal hypertensionÀ¸·Î ÁøÇàÇÑ´Ù.
2. Choledochal cyst
adenocarcinoma risk°¡ ³ô´Ù.
surgical excision & biliary-enteric anastomosis°¡ ÇÊ¿äÇÏ´Ù.
3) Congenital Biliary Ectasia : intrahepatic duct dilatation
+- major duct dilatation : Caroli's disease
+- interlobular, intralobular duct : congenital hepatic fibrosis
¨ç ÀÓ»óÁõ»ó : recurrent cholangitis, abscess formation, gallstone formation
¨è ÇÕº´ÁõÀÌ ÈçÇÏ´Ù.
i) secondary biliary cirrhosis with portal hypertension
ii) amyloidosis
iii) extrahepatic biliary obstruction
iv) cholangiocellular carcinoma
v) recurrent episodes of sepsis with hepatic abscess formation
CT³ª cholangiogramÀ¸·Î Áø´ÜÇÒ¼ö ÀÖÀ¸¸ç Áö¼ÓÀû Ç×»ýÁ¦ Ä¡·á°¡ ÇÊ¿äÇÏ´Ù.
II. Choledocholithiasis
1. º´Å»ý¸®
GB stone -> CBD passage :10-15%
³ªÀ̰¡ µé¸é¼ Áõ°¡ÇÏ¿© elderly ptÀÇ 25%¿¡¼ common duct stoneÀ» °¡Áø´Ù.
´ëºÎºÐ ´ã³¶¿¡¼ Çü¼ºµÈ cholesterol or mixed stoneÀÌ´Ù.
±×·¯³ª de novo primary stoneÀÎ °æ¿ì ÈçÈ÷ pigment stoneÀε¥ Àß »ý±â´Â Á¶°ÇÀº
i) chronic hemolytic disease
ii) hepatobiliary parasitism or chronic, recurrent cholangitis
iii) congential anaomaly of the bile duct(ƯÈ÷ Caroli's disease)
iv) dilated, sclerosed, or strictured ducts
2. ÇÕº´Áõ
¨ç cholangitis
¨è obstructive jaundice
cholecystitisȯÀÚ¿¡¼ bilirubin¤Ó 5 mg/dlÀÌ»ó ¿Ã¶ó°¥ ¶§ CBD stoneÀ» ÀǽÉÇÑ´Ù.
ÃÖ´ë 15 mg/dl±îÁö ¿Ã¶ó°¥¼ö ÀÖÀ¸¸ç ±× ÀÌ»óÀº µå¹°´Ù.
20 mg/dLÀÌ»óÀº neoplastic obstructionÀ» ÀǹÌÇÑ´Ù.
s-ALPÀº °ÅÀÇ Ç×»ó Áõ°¡Çϸç ÀÓ»óÀû Ȳ´ÞÀÌ ¹ß»ýÇϱâ Àü¿¡ ¸ÕÀú »ó½ÂÇÑ´Ù.
obstructionÀÌ Ç®¸°ÈÄ LabÈ£Àü¼ø¼
AST/ALT(Áï½Ã È£Àü) -> Bil (1-2ÁÖ) -> ALP
¨é pancreatitis
nonalcoholic acute pancreatitisÀÇ °¡Àå ÈçÇÑ ¿øÀÎÀº biliary tract diseaseÀÌ´Ù.
pancreatitisÀÇ 15%¿¡¼ acute cholecystitis°¡ µ¿¹ÝµÇ°í
30%À̻󿡼 choledocholithiasis°¡ µ¿¹ÝµÈ´Ù.
* cholecystitisȯÀÚ¿¡¼ coexisting pancreatitis¸¦ ÀǽÉÇÏ´Â °æ¿ì
i) back pain or abdominal midlineÀÇ ¿ÞÂÊÀ¸·Î pain
ii) prolonged vomiting with paralytic ileus
iii) pleural effusion(ƯÈ÷ ¿ÞÂÊ)
¨ê secondary biliary cirrhosis : ÀÏ´Ü »ý±â¸é Æó¼â°¡ Ç®¸®´õ¶óµµ °è¼Ó ÁøÇàµÇ¾î LC, portal
hypertension, liver failure·Î ÀÌÇàÇÑ´Ù.
3. Áø´Ü ¹× Ä¡·á
* gallstoneȯÀÚ¿¡¼ ERCP ÀûÀÀÁõ
i) jaundice or pancreatiti history(+)
ii) LFT abnormality
iii) USG»ó CBD dilatation or stone(+)
III. Trauma, Stricture & hemobilia
IV. Hepatobiliary parasitism
V. Sclerosing cholangitis(primary or idiopathic)
1. Á¤ÀÇ ¹× °³¿ä
progressive, inflammatory, sclerosing and obliterative process affecting the extrahepatic
and/or the intrahepatic bile ducts
70%¿¡¼ IBD(ƯÈ÷ UC)¿Í µ¿¹ÝµÈ´Ù.
µå¹°±ä ÇÏÁö¸¸ ¶ÇÇÑ multifocal firosclerosis syndrome°ú µ¿¹ÝµÇ±âµµ ÇÑ´Ù.
: retroperitoneal, mediastinal and/or periureteral fibrosis, Riedel's struma or
pseudotumor of the orbit
* AIDS¿¡¼ÀÇ biliary tract lesion
i) diffuse involvement of intrahepatic bile ducts alone
ii) intra- and extrahepatic bile duct µÑ´Ù ħ¹ü
iii) ampullary stenosis
iv) CBDÀÇ intrapancreatic portion stricture
v) pancreatic duct involvement
°ü·ÃµÈ °¨¿°±ÕÀº Cryptosporidium, MAI, CMV, Microspordia, and Isospora
±× ¿Ü acalculous cholecystitis°¡ 10%¿¡¼ ¹ß»ý
2ndary sclerosing cholangitis´Â choledocholithiasis, CCC, op or traumatic biliary surgery
or continuous inflammatory processÀÇ long-term complicationÀÌ´Ù.
2. primary sclerosing cholangitisÀÇ sx & signs
chronic or intermittent biliary obstructionÀÇ sx & signs
: jaundice, pruritus, RUQ pain or acute cholangitis
Èı⿡ complete biliary obstruction, secondary biliary cirrhosis, hepatic failure
or portal hypertension with bleeding varices
3. Áø´Ü
cholangiography»ó beaded appearance
: multifocal, diffusely, distributed stricture with intervening segments of normal or dilated
ducts
4. Ä¡·á
i) pruritus¿¡´Â cholestyramineÀÌ µµ¿ò
ii) cholangitis¶§´Â antibiotics
iii) bone mass°¨¼Ò¸¦ ¿¹¹æÇϱâ À§ÇØ vit D & Ca
iv) glucocorticoid, MTX, cyclosporineÀº È¿°ú°¡ ¾ø´Ù.
v) UDCA´Â LFT¸¦ È£Àü½ÃŰÁö¸¸ »ýÁ¸·ü Çâ»ó¿¡ ´ëÇØ¼´Â ÀÔÁõµÈ ¹Ù ¾ø´Ù.
vi) complete or high-grade biliary obstrution¶§ dominant stricture¿¡ ´ëÇÏ¿©
balloon dilatation, stentingÀ» ½ÃÇàÇÒ¼ö ÀÖ´Ù.
vii) liver transplantation: ÇöÀç °¡Àå ÈçÇÑ ÀûÀÀÁõ
5. ¿¹ÈÄ: ºÒ·®Çϸç Áß°£»ýÁ¸·üÀÌ 9-12³â
* »ýÁ¸·ü°ú °ü·ÃµÈ 4°¡Áö º¯¼ö: age, bilirubin, histologic stage, splenomegaly