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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