순천향의대논문집 : 제 14 권 1 호 2008 J. Soonchunhyang Med. Coll. 14(1), p , 200 총담관낭종과동반된췌담관이상합류의영상학적소견순천향대학교부천병원영상의학과, 소화기내과 2 이아름, 이혜경, 박성진, 이범하, 문종호 2,

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1 순천향의대논문집 : 제 14 권 1 호 2008 J. Soonchunhyang Med. Coll. 14(1), p , 200 총담관낭종과동반된췌담관이상합류의영상학적소견순천향대학교부천병원영상의학과, 소화기내과 2 이아름, 이혜경, 박성진, 이범하, 문종호 2, 홍현숙, 김대호 Radiological findings of choledochal cyst with associated anomalous union of pancreaticobilary duct Lee A Leum, Lee Hae Kyung, Park Seong Jin, Yi Boem Ha, Moon Jong Ho 2, Hong Hyun Sook, Kim Dae Ho = Abstract = Department of Radiology, Department of gastroenterology 2 Soonchunhyang University Bucheon Hospital Purpose: This retrospective study was designed to classify choledochal cysts and an anomalous union of pancreaticobilary duct (AUPBD) on the basis of imaging features and to evaluate associated findings. Materials and Methods: Thirty-one patients with choledochal cysts (23 female, 8 male; mean age, 46.1 years) were classified according to the types of Todani and Komi classification based on the findings of CT, MRCP and ERCP in a hospital. Two radiologists reviewed and classified the types of choledochal cysts, AUPBDs and associated findings. Results: The most common type was IVA in 14 patients according to Todani classification. Type IA, IC, III, IB and V were as follows. According to the Komi classification, the most common type was Ia in 9 patients, and type IIb, Ib, IIa, IIIc2, IIIc3 were as follows. The associated abnormal findings were GB cancer, cholangiocarcinoma, ampulla of Vater cancer, and stones. Conclusion: The most common types of the choledochal cyst and an AUPBD are type IVA in Todani and Ia in Komi classification. GB cancer is the most common associated malignancy. MRCP and coronal reformatted images of MDCT are very useful modality in evaluating the AUPBD. Index words : Bile duct, Choledochal cyst, MR, CT, Cholangiography Corresponding author: 이혜경경기도부천시원미구중동순천향대학부천병원영상의학과 T.P: Fax: hklee@schbc.ac.kr 서 총담관낭종은간내및간외담도가낭성또는방추형으로확장되는질환으로전세계적으로드물게보고가 론

2 되고있으며, 서양에서는 100,000~150,000 명당한명꼴로발병되며, 서구에비해일본을비롯한동양에서 발생빈도가높으며여성에서남성보다 3~4 배더많이발생한다고알려져있다. 1) 췌담관이상합류는 총담관낭종발생의근본원인으로알려져있으며, 이들이담석및담관석, 췌장염, 간농양, 담관경화, 간문맥 고혈압과간및담관암등의여러합병증을유발하는것으로알려져있어적극적인진단과치료의 필요성이제기되고있다. 2-6) 총담관낭종과췌담관이상합류를진단하기위하여초음파검사, 전산화단층촬영 (CT), 내시경적역행성 담췌관조영술 (ERCP), 자기공명영상담관조영술 (MRCP), 경내시경적도관초음파등이이용된다. CT 는최근 다중검출영상획득기법의도입으로해상도가증가하였고, 여러가지방향으로직선뿐만아니라곡선형태로 영상을재구성하여보여줌으로써복잡한해부학적구조를이해할수있는데큰도움을주고있으며, MRCP 는 최근고자장과고속영상획득이가능한장비의도입으로고해상도의검사가가능하게되었다. 이에저자들은 저자들의경험을바탕으로 CT, MRCP, ERCP 에서관찰되는총담관낭종및췌담관이상합류에대한각종류별 영상소견들과이에동반되는질환들에대해기술하고자한다. 대상및방법 2001 년 2 월부터 2007 년 1 월까지본원에서총담관낭종으로진단받은환자 31 명을대상으로하였고, 남자가 8 명 (25.8%), 여자가 23 명 (74.2%) 이었으며나이는 20 세에서 97 세까지평균 46.6 세였다. 환자의주증상은 복통및복부불쾌감이가장많았고그외발열, 소양감, 체중감소등이있었다. 전체 31 명의환자중 28 명에서 CT 를시행하였으며, 21 명 (67.7%) 에서 MRCP 를, 27 명 (87.1%) 에서 ERCP 를 시행하였다. CT 는모든환자에서조영증강후각각동맥기, 문맥기, 평형기영상을획득하였으며, 조영 증강은조영제 120~150 ml 를자동주입기를통하여초당 3~3.5 ml 의속도로주입하였고, 상행대동맥에서 덩어리추적방법 (bolus tracking method) 을이용하여조영증강시기를결정하였다. CT 장비는나선형 CT 장비 (Hispeed CT-i pro; GE Medical System, Milwaukee, USA) 와 16 채널다중검출전산화단층촬영장비 (MDCT: Somatom Sensation 16; Siemens Medical System, Erlangen, Germany) 를이용하였고, 영상획득은 횡격막에서하복부까지머리에서다리방향으로얻었으며, 나선형 CT 장비에서절편두께 5mm, 피치 (pitch) 1, 관전압 120kVp, 관전류 200mAs 이용하였으며, MDCT 장비에서검출기폭조절은 mm, 피치 1.5:1, 관전압 120kVp, 관전류 160mAs 를이용하였다. MDCT 장비에서영상의재구성은횡단면영상, 시상영상모두절편두께 5mm 로구성하였다. 필요한경우추가로절편두께 3mm 로다평면재구성을하였다. MRCP 는 3T (GE SIGNA HDx, GE Medical System, Milwaukee, USA) 기기와 1.5T (GE SIGNA, GE Medical System) 장비를이용하였으며, 각각 8 채널과 4 채널몸통코일배열 (torso array coil) 을이용하여호흡정지상태에서단발포고속스핀에코 (Single Shot Fast Spin Echo) 기법으로원천절편영상 (source image) 횡단면과관상면영상, 삼차원 투사영상 (3-D projection image) 을얻었다. 사용한파라미터는 TR/ TE eff 45000/900msec, 절편두께 4mm, 메트릭스크기 , 영상범위 28cm 등이었으며, 원천절편횡단면영상은간부터총담관까지 3-5mm 간격으로 40 개의영상을얻었고, 관상면영상은간문부를중심으로하여 3-5mm 간격으로 20 개의영상을, 삼차원투사영상은총담관을중심으로 5cm 두께의관상면영상을기준하여 15 도간격으로총 12 개의 회전영상을얻었다. 얻어진영상은 PACS (Picture Archiving and Communications System) (π -view STAR, Infinitt, Seoul, Korea) 상에서두명의영상의학과의사가독립적으로분석하였으며이견이있는경우는합의하여판정하였다. 각영상의분석은 CT 와 MRCP 의소견을 ERCP 와비교하여 Todani 분류 7,8) 에따라확장된담관의부위, 범위및

3 간내담관의확장여부에따라총담관낭종의종류를나누었고췌담관이상합류유무를확인한뒤 Komi 분류 9) 에의거하여나누었다. 그외동반질환의발생유무및종류를확인하고, 동반질환이있는경우 이들과췌담관이상합류, 총담관낭종의종류에따른연관성을알아보았다. 결과 CT와 MRCP를이용하여총담관낭종및췌담관이상합류를확인하고분류하는것은모든환자에서가능하였으며, 이들소견은 ERCP와일치하였다. Todani 분류에따른총담관낭종은전체환자 31명중 14명 (45.2%) 에서 IVA형 (Fig. 1) 이가장많았으며, IA형 (Fig. 2) 이 7명 (22.6%) 으로그다음으로많았다. 이외 IC형 (Fig. 3) 과 III형 (Fig. 4) 이 4명, IB형 (Fig. 5) 과 V (Fig. 6) 형이각각 1명씩있었다. Komi 분류에따른췌담관이상합류는전체환자 31중 26명 (83.9%) 에서확인가능하였으며, 췌담관이상합류가총담관낭종의원인이되지않는 III형과 V형의총담관낭종환자 5명을제외한모든환자에서분류가가능하였다. 췌담관이상합류의종류는 Ia형 (Fig. 1B) 이 9명 (34.6%) 으로가장많았고, IIb형 (Fig. 7) 이 5명 (19.2%) 으로그다음으로많았다. 이외 Ib형 (Fig. 8) 이 4명, IIa형 (Fig. 2) 과 IIIc2형 (Fig. 9) 이각각 3명, IIIc3형 (Fig. 10) 이 2명이었다 (Table 1). 동반질환으로악성종양이 9명 (29.0%) 으로가장많았으며, 이들중담낭암 (Fig. 2, 5) 이 4명 (12.9%) 으로가장흔하였다. 그외총담관낭종에서발생한담도암 (Fig. 11) 및담낭암과담도암이동시에발생한경우 (Fig. 7) 가각각 2명 (6.5%) 이었고, 바터팽대암 (Fig. 4C) 이 1명에서발생하였다. 양성질환으로는담도계결석 (Fig. 8) 이 5명 (16.2%) 에서나타났다. 이들동반질환과총담관낭종및췌담관이상합류의연관은각형태별로다양하게나타났다 (Table 2). 고찰 총담관낭종은비교적드문질환으로, 간내외담관의낭성확장을포함한여러가지형태의확장된상태를말하며 1969년 Babbit 등 6) 이총담관낭종의발생기전으로총통로설을내세운이후췌담관이상합류가대표적인원인으로알려져있다. 췌담관이상합류는총담관과췌관이십이지장벽의바깥에서서로합류하는것으로이런경우에 Oddi 관보다더바깥에서합류됨으로써총담관과췌관이 Oddi 관의정상적인기능에영향을받지않게된다. 3) Todani 등은췌담관이상합류에의해총담관낭이형성된환자들에서췌담관이바터팽대부의윤주근보다상방에서이상각도로총담관과합류되어촘담관낭종이형성되는것으로설명하였다. 6-8) 총담관낭종은선천적으로발생하는것으로알려져있으며, 환자의 60% 이상이 10세이하에서진단되며, 20% 정도만이 20대이후에진단되는것이이러한가설을뒷받침한다. 10) 그러나 Yamashiro 등 11) 은삼분의일에서는선천성질환이나, 나머지삼분의이에서는선천적인원인과후천적인원인이동반되어나타나는것으로설명하고있으며, 여러임상적및실험적연구에서췌담관이상합류가후천적원인의중요한인자인것으로설명되고있다. Kimura 등 12,13) 은연속적인담관조영술을이용하여췌담관이상합류에서괄약근의작용이그하방에서일어나서췌액이담관으로역류되는것을, 한 14) 등은췌담관이상합류가있는총담관이소아에서정상인총담관이점점확장되어총담관낭종이형성되는것을확인하였다. 이러한췌담관이상합류는총담관낭종이있는환자에서많게는 92% 까지동반되는것으로보고가되고있어가장큰원인으로판단되며, 이는총담관이그길이가 2cm 가넘을때의미가있으며, 이경우에췌장염, 바터팽대부의협착, 총담관낭종등이발생하는것으로알려져있다. 총담관의길이가 2cm를넘는경우에도총담관낭종이발생하지않기도하나, 췌담관이상합류가있는경우에는트립신등을포함한

4 압력이높은췌액이총담관으로역류하게되어염증반응과내벽의파괴등을통하여결국섬유화를유발하게된다. 또한췌담관이상합류의모양에따라발생하는총담관낭종의모양이다르다고알려져있는데, 직각으로합류하는경우에는낭성확장, 예각으로합류하는경우에는원추형확장으로나타난다. 1,15,16) 총담관낭에대한분류는병변의위치와모양에따라여러가지로나뉘는데초기 Alonso-Lej 등에의하여이루어졌으며, 이후 Todani 등이분류한방법이가장늘리이용되고있다. 7,8,17) 이분류는가장흔한것으로알려진제 I형에서 Caroli 병으로불리는 V형까지다섯가지종류로크게나뉘며, 제 I형은다시 A, B, C의세가지아형으로나뉜다. IA형은총담관의낭성확장, IB형은부분적, 분절형확장, IC형은원추형의확장이발생한경우를말하며, II형은약 2% 정도의빈도로알려져있으며, 총담관게실이나타난경우, III형은총담관류 (choledochocele) 가발생한경우를말한다. IV형은 A 와 B, 두개의아형으로나뉘며, IVA는 20% 내외의빈도로발생하는것으로알려져있는데, 간내담관과간외담관에다수의낭성확장이동반된경우를말하며, IVB는간외담관에만낭성확장이동반된경우를말하고, IVA에비해드문것으로알려져있다. 간내담관만낭성확장된경우를 Caroli 병이라고도부르며 V형으로분류된다. 본연구에서는총 31명총담관낭종환자중 IVA형이 14명 (45.2%) 으로가장흔한것으로나타나다른보고와는상이한소견을보였으며, type I이 12명 (38.7%) 으로그다음으로흔하였다. Type I 내에서는 IA가 7명 (22.6%) 으로가장많았다. 췌담관이상합류는총통로의길이와내경, 췌담관합류시합류점의각도에따라 1977년 Komi 등에의하여최초로분류되었으며, 1991년일부수정된 Komi 분류가널리사용되고있다. 9,18) 이분류에의하면, 췌담관이상합류는제 I형에서 III형까지의주분류와총아홉가지의분류로나뉘며, 제 I형은총담관이췌관과직각을이루어합쳐지는형태이며, 총담관의모양에따라 a형및 b형으로나뉜다. a형은총담관이가는형태이고, b형은확장된형태이다. 제 II형은총담관과췌관이예각으로합류하는경우이며, 총담관의형태에따라 a, b의두가지형태로나뉜다. 제 III형은부췌관이있는형태로, 주췌관과부췌관의형태에따라, a, b, c형으로나뉜다. IIIa형은주췌관과부췌관이잘구분되는전형적인췌장분할 (pancreas divisum) 이며, IIIb형은주췌관이없는췌장분할이며, IIIc형은주췌관과부췌관이서로연결되어있는형으로, 연결관의모양에따라 1, 2, 3형으로다시나뉜다. IIIc1형은연결관이미미한정도로보이는경우이며, IIIc2형은연결관이주췌관이나부췌관처럼확장된경우이며, IIIc3형은췌관및담관이전체적으로또는일부가확장된경우이다. 각형태별발병빈도에대한보고는없으나, 본연구에서는분류가가능하였던 26명의환자중 I형이그절반으로 13명 (50%) 를차지하였고, 이중 Ia형이 9명 (34.6%) 으로가장흔한것으로나타났으며, 그외다양한종류의형태가나타나는것으로보였다. 그러나 II형및 IIc1형은단한명에서도관찰되지않았다. 이는이들형이상대적으로빈도가적을뿐아니라, 본연구에포함된환자의수가비교적적어모든형이관찰되지않았을가능성이높을것으로예상된다. 환자의증상은발현되지않는경우에서부터담관의파열에의한심한복막염까지다양하게나타나며, 나이에따라발현되는증상이다른것으로알려져있다. 성인의경우증상은비특징적이며, 복통이가장흔한것으로알려져있다. 19,20) 복통, 황달, 그리고이와동반된종괴등의전형적인삼대증상은잘나타나지않으며, Liu 등 2) 에의하면총담관낭종환자의 18.3% 에서만이이러한전형적이 3대증상이나타난것으로알려져있다. 전형적인증상을보이지않는이유로건강에대한관심의증가로조기진단및치료를받기때문인것으로추정된다. 16) 그러나일찍진단되지않을경우성인이되어서담관결석, 담관염, 췌장염

5 등으로나타나거나심지어담관암및췌장암등으로진단되어나타나기도한다. 한등 14) 은담즙앙금이총담관내에정체되어, 담즙의흐름이원활하지못하여복통및 hyperamy lasemia, hyperlipasemia, 및일시적인 hyperaminotra nsferasemia 등을일으켜이와동반된증상을나타내는것으로설명하고있으며, 본연구에서도대부분의환자가주로복통및복부불쾌감증상을보여비슷한결과를나타내었다. 총담관낭종을진단하는방법으로초음파가가장쉽고, 유용하게사용될수있으며, 간외또는간내담관의국소적인확장의형태로나타난다. 그러나다른원인에의한담관폐색의경우에서도비슷한모양의담관확장을동반할수있다. CT에서총담관낭종은담관의주위에서내부에물음영을포함하는종괴로관찰되며, 담관의낭성확장이외에동반되어있는췌담관이상합류도잘보여주는것으로알려져있고, 특히조영증강이후 MDCT를통하여촬영을하여영상을재구성함으로써이러한형태를쉽게진단할수있게도움을준다. 21,22) 또한 CT는총담관낭종뿐만아니라주위의췌장및여러다른구조물들을쉽게관찰할수있는데용이하며, 담관암의진단, 담낭의내벽의비후등을진단하는데도움을준다. MRCP는췌담관이상합류를진단하는데있어정확한방법으로알려져있으며, 총담관낭종의각형태와병발부위를잘보여주는것으로알려져있다. 23,24) ERCP도비슷한정도이상의영상소견을보이는것으로알려져있으나, 방사선조사에따른위해성이있고내시경기구및보조기구를이용하여몸속으로삽입을하여야하는점등에서보다침습적이며, 정확한검사를위해서는시술자의경험이중요하며, 25) 시술자에따라성공률과합병증이병발할수있으며, 특히소아등에서는시술하기가어려운단점등이있다. 비슷한방법으로내시경적초음파시술법이있으나시술자에따라성공률이다른것으로알려져있다. 26) 방사선동위원소촬영법 27) 은해부학적인구조외에기능적인면을보여줄수있어유용한것으로알려져있으며, 진단률도비교적높다. 그러나미세한구조를보여주지못하여양성낭종과총담관낭종을구분하기어려운것으로알려져있다. 총담관낭종은담석및담관석, 췌장염, 간농양, 담관경화, 간문맥고혈압과간및담관암등의여러가지합병증을동반하는것으로알려져있는데, 암발생빈도는보고자에따라다양하며, 2.5~28% 까지보고되고있다. 3,9,21) 20대에서암발병률은 2% 정도이며, 60대에서 43% 까지증가하는것으로알려져있으며, 4,5,7,28) 본연구에서는 40대에서가장많이발생하였다. 총담관낭종이있는환자에서암발생률이높은이유로역류된췌장액에의한담관내벽의지속적인자극에의해발생하는것으로추정되며, 동반된담관석의영향도있는것으로알려져있다. 29,30,31) Liu 등에의하면췌담관이상합류만있는환자의 62.5% 에서담관결석이동반되는것으로보고하였으며, 절반이담낭암과담관결석을같이동반하는것으로보고하였다. 2) 본연구에서도담낭암이가장많았으며또한총담관낭종암과담낭암이동시에발병한경우가 2명 (6.5%) 에서발견되었다. 또한암발생률은 Todani분류에따라조금씩다른데제 I형과 IV형에서가장높은것으로알려져있으며, 본연구에서도제 I형에서 4명 (12.9%), 제 IV형에서 3명 (9.7%) 의발생률을보여가장많은것으로나타났다. Komi 분류에따른암발생률에대한연구는알려져있지않은데본연구에서는췌담관이상합류가있는 26명의환자중 Ia형에서 3명 (11.5%) 으로가장많았고, 이는전체 Ia형환자중의 1/3을차지하였다. 그러나대상이적어결론을내리는데는무리가있을것으로생각한다. 본연구의제한점은총담관낭종및췌담관이상합류가드물게나타나는질환이며, 다양한형태로나타나기때문에연구대상이적어환자의발병빈도를정확히분류하는데어려움이있었으나저자들은다양한영상학적기법을통하여비교적많은종류의총담관낭종과췌담관이상합류를진단하고분류할수있었다.

6 결론적으로총담관낭종은 Todani 분류에의해 IVA 형이가장많았으며, 췌담관이상합류는 Komi 분류에의해 Ia 형이가장많았다. 동반질환은담낭암이가장많았다. 또한 MRCP 와 MDCT 에의한재건영상은췌담관이상합류를진단하는데매우유용하였다. 참고문헌 1. De Vries JS, De Vries S, Aronson DC, Bosman DK, Rauws EAJ, Bosma A, et al : Choledochal cysts: age of presentation, symptoms, and late complications related to Todani s classification. J Pediatr Surg 37: , Liu Y, Wang J, Khagendra RD, JI Z, LI J, WANG X, et al : Congenital choledochal cysts in adults: twenty-five-year experience. Chin Med J (Engl) 120: , Savader SJ, Benenati JF, Venbrux AC, Mitchell SE, Widbus DM, Cameron JL, et al : Choledochal cysts: classification and cholangiographic appear ance. Am J Roentgenol 156: , Visser BC, Suh I, Way LW, Kang SM : Conge nital choledochal cysts in adults. Arch Surg. 139: , Watanabe Y, Toki A, Todani T : Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 6: , Babbitt DP : Congenital choledochal cysts: New etio logical concept based on anomalous relations hips of the CBD and pancreatic bulb. Ann Radio 12: , Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K : Congenital bile duct cysts: Classifi cation, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134: , Todani T, Watanabe Y, Fujii T, Uemura S. Ano malous arrangement of the pancreatobiliary ductal system in patients with a choledochal cyst. Am J Surg 147: , Komi N, Udaka H, Ikeda N, Kashiwagi Y : Co ngenital dilatation of the biliary tract; new clas sification and study with particular reference to anomalous arrangement of the pancreatico biliary ducts. Gastroenterol Jpn 12(4): , Flanigan DP : Biliary cysts. Ann Surg 182: , Yamashiro Y, Sato M, Shimizu T, Oguchi S, Miyano T : How great is the incidence of truly congenital common bile duct dilatation? J Pediatr Surg 28: , Matos C, Nicaise N, Deviere J, Cassart M, Metens T, Struyven J, et al : Choledochal cysts: comparison of findings at MR cholangiopancrea tography and endoscopic retrograde cholangiopan creatography in eight patients. Radiology 209: , Kimura K, Ohto M, Saisho H, Unozawa T, Tsuchiya Y, Morita M, et al : Association of gallbladder carcinoma and anomabus pancreatico biliary ductal union. Gastroenterology 89: , 1985; 14. Han SJ, Hwang EH, Chung KS, Kim MJ, Kim H : Acquired choledochal cyst from anomalous pancreatobiliary duct union. J Pediatr Surg 32: , Todani T, Watanabe Y, Fujii T, Toki A, Uemura S, Koike Y : Cylindrical dilatation of the chole dochus: a special type of congenital bile duct dilatation. Surgery 98: , 1985;

7 16. Lindberg CG, Hammarstro LE, Holmin T, Lun dstedt C : Cholangiographic appearance of bile- duct cysts. Abdom Imaging 23: , Todani T : Congenital choledochal dilatation: cla ssi fication, clinical features, and long-term results. J Hepatobiliary Pancreat Surg 4: , Komi N, Takehara H, Kunitomo K, Miyoshi Y, Yagi T : Does the type of anomalous arrange ment of pancreaticobiliary ducts influence the surgery and prognosis of choledochal cyst? J Pediatr Surg 27: , Lai HS, Duh YC, Chen WJ, Chen CC, Hung WT, Lee PH, et al : Manifestations and surgical treatment of choledochal cyst in different age group patients. J Formos Med Assoc. Apr;96: 242-6, Okada A, Nakamura T, Higaki J, Okumura K, Kamata S, Oguchi Y : Congenital dilatation of the bile duct in 100 instances and its relationship with anomalous junction. Surg Gynecol Obstet 171: , Kim SH, Han JK, Choi BI, Kim TY, Yeon KM : CT findings of malignancies occurring in chole dochal cysts : differential points with benign inflammations. J Korean Radiol Soc 35: , Sugiyama M, Haradome H, Takahara T, Abe N, Tokuhara M, Masaki T, et al : Anomalous pan creaticobiliary junction shown on multidetector CT. AJR Am J Roentgenol 180: , Sugiyama M, Baba M, Atomi Y, Hanaoka H, Mizutani Y, Hachiya J : Diagnosis of anomalous pancreaticobiliary junction: value of magnetic resonance cholangiopancreatography. Surgery 123: , Govil S, Justus A, Korah I, Perakath A, Za chariah N, Sen S : Choledochal cysts: evaluation with MR cholangiography. Abdom Imaging 3: , Kimura K, Ohto M, Saisho H, Unozawa T, Tsuchiya Y, Morita M, et al : Association of gallbladder carcinoma and anomalous pancreatico biliary ductal union. Gastroenterology 89: , Sugiyama M, Atomi Y : Endoscopic ultrasono graphy for diagnosing anomalous pancreatico biliary junction. Gastrointest Endosc 45: , Rajnish A, Gambhir S, Das BK, Saxena R : Classifying choledochal cysts using hepatobiliary scintigraphy. Clin Nucl Med 25: , Todani T, Tabuchi K, Watanabe Y, Kobayashi T. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 44: , Flanigan DP : Biliary carcinoma associated with biliary cysts. Cancer 40: , Yoshida H, Itai Y, Minami M, Kokubo T, Ohto mo K, Kuroda A : Biliary malignancies occurring in choledochal cysts. Radiology 173: , Voyles CR, Smadja C, Shands WC, Blumgart LH : Carcinoma in choledochal cysts. Age-related incidence. Arch Surg 118: , 1983.

8 Table 1. Classification of choledochal cysts and anomalous union of pancreaticobiliary duct according to the Todani and Komi classification Todani Komi IA IB IC IVA total Ia Ib IIa IIb IIIc IIIc Total Table 2. Associated radiological findings according to the Todani and Komi classification. * Combined = choledochal cyst and GB No. Associated findings Todani classification Komi classification 1 GB cancer IVA Ia 2 GB cancer IC IIa 3 GB cancer IC IIa 4 GB cancer IVA IIb 5 Choledochal cyst cancer 1A Ia 6 Choledochal cyst cancer III * 7 Combined cancer * IB Ia 8 Combined cancer * IVA IIb 9 Ampulla of Vater cancer III * 10 Choledochal cyst stone IVA Ia 11 Choledochal cyst stone IA Ib 12 Choledochal cyst stone IVA Ib 13 GB stone III *

14 Combined stone * IVA IIIc3 Fig.1. A 43-year-old female with a type IVA choledochal cyst and a type Ia AUPBD. A. Ultrasound image shows segmental dilatation of intrahepatic bile ducts and cystic dilatation of the cmon bile duct.

A 20-year-old female with a type IA choledochal cyst and a type IIa AUPBD.

9 14 Combined stone * IVA IIIc3 Fig.1. A 43-year-old female with a type IVA choledochal cyst and a type Ia AUPBD. A. Ultrasound image shows segmental dilatation of intrahepatic bile ducts and cystic dilatation of the cmon bile duct. B. ERCP image shows cystic dilatation of the common bile duct with abrupt narrowing at distal end joining with the pancreatic duct at the right angle. Fig. 2. A 20-year-old female with a type IA choledochal cyst and a type IIa AUPBD. MRCP image shows cystic dilatation of the common bile duct and the common hepatic duct without dilatation of intrahepatic bile ducts. It also shows the common bile duct is joined with the pancreatic duct at the right angle.

There is an irregular polypoid enhancing mass in the GB, confirmed to GB cancer after an operation. Fig. 4. A 39-year-old female with choledochocele combined with ampulla of Vater cancer.

10 Fig. 3. A 64-year-old female with a type IC choledochal cyst and an AUPBD combined with GB cancer. CT (A, B) and MRCP (C) images show fusiform dilatation of the common bile duct joining with the pancreatic duct at the right angle. There is an irregular polypoid enhancing mass in the GB, confirmed to GB cancer after an operation. Fig. 4. A 39-year-old female with choledochocele combined with ampulla of Vater cancer. On MRCP (A) image, the distal common bile duct is bulbously dilated (arrow) and the proximal common bile duct and the pancreatic duct are slightly dilated. On ERCP image, the distal common bile duct shows saccular dilatation (B) and then collapse during the procedure (C), and the ampulla of Vater shows bulging and hyperemic mucosal change near the orifice (D, E).

nodular enhancing mass in the GB. Fig. 6. A

11 Fig. 5. A 33-year-old female with a type IIA choledochal cyst and a type Ib AUPBD combined with GB cancer. ERCP and MRCP (A, B) images show the fusiform dilatation of the common bile duct and a a filling defect in the GB. CT (C) image shows a. nodular enhancing mass in the GB. Fig. 6. A 40-year-old female with Caroli s disease. A. MRCP image shows multifocal irregular dilatations of both intrahepatic bile ducts without dilatation of the common bile duct. B. CT image shows multifocal cystic dilated lesions in both lobes of the liver. There are dot-like enhancing foci adjacent to dilated intrahepatic bile ducts.

Fig. 7. A 62-year-old female with a type IVA choledochal cyst and a type IIb AUPBD, combined with cancer in the GB and the choledochal cyst. A. ERCP image shows irregularly dilated common bile duct, common hepatic duct and intrahepatic bile ducts.

CT image shows an enhancing mass in the GB and irregular wall thickening with a mass in the common bile duct. C.

12 Fig. 7. A 62-year-old female with a type IVA choledochal cyst and a type IIb AUPBD, combined with cancer in the GB and the choledochal cyst. A. ERCP image shows irregularly dilated common bile duct, common hepatic duct and intrahepatic bile ducts. The common bile duct is joined with the pancreatic duct at an acute angle and a common channel is also dilated. B. CT image shows an enhancing mass in the GB and irregular wall thickening with a mass in the common bile duct. C. On PET-CT image, focal increased FDG uptakes are seen around the GB and the common bile duct. Fig. 8. A 44-year-old female with a type IVA choledochal cyst and a type Ib AUPBD combined with a stone in the choledochal cyst. MRCP (A) and ERCP (B) images show a segmental dilatation of the intrahepatic bile duct and a marked dilatation of the common bile duct, joined with the pancreatic duct at the right angle. There is a large filling defect

of the common bile duct and the common hepatic duct with a small round filling defect (arrow) at the common bile duct.

13 of stone in the common bile duct and the dilated common channel. Fig. 9. A 67-year-old female with a type IVA choledochal cyst and a type IIIc2 AUPBD combined with the stone in the choledochal cyst ERCP (A) and MRCP (B) images show a diffuse dilatation of the common bile duct and the common hepatic duct with a small round filling defect (arrow) at the common bile duct. Comminuted main and accessory pancreatic ducts are also noted. Fig. 10. A 32-year-old female with a type IA choledochal cyst and a type IIIc3 AUPDB. There is a sacular dilatation of the common bile duct, common hepatic duct and cystic duct on ERCP image. And the duct system of common channel shows dilatation of main and accessory duct with AUPBD.

Fig. 11. A 40-year-old male with a type IA choledochal cyst and a type Ia AUPDB combined with choledochal cyst cancer. A. MRCP image shows a cystic dilatation of common bile duct without intrahepatic duct dilatation, and an irregular shaped low signal intensitic lesion (arrow) inside of the cyst.

14 Fig. 11. A 40-year-old male with a type IA choledochal cyst and a type Ia AUPDB combined with choledochal cyst cancer. A. MRCP image shows a cystic dilatation of common bile duct without intrahepatic duct dilatation, and an irregular shaped low signal intensitic lesion (arrow) inside of the cyst. B. On ultrasound image, it notes an echogenic mass (arrow) attached to the anterior wall of the cyst.

Cholangiocarcinoma Arising from Remnant Choledochal Cyst 증례 Todani 1 (Fig. 1), (Roux-en-Y choledochojejunostomy). 1., 118/88 mmhg, 75 /

Cholangiocarcinoma Arising from Remnant Choledochal Cyst 증례 Todani 1 (Fig. 1), (Roux-en-Y choledochojejunostomy). 1., 118/88 mmhg, 75 / Case Report The Korean Journal of Pancreas and Biliary Tract 2017;22:147-151 https://doi.org/10.15279/kpba.2017.22.3.147 pissn 1976-3573 eissn 2288-0941 선천성담관낭종수술후잔류낭종에서 15 년만에발생한담관암 울산대학교의과대학서울아산병원 1