대 한 방 사 선 의 학 회 지 1992; 28(1) : 124~129 Journal of Korean Radiological Society, January, 1992

Caroli ’s Disease: Hepatic Arterial Color Doppler Signals in the Communicating Dlated Bile Ducts

Moon-Gyu Lee, Boo Kyung Han, Seong Yon Bael‘, Kyoung Sik Cho, Yong Ho Auh, Myung Hwan Kim* , Eun Sil Yu**

Department o[ Diagnostic Radiology, Asan Medical center. University o[ Ulsan College o[ Medicine

- Abstract-

Three siblings with congenital dilatation of the intrahepatic bile ducts (Caroli ’ s disease) are presented . Bile duct

pathology was associated with congenital hepatic fibrosis and polycystic renal disease in all three patients. On color

Doppler imaging (CD imaingJ, multiple small color Doppler signaJs were observed in or near the vascular radicJes

within the dilated bile ducts. besides other well-known sonographic findings such as bile duct dilatations. biliary

calcul i. Dopper frequency spectral analysis confirmed all these color Doppler signals as arterial origin in all patients.

showing pulsatile wave pattern. Although portal venous radicles are well known in conventional sonograms or com-

puted tomotraphy(CT). continuous wave pattems were not detected in all patients. ln addition to previously reported

sonographic findings about Caroli ‘s disease. color Doppler signals showing arterial wave pattern in or around the

portal venous radicles within dilated ducts are another helpful diagnostic criteria and these findings are easily depicted

on routine sonograms with color mapping.

Index Words: Bile ducts‘ abnormalites ‘ 765.1311

Bile ducts. disease. 765 .28

Bile ducts. Doppler sludies. 765.12984

Ultrasound(US) . Doppler studies

Hepatic artery. US studies. 952.12984

across dilated 1umina(2). The patho10gic features of

INTRODUCTION porta1 radic1es. partia l1y or comp1ete1y surrounded by

dilated bile ducts. were a 1so reported with US( 10) and

Since the first report by Caroli et a l( 1), there have CT( 14).

been several repoπs dealing with Caroli ’s disease or By looking into vascular pate ncy and the status

congenital dilatation of the intrahe patic bile of blood flow within vascular radicles (or porta1

ducts(2-9). Recently new diagnostic modalities of radicles), color Doppler Imaging(CD imaging) or con-

u1trasonography(US)( 10-13) and computed ventiona1 pu1sed Dopp1er US may p1ay an additiona1

tomography(CTHI4-17) were applied for the diagnosis role in this disease entity. We describe new1y observ-

of this disease entity. ed co1or Dopp1er signa1s representing arterial wave

Besides congenital dilatation of intrahepatic bile pattems within the dilate d bile ducts. which may be

ducts(bile cysts l. sonograms of the liver showed por- another diagnostic c\ue in diagnosing Caroli ’s disease

ta1 venous radic\es. which were variab1y described as and/or Grumbach ‘ s disease.

intraluminal bulbar protrusions or bridge formation

*울산대학교 의과대학 서울중앙병원 내과

* Department o[ Internal Medicine. Asan Medical Center. University o[ Ulsan ColJege o[ Medicine

**울산대학교 의과대학 서울중앙병원 해부병리과

* *Department o[ Pathology. Asan M edicaJ Center. University o[ Ulsan ColJege o[ Medicine

이 논문은 1991년 9월 20일 접수하여 1991년 10월 23일에 채택되었음

Received September 20. Accepted October 23. 199 1.

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CASE REPORTS

Case 1.

A 33 year-old man was admitted because ofrecur-

rent fever and right upper quadrant pain. On physical

examination. the liver was palpable 3cm below the

right costal margin . He had had several episodes of

fever. c hills. and right upper quadrant pain. Past

history revealed splenomegaly from birth and he had

suffered from easy fatigability. He and his younger

Moon-Gyu Lee, et al Caroli ’s Disease

brother(Case 2) were diagnosed as having congenital a

spherocytosis. Due to splenomegaly ‘ pancytopenia.

and increased peripheral ovalocytes(26% J, splenec-

tomy was done at a private hospital three years ago.

The following abnormallaboratory values were ob-

tained: white blood cell count. 13.800/mm'; mildly

elevated serum alkaline phosphatase. 272IU/L

Serum total bilirubin was normal. Urinalysis was

norma l.

Abdominal CT and US revealed dilatations of in-

trahepatic bile ducts in the entire liver with a hyper-

trophied left lobe. Vascular radicles in the ductal

lumen were also seen. Intrahepatic stones and por-

tal vein thrombosis were also evident. Bilateral small

renal cysts were associated. CD imaging

demonstrated multiple color Doppler signals within

dilated saccular bile ducts. appearing within or along

portal ra dicles. Doppler spectral analysis confirmed

these color signals as arterial origin by demonstrating

a pulsatile wave pattern. Wedge biopsy of the liver

confirmed Caroli ’s disease. associated with congenital

hepatic fibrosis (Fig. 1).

Case 2.

The younger brother. 27 years of age. had a

palpable spleen from birth. He had a history of

poliomyelitis with residual sequellae including

scoliosis. He also received spleectomy under the im-

pression of congenital spherocytosis at a private

hospital three years ago. At operation. a nodular and

enlarged liver was found. He was admitted this time

with hematemesis. Gastric endoscopy disclosed

severe esophageal varices. Laboratory tests were nor-

m a l. Pathologic slide review was not available in this

patient. Abdominal CT and US showed moderately

severe dilatation of the intrahepatic bile ducts in both

c

Fig. 1. (Case one). (a) Enhanced CT shws mu1tiple small bile cysts of dilated bile ducts mainly in the right lobe Multiple septations a re seen within cysts. (b) Transverse CD image of dilated bile ducts in the right lobe demonstrates color signals near the vascular radicle and across the lumen. (c) By Iight microscopy. dila ted a nd distorted bile ducts are arranged along the fibrous sep-tation of nodules. Within the expanded area. bile duct is irregularly dilated

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Journal of Korean Radiological Society 1992 ; 28(1) : 124~ 129

b

c

Fig. 2. (Case two). (a) Enhanced CT of case 2. Similar features to figure l(a ) are seen. (b) Oblique CD image displays a few dilated bile ducts. Color signal is depicted near the vascular radicle(arrow). (c) Pulasatile wave pat. tern is obtained in Doppler spectral analysis ‘ identifying the color signal as arterial origin

Fig. 3. (Case three) . (a) Enhanced CT reveals multiple dilated bile ducts in the right lobe and the medial segment ofthe left lobe. Note the intraluminallinear density within the cyst of m edial segment(arrow). (b) Transverse duplex CD image of the same cyst in the medial segment shows vascular radicle. Doppler spectral analysis at the tip reveals arterial pulsatile wave . (c) Longitudinal CD images of(b) demonstrate color signals , encoded red , around bulbar pro trusion. (d) Arterial wave form is appeared in a Doppler spec. tral display in the red co!or signal

a

b

c

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Moon-Gyu Lee, et al Carol i’s Disease

lobes with small dilated sacc비ar bile ducts in the deve1.:얘ed the concept of the ductal plate malforma-

right lobe. The left lobe of the liver was hypertrophied. tion to explain the findings in autosomal recessive

Bilateral renal cysts were also found Superior polycystic kidney disease and congenital hepatic

m esenteric arterial portography showed multiple fibrosis. An additional finding . visible on the

periportal collaterals without visuallZatlon of the macr。scoplc phqtographs , lS the presence ofvascular

main portal vein. cn imaging showed multiple color radicles including both hepatic arterial and portal signals in dilated small bile ducts near the portal venous ones. partially or completely surrounded by

radicles. and Doppler spectral analysis revealed all dilated bile ducts. These structures correspond to nor-

these color signals to be of arterial origin(Fig. 2). Ex- mal sized veins and may be hepatic arteries on

pected continous wave patterns from the in- sonograms. located in bulbar protrusions(6. 1O). But.

traluminal portal venous flow within the portal hepatic arterial radicles are not easy to document on

radicles were not observed. conventional grayscale sonograms.

To date. reported sonographic findings were

Case 3. dilated bile ducts with vascular radicles appearing as

The eldest brother . a 35-year-old. also have had intraluminal bulbar protrusions. bridge formation

splenomegaly since birth. When he was 25 years old. across lumen. or portal radicles in the center of the

he had an exploratory laparotomy because of sudden dilated ducts . which is regarded as specific findings

severe abdominal pain. At that time the \iver was for the diagnosis. These findings were regarded as to

grossly cirrhotic. He did not receive a splenectomy. support the hypothesis of arrested normal em-

Thereafter he has suffered from recurrent right up- bryogenesis of intrahepatic bile ducts for t l1e

per quadrant pain and feve r. Abdominal CT showed pathogenesis of this disease(l O) . CT can reveal fin-

dilated m비tiple bile ducts with intrahepatic and ex- dings similar to sonograms( 14-17).

trahepatic biliary \ithiasis . Gallbladder stones were However. CD imaging can not only depict dilated

also found. Both kidneys showed multiple small bile ducts with intraluminal vascular radicles but also

cysts . Small dots of color Doppler signals were found easily identify intraluminal color Doppler signals

in the cente'r

phasized the presence ofvascular radicles by quoting

Nakanuma ’ s previous macroscopic and microscopic

DISCUSSION photographs(2) demonstrating the po다al vein and ac-

companying hepatic artery in the central fibrous

Congenita l ble duct dilatation is an autosomal core(so-called the portal radicles) of dilated bile ducts

recessive inherited disease . Pathologically. tortuous In this aspect. the term of the vascular radicles seems

dilated , dysplastic bile ducts are found. A common to be more comprehensive than the portal radicles

additional finding is an infantile polycystic kidney. to understand and explain the embyogenesis of this

In the pathogenetic analysis of Caroli ’ s disease by disease

Nakanuma et a I(2). they consider the malformation The location of the arteral color signals did not

as a developmental abnormalities. probably a com- match well with portal radicles : no arterial echoes

b ination of overgrowth of biliary epithelium and of could be identified in the lumen of bile ducts after

its supporting portal connective tissues . Dispropor- turning off the color e ncoding. The reason for non-

tionate speed and extent of growth of these two tissue visualization of the hepatic arterial radicles may be

components in the fetus and possibly after birth explained by the fact that:relatively high velocity

might result in elongation. tortuosity . and irreg비ar arteries are not imaged on gray scale sonograms

dilatation of the bile ducts. with bulbar protrusions because of small caliber. At presen t. we are lacking and bridge formation of ductal walls . Jorgensen(3 .4) in proof for the explanation of the non-visualization

1'/7 -

Journal of Korean Radi이 ogical Society 1992 ; 28( 1) : 124~ 129

of the venous color signals. The lower velocity than 6. Hoglund M. Muren C. Schmidt D. Caroli ‘ s disease

that of arteries. inappropriate scale for velocity in two sisters. diagnosis by ultrasonography ahd

search. or possible portal venous thrombosis can be computed tomography. Acta Raiologica

factors that could explain this phenomenon. 1989:30:459-462

We believe these arterial color signa1s are very 7. Mall JC. Ghahremani GG. Boyer JB. Caroli's disease

specific for the diagnosis of Caroli ’ s disease. Although associated with congenital hepatic fibrosis and renal

our case number were limited. we did not see such tubular ectasia. Gastroenterology 1974:66:

color signals in patients with hepatic simple cysts or 1029-1035

dilated bile ducts in patients with obstructive jaun- 8. Mujahed Z. Glenn F . Evans JA. Communicating

dice. Some background noise signals in the lumen cavernous ectasia of the intrahepatic ducts(Caroli ’s

ofhepatic cysts may mimik Carol ’sintraluminalcolor disease). AJR 1971 :113:21-26

signals. This can be avoided with apprpriate color set- 9. Lucaya J . Gomez JL. Molino C. Atienza JG. Con-

tings prior to examination. CD imaging may play an genital dilatation of the intrahepatic bile

additive or hopefully confirmative diagnostic tool in ducts(Caroli ’s disease). Radiology 1978: 127:746.778

evaluating Carolïs disease and give clues to the 10. Marchal GJ. Desmet VJ. Pro esmans WC. et a l.

pathogenesis of this unique disease entity. Caroli disease:high-frequency US and pathologic fin-

dings. Radiology 1986: 158:507 -511

REFERENCES

11. Takaki Y‘ Takahashi M. Ueno S. Fukui K. Radiologic

demonstration of Caroli ’ s disease:a case report. J

Comput Tomogr 1986:10:153-156

1. Caroli J. Soupault R. Kossakowski J. et a l. La dilata- 12. Mittelstaedt CA. Volberg FM. Fischer GJ . McCart-

tion polykystique congenitale des voies biliaires ney WH. Caroli ‘s disease:sonographic findings. AJR

intra-hepatiques:essai de classification. Semin Hop 1980: 134:585-587

Paris 1958:34:128-135 13. Bass EM ‘ Funston MR. Shaff MI. Caroli ‘ s disease:an

2. Nakanuma Y. Terada T. Ohta G. Kurachi M. Mat- ultrasonic diagnosis. Br J Radiol 1977:50:366-369

subara F. Caroli ’ s disease in congenital hepatic 14. Choi BL Yeon KM. Kim SH. Han MC. Caroli

fibrosis and infantile polycystic disease. Liver disease:central dot sign in CT. Radiology

1982 ‘ 2 :346-354 1990:174:161-163

3. Jorgensen MJ. Three-dimensional reconstruction of 15. Hopper KD. The role of computed tomography in the

intrahepatic bile ducts in a case of polycystic disease e、raluation of Caroli ‘ s disease. Clin Imaging

of the liver in an infan t. Acta Pathol Microbiol Im- 1989 ‘ 13:68-73

munol Scand(A) 1972:80:201-206 16. Kaiser JA. Mall JC. Salmen BJ. Parker JJ. Diagnosis

4. Jorgensen MJ. A stereological study ofintrahepatic of Caroli disease by computed tomography:report

bile ducts in congenital hepatic fibrosis. Acta Pathol of two cases. Radiolog 1979: 132:661-664

Microbiol Immunol Scand(A) 1974:82:21-29 17. Musante F. Derchi LE. bonati P. CT cholangiography

5. Caroli J. Disease of the intrahepatic biliary tree. Clin in suspected Caroli ’ s disease. J Comput Assist

GastroenteroI1973:2:147-161 Tomogr 1982:6:482-485

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Moon-Gyu Lee, et al Caroli 's Disease

〈 국문 요약>

Caroli ’s Disease : Hepatic Arterial Color Signals in the Communicating Dilated

Bile Ducts

울산대학교 의과대학 서울중앙병원 진단방사선과， 내과* 해부병리과**

李故圭 • 韓笑覆 • 白承뼈 • 趙京植 • 吳勇鎬 • 金明煥* • 劉股첼**

저자들은 선천적 간내담관 확장을 동반한 3명의 형제를 대상으로 color Doppler flow imaging (이하 CD imaging로

함)을 시행하였다. 담관 병리조직 소견상 및 임상적으로 간 섬유증과 다낭성 신질환이 세환자 모두에서 있었다. CDimag.

mg상 다수의 색신호(color signal)를 보였고 그외에 담관확장， 간내담석， 담액낭내의 문맥기 (portal venous radicle)를 의

미하는 구상형 돌출(bulbar pro trusion)을 보였다. 세명의 환자에서 세로이 발견된 색신호가 스펙트럼 분석에서 박동성 파

장을 보이는 동맥인 것으로 판명되었다. 따라서 CD imaging상 동맥 색신호가 보이는 경우는 다른 간의 낭성질환에서는

볼 수 없는 이 질환의 특징적인 소견이라고 생각된다.

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