Congenital Bile Duct Dilatation in Children

By Mei-Hwei Chang, Teh-Hong Wang, Chiu-Chian Chen, and Wen-Tsung Hung

Taipei, Taiwan

�9 Fifteen children under the age of 15 were identified to have congenital dilatation of the biliary tree. Thirteen of them (87%) had dilatation of both intrahepatic and extra- hepatic bile ducts, while two had extrahepatic biliary dilatation only. Visualization of both intrahepatic and extra- hepatic biliary tree in small children before operation is possible by ultrasonography, endoscopic retrograde cho- langio-pancreatography, or percutaneous transhepatic cholangiography. The advantage and disadvantage of those methods of examinations in children are discussed here. Dilatation of intrahepatic bile ducts is common in children with extrahepatic biliary dilatation. Assessment of both the intrahepatic and extrahepatic bile ducts bears a crucial role in the management of congenital biliary dilatation at operation and during the postoperative follow-up. Roux- en-Y hepaticojejunostomy with hepaticocutaneous arm was performed in our patients with satisfactory results. Residual intrahepatic stones could be removed through the hepaticocutaneous arm by choledochoscopy. �9 1986 by Grune & Strat ton, Inc.

INDEX WORDS: Intrahepatic biliary dilatation; endoscopic retrograde cholangio-pancreatography (ERCP); percuta- neous transhepatic cholangiography (PTC).

C O N G E N I T A L DILATATION of the common bile duct (CBD), or choledochal cyst, is not rare.

Oriental peoples are particularly affected) However, dilatation of the intrahepatic biliary tree in addition to the extrahepatic bile duct had not been carefully examined in the past until about one decade ago. 2 The intrahepatic biliary tree was usually described as nor- mal without detailed examination. 3 5 During the fol- low-up course of operated cases with choledochal cysts, problems such as intrahepatic stones or biliary tree infections may occur in the dilated intrahepatic ducts. It is, therefore, important to visualize both the intrahe- patic and extrahepatic biliary tree clearly before or during operation.

During the past five years, we have encountered 18 cases of congenital biliary dilatation in children under the age of 15. Three of them were diagnosed by ultrasonography examination only. Only the 15 cases who were investigated by both ultrasonography and cholangiography were included in this study. Their

From the Departments of Pediatrics, Internal Medicine, and Surgery, College of Medicine, National Taiwan University, Taipei.

Address reprint requests to Mei-Hwei Chang, MD, Department of Pediatrics, National Taiwan University Hospital, Taipei, Tai- wan, ROC.

�9 1986 by Grune & Stratton, Inc. 0022-3468/86/2102-0003503.00/0

clinical manifestations, interesting diagnostic features, and courses are discussed.

MATERIALS AND METHODS

From January 1979 to December 1983, 15 patients under the age of 15 were identified as having congenital dilatation of the biliary tree at the Department of Pediatrics, National Taiwan University Hospital. Serum protein, aspartate aminotransferase (AST), alanine aminotransferase (ALT) activities, direct-reacting and total biliru- bin, alkaline phosphatase, gammaglutamyl transpeptidase, amylase, and creatinine were studied and followed at weekly intervals.

Abdominal ultrasonography was performed as a screening test in all 15 cases. A real time scanner with a 3.5 MHz and a 5.0 MHz transducer (Sonolayer-V Model SSL-53M, Toshiba Corp, Japan) and/or a contact B mode scanner with a 2.25 MHz transducer (Type 5550, Rohe Scientific Corp, Calif) were used.

Endoscopic retrograde cholangio-pancreatography using the Olympus JF-BII duodenoscope (Olympus Optical Co, Tokyo) was attempted in six and succeeded in five patients. The youngest one was a 4-year-old girl. Percutaneous transhepatic cholangiography was performed in six patients using the Chiba needle (Teruma Corp, Tokyo). The youngest one was a 22-month-old girl. Operative cholangiography was performed in seven patients. Fourteen children underwent laparotomy. Bite was sent for bacte6ai culture and amylase determination. Wedge biopsy of the liver was performed in 11 cases.

According to the cholangiographic findings, dilatation of the biliary tree was classified by using Saito's classification, which consists of five main types: (1) dilatation of the common bile duct; (2) dilatation of the hepatic duct; (3) dilatation of the peripheral intrahepatic ducts; (4) dilatation of the intramural choledochus; and (5) miscellaneous. There were also subtypes according to the form and number of dilatation: (c) cystic, (f) fusiform, (d) diverticular, (m) multiple; and appending types to the main and subtypes: (p) anomalous arrangement of pancreatic duct, (h) hepatic hilum stenosis, and (s) bile stone, calculi, mud.6'7

RESULTS

Clinical Manifestat ions and

Laboratory Examinat ions

There were three boys and 12 girls. The age of onset was before one year in 4, 1 to 3 years in four, 3 to 6 years in four, 6 to 10 years in one, and 10 to 15 years in two patients. Abdominal pain and jaundice were the two most common symptoms. Each appeared in 12 (80%) and 11 (73%) of the 15 patients separately. Ten patients had clay-colored stool, seven had fever, and only four had a palpable abdominal mass. Table 1 summarizes the clinical features. The classic triad of choledochal cyst--abdominal mass, pain, and jaun- d i c e - w a s present in only three patients. An eight- year-old girl presenting with only recurrent abdominal pain was diagnosed initially as having hepatitis accord- ing to the elevated transaminase as well as normal

112 Journal of Pediatric Surgery, Vol 21, No 2 (February), 1986: pp 112-I 17

CONGENITAL BILE DUCT DILATATION

Table 1. Clinical Man i fes ta t ions o f the 15 Chi ldren Wi th

Congenital Dilatation of the Biliary Tree

Clinical Manifestations Case No.

Abdominal pain only 3

Jaundice only 2

Abdominal pain and jaundice 2

Abdominal pain and fever 1

Abdominal pain, jaundice, and abdominal mass 1

Abdominal pain, jaundice, and fever 3

Abdominal mass, jaundice, and fever 1

Abdominal mass, abdominal pain, jaundice,

and fever 2

Total 15

bilirubin and alkaline-phosphatase. Ultrasonography revealed fusiform dilatation of the biliary tree. It was further confirmed by cholangiography.

No one had completely normal liver function. Ten patients had conjugated hyperbilirubinemia. Elevation of alkaline phosphatase above 30 King-Armstrong units, the upper normal limit of our children, was found in 11 of the 15 cases. Alanine aminotransferase was greater than 80 Karmen units in 11 cases. Five patients had serum amylase greater than 500 Somogyi units (less than 250 Somogyi units in a normal sub- ject). Four of them had clinical manifestations of pancreatitis including characteristic severe abdominal pain, vomiting, and serum amylase were all greater than 900 Somogyi units.

Cholangiography and Cholangiopancreatography

Thirteen cases (87%) had dilatation of both the intrahepatic and extrahepatic bile ducts and two cases

113

had dilatation of only CBD and common hepatic duct (case 1 and 2).

Using Saito's classification (see MATERIALS AND METHODS), our 15 cases could be classified as [c + Ilc in 4 cases, Ic + [If in 4 cases, If in 2 cases, I f + I | f in 2 cases, Ic + IIc+ I l ICin 1 case, I f + I I f + l I I f in 1 case, and Ic + I l f + IIIC in 1 case (Fig 1).

In the 13 cases with intrahepatic biliary dilatation, only one case had dilatation limited to the left hepatic duct (Fig 2). The remaining 12 cases had both left and right hepatic ducts and their branches dilated. The dilatation of the left hepatic duct was more prominent than right in eight cases.

The junction of the pancreatic duct and CBD was visualized in five cases. A long common channel was found in four cases. There were three types of the junction: pancreatic duct drained into CBD in 3 cases (Fig 3A), CBD drained into pancreatic duct in one case (Fig 3B), and pancreatic duct and CBD drained into the duodenum separately in one case (Fig 4D). Five patients had biliary stones. Two patients had big stones shown on cholangiography. In three patients, fine stones were found during operation but not on cholangiography.

Ultrasonography

When the ratio of the diameter of the bile duct to its adjacent portal vein is greater than one, the bile duct is judged as dilated. Dilatation of the biliary tree was demonstrated in all of the 15 cases by ultrasonography. Both intrahepatic and extrahepatic biliary dilatation could be demonstrated by ultrasonography using real

Fig 1. Scheme of cholan- giographic findings in 15 chi l - dren. The dilatation was classi- fied using Saito's classification. (1: CBD di latat ion; I|: hepatic duct di latat ion; II1: peripheral bile duct dilatation; c: cystic; f: fusiform; p: anomalous ar- rangement of pancreatic duct; m: mult ip le).

CASE 1 2

T Y P E i f I f P

3 4 5 6 7 8

I f l l f m Ic I lcmll lcm If l i fml l l fm Ic I l fm Ic l lcm Ic Ilcm P P

CASE 9 10 11 12 13 14 15

T Y P E I c l f fm Ic l l fm Ic l l cm Ic II fm If I l fm Ic I l fml| lc Ic l lcm P

114 CHANG ET AL

A B ( ~

C

Fig 2. Ultrasonographic (A and B) and operative cholangio- graphic f indings (C) in case 11. (A) Right hepatic duct was not dilated. (B) Left hepatic duct was dilated. (C) Cholangiography showed bead-like dilatation of the left hepatic duct, (Abbrevia- tions: R, r ight hepatic duct; CHD, common hepatic duct; p, portal vein; LHD, lef t hepatic duct).

time scanner (Fig 4). We found dilated CBD and common hepatic duct in 15 cases, dilated left hepatic duct in 13 cases, and dilated right hepatic duct in 12 c a s e s .

Liver Pathology

By gross and microscopic examinations of the liver, biliary cirrhosis was found in the only patient who died. Severe portal fibrosis was found in one patient. Promi- nent periportal fibrosis was found in eight patients. No one showed pathological changes of congenital hepatic fibrosis.

Course and Treatment

Laparotomy was performed in 14 patients. One patient refused operation and was lost to follow-up. Excision of the extrahepatic bile duct and Roux-en-Y

Fig 3. Different types of pancreatico-choledochal junction in two cases, (A) Pancreatic duct (p) drained into CBD. (B) CBD drained into pancreatic duct. (The arrow shows the junction of CBD and pancreatic duct).

hepaticojejunostomy was performed in 13 cases. Among them, the jejunum was vented at the hepatic limb and converted into double Roux-en-Y in eight latter patients with satisfactory results. Residual stones in the intrahepatic bile ducts could be removed by choledochoscopy through the cutaneostomy. One patient underwent temporary cholecystostomy and bile drainage only because of severe biliary infection. She died of sepsis before hepaticojejunostomy could be done.

Fourteen patients were followed for from 5 months to 9 years with a mean duration of 30 months. They are all well. The patient with multiple bead-like dilatation of the left hepatic duct had fever and jaundice 8 years after excision of the choledochal cyst and Roux-en-Y hepaticojejunostomy (case 11). Double Roux-en-Y with a hepaticocutaneous arm was then performed and the patient remained well 18 months after the second operation. Case 5 had residual intrahepatic stones

CONGENITAL BILE DUCT DILATATION 1 15

A B

Fig 4. Ultrasonographic (A and B) and cholangiographic (C and D) findings in case 13. (A) CBD and right hepatic duct were dilated. (B) Left hepatic duct is dilated (arrow: portal vein). (C) PTC showed dilated CBD, right and left hepatic duct, (D) Pancreatic duct and CBD drained separately into duodenum (arrow). (R: right hepatic duct, GB: gall bladder, L: left hepatic duct, S: spine, C: CBE), P: pancreatic duct).

C D

after operation. Choledochoscopic removal of the stones through the hepaticocutaneous arm was suc- cessful, The patient had no more biliary tree infection during the 28-month follow-up. Liver function test returned to normal in all of the 14 patients followed. Follow-up ultrasonographies were performed in 12 cases and revealed normal sized intrahepatic bile ducts in ten cases (Fig 5). The other two patients still showed some degree of dilatation, but it was milder than before. Those four patients with pancreatitis also had no more attacks of abdominal pain and normal serum amylase.

DISCUSSION

Dilatation of the intrahepatic bile ducts was consid- ered to be extremely rare until about one decade ago. It was found in 10% to 83% of the patients with chole- dochal cyst in the literature, 8-11 Our 15 cases showed a rate of 87% of intrahepatic dilatation. This may sug- gest we have a higher incidence of intrahepatic involve- ment but other important factors are the different criteria in interpreting intrahepatic bitiary dilatation and the thoroughness of examination in different cen- ters.

The size of the hepatic ducts in all of our 13 cases with intrahepatic biliary dilatation was greater than the upper limit of the hepatic duct caliber in normal Chinese adults and children, ~2'~3 Therefore, the intra-

hepatic biliary dilatation in our cases is not overdiag- nosed. Caroli et al first described a special form of biliary malformation with generalized cystic dilatation of the intrahepatic bile duct, 14 Congenital hepatic fibrosis was common in this disease. In our 15 cases, only one (case 4, Fig 1) had such diffuse cystic dilatation of the intrahepatic bile ducts. None of our cases had congenital hepatic fibrosis of the liver.

The etiology of congenital dilatation of the biliary tree is still controversial. The common channel theory proposed by Babbit et al (1969) is the most widely accepted one at present, though the other two, the malformation theory and the viral theory, are also supported by some other authors. ~5-2~ Kato and Ohkawa both successfully produced choledochal cyst with mildly dilated intrahepatic bile ducts in puppies by anastomosing the pancreatic duct and biliary t r e e . 22,23

A reflux of the pancreatic juice into the biliary system was thought to be a possible cause of chole- dochal cyst. Excision of the cyst with hepaticojejunos- tomy seems to terminate the reflux and to be the treatment of choice. 24-26 In our five cases with visible junction of the CBD and the pancreatic duct, four had a long common channel and one had separate channels. Questions remained unexplained in those with periph- eral or asymmetric dilatation of the intrahepatic bile ducts as in our case 11, or those without a common

116 CHANG ET AL

A

BEFORE OPERAT tON I

15 MONTHS LATER

B ]1

Fig 5. Ul t rasonographic fo l - l ow-up in case 4. (A) Before operation, right hepatic duct and its branches were markedly di- lated. It became smaller 15 months after operation (a r row) . (B) Same f inding was also noted in le f t hepatic duct and its branches ( a r r o w ) . (Abb rev ia - t ions: RHD, right hepatic duct; p, portal vein; LHD, le f t hepatic duct).

channel as in our case 1 3 if common channel theory is used. Unexplained pancreatitis in children should lead us to investigate the condition of the biliary tree. zv'28 Patients with biliary dilatation also should be screened for pancreatitis. Preoperative assessment of both intra- hepatic and extrahepatic biliary tree could save opera- tion time and give the surgeon a guide to plan the operative procedure.

The comparison of different diagnostic methods before operation is shown in Table 2. Ultrasonography is the best screening method. 29 3~ Compared to cholan- giography, ultrasonography is simple, rapid, noninva- sive, easily reproducible, and gives good information for both intrahepatic and extrahepatic bile duct dilata- tion. The disadvantages of ultrasonography are the following: peripheral bile ducts are not clearly visual-

Table 2. Comparisons of Ultrasonography, PTC, and ERCP in the Preoperat ive Diagnosis o f Bi l iary Di la tat ion

Ultrasonography PTC ERCP

Invasiveness + +

Technical difficulty in infants and small -- -- +

children

Image of biliary tree fragmented integrated integrated

Common bile duct partially obliterated by clearly seen clearly seen

gas in GI tract

Relationship between biliary tree and not clear not clear clearly seen

pancreatic duct

Disadvantage

Irradiation + +

Risk of biliary infection - + + +

Pancreatitis no no occasional

Allergy to contrast medium no rare rare

Conditions not suitable for examina- none bleeding tendency, current biliary infection

tion current biliary infec- or pancreatitis, al- tion, allergy to con- lergy to contrast

trast medium medium

CONGENITAL BILE DUCT DILATATION 1 17

ized in mi ld ly d i la ted or no rma l cases espec ia l ly in

smal l infants ; par t of the C B D was of ten obscured by

gas in t he gas t ro in tes t ina l t rac t ; and the i m a g e was

r a the r f r a g m e n t e d and needed to be i n t eg ra t ed by the

person who was r ead ing the u l t r a sonography . C h o l a n -

g iog raphy is more cont inuous , showing the en t i re bil-

iary t ree as a whole. T h e re la t ionsh ip be tween the

b i l ia ry t ree and panc rea t i c duc t also could be d e m o n - s t ra ted . 3z

U l t r a s o n o g r a p h y could not t ake the p lace o f cho lan-

g iography . P reope ra t i ve c h o l a n g i o g r a p h y should be

a t t emp ted . For those wi th h igh risk or in w h o m P T C or

E R C P fails, ope ra t ive c h o l a n g i o g r a p h y or cho lang io -

g r a p h y t h rough a cho lecys to s tomy tube should be

pe r fo rmed .

D o u b l e R o u x - e n - Y p r o c e d u r e wi th h e p a t i c o c u t a -

neous a r m is su i t ab le for pa t ien ts wi th i n t r ahepa t i c

d i l a t a t ion espec ia l ly for those wi th mu l t i p l e d i l a t a t ion

and stenosis or s tone fo rma t ion . Even if s tones occur

la ter or res idual s tones a re found, such as in case 5,

cho ledochoscopy t h r o u g h the h e p a t i c o c u t a n e o u s a r m

can be pe r fo rmed .

A C K N O W L E D G M E N T

We thank Drs Donald V. Eitzman and B.J. Lin for reviewing this manuscript.

REFERENCES

1. Chen W J, Chang CH, Hung WT: Congenital choledochal cyst: With observation on rupture of the cyst and intrahepatic ductal dilatation. J Pediatr Surg 8:529-638, 1973

2. Tsuchida Y, lshida M: Dilatation of the intrahepatic bile ducts in congenital cystic dilatation of the common bile duct. Surgery 69:776-781, 1971

3. Alonso-Lej F, Rever WB, Pessagno D J: Congenital chole- dochal cyst, with a report of two and an analysis of 94 cases, lnt Abstr Surg 108:1-30, 1959

4. Lee SS, Min PC, Kim GS, et al: Choledochal cyst, a report of 9 cases and review of the literature. Arch Surg 99:19-28, 1969

5. Shallow TA, Eger SA, Wagner FB: Congenital cystic dilata- tion of the common bile duct. Ann Surg 117:355-386, 1943

6. Saito S: A proposal of new classification of congenital biliary dilatation. Jpn J Pediatr Surg 9:1176-1179, 1977

7. Saito S: Surgical treatment and long-term follow-up results of congenital dilatation of the biliary ducts, in Kasai M, Shiraki K (eds): Cholestasis in Infancy: Its Pathogenesis, Diagnosis and Treat- ment. Tokyo, University of Tokyo Press; and Baltimore, University Park Press, 1980, pp 325-332.

8. Klotz D, Cohn BD, Kottmeier PK: Choledochal cysts: Diag- nostic and therapeutic problems. J Pediatr Surg 8:273-283, 1973

9. Longmire WP, Mandiola SA, Gordon HE: Congenital cystic disease of the liver and biliary system. Ann Surg 174:711 726, 1971

10. Glenn F, McSherry CK: Congenital segmental cystic dilata- tion of the biliary ductal system. Ann Surg 177:705-713, 1973

11. Todani T, Watanabe Y, Narusue M, et al: Congenital bile duct cysts: Classification, operative procedures, and review of thirty- seven cases including cancer arising from choledochal cyst. Am J Surg 134:263 269, 1977

12. Wu CH, Wang TH, Sung JL, et al: Normal calibers of pancreatic duct and bile ducts on ERCP in Chinese, in Proceedings of the Third Asian-Pacific Congress of Digestive Endoscopy, Taipei, Gastroenterological Society of the Republic of China, 1980, p 705

13. Hsu GY, Chang MH: unpublished personal data, 1984 14. Caroli J, Couinaud C: Une affection nouvelle sans doute

cong6nitale, desvoies biliaires: La dilatation Kystique unilobaire des canaux h6patiques. Semin Hop Paris 34:496 502, 1958

15. Babbitt DP, Starshak R J, Clemett AR: Choledochal cyst: A concept of etiology. Am J Roentgenot 199:57-62, 1973

16. Miyata S, Shibue T, Toshikazu O, et al: A clinical investiga- tion on 17 cases of idiopathic choledochus dilatation in adult diagnosed by ERCP with special reference to cholangiogram and anomalous pancreatico-biliary ductal anastomosis. Gastroenterol Endosc 21:430-439, 1979

17. Urakami Y, Seki H, Kishi S, et al: Congenital choledochal cyst in children diagnosed by endoscopic retrograde cholangiopan- creatography. Gastroenterol Endosc 18:609-617, 1976

18. Tsuchida Y, Saito S, Honna T, et al: Morphological studies on congenital bile duct cyst with reference to the intrahepatic involvement, in Kasai M, Shiraki K (eds): Cholestasis in Infancy: Its Pathogenesis, Diagnosis and Treatment. Tokyo, University of Tokyo Press; and Baltimore, University Park Press, 1980, pp 207-216

19. Miyano T, Suruga K, Suda K: Abnormal choledocho- pancreatico-ductal junction related to the etiology of infantile obstructive jaundice disease. J Pediatr Surg 14:16-26, 1979

20. Arima E, Akita H: Congenital biliary tract dilatation and anomalous junction of the pancreatico-biliary ductal system. J Pediatr Surg I4:%15, I979

21. Kimura K, Ohto M, Ono T, et al: Congenital cystic dilatation of the common bile duct: Relationship to anomalous pancreaticobil- iary ductal union. Am J Roentgenol 128:571-577, 1977

22. Kato T: The etiology of congenital choledochal cyst, in Kasai M and Shiraki K (eds): Cholestasis in Infancy: Its Pathogenesis, Diagnosis and Treatment. Tokyo, University of Tokyo Press; and Baltimore, University Park Press, 1980, pp 241-250

23. Ohkawa H, Sawaguchi S, Yamazaki Y, et al: Experimental analysis of anomalous pancreaticobiliary ductal union. J Pediatr Surg17:7 13, 1982

24. Lilly JR: Surgery of coexisting biliary malformations in choledochal cyst. 3 Pediatr Surg 14:643-647, 1979

25. Kimura K, Tsugawa C, Ogawa K, et al: Choledochal cyst: Etiological considerations and surgical management in 22 cases. ArchSurg 113:159 163, 1978

26. Todani T, Narusue M, Watanabe Y, et al: Management of congenital choledochal cyst with intrahepatic involvement. Ann Surg 187:272-280, 1978

27. Raffensperger JG, Given GZ, Warner RA: Fusiform dilata- tion of the common bile duct with pancreatitis. J Pediatr Surg 8:907-910, 1973

28. Stringer G, Filler RM: Fictious pancreatitis in choledochal cyst. J Pediatr Surg 17:359-361, 1982

29. Nishina T, Saito S, Bandai Y: Congenital biliary dilatation. lgaku No Ayumi 120:U-57-68, 1982

30. Hadidi A: Types I and 1II choledochal cyst--preoperative diagnosis by ultrasound. Am J Dis Child 137:663-665, 1983

31. Weill F, Eisencher A, Zeltner F: Ultrasonic study of the normal and dilated biliary tree. Radiology 127:221-224, 1978

32. Kato O: Clinical significance of anomalous pancreatico- biliary union. Gastroenterol Endosc 24:511-518, 1982