The role of ERCP in biliary atresia

0016-5107/97/4505-036555.00 + 0

GASTROINTESTINALENDOSCOPY Copyright© 1997by the AmericanSocietyfor GastreintestinalEndoscopy

The role of ERCP in biliary atresia Naomi Ohnuma, MD, Hideyo Takahashi, MD, Masahiro Tanabe, MD, Hideo Yoshida, MD, Jun Iwai, MD Chiba, Japan

Background: It is not easy to discriminate between infantile hepatitis and biliary atresia in spite of several diagnostic tests including laboratory analyses, ultrasound, and hepatobiliary scans. ERCP is the most useful procedure for visualization of the extrahepatic biliary system, but ERCP is still an uncommon procedure in children. Methods: ERCP examination was performed in 52 infants with biliary atresia (10 with infantile hepatitis, 5 with congenital biliary dilatation, 3 with paucity of intrahepatic bile duct, 2 with duodenal atresia, and I with postoperative jaundice of hepatoblastoma) aged from 8 days to 300 days (mean, 71 days). Results: ERCP was successful in 47 with biliary atresia, in 9 with infantile hepatitis, and 10 with another disease. Liver biopsy was performed in I infant with hepatitis in whom the cannulation failed; in 9 with hepatitis in whom the cannulation was successful, exploratory laparotomy could be avoided. The ERCP findings in 46 patients with biliary atresia (excluding 1 in whom evaluation could not be performed because of poor x-ray quality) were classified into four patterns. Conclusions: A success rate of ERCP examinations in infants was 88%, so ERCP is recommended to make a correct decision regarding the need for surgery in cholestatic disorders. (Gastrointest Endosc 1997;45:365-70.)

Biliary a t r e s i a a n d infantile hepatitis are two imp o r t a n t causes of h y p e r b i l i r u b i n e m i a in the infantile period. T h e distinction b e t w e e n these two disorders m a y be difficult, b u t it is i m p o r t a n t because of the diff e r e n t t h e r a p e u t i c options a n d prognoses. T h e detection of the p a t e n c y of the biliary duct is the p r i m a r y goal of diagnostic investigations in infants w i t h cholestasis. E R C P is the most useful procedure for visualization of the e x t r a h e p a t i c biliary passage, b u t it h a s r a r e l y been r e p o r t e d in infants because of the unavailability of sufficient]y small i n s t r u m e n t s . The recent d e v e l o p m e n t of a prototype pediatric duodenoscope has p e r m i t t e d E R C P e x a m i n a t i o n s in infants.l-5 The p r e s e n t s t u d y was u n d e r t a k e n (1) to assess the ro]e of E R C P in the diagnosis of biliary a t r e s i a in inReceived January 24, 1996. For revision April 12, 1996. Accepted November 21, 1996. From the Department of Pediatric Surgery, Chiba University, School of Medicine, Chiba, Japan. Reprint requests: Naomi Ohnuma, MD, Pediatric Surgery, Chiba University, School of Medicine 1-8-1, Inohana, Chuo-ku, Chiba-city, Chiba, 260, Japan. 37/1/79515

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fants a n d (2) to r e p o r t the E R C P findings of the long c o m m o n channel in the pancreaticobiliary duct seen in some types of biliary atresia.

MATERIALS AND METHODS Patients ERCP examination was performed a total of 75 times in 73 infant patients at the Pediatric Surgical Department of Chiba University, School of Medicine, between March 1977 and March 1995. The infants included 30 boys and 43 girls ranging in age from 8 days to 300 days (mean, 71 days); 61 infants were less than 3 months ofage. The 73 patients consisted of 52 with biliary atresia, 10 with infantile hepatitis, 5 with congenital biliary dilatation, 3 with paucity of intrahepatic bile duct, 2 with duodenal atresia, and 1 with postoperative jaundice of hepatoblastoma. The main purpose of the ERCP examination in these infants was the differential diagnosis between biliary atresia and infantile hepatitis. In patients with infantile hepatitis in whom the results of several diagnostic tests (including laboratory tests, ultrasound, and hepatobiliary scan) were equivocal, the biliary tree was opacified with ERCP and biliary atresia excluded. Thus the patient was diagnosed not to have surgical disorders and was indicated to be treated medically for infantile hepatitis. GASTROINTESTINAL ENDOSCOPY 365

All patients with biliary atresia diagnosed by ERCP underwent laparotomy with operative cholangiogram and histologic evaluation of the biliary duct to decide Kasai's classification of bi]iary atresia. The instrument used was the Machida FGS-PEII (Machida Endoscope Co. Ltd., Tokyo, Japan) with the diameter of 8.0 mm (before 1979) and the Olympus PJF (Olympus Corp., Tokyo, Japan) with the diameter of 8.8 mm (since 1980), both of which are small enough to be used in infants without hazard. Since 1990 the Olympus XPJF 7.5E has been used. These small endoscopes are fragile and break easily.

ERCP procedure Informed consent for ERCP was obtained from parents in all cases. All ERCP examinations for the infantile cases were performed with the patient under general anesthesia with endotracheal intubation to prevent airway obstruction and dyspnea due to abdominal distension during the procedure. ERCP examinations were performed on a fluoroscopic table. The patient was placed in ]eft lateral position. The endoscope was inserted and advanced to the duodenum by the method identical to that used for an adult. After the recognition of the papilla of Vater, the scope was moved to a position such that the papilla appeared in the center of the visum field. Then the cannulation was attempted. An Olympus tapered Teflon catheter (PR-10Q) was used in cannulation and no antispasmodic agent was used to assist in cannulation. If the cannula was inserted into the orifice of papilla, a small amount of 60% Urografin contrast medium was injected slowly under fluoroscopy to obtain the cholangiopancreatographic images. The most important point in cannulation was to avoid insertion of the cannula too far into the orifice of the papilla. The optimum position is one in which a small amount of the contrast medium flows out around the cannula. We usually call it "push technique," wherein a shallow common channel injection fills both the pancreatic duct and the biliary duct. High injection pressure is an important part of this technique, so slow administra-

Hepatic Pancreatic Duct

Common Bile Duct I

I

Figure 1. In a 66-day-old girl diagnosed with infantile hepatitis, the common bile duct, the gallbladder, the intrahepatic bile duct and the pancreatic duct are visualized with ERCP.

ERCP No Bile Duct Distal End of Entire Length of CommonBile Duct »ancreaticDuctOnly CornrnonBile Duct CornmonBile Duct Gallbladder PancreaticDuct PancreaticDuct PancreaticDuct Number of Patients 35 1 2 8 Findings

1Trb 2 8 ~ KASAI's Ctassification

]]Ic

1~

]]Id

1 ~lll~r

I¢yst

ffr a

]]I a

Icyst 5 ~ ,

Figure 2. ERCP findings of biliary atresia. 366 G A S T R O I N T E S T I N A L E N D O S C O P Y

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l~

~~ reatic Duct

J~ co~~o~~,,~ouc~~~ Distal End of

(

/

ancreatic Duct

)~~1~

Figure 3. In a 40-day-old boy with biliary atresia, the bile duct

is not visualized with ERCP. Surgery confirmed this to be a type IIIb according to Kasai's classification.

tion of Urografin is performed by using a 2 or 3 ml syringe. ERCP examinations were performed by several endoscopists skilled at ERCP in adults. The time for total examination (from introduction ofduodenoscope to withdrawal of the instrument) varied from 30 minutes to 1 hour. After the examination, oral intake was withheld for 24 hours. Blood and urine amylase levels were monitored. RESULTS

The papilla of Vater was identified and cannu]ated in 66 of 75 (88%) ERCP examinations. In 5 examinations of biliary atresia, 2 of infantile hepatitis, 1 of congenital biliary dilatation, and 1 of duodenal atresia, the papilla could not be cannulated because of technical problems with the endoscope and catheter. In 9 of 10 infants with suspected infantile hepatitis in whom the results of several diagnostic tests were equivocal, ERCP examinations revealed biliary ductal system opacification (Fig. 1). Liver biopsy was performed in I infant in whom the cannulation failed, but in 9 in whom the cannulation was successful exploratory laparotomy was avoided. During the follow-up period, cholestasis diminished spontaneously in all 10 infants with infantile hepatitis. In 47 of the 52 infants with biliary atresia, the procedure was completed successfully. The ERCP findV O L U M E 45, NO. 5, 1997

Common Channel Figure 4. In a 71-day-old girl with biliary atresia, the short and dilated distal end of the common bile duct with the pancreatic duct are visualized with ERCP. Surgical exploration confirmed this to be a type I cyst according to Kasai's classification.

ings in 46 of them (excluding one whose ERCP findings could not be evaluated because of poor x-ray image quality) were classified into four patterns according to various parameters such as the filling with contrast medium of portions of the biliary duct and the pancreatic duct and the diameter of the common bile duct (Fig. 2). Pattern 1, in which only the pancreatic duct alone or the pancreatic duct and a choledochal cyst could be demonstrated and no other bile duct was visualized (Fig. 3), was seen in 35 patients with biliary atresia. Five had type I cysts, 28 had IIIb, 1 had IIIc, and 1 had IIId according to Kasai's classification. Pattern 2, in which only the short and dilated end of the common bile duct with the pancreatic duct was visualized, was seen in one patient with biliary atresia with type I cyst according to Kasai's classification (Fig. 4). Pattern 3, in which the entire length of the common bile duct with the pancreatic duct was visualized (without the gallbladder and the common hepatic GASTROINTESTINAL ENDOSCOPY

3{}7

Figure 6. This 34-day-old boy was diagnosed with biliary atresia. The gallbladder, the common bile duct, and the pancreatic duct are visualized with ERCP. Surgical exploration confirmed this to be a type lila according to Kasai's classification.

Figure 5. In an 83-day-old boy with biliary atresia, the entire length of the common bile duct with the pancreatic duct are visualized with ERCP. Surgery confirmed a type lila according to Kasai's classification.

duct), was noted in 2 patients with biliary atresia. These are IIIa according to Kasai's classification (Fig. 5). Pattern 4, in which the common bile duct and the gallbladder with the pancreatic duct were visua]ized (without the common hepatic duct) was observed in 8 patients with biliary atresia. These are IIIa according to Kasai's classification (Fig. 6). The long common channel of the pancreaticobiliary duct was observed in 11 patients with biliary atresia in whom the common bile duct was visualized with ERCP (one with type I cyst and 10 with type IIIa according to Kasai's classification). We identified the 368 G A S T R O I N T E S T I N A L ENDOSCOPY

common channel in 11 patients with biliary atresia and measured the length. The common channel length ranged from 2 mm to 16 mm; it was shorter than that in congenital biliary dilatation but longer than that in infantile hepatitis (Tab]e 1). During the study period, we monitored the urinary and serum amylase levels and observed carefully for signs of infection, pain, and vomiting. There were no apparent complications caused by ERCP or anesthesia. DISCUSSION Infantile hepatitis and biliary atresia are two important causes of hyperbilirubinemia in infants. The distinction between these two disorders may be difficult but is important because the prognosis and management of these clinical entities differ significant]y. It is well known that the Kasai procedure (portoenterostomy) is a life-saving operation in the patient with biliary atresia, particularly if performed before the age of 60 days. 6-9 However, no single test or combination of tests is consistently reliable in differentiating intrahepatic from extrahepatic forms of cholestasis, and in some VOLUME 45, NO. 5, 1997

ERCP Findings Number of Patients

35

1

2

8

(Ohnuma's series) Number of Patients

7

7

6

(Guelrud's series)

Figure 7. Schematic representation of the radiologic findings at ERCP in biliary atresia that compares our series with the series of Guelrud et al.4 patients explorative laparotomy is performed to establish a final diagnosis. Several procedures have been used to differentiate these entities. Imaging techniques such as ultrasonography and hepatobiliary scan are useful procedures, but are often inconclusive. The presence or absence of the gallbladder on ultrasound is an ambiguous finding in biliary atresia. 1o The discriminating value of hepatobiliary scan is good, but nonexcretion may also be caused by severe cholestasis due to intrahepatic disease. Hepatobiliary scan combined with analysis of bilirubin and bile acids in duodenal aspirates is helpful but still shows considerable overlap between infantile hepatitis and biliary atresia, u Histologic evaluation of a percutaneous liver biopsy specimen is still a very important procedure in the diagnosis ofinfantile cholestasis. Although there are no specific criteria for biliary atresia, an overall diagnostic accuracy of 86% can be achieved in differentiating bile duct obstruction from intrahepatic disease. 12 At present, an accurate preoperative diagnosis can be established in most patients when a combination of several diagnostic tests is used. These extensive programs take time and are expensive. With the use of ERCP, it is possible to visualize the biliary tree so that a correct decision regarding the need for surgery can be made. ERCP is still an uncommon procedure in children. This is due to a number of factors, which include a lack of pediatric gastroenterologists skilled in this technique and, until recently, unavailability of sufficiently small endoscopes. ERCP is a useful procedure for visualization of the extrahepatic biliary passage. In the present study, the biliary tree was visualized successfully with ERCP in 90% of the infants with infantile hepatitis, preventing unnecessary surgery. ERCP was thought to be useful in 47 of the 52 infants with biliary atresia in our study in spite of the VOLUME 45, NO. 5, 1997

Table 1. Common channel length in ERCP examination Patient Age (d) no.

Diagnosis

1 2 3 4

60 70 111 143

Congenital Congenital Congenital Congenital

1 2 3 4 5 6 7 8 9 10 11

83 71 43 51 98 55 138 54 34 45 27

Biliary Bfliary Bi]iary Bfliary Biliary Biliary Biliary Biliary Biliary Biliary Biliary

1 2 3

52 73 45

biliary biliary biliary biliary

atresia atresia atresia atresia atresia atresia atresia atresia atresia atresia atresia

dilatation dilatation dilatation dilatation

IIIa I cyst IIIa IIIa IIIa III a IIIa IIIa IIIa III a IIIa

Neonatal hepatitis Neonatal hepatitis Neonatal hepatitis

Common channel length (mm) 22 20 10 10 16 9 7 6 5 5 5 5 4 4 2 3 2 2

remaining uncertainty as to the diagnosis when a cholangiogram cannot be obtained via the papilla. In 46 of them, one of the four radiologic patterns typical of biliary atresia was observed. Guelrud et al. 4 reported three radiologic types based on the ERCP findings in 20 cases ofbiliary atresia. There are slight differences between the radiologic findings consistent with biliary atresia in our study and those reported by Guelrud et al. 4 (Fig. 7) Although absence of visualization of the biliary tree (out pattern 1) and opacification of the distal common duct and gallbladder without visualization of the main hepatic duct (our pattern 4) were found in out study and the study by Guelrud et al. 4 (and Derkx et al.5), we did not observe a pattern ofopacification of the distal common duct, gallbladder, GASTROINTESTINAL ENDOSCOPY

369

and a segment of the main hepatic duct with biliary lakes at the porta hepatis like that observed by Gue]rud et al. 4 On the other hand, unlike Guelrud et al., 4 we did observe opacification of the slightly dilated distal end of the common bile duct (our pattern 2) and opacification of almost the entire length of the common bile duct without the gallbladder and common hepatic duct (our pattern 3). Some observational differences may relate to the techniques of high versus low pressure of injection of contrast materials. Although proposed hypotheses as to the cause of bfliary atresia include injury of the vascular supply to the bile ducts, abnormal bile acids, and viral infect i o n , 13-17 the cause remains unknown. Recently, Toyosaka et al. is reported the results ofhisto]ogic investigation of the area around the papilla of Vater of the duodenum, including the pancreaticobiliary ductal system, using serial section in eight autopsy cases of biliary atresia. They found a long common channel of the pancreaticobiliary ductal junction in all eight cases. Conversely, Kimura et al. 19reported the resu]ts of similar histologic investigations and found normal length of the common channel in neonates and young infants. We identified a shorter common channel in biliary atresia than that in congenital biliary dilatation in ERCP findings. The number of patients in our study is admitted]y small. These ERCP findings suggest that some cases of bi]iary atresia have etiological resemblance to congenital biliary dilatation as the histological results of the study by Toyosaka et al. also suggest. In summary, ERCP is a helpful diagnostic tool in the evaluation of cholestasis of infancy. The quality of endoscopes for this task is improving. Classifications of biliary atresia need to be merged and refined. REFERENCES 1. Gue]rud M, Jaen D, Torres P, Mujica C, Mendoza S, Rivero E, et al. Endoscopic cholangiopancreatography in the infant; evaluation of a new prototype pediatric duodenoscope. Gastrointest Endosc 1987;33:4-8. 2. Heyman MB; Shapiro HA, Thaler MM. Endoscopic retrograde

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