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The role of endoscopic retrograde cholangiopancreatography in infants with cholestasis
The Role of Endoscopic Retrograde Cholangiopancreatography in Infants With Cholestasis By Yasushi Iinuma, Rintaro Narisawa, Makoto Iwafuchi, Masanori Uchiyama, Masafumi Naito, Minoru Yagi, Satoshi Kanada, Masahiro Otaki, Satoru Yamazaki, Terasu Honma, Hirotaka Motoyama, and Yasuyuki Baba Niigata City, Japan
Background/Purpose: Endoscopic retrograde cholangiopancreatography (ERCP) was assessed in the diagnosis of cholestatic liver disease in infants. Methods: ERCP was performed in 50 infants who had prolonged cholestasis. Their ages ranged from 25 to 274 days (mean, 69 days), and their weight ranged from 2.6 to 6.7 kg (mean, 4.7 kg). Incomplete visualization of the biliary tree or visualization of only the pancreatic duct was followed by exploratory laparotomy. In those in whom the biliary tree was visualized completely, the caliber of the bile duct was compared with that of the pancreatic duct. Results: ERCP was completed in 43 patients (success rate, 86%) without complications. In the 7 patients in whom ERCP failed, 6 had biliary atresia (BA) diagnosed by exploratory laparotomy. The other patient had congenital biliary dilatation (CBD). In 29 of the 43 patients, the biliary tree was seen partially or only the pancreatic duct was visualized. These
D
ESPITE ADVANCES in diagnostic techniques, the differential diagnosis of cholestatic liver disease in infants is difficult. Demonstration of the patency of the bile duct is particularly important in the differentiation of biliary atresia (BA) from other cholestatic diseases. Endoscopic retrograde cholangiopancreatography (ERCP) is the most reliable and useful examination to establish the patency of the bile duct. However, there are few reports of ERCP in the evaluation of cholestasis in infants because of technical difficulties. In this study, the utility of ERCP in the diagnosis of cholestatic liver disease in infants was assessed. Characteristic findings were evaluated. MATERIALS AND METHODS
Patients Between April 1982 and December 1996, a total of 65 infants with prolonged cholestasis who were thought to have obstructive jaundice were admitted to our hospital. ERCP was performed in 50 of these patients. The remaining 15 patients underwent exploratory laparotomy. In 46 of the 50 patients, other diagnostic modalities including laboratory tests, hepatobiliary scintigraphy, and ultrasonography were not conclusive. The remaining 4 patients had a diagnosis of congenital biliary dilatation (CBD) by ultrasonography or computed tomography. In the patients with CBD, ERCP was performed to define the pattern of the anomalous pancreaticobiliary junction before biliary reconstruction. The patients included 23 boys and 27 girls, ranging in age from 25 to 274 days (mean, 69 days), ranging in weight from 2.6 to 6.7 kg (mean, Journal of Pediatric Surgery, Vol 35, No 4 (April), 2000: pp 545-549
patients had BA diagnosed by laparotomy. Complete visualization of the biliary tree was obtained in 14 patients. Of these, 9 had neonatal hepatitis (NH), 2 had a paucity of intrahepatic bile ducts (PIBLD), and 3 had CBD. In all of the patients with NH, cholestasis improved spontaneously. The 2 patients with PIBLD had biopsy-proven disease. The caliber of the bile duct was larger than that of the pancreatic duct in NH. This relationship was not observed in PIBLD.
Conclusions: ERCP is safe in infants. It is useful in the diagnosis of prolonged cholestasis. J Pediatr Surg 35:545-549. Copyright 娀 2000 by W.B. Saunders Company. INDEX WORDS: Endoscopic retrograde cholangiopancreatography, biliary atresia, congenital biliary dilatation, neonatal hepatitis, paucity of intrahepatic bile ducts.
4.7 kg). The patients who had incomplete visualization of the biliary tree or demonstration of only the pancreatic duct underwent exploratory laparotomy after ERCP. The differential diagnosis in the patients in whom the biliary tree was completely visualized was based on the comparison between the caliber of the bile duct and that of the pancreatic duct.
ERCP Procedure Informed consent was obtained from the parents before ERCP. All examinations were performed by one endoscopist. An Olympus PJF/ PJF 7.5 duodenoscope (Olympus Co, Tokyo, Japan) was used. All examinations were performed on a fluoroscopic table, under general anesthesia, in an operating room. The actual procedure was the same as that used in adults. The endoscope was inserted into the second portion of the duodenum with the patient in the left lateral decubitus position. The insertion was assisted by pressing on the area on the abdominal wall corresponding to the greater curvature of the stomach to straighten the fiberscope. The papilla of Vater was selectively cannulated with a tapered Teflon catheter. The pancreatic and biliary ducts were visualized with fluoroscopy during the injection of 60% Urograpffin (Japan Schering Co, Osaka, Japan) containing 0.25 g of Cefoperazone sodium (Toyama Chemical Co, Tokyo, Japan). The time for total examination
From the Department of Pediatric Surgery and the Third Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan. Address reprint requests to Yasushi Iinuma, MD, Department of Pediatric Surgery, Niigata University, 1-757 Asahimachi-dori, Niigata City, 951-8520, Japan. Copyright 娀 2000 by W.B. Saunders Company 0022-3468/00/3504-0002$03.00/0 545
546
IINUMA ET AL
from the introduction of the duodenoscope to its withdrawal varied from 0.5 to 1 hour. After ERCP, antibiotics and synthetic protease inhibitors were given, and the blood amylase levels were monitored. An oral diet was started 24 hours after examination.
RESULTS
ERCP was completed in 43 of 50 patients (success rate, 86%). In the remaining 7 patients, cannulation of the papilla failed. Of the 7 patients in whom ERCP failed, 6 had BA diagnosed by exploratory laparotomy. The other patient had CBD. Unsuccessful ERCP was caused by gastric volvulus. Of the 43 patients, 14 patients had complete visualization of the biliary tree (including 3 patients with CBD). In the remaining 29 patients, complete visualization of the biliary tree was not achieved, or only the pancreatic duct was visualized (Fig 1). All 29 patients in whom complete visualization of the biliary tree was not achieved had BA diagnosed by exploratory laparotomy. The most common ERCP finding in BA was visualization of only the pancreatic duct (24 of 29 patients). The common bile duct, gallbladder, and pancreatic duct were visualized in 4 patients (Fig 2). The short distal end of the common bile duct and the pancreatic duct were visualized in 1 patient (Fig 3). The caliber of the bile ducts in these 5 patients was smaller than that of the pancreatic ducts. In 9 of the 14 patients in whom the biliary tree was completely visualized, the caliber of the bile duct was larger than that of the pancreatic duct. These patients had neonatal hepatitis (NH). Cholestasis improved spontaneously during the follow-up period. Unnecessary laparotomy was avoided (Fig 4). In 2 of the 5 remaining patients, the caliber of the bile duct was smaller than that of the pancreatic duct. These patients had a paucity of
Fig 2. Imaging in a 68-day-old girl with a diagnosis of biliary atresia in whom the gallbladder, common bile duct, and pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). Laparotomy after ERCP confirmed the diagnosis.
intrahepatic bile ducts (PIBLD; Fig 5). These patients underwent liver biopsy. Histological examination confirmed the diagnosis. The other 3 patients, who had CBD, had a dilated common bile duct with an anomalous pancreaticobiliary junction (Fig 6). DISCUSSION
Fig 1. Results of endoscopic retrograde cholangiopancreatography (ERCP). ERCP examination was successful in 43 of 50 infants.
Cholestatic disease in infants remains a major diagnostic challenge, despite increasing knowledge regarding the pathogenesis. The differentiation of BA from other cholestatic diseases in infants is one of the most difficult problems for the pediatric gastroenterologist. In many infants with cholestasis, conventional liver function tests, hepatobiliary scintigraphy, ultrasonography, and liver biopsy are not conclusive. Therefore, confirmation of the patency of the bile duct is critical to differentiate BA from other forms of cholestasis. Recently, magnetic resonance cholangiopancreatography (MRCP) has been used to
ERCP IN INFANTS WITH CHOLESTASIS
547
admission. Recently, we have begun to omit ERCP in patients over 60 days old whom we suspect may have BA. In the diagnosis of BA, the echogenic density of the fibrous remnant in the porta hepatis, which is known as the triangular cord (TC), is a useful finding on ultrasonography.5 But the detection of a TC is not easy. Furthermore, lack of a TC does not exclude the possibility of BA. When the biliary tree is partially visualized or only the pancreatic duct is seen, BA should be suspected. Explor-
Fig 3. Imaging in a 80-day-old girl with a diagnosis of biliary atresia in whom the short distal end of common bile duct and the pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). Laparotomy after ERCP confirmed the diagnosis.
visualize pancreaticobiliary ducts. In small infants, however, the usefulness of MRCP has not been established.1 ERCP is the most useful procedure in these patients, but reported use of the procedure in cholestatic infants has been limited because of technical difficulties. The success rate of ERCP in children under 1 year of age (27% to 95%) is lower than that in adults.2-4 Our success rate of 86% in infants was satisfactory. The principles of cannulation of the papilla are the same as those in the adult, except that infants have limited duodenal space. Therefore, it is important to assist the insertion of the endoscope by pressing on the greater curvature of the stomach from the abdominal wall. This stretches the fiberscope and brings the papilla directly in view. Also, one must avoid insertion of the cannula too deeply into the papilla (not to exceed 2 mm). We perform ERCP in all infants with cholestasis immediately after
Fig 4. Imaging in a 78-day-old boy with a diagnosis of neonatal hepatitis in whom the gallbladder, intrahepatic duct, and the pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). The caliber of the bile duct is larger than that of the pancreatic duct. Jaundice improved spontaneously. Unnecessary laparotomy was avoided.
548
IINUMA ET AL
We believe that the relationship between the caliber of the common bile duct and that of the pancreatic duct is more important in distinguishing NH from PIBLD. Using this information, unnecessary laparotomy can be avoided in patients with NH. ERCP was useful in defining the pattern of the anomalous pancreaticobiliary junction before surgery in the patients with CBD. This anatomic information can help avoid pancreatic duct injury during surgery. ERCP is a safe and useful diagnostic tool for the differential diagnosis of cholestatic diseases in infants. In the diagnosis of NH, unnecessary laparotomy can be avoided. The caliber of the bile duct relative to that of pancreatic duct is important for distinguishing PIBLD from NH. When the biliary tree is seen partially or only the pancreatic duct is seen, BA should be suspected. Exploratory laparotomy is indicated in these patients. In patients with CBD, ERCP helps define the pattern of the anomalous pancreaticobiliary junction before surgery.
Fig 5. Imaging in a 80-day-old boy with a diagnosis of paucity of intrahepatic bile ducts in whom the gallbladder, intrahepatic ducts, and pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). The caliber of the bile duct is smaller than that of the pancreatic duct. Open liver biopsy confirmed the diagnosis.
atory laparotomy is indicated in these patients. Guelrud et al6 and Ohnuma et al3 have reported the ERCP findings of BA. Our ERCP findings resembled those of Ohnuma et al rather than those of Guelrud et al. Such observational differences might be related to different injection pressures of contrast medium into the pancreaticobiliary duct. In the infants with cholestasis from diseases other than BA, when the biliary tree was completely visualized, simultaneous visualization of the pancreatic duct was obtained. In all patients with NH, the caliber of the common bile duct was larger than that of the pancreatic duct. In contrast, the caliber of the common bile duct was smaller than that of the pancreatic duct in the patients with PIBLD. This relationship was also observed in the 5 patients with BA in whom the biliary tree was partially visualized. Reports of cholangiographic findings in NH and PIBLD are rare. The ERCP findings of PIBLD have been reported to include marked and diffuse narrowing of the intrahepatic bile ducts, a reduced number of intrahepatic branches, and a normal, thin, straight extrahepatic duct.2,7
Fig 6. Imaging in a 80-day-old girl with a diagnosis of congenital biliary dilatation by ultrasonography and computed tomography in whom the dilated common bile duct, anomalous pancreaticobiliary junction, and pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP).
ERCP IN INFANTS WITH CHOLESTASIS
549
REFERENCES 1. Yamataka A, Kuwatsuru R, Shima H, et al: Initial experience with non-breath-hold magnetic resonance cholangiopancreatography: A new non-invasive technique for the diagnosis of choledochal cyst in children. J Pediatr Surg 32:1560-1562, 1997 2. Guelrud M, Carr-Locke DL, Fox VL: ERCP in Pediatric practice: Diagnosis and Treatment. Oxford, Isis Medical Media, 1997, pp 1-50 3. Ohnuma N, Takahashi H, Tanabe M, et al: The role of ERCP in biliary atresia. Gastrointest Endosc 45:365-375, 1997 4. Shirai Z, Toriya H, Maeshiro K, et al: The usefulness of endoscopic retrograde cholangiopancreatography in infants and small children. Am J Gastroenterol 88:536-541, 1993
5. Park WH, Choi SO, Lee HJ, et al: A new diagnostic approach to biliary atresia with emphasis on ultrasonographic triangular cord sign: Comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg 32:1555-1559, 1997 6. Guelrud M, Jaen D, Mendoza S, et al: ERCP in the diagnosis of extrahepatic biliary atresia. Gastrointest Endosc 37:522-526, 1991 7. Torres WH, Lind CD, et al: Extra-hepatic biliary hypoplasia associated with alpha1-antitripsin deficiency. Gastrointest Endosc 40: 360-361, 1994
Background/Purpose: Endoscopic retrograde cholangiopancreatography (ERCP) was assessed in the diagnosis of cholestatic liver disease in infants. Methods: ERCP was performed in 50 infants who had prolonged cholestasis. Their ages ranged from 25 to 274 days (mean, 69 days), and their weight ranged from 2.6 to 6.7 kg (mean, 4.7 kg). Incomplete visualization of the biliary tree or visualization of only the pancreatic duct was followed by exploratory laparotomy. In those in whom the biliary tree was visualized completely, the caliber of the bile duct was compared with that of the pancreatic duct. Results: ERCP was completed in 43 patients (success rate, 86%) without complications. In the 7 patients in whom ERCP failed, 6 had biliary atresia (BA) diagnosed by exploratory laparotomy. The other patient had congenital biliary dilatation (CBD). In 29 of the 43 patients, the biliary tree was seen partially or only the pancreatic duct was visualized. These
D
ESPITE ADVANCES in diagnostic techniques, the differential diagnosis of cholestatic liver disease in infants is difficult. Demonstration of the patency of the bile duct is particularly important in the differentiation of biliary atresia (BA) from other cholestatic diseases. Endoscopic retrograde cholangiopancreatography (ERCP) is the most reliable and useful examination to establish the patency of the bile duct. However, there are few reports of ERCP in the evaluation of cholestasis in infants because of technical difficulties. In this study, the utility of ERCP in the diagnosis of cholestatic liver disease in infants was assessed. Characteristic findings were evaluated. MATERIALS AND METHODS
Patients Between April 1982 and December 1996, a total of 65 infants with prolonged cholestasis who were thought to have obstructive jaundice were admitted to our hospital. ERCP was performed in 50 of these patients. The remaining 15 patients underwent exploratory laparotomy. In 46 of the 50 patients, other diagnostic modalities including laboratory tests, hepatobiliary scintigraphy, and ultrasonography were not conclusive. The remaining 4 patients had a diagnosis of congenital biliary dilatation (CBD) by ultrasonography or computed tomography. In the patients with CBD, ERCP was performed to define the pattern of the anomalous pancreaticobiliary junction before biliary reconstruction. The patients included 23 boys and 27 girls, ranging in age from 25 to 274 days (mean, 69 days), ranging in weight from 2.6 to 6.7 kg (mean, Journal of Pediatric Surgery, Vol 35, No 4 (April), 2000: pp 545-549
patients had BA diagnosed by laparotomy. Complete visualization of the biliary tree was obtained in 14 patients. Of these, 9 had neonatal hepatitis (NH), 2 had a paucity of intrahepatic bile ducts (PIBLD), and 3 had CBD. In all of the patients with NH, cholestasis improved spontaneously. The 2 patients with PIBLD had biopsy-proven disease. The caliber of the bile duct was larger than that of the pancreatic duct in NH. This relationship was not observed in PIBLD.
Conclusions: ERCP is safe in infants. It is useful in the diagnosis of prolonged cholestasis. J Pediatr Surg 35:545-549. Copyright 娀 2000 by W.B. Saunders Company. INDEX WORDS: Endoscopic retrograde cholangiopancreatography, biliary atresia, congenital biliary dilatation, neonatal hepatitis, paucity of intrahepatic bile ducts.
4.7 kg). The patients who had incomplete visualization of the biliary tree or demonstration of only the pancreatic duct underwent exploratory laparotomy after ERCP. The differential diagnosis in the patients in whom the biliary tree was completely visualized was based on the comparison between the caliber of the bile duct and that of the pancreatic duct.
ERCP Procedure Informed consent was obtained from the parents before ERCP. All examinations were performed by one endoscopist. An Olympus PJF/ PJF 7.5 duodenoscope (Olympus Co, Tokyo, Japan) was used. All examinations were performed on a fluoroscopic table, under general anesthesia, in an operating room. The actual procedure was the same as that used in adults. The endoscope was inserted into the second portion of the duodenum with the patient in the left lateral decubitus position. The insertion was assisted by pressing on the area on the abdominal wall corresponding to the greater curvature of the stomach to straighten the fiberscope. The papilla of Vater was selectively cannulated with a tapered Teflon catheter. The pancreatic and biliary ducts were visualized with fluoroscopy during the injection of 60% Urograpffin (Japan Schering Co, Osaka, Japan) containing 0.25 g of Cefoperazone sodium (Toyama Chemical Co, Tokyo, Japan). The time for total examination
From the Department of Pediatric Surgery and the Third Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan. Address reprint requests to Yasushi Iinuma, MD, Department of Pediatric Surgery, Niigata University, 1-757 Asahimachi-dori, Niigata City, 951-8520, Japan. Copyright 娀 2000 by W.B. Saunders Company 0022-3468/00/3504-0002$03.00/0 545
546
IINUMA ET AL
from the introduction of the duodenoscope to its withdrawal varied from 0.5 to 1 hour. After ERCP, antibiotics and synthetic protease inhibitors were given, and the blood amylase levels were monitored. An oral diet was started 24 hours after examination.
RESULTS
ERCP was completed in 43 of 50 patients (success rate, 86%). In the remaining 7 patients, cannulation of the papilla failed. Of the 7 patients in whom ERCP failed, 6 had BA diagnosed by exploratory laparotomy. The other patient had CBD. Unsuccessful ERCP was caused by gastric volvulus. Of the 43 patients, 14 patients had complete visualization of the biliary tree (including 3 patients with CBD). In the remaining 29 patients, complete visualization of the biliary tree was not achieved, or only the pancreatic duct was visualized (Fig 1). All 29 patients in whom complete visualization of the biliary tree was not achieved had BA diagnosed by exploratory laparotomy. The most common ERCP finding in BA was visualization of only the pancreatic duct (24 of 29 patients). The common bile duct, gallbladder, and pancreatic duct were visualized in 4 patients (Fig 2). The short distal end of the common bile duct and the pancreatic duct were visualized in 1 patient (Fig 3). The caliber of the bile ducts in these 5 patients was smaller than that of the pancreatic ducts. In 9 of the 14 patients in whom the biliary tree was completely visualized, the caliber of the bile duct was larger than that of the pancreatic duct. These patients had neonatal hepatitis (NH). Cholestasis improved spontaneously during the follow-up period. Unnecessary laparotomy was avoided (Fig 4). In 2 of the 5 remaining patients, the caliber of the bile duct was smaller than that of the pancreatic duct. These patients had a paucity of
Fig 2. Imaging in a 68-day-old girl with a diagnosis of biliary atresia in whom the gallbladder, common bile duct, and pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). Laparotomy after ERCP confirmed the diagnosis.
intrahepatic bile ducts (PIBLD; Fig 5). These patients underwent liver biopsy. Histological examination confirmed the diagnosis. The other 3 patients, who had CBD, had a dilated common bile duct with an anomalous pancreaticobiliary junction (Fig 6). DISCUSSION
Fig 1. Results of endoscopic retrograde cholangiopancreatography (ERCP). ERCP examination was successful in 43 of 50 infants.
Cholestatic disease in infants remains a major diagnostic challenge, despite increasing knowledge regarding the pathogenesis. The differentiation of BA from other cholestatic diseases in infants is one of the most difficult problems for the pediatric gastroenterologist. In many infants with cholestasis, conventional liver function tests, hepatobiliary scintigraphy, ultrasonography, and liver biopsy are not conclusive. Therefore, confirmation of the patency of the bile duct is critical to differentiate BA from other forms of cholestasis. Recently, magnetic resonance cholangiopancreatography (MRCP) has been used to
ERCP IN INFANTS WITH CHOLESTASIS
547
admission. Recently, we have begun to omit ERCP in patients over 60 days old whom we suspect may have BA. In the diagnosis of BA, the echogenic density of the fibrous remnant in the porta hepatis, which is known as the triangular cord (TC), is a useful finding on ultrasonography.5 But the detection of a TC is not easy. Furthermore, lack of a TC does not exclude the possibility of BA. When the biliary tree is partially visualized or only the pancreatic duct is seen, BA should be suspected. Explor-
Fig 3. Imaging in a 80-day-old girl with a diagnosis of biliary atresia in whom the short distal end of common bile duct and the pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). Laparotomy after ERCP confirmed the diagnosis.
visualize pancreaticobiliary ducts. In small infants, however, the usefulness of MRCP has not been established.1 ERCP is the most useful procedure in these patients, but reported use of the procedure in cholestatic infants has been limited because of technical difficulties. The success rate of ERCP in children under 1 year of age (27% to 95%) is lower than that in adults.2-4 Our success rate of 86% in infants was satisfactory. The principles of cannulation of the papilla are the same as those in the adult, except that infants have limited duodenal space. Therefore, it is important to assist the insertion of the endoscope by pressing on the greater curvature of the stomach from the abdominal wall. This stretches the fiberscope and brings the papilla directly in view. Also, one must avoid insertion of the cannula too deeply into the papilla (not to exceed 2 mm). We perform ERCP in all infants with cholestasis immediately after
Fig 4. Imaging in a 78-day-old boy with a diagnosis of neonatal hepatitis in whom the gallbladder, intrahepatic duct, and the pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). The caliber of the bile duct is larger than that of the pancreatic duct. Jaundice improved spontaneously. Unnecessary laparotomy was avoided.
548
IINUMA ET AL
We believe that the relationship between the caliber of the common bile duct and that of the pancreatic duct is more important in distinguishing NH from PIBLD. Using this information, unnecessary laparotomy can be avoided in patients with NH. ERCP was useful in defining the pattern of the anomalous pancreaticobiliary junction before surgery in the patients with CBD. This anatomic information can help avoid pancreatic duct injury during surgery. ERCP is a safe and useful diagnostic tool for the differential diagnosis of cholestatic diseases in infants. In the diagnosis of NH, unnecessary laparotomy can be avoided. The caliber of the bile duct relative to that of pancreatic duct is important for distinguishing PIBLD from NH. When the biliary tree is seen partially or only the pancreatic duct is seen, BA should be suspected. Exploratory laparotomy is indicated in these patients. In patients with CBD, ERCP helps define the pattern of the anomalous pancreaticobiliary junction before surgery.
Fig 5. Imaging in a 80-day-old boy with a diagnosis of paucity of intrahepatic bile ducts in whom the gallbladder, intrahepatic ducts, and pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP). The caliber of the bile duct is smaller than that of the pancreatic duct. Open liver biopsy confirmed the diagnosis.
atory laparotomy is indicated in these patients. Guelrud et al6 and Ohnuma et al3 have reported the ERCP findings of BA. Our ERCP findings resembled those of Ohnuma et al rather than those of Guelrud et al. Such observational differences might be related to different injection pressures of contrast medium into the pancreaticobiliary duct. In the infants with cholestasis from diseases other than BA, when the biliary tree was completely visualized, simultaneous visualization of the pancreatic duct was obtained. In all patients with NH, the caliber of the common bile duct was larger than that of the pancreatic duct. In contrast, the caliber of the common bile duct was smaller than that of the pancreatic duct in the patients with PIBLD. This relationship was also observed in the 5 patients with BA in whom the biliary tree was partially visualized. Reports of cholangiographic findings in NH and PIBLD are rare. The ERCP findings of PIBLD have been reported to include marked and diffuse narrowing of the intrahepatic bile ducts, a reduced number of intrahepatic branches, and a normal, thin, straight extrahepatic duct.2,7
Fig 6. Imaging in a 80-day-old girl with a diagnosis of congenital biliary dilatation by ultrasonography and computed tomography in whom the dilated common bile duct, anomalous pancreaticobiliary junction, and pancreatic duct are visualized with endoscopic retrograde cholangiopancreatography (ERCP).
ERCP IN INFANTS WITH CHOLESTASIS
549
REFERENCES 1. Yamataka A, Kuwatsuru R, Shima H, et al: Initial experience with non-breath-hold magnetic resonance cholangiopancreatography: A new non-invasive technique for the diagnosis of choledochal cyst in children. J Pediatr Surg 32:1560-1562, 1997 2. Guelrud M, Carr-Locke DL, Fox VL: ERCP in Pediatric practice: Diagnosis and Treatment. Oxford, Isis Medical Media, 1997, pp 1-50 3. Ohnuma N, Takahashi H, Tanabe M, et al: The role of ERCP in biliary atresia. Gastrointest Endosc 45:365-375, 1997 4. Shirai Z, Toriya H, Maeshiro K, et al: The usefulness of endoscopic retrograde cholangiopancreatography in infants and small children. Am J Gastroenterol 88:536-541, 1993
5. Park WH, Choi SO, Lee HJ, et al: A new diagnostic approach to biliary atresia with emphasis on ultrasonographic triangular cord sign: Comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg 32:1555-1559, 1997 6. Guelrud M, Jaen D, Mendoza S, et al: ERCP in the diagnosis of extrahepatic biliary atresia. Gastrointest Endosc 37:522-526, 1991 7. Torres WH, Lind CD, et al: Extra-hepatic biliary hypoplasia associated with alpha1-antitripsin deficiency. Gastrointest Endosc 40: 360-361, 1994