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Biliary stenting in an infant with malignant obstructive jaundice
Biliary stenting in an infant with malignant obstructive jaundice Moises Guelrud, Sonia Mendoza, Anton Zager, Carlos Noguera,
MD MD MD MD
ERCP permits precise identification of pathological processes within the biliary tract and is commonly used diagnostically in adults. 1- 3 Recently, it has been used for therapeutic indications such as retained duct stones.4.5 In 1979, Soehendra and Reynders-Frederix 6 described endoscopic biliary stenting in which a plastic tube is positioned in the biliary tree to bridge a stricture, thereby restoring bile drainage into the duodenum. The following report about an infant represents an unusual case of extrahepatic biliary obstruction caused by neuroblastoma and illustrates the usefulness of ERCP in the diagnosis and treatment of this problem. CASE REPORT
After 3 days the total bilirubin was 5.2 mg/liter and over the next 2 weeks, cyclophosphamide (150 mg/m 2 ) and doxorubicin (adriamycin, 35 mg/m2 ) were administered. On day 13 of chemotherapy, the total bilirubin was 1.1 mg/liter. The combination chemotherapy was repeated every 28 days for 3 months. A CAT scan after 4 months showed a 70% reduction in size of the mass and no evidence for biliary tract obstruction. ERCP examination using a prototype pediatric duodenoscope Olympus JFP confirmed disappearance of the common hepatic compression (Fig. 3) and the biliary stent was removed. After a 3-month follow-up, the child is asymptomatic, anicteric, and has normal liver chemistries.
DISCUSSION
Neuroblastoma generally is a large tumor, often greater than 10 cm in diameter. Approximately 80% of cases are diagnosed before 5 years of age, and 30% manifest clinically in children less than 1 year of age. The adrenal medulla is the commonest site of the neoplasm, but it may develop at any location where there are autonomic ganglion cells. Extrahepatic bili-
A 21-month-old male was admitted to the hospital presenting with 1 week of jaundice and an abdominal mass. At 24 days of age an adrenal neuroblastoma had been resected. It was well circumscribed and thought to be stage 1. No further treatment was given. The child remained asymptomatic with normal growth until 1 week prior to admission when his mother noted dark urine and scleral icterus. An enlarged, tender liver and a hard nodular mass 3 cm in diameter was palpated in the right upper quadrant of the abdomen. Laboratory tests revealed total bilirubin, 29.6 mg/ liter; direct bilirubin, 25.1 mg/liter; aspartate aminotransferase, 115 milliunits/ml; alanine amirotransferase, 280 milliunits/ml; alkaline phosphatase, 818 mu/ml; and prothrombin time, 12/12 sec (patient/control). An abdominal CAT scan revealed hepatomegaly and dilated intrahepatic ducts, suggesting an obstruction of the common bile duct by a right paravertebral retroperitoneal mass of 8 by 6 cm that was also compressing the inferior vena cava and the portal vein. Prior to chemotherapy, an attempt was made to relieve the hepatic obstruction. Under general anesthesia, ERCP was performed using the Olympus JF-1T duodenoscope. The common bile duct was normal caliber and the gallbladder filled. At the common hepatic duct, there was a narrowed stricture with dilation of both hepatic ducts and intrahepatic branches (Fig. 1). A small endoscopic papillotomy was done and a guide wire was inserted into the left hepatic duct. The ERCP catheter was withdrawn and a 7 French double pigtail stent was introduced under fluoroscopic control with a pusher catheter. The guide wire was removed while holding the stent in place with the pusher (Fig. 2). The whole procedure lasted 25 min, was well tolerated, and the patient was able to eat immediately after recovery from the anesthesia. From the Departments of Gastroenterology, Pediatric Surgery, and Oncology, Policlinica Metropalitana, Caracas, Venezuela. Reprint requests: Moises GuelrOO, MD, Policlinica Metropolitana, Urb. Caurimare, Caracas, Venezuela. VOLUME 35, NO.3, 1989
Figure 1. Endoscopic retrograde cholangiogram showing a stricture at the common hepatic duct (arrow). There is dilation of the hepatic duct and intrahepatic biliary ducts. The common bile duct below the cystic duct and the gallbladder is normal.
259
Figure 2. A, A guide wire is introduced into the left hepatic duct. B, A 7 French double pigtail biliary stent is placed in the left
hepatic duct bridging the obstruction.
Figure 3. A, With the stent in place, an ERCP catheter is introduced into the common duct and a cholangiogram shows dissapearance of the stricture at the common hepatic duct. B, After removing the biliary stent, the common hepatic duct is slightly dilated without stricture.
ary obstruction has not been described. 7 Metastases occur primarily to abdominal lymph nodes, liver, lungs, bone marrow, and skin. The prognosis is related to the age of the patient at diagnosis, the site of the primary tumor, and the degree of tumor differentiation. The prognosis for children who are less than 1 260
year of age is better than for older children with comparable tumors. Extraabdominal tumors and those containing differentiated, mature ganglion cells8 carry a better prognosis. ERCP, first performed in children in 19769 , has been successful in infants weighing as little as 2.5 kg. 10 GASTROINTESTINAL ENDOSCOPY
The pediatric indications for ERCP have included evaluation of neonatal cholestasis,lO relapsing pancreatitis' II and the diagnosis of extrahepatic biliary obstruction. 12 Significant complications, including cholangitis, bacteremia, and pancreatitis, have been rare in children. Minor acute duodenal erosions were observed in 7 of 23 neonates who experienced no apparent clinical consequences. 10 In adults, endoscopic biliary stenting has been used for palliation of unresectable biliopancreatic tumors, preoperative drainage of tumors with potentially resectable lesions, and as a temporary biliary decompression in patients with large common duct stones or benign stricture.l.13-15 There are no reports of stent placement in infancy. In this child, biliary drainage prior to chemotherapy was important because adriamycin is excreted in bile and substantial morbidity is associated with severe jaundice. 16 ERCP, endoscopic papillotomy, and biliary stenting should be performed in children only by experienced endoscopists who have a high success rate in placing biliary stents. Although indications are rare in children, the technical capability now exists to consider this form of therapy in selected cases. ACKNOWLEDGMENT
The authors thank Dr. Harland S. Winter, Children's Hospital, Harvard Medical School for reviewing the manuscript. REFERENCES 1. Takagi K, Ikeda S, Nakagawa Y, et al. Endoscopic sphincterotomy of the ampulla of Vater. Endoscopy 1970;2:107-15.
VOLUME 35, NO.3, 1989
2. Cotton PB, Beales JSM. The role of endoscopic retrograde cholangiopancreatography (ERCP) in patients with jaundice. Acta Gastroenterol Belg 1973;36:689-92. 3. Vennes JA, Jacobsen JR, Silvis SE. Endoscopic cholangiography for biliary system diagnosis. Ann Intern Med 1974;80:614.
4. Kawai K, Akasaka Y, Murakami K, et al. Endoscopic sphincterotomy of the ampulla of Vater. Gastrointest Endosc 1974;20:148-51. 5. Classen M, Safrany L. Endoscopic papillotomy and removal of gallstones. Br Med J 1975;4:371-4. 6. Soehendra N, Reynders-Frederix V. Palliative bile duct drainage. A new endoscopic method of introducing a transpapillary drain. Endoscopy 1980;12:8-11. 7. Kissane JM. Anderson's pathology. 8th ed. St. Louis: CV Mosby, 1985:1435. 8. Triche SJ, Askin FB, Kissane JM. Neuroblastoma, Ewing's sarcoma, and the differential diagnosis of small-round, bluecell tumors. In: Finegold M, ed. Pathology of neoplasia in children and adolescents. Philadelphia: WB Saunders, 1986:145 (Major problems in pathology; Vol 18). 9. Waye JD. Endoscopic retrograde cholangiopancreatography in the infant. Am J GastroenteroI1976;65:461-3. 10. Guelrud M, Jaen D, Torres P, et al. Endoscopic cholangiopancreatography in the infant: evaluation of a new prototype pediatric duodenoscope. Gastrointest Endosc 1987;33:4-8. 11. Alwniack A, Jonson G, Mattsson K. Chronic relapsing pancreatitis in a child. Endoscopic diagnosis. Acta Chir Scand 1977;143:153-5. 12. Urakami V, Seki H, Kishi S. Endoscopic retrograde cholangiopancreatography (ERCP) performed in children. Endoscopy 1977;9:86-91. 13. Wurbs D, Phillip J, Classen M. Experiences with long-standing nasobiliary tube in biliary disease. Endoscopy 1980;12:219-23. 14. Huibregtse K, Tytgat GN. Palliative treatment of obstructive jaundice by transpapillary introduction of a large bore bile duct endoprosthesis. Gut 1982;23:371-5. 15. Siegel JH, Yatto RP. Biliary endoprostheses for the management of retained common bile duct stones. Am J Gastroenterol 1984;79:50-4. 16. Bachur NR. Adriamycin (NSC-123127) pharmacology. Cancer Chemother Rep 1975;3:153-8.
261
MD MD MD MD
ERCP permits precise identification of pathological processes within the biliary tract and is commonly used diagnostically in adults. 1- 3 Recently, it has been used for therapeutic indications such as retained duct stones.4.5 In 1979, Soehendra and Reynders-Frederix 6 described endoscopic biliary stenting in which a plastic tube is positioned in the biliary tree to bridge a stricture, thereby restoring bile drainage into the duodenum. The following report about an infant represents an unusual case of extrahepatic biliary obstruction caused by neuroblastoma and illustrates the usefulness of ERCP in the diagnosis and treatment of this problem. CASE REPORT
After 3 days the total bilirubin was 5.2 mg/liter and over the next 2 weeks, cyclophosphamide (150 mg/m 2 ) and doxorubicin (adriamycin, 35 mg/m2 ) were administered. On day 13 of chemotherapy, the total bilirubin was 1.1 mg/liter. The combination chemotherapy was repeated every 28 days for 3 months. A CAT scan after 4 months showed a 70% reduction in size of the mass and no evidence for biliary tract obstruction. ERCP examination using a prototype pediatric duodenoscope Olympus JFP confirmed disappearance of the common hepatic compression (Fig. 3) and the biliary stent was removed. After a 3-month follow-up, the child is asymptomatic, anicteric, and has normal liver chemistries.
DISCUSSION
Neuroblastoma generally is a large tumor, often greater than 10 cm in diameter. Approximately 80% of cases are diagnosed before 5 years of age, and 30% manifest clinically in children less than 1 year of age. The adrenal medulla is the commonest site of the neoplasm, but it may develop at any location where there are autonomic ganglion cells. Extrahepatic bili-
A 21-month-old male was admitted to the hospital presenting with 1 week of jaundice and an abdominal mass. At 24 days of age an adrenal neuroblastoma had been resected. It was well circumscribed and thought to be stage 1. No further treatment was given. The child remained asymptomatic with normal growth until 1 week prior to admission when his mother noted dark urine and scleral icterus. An enlarged, tender liver and a hard nodular mass 3 cm in diameter was palpated in the right upper quadrant of the abdomen. Laboratory tests revealed total bilirubin, 29.6 mg/ liter; direct bilirubin, 25.1 mg/liter; aspartate aminotransferase, 115 milliunits/ml; alanine amirotransferase, 280 milliunits/ml; alkaline phosphatase, 818 mu/ml; and prothrombin time, 12/12 sec (patient/control). An abdominal CAT scan revealed hepatomegaly and dilated intrahepatic ducts, suggesting an obstruction of the common bile duct by a right paravertebral retroperitoneal mass of 8 by 6 cm that was also compressing the inferior vena cava and the portal vein. Prior to chemotherapy, an attempt was made to relieve the hepatic obstruction. Under general anesthesia, ERCP was performed using the Olympus JF-1T duodenoscope. The common bile duct was normal caliber and the gallbladder filled. At the common hepatic duct, there was a narrowed stricture with dilation of both hepatic ducts and intrahepatic branches (Fig. 1). A small endoscopic papillotomy was done and a guide wire was inserted into the left hepatic duct. The ERCP catheter was withdrawn and a 7 French double pigtail stent was introduced under fluoroscopic control with a pusher catheter. The guide wire was removed while holding the stent in place with the pusher (Fig. 2). The whole procedure lasted 25 min, was well tolerated, and the patient was able to eat immediately after recovery from the anesthesia. From the Departments of Gastroenterology, Pediatric Surgery, and Oncology, Policlinica Metropalitana, Caracas, Venezuela. Reprint requests: Moises GuelrOO, MD, Policlinica Metropolitana, Urb. Caurimare, Caracas, Venezuela. VOLUME 35, NO.3, 1989
Figure 1. Endoscopic retrograde cholangiogram showing a stricture at the common hepatic duct (arrow). There is dilation of the hepatic duct and intrahepatic biliary ducts. The common bile duct below the cystic duct and the gallbladder is normal.
259
Figure 2. A, A guide wire is introduced into the left hepatic duct. B, A 7 French double pigtail biliary stent is placed in the left
hepatic duct bridging the obstruction.
Figure 3. A, With the stent in place, an ERCP catheter is introduced into the common duct and a cholangiogram shows dissapearance of the stricture at the common hepatic duct. B, After removing the biliary stent, the common hepatic duct is slightly dilated without stricture.
ary obstruction has not been described. 7 Metastases occur primarily to abdominal lymph nodes, liver, lungs, bone marrow, and skin. The prognosis is related to the age of the patient at diagnosis, the site of the primary tumor, and the degree of tumor differentiation. The prognosis for children who are less than 1 260
year of age is better than for older children with comparable tumors. Extraabdominal tumors and those containing differentiated, mature ganglion cells8 carry a better prognosis. ERCP, first performed in children in 19769 , has been successful in infants weighing as little as 2.5 kg. 10 GASTROINTESTINAL ENDOSCOPY
The pediatric indications for ERCP have included evaluation of neonatal cholestasis,lO relapsing pancreatitis' II and the diagnosis of extrahepatic biliary obstruction. 12 Significant complications, including cholangitis, bacteremia, and pancreatitis, have been rare in children. Minor acute duodenal erosions were observed in 7 of 23 neonates who experienced no apparent clinical consequences. 10 In adults, endoscopic biliary stenting has been used for palliation of unresectable biliopancreatic tumors, preoperative drainage of tumors with potentially resectable lesions, and as a temporary biliary decompression in patients with large common duct stones or benign stricture.l.13-15 There are no reports of stent placement in infancy. In this child, biliary drainage prior to chemotherapy was important because adriamycin is excreted in bile and substantial morbidity is associated with severe jaundice. 16 ERCP, endoscopic papillotomy, and biliary stenting should be performed in children only by experienced endoscopists who have a high success rate in placing biliary stents. Although indications are rare in children, the technical capability now exists to consider this form of therapy in selected cases. ACKNOWLEDGMENT
The authors thank Dr. Harland S. Winter, Children's Hospital, Harvard Medical School for reviewing the manuscript. REFERENCES 1. Takagi K, Ikeda S, Nakagawa Y, et al. Endoscopic sphincterotomy of the ampulla of Vater. Endoscopy 1970;2:107-15.
VOLUME 35, NO.3, 1989
2. Cotton PB, Beales JSM. The role of endoscopic retrograde cholangiopancreatography (ERCP) in patients with jaundice. Acta Gastroenterol Belg 1973;36:689-92. 3. Vennes JA, Jacobsen JR, Silvis SE. Endoscopic cholangiography for biliary system diagnosis. Ann Intern Med 1974;80:614.
4. Kawai K, Akasaka Y, Murakami K, et al. Endoscopic sphincterotomy of the ampulla of Vater. Gastrointest Endosc 1974;20:148-51. 5. Classen M, Safrany L. Endoscopic papillotomy and removal of gallstones. Br Med J 1975;4:371-4. 6. Soehendra N, Reynders-Frederix V. Palliative bile duct drainage. A new endoscopic method of introducing a transpapillary drain. Endoscopy 1980;12:8-11. 7. Kissane JM. Anderson's pathology. 8th ed. St. Louis: CV Mosby, 1985:1435. 8. Triche SJ, Askin FB, Kissane JM. Neuroblastoma, Ewing's sarcoma, and the differential diagnosis of small-round, bluecell tumors. In: Finegold M, ed. Pathology of neoplasia in children and adolescents. Philadelphia: WB Saunders, 1986:145 (Major problems in pathology; Vol 18). 9. Waye JD. Endoscopic retrograde cholangiopancreatography in the infant. Am J GastroenteroI1976;65:461-3. 10. Guelrud M, Jaen D, Torres P, et al. Endoscopic cholangiopancreatography in the infant: evaluation of a new prototype pediatric duodenoscope. Gastrointest Endosc 1987;33:4-8. 11. Alwniack A, Jonson G, Mattsson K. Chronic relapsing pancreatitis in a child. Endoscopic diagnosis. Acta Chir Scand 1977;143:153-5. 12. Urakami V, Seki H, Kishi S. Endoscopic retrograde cholangiopancreatography (ERCP) performed in children. Endoscopy 1977;9:86-91. 13. Wurbs D, Phillip J, Classen M. Experiences with long-standing nasobiliary tube in biliary disease. Endoscopy 1980;12:219-23. 14. Huibregtse K, Tytgat GN. Palliative treatment of obstructive jaundice by transpapillary introduction of a large bore bile duct endoprosthesis. Gut 1982;23:371-5. 15. Siegel JH, Yatto RP. Biliary endoprostheses for the management of retained common bile duct stones. Am J Gastroenterol 1984;79:50-4. 16. Bachur NR. Adriamycin (NSC-123127) pharmacology. Cancer Chemother Rep 1975;3:153-8.
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