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Cholelithiasis and choledocholithiasis associated with anomalous junction of the cystic duct in a child
Journal of Pediatric Surgery (2007) 42, E17–E19
www.elsevier.com/locate/jpedsurg
Cholelithiasis and choledocholithiasis associated with anomalous junction of the cystic duct in a child Satohiko Yanagisawa a,⁎, Takaharu Oue b , Takayuki Odashima a , Masaaki Kuda a , Yoshihide Tanabe a , Kinji Yokomori a a
Department of Pediatric Surgery, Jichi Medical University, Tochigi 329-0498, Japan Department of Pediatric Surgery, Osaka University, Osaka 565-0871, Japan
b
Index words: Cholelithiasis; Choledocholithiasis; Anomaly of cystic duct; 3-dimensional computed tomography
Abstract We report an extremely rare case of cholelithiasis, presumably owing to cholestasis resulting from an anomalous course of the cystic duct. A 10-year-old girl visited our hospital because of right epigastric pain and fever. Cholelithiasis and choledocholithiasis were diagnosed by ultrasound examination. Magnetic resonance cholangiopancreatography showed no pancreaticobiliary maljunction but confirmed a dilated, tortuous cystic duct anomalously draining into the right hepatic duct. Because cholangitis and obstructive jaundice progressed after admission, emergent endoscopic retrograde cholangiopancreatography was performed, and a common bile duct stone was removed endoscopically. It was a bilirubin stone. At a later date, laparoscopic cholecystectomy was performed for cholelithiasis. Preoperative 3-dimensional computed tomography and intraoperative cholangiography enabled us to treat the cystic duct safely. © 2007 Elsevier Inc. All rights reserved.
Cholelithiasis and choledocholithiasis rarely occur in children. Patients often have underlying disorders causing cholelithiasis. Such disorders include hemolytic disease, cholestasis, enterohepatic circulation disturbance after enterectomy, and long-term total parental nutrition [1-5], but some cases occur without underlying disorders as idiopathic cholelithiasis [1-6]. We report a 10-year-old patient with cholecystocholedocholithiasis associated with an anomalous junction of the cystic duct, along with a review of the literature.
1. Case report A 10-year-old girl visited our hospital with chief complaints of right epigastric pain and fever. She had ⁎ Corresponding author. Tel.: +81 285 58 7371; fax: +81 285 44 3234. E-mail address: [email protected] (S. Yanagisawa). 0022-3468/$ – see front matter © 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.07.032
noted preprandial right flank pain every 2 months for 1 year. Severe right epigastric pain started several days before this visit. Because the pain persisted and was accompanied by fevers of between 37°C and 38°C, she visited our hospital. Physical examination revealed epigastric tenderness but no guarding. Blood tests showed evidence of mild inflammation (white blood cell count, 7100/μL; C-reactive protein, 0.25 mg/dL) and hepatic dysfunction (total bilirubin [T-bil], 1.63 mg/dL; aspartate aminotransferase, 346 mU/mL; alanine aminotransferase, 371 mU/mL). Serum amylase level was normal. Ultrasound revealed a hyperechoic lesion suggestive of cholelithiasis. A gallstone 7 mm in diameter was present in the common bile duct and obstructed the latter, causing diffuse dilation of the biliary tract from the common bile duct to intrahepatic bile ducts. Suspecting pancreaticobiliary maljunction, we performed magnetic resonance cholangiopancreatography. No filling defect could be identified in the common bile duct, but a gallstone was seen as a filling defect
E18 in the gallbladder. Neither pancreatic duct dilation nor cystic choledochal dilatation was found. A dilated, tortuous cystic duct ascended and joined the right hepatic duct. These imaging findings led to the diagnosis of cholecystocholedocholithiasis associated with an anomalous junction of the cystic duct. The patient was admitted, fasted, and started on fluid replacement therapy. On the second hospital day, the abdominal pain subsided; but blood tests showed evidence of obstructive jaundice (T-bil, 4.83 mg/dL; direct bilirubin [D-bil], 3.81 mg/dL) and exacerbation of liver function (aspartate aminotransferase, 673 mU/mL; alanine aminotransferase, 921 mU/mL; alkaline phosphatase, 2099 mU/ mL; γGT, 537 mU/mL). Therefore, endoscopic retrograde cholangiopancreatography was performed under general anesthesia (Fig. 1). A gallstone was noted in the common bile duct as a filling defect and was removed with a basket catheter. It was a 5 × 3-mm black stone, the analysis of which identified it as a bilirubin stone. We had confidence in our diagnosis after seeing endoscopic retrograde cholangiopancreatography findings. The insertion of an endoscopic nasobiliary drainage tube resulted in the relief of the obstructive jaundice and cholangitis. Considering surgery for cholelithiasis, we performed 3-dimensional computed tomography to confirm the 3-dimensional structure of the biliary tract (Fig. 2) and found that the cystic duct anomalously drained into the right posterior segmental branch of the biliary tract. At a later date, laparoscopic cholecystectomy for cholelithiasis was performed. The cystic duct was adequately exposed, and intraoperative cholangiography was performed to identify the site of the cystic-hepatic duct junction. The junction was located in the liver parenchyma, and the cystic duct was clipped at approximately 1.5 cm
S. Yanagisawa et al.
Fig. 2 Three-dimensional computed tomography: The cystic duct was dilated and tortuous and joined the right posterior segmental branch. An anomalous junction of intrahepatic bile ducts was also noted.
from the junction with the intrahepatic right bile duct and sectioned. After the cystic artery was ligated, the gallbladder was detached from the gallbladder fossa of the liver. The cholecystectomy was accomplished safely without biliary injury. The gallbladder contained minute, black stones. The resected gallbladder specimen had a smooth mucosal surface without elevated lesions. Pathologic examination showed mild infiltration of inflammatory cells in the mucosa, with no evidence of malignancy. The patient's postoperative course was uneventful, and she was discharged on the fourth postoperative day. She is currently asymptomatic and is being followed up as an outpatient.
2. Discussion
Fig. 1 Endoscopic retrograde cholangiopancreatography. The cystic duct ascended and drained into the right hepatic duct.
Cholelithiasis in children is rare and occurs in individuals ranging from newborns to 20-year-olds, particularly in those older than 10 years [1-6]. Its prevalence ranges from 0.13% to 1.9%, depending on the study [4-7], and is lower than that reported in adults (5%-10%). Characteristically, cholelithiasis in children is triggered by underlying conditions, including hemolytic diseases such as hereditary spherocytosis, cholestasis associated with biliary infection, biliary anomalies, enterohepatic circulation disturbance owing to ileocecal resection or short bowel syndrome, long-term total parental nutrition [2], and pregnancy during puberty [1-6]. In the absence of underlying conditions, cholelithiasis is considered idiopathic [4], and the incidence of idiopathic cholelithiasis has recently increased [5,6]. Cholestasis owing to anatomical abnormalities, such as pancreaticobiliary maljunction and choledochal dilatation, has frequently been
Cholelithiasis and choledocholithiasis associated with anomalous junction of the cystic duct
E19
Fig. 3 Classification of the branching types of the biliary tree. Right anterior duct was represented by A; right posterior duct, by P; right hepatic duct, by R; left hepatic duct, by L; the first branching duct of lateral segment, by L1; and the second branching one, by L2 as described by Yoshida et al [8].
reported to be complicated by cholecystolithiasis. However, cholelithiasis owing to an anomalous course of the cystic duct has very rarely been reported. In contrast to normal biliary branching patterns, an anomalous junction of the cystic duct coexisted with an intrahepatic biliary branching anomaly in this patient. Because of the low incidence of biliary branching anomalies, it is difficult to show incidences by the anatomical branching pattern. Therefore, we discuss this case based on the classification by Yoshida et al [8] of the branching patterns of the biliary tree (Fig. 3). When their anomalous course is classified into (1) anomalous intrahepatic biliary branching patterns and (2) anomalous junction of the cystic duct, the biliary anomalies in this patient correspond to L1-RL2 (incidence, 0.5%) in “others” under (1) and to P-AL(P-C) (incidence, 0.45%) under (2). Thus, the present case is extremely rare in that these 2 types of anomalies coexisted. In the present case, anomaly of cystic duct and biliary tree may cause relapse of common bile duct stones and cholangitis with repeated abdominal pain in the future; therefore, we decided to perform subsequent laparoscopic cholecystectomy. Cholecystectomy in adults is complicated, at frequencies of 0.6% to 1% [9], by biliary duct injury owing to poor surgical skills and the anomalous course of the cystic and common bile ducts. In patients with an anomalous course of bile ducts, the use of intraoperative cholangiography has been reported to minimize intraoperative bile duct injury [9]. In this case,
preoperative imaging studies, including 3-dimensional computed tomography, and intraoperative cholangiography enabled us to perform safe surgery.
References [1] Pokorny WJ, Saleem M, O'Gorman RB, et al. Cholelithiasis and cholecystitis in childhood. Am J Surg 1984;148:742-4. [2] Rief S, Sloven DG, Lebenthal E. Gallstone in children. AJDC 1991; 145:105-8. [3] Roslyn JJ, Pitt HA, Mann L, et al. Parenteral nutrition-induced gallbladder disease: a reason for early cholecystectomy. Am J Surg 1984;148:58-63. [4] Palasciano G, Portincasa P, Vinciguerra V, et al. Gallstone prevalence and gallbladder volume in children and adolescents: an epiderminological ultrasonographic survey and relationship to body mass index. Am J Gastroenterol 1989;84:1378-82. [5] Wesdorp I, Bosman D, Graaff AD, et al. Clinical presentation and predisposing factors of cholelithiasis and sludge in children. JPGN 2000;31:411-7. [6] Bailey PV, Connors RH, Tracy TF, et al. Changing spectrum of cholelithiasis and cholecystitis in infants and children. Am J Surg 1989;158:585-8. [7] Stringer MD, Taylor DR, Soloway RD. Gallstone comparison: are children different? J Pediatr 2003;142:435-40. [8] Yoshida J, Chijiiwa K, Yamaguchi K, et al. Practical classification of the branching types of the biliary tree: an analysis of 1094 consecutive direct cholangiograms. J Am Coll Surg 1996;182:37-40. [9] Kullman E, Borch K, Lindstrom E, et al. Value of routine intraoperative cholangiography in detecting aberrant bile ducts and bile duct injuries during laparoscopic cholecystecomy. Br J Surg 1996;83:171-5.
www.elsevier.com/locate/jpedsurg
Cholelithiasis and choledocholithiasis associated with anomalous junction of the cystic duct in a child Satohiko Yanagisawa a,⁎, Takaharu Oue b , Takayuki Odashima a , Masaaki Kuda a , Yoshihide Tanabe a , Kinji Yokomori a a
Department of Pediatric Surgery, Jichi Medical University, Tochigi 329-0498, Japan Department of Pediatric Surgery, Osaka University, Osaka 565-0871, Japan
b
Index words: Cholelithiasis; Choledocholithiasis; Anomaly of cystic duct; 3-dimensional computed tomography
Abstract We report an extremely rare case of cholelithiasis, presumably owing to cholestasis resulting from an anomalous course of the cystic duct. A 10-year-old girl visited our hospital because of right epigastric pain and fever. Cholelithiasis and choledocholithiasis were diagnosed by ultrasound examination. Magnetic resonance cholangiopancreatography showed no pancreaticobiliary maljunction but confirmed a dilated, tortuous cystic duct anomalously draining into the right hepatic duct. Because cholangitis and obstructive jaundice progressed after admission, emergent endoscopic retrograde cholangiopancreatography was performed, and a common bile duct stone was removed endoscopically. It was a bilirubin stone. At a later date, laparoscopic cholecystectomy was performed for cholelithiasis. Preoperative 3-dimensional computed tomography and intraoperative cholangiography enabled us to treat the cystic duct safely. © 2007 Elsevier Inc. All rights reserved.
Cholelithiasis and choledocholithiasis rarely occur in children. Patients often have underlying disorders causing cholelithiasis. Such disorders include hemolytic disease, cholestasis, enterohepatic circulation disturbance after enterectomy, and long-term total parental nutrition [1-5], but some cases occur without underlying disorders as idiopathic cholelithiasis [1-6]. We report a 10-year-old patient with cholecystocholedocholithiasis associated with an anomalous junction of the cystic duct, along with a review of the literature.
1. Case report A 10-year-old girl visited our hospital with chief complaints of right epigastric pain and fever. She had ⁎ Corresponding author. Tel.: +81 285 58 7371; fax: +81 285 44 3234. E-mail address: [email protected] (S. Yanagisawa). 0022-3468/$ – see front matter © 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.07.032
noted preprandial right flank pain every 2 months for 1 year. Severe right epigastric pain started several days before this visit. Because the pain persisted and was accompanied by fevers of between 37°C and 38°C, she visited our hospital. Physical examination revealed epigastric tenderness but no guarding. Blood tests showed evidence of mild inflammation (white blood cell count, 7100/μL; C-reactive protein, 0.25 mg/dL) and hepatic dysfunction (total bilirubin [T-bil], 1.63 mg/dL; aspartate aminotransferase, 346 mU/mL; alanine aminotransferase, 371 mU/mL). Serum amylase level was normal. Ultrasound revealed a hyperechoic lesion suggestive of cholelithiasis. A gallstone 7 mm in diameter was present in the common bile duct and obstructed the latter, causing diffuse dilation of the biliary tract from the common bile duct to intrahepatic bile ducts. Suspecting pancreaticobiliary maljunction, we performed magnetic resonance cholangiopancreatography. No filling defect could be identified in the common bile duct, but a gallstone was seen as a filling defect
E18 in the gallbladder. Neither pancreatic duct dilation nor cystic choledochal dilatation was found. A dilated, tortuous cystic duct ascended and joined the right hepatic duct. These imaging findings led to the diagnosis of cholecystocholedocholithiasis associated with an anomalous junction of the cystic duct. The patient was admitted, fasted, and started on fluid replacement therapy. On the second hospital day, the abdominal pain subsided; but blood tests showed evidence of obstructive jaundice (T-bil, 4.83 mg/dL; direct bilirubin [D-bil], 3.81 mg/dL) and exacerbation of liver function (aspartate aminotransferase, 673 mU/mL; alanine aminotransferase, 921 mU/mL; alkaline phosphatase, 2099 mU/ mL; γGT, 537 mU/mL). Therefore, endoscopic retrograde cholangiopancreatography was performed under general anesthesia (Fig. 1). A gallstone was noted in the common bile duct as a filling defect and was removed with a basket catheter. It was a 5 × 3-mm black stone, the analysis of which identified it as a bilirubin stone. We had confidence in our diagnosis after seeing endoscopic retrograde cholangiopancreatography findings. The insertion of an endoscopic nasobiliary drainage tube resulted in the relief of the obstructive jaundice and cholangitis. Considering surgery for cholelithiasis, we performed 3-dimensional computed tomography to confirm the 3-dimensional structure of the biliary tract (Fig. 2) and found that the cystic duct anomalously drained into the right posterior segmental branch of the biliary tract. At a later date, laparoscopic cholecystectomy for cholelithiasis was performed. The cystic duct was adequately exposed, and intraoperative cholangiography was performed to identify the site of the cystic-hepatic duct junction. The junction was located in the liver parenchyma, and the cystic duct was clipped at approximately 1.5 cm
S. Yanagisawa et al.
Fig. 2 Three-dimensional computed tomography: The cystic duct was dilated and tortuous and joined the right posterior segmental branch. An anomalous junction of intrahepatic bile ducts was also noted.
from the junction with the intrahepatic right bile duct and sectioned. After the cystic artery was ligated, the gallbladder was detached from the gallbladder fossa of the liver. The cholecystectomy was accomplished safely without biliary injury. The gallbladder contained minute, black stones. The resected gallbladder specimen had a smooth mucosal surface without elevated lesions. Pathologic examination showed mild infiltration of inflammatory cells in the mucosa, with no evidence of malignancy. The patient's postoperative course was uneventful, and she was discharged on the fourth postoperative day. She is currently asymptomatic and is being followed up as an outpatient.
2. Discussion
Fig. 1 Endoscopic retrograde cholangiopancreatography. The cystic duct ascended and drained into the right hepatic duct.
Cholelithiasis in children is rare and occurs in individuals ranging from newborns to 20-year-olds, particularly in those older than 10 years [1-6]. Its prevalence ranges from 0.13% to 1.9%, depending on the study [4-7], and is lower than that reported in adults (5%-10%). Characteristically, cholelithiasis in children is triggered by underlying conditions, including hemolytic diseases such as hereditary spherocytosis, cholestasis associated with biliary infection, biliary anomalies, enterohepatic circulation disturbance owing to ileocecal resection or short bowel syndrome, long-term total parental nutrition [2], and pregnancy during puberty [1-6]. In the absence of underlying conditions, cholelithiasis is considered idiopathic [4], and the incidence of idiopathic cholelithiasis has recently increased [5,6]. Cholestasis owing to anatomical abnormalities, such as pancreaticobiliary maljunction and choledochal dilatation, has frequently been
Cholelithiasis and choledocholithiasis associated with anomalous junction of the cystic duct
E19
Fig. 3 Classification of the branching types of the biliary tree. Right anterior duct was represented by A; right posterior duct, by P; right hepatic duct, by R; left hepatic duct, by L; the first branching duct of lateral segment, by L1; and the second branching one, by L2 as described by Yoshida et al [8].
reported to be complicated by cholecystolithiasis. However, cholelithiasis owing to an anomalous course of the cystic duct has very rarely been reported. In contrast to normal biliary branching patterns, an anomalous junction of the cystic duct coexisted with an intrahepatic biliary branching anomaly in this patient. Because of the low incidence of biliary branching anomalies, it is difficult to show incidences by the anatomical branching pattern. Therefore, we discuss this case based on the classification by Yoshida et al [8] of the branching patterns of the biliary tree (Fig. 3). When their anomalous course is classified into (1) anomalous intrahepatic biliary branching patterns and (2) anomalous junction of the cystic duct, the biliary anomalies in this patient correspond to L1-RL2 (incidence, 0.5%) in “others” under (1) and to P-AL(P-C) (incidence, 0.45%) under (2). Thus, the present case is extremely rare in that these 2 types of anomalies coexisted. In the present case, anomaly of cystic duct and biliary tree may cause relapse of common bile duct stones and cholangitis with repeated abdominal pain in the future; therefore, we decided to perform subsequent laparoscopic cholecystectomy. Cholecystectomy in adults is complicated, at frequencies of 0.6% to 1% [9], by biliary duct injury owing to poor surgical skills and the anomalous course of the cystic and common bile ducts. In patients with an anomalous course of bile ducts, the use of intraoperative cholangiography has been reported to minimize intraoperative bile duct injury [9]. In this case,
preoperative imaging studies, including 3-dimensional computed tomography, and intraoperative cholangiography enabled us to perform safe surgery.
References [1] Pokorny WJ, Saleem M, O'Gorman RB, et al. Cholelithiasis and cholecystitis in childhood. Am J Surg 1984;148:742-4. [2] Rief S, Sloven DG, Lebenthal E. Gallstone in children. AJDC 1991; 145:105-8. [3] Roslyn JJ, Pitt HA, Mann L, et al. Parenteral nutrition-induced gallbladder disease: a reason for early cholecystectomy. Am J Surg 1984;148:58-63. [4] Palasciano G, Portincasa P, Vinciguerra V, et al. Gallstone prevalence and gallbladder volume in children and adolescents: an epiderminological ultrasonographic survey and relationship to body mass index. Am J Gastroenterol 1989;84:1378-82. [5] Wesdorp I, Bosman D, Graaff AD, et al. Clinical presentation and predisposing factors of cholelithiasis and sludge in children. JPGN 2000;31:411-7. [6] Bailey PV, Connors RH, Tracy TF, et al. Changing spectrum of cholelithiasis and cholecystitis in infants and children. Am J Surg 1989;158:585-8. [7] Stringer MD, Taylor DR, Soloway RD. Gallstone comparison: are children different? J Pediatr 2003;142:435-40. [8] Yoshida J, Chijiiwa K, Yamaguchi K, et al. Practical classification of the branching types of the biliary tree: an analysis of 1094 consecutive direct cholangiograms. J Am Coll Surg 1996;182:37-40. [9] Kullman E, Borch K, Lindstrom E, et al. Value of routine intraoperative cholangiography in detecting aberrant bile ducts and bile duct injuries during laparoscopic cholecystecomy. Br J Surg 1996;83:171-5.