BACKGROUND Giant congenital biliary dilation(CBD)is a rare condition observed in clinical practice.Infants born with this condition often experience a poor overall health status,and the disease progresses rapidly,lead...BACKGROUND Giant congenital biliary dilation(CBD)is a rare condition observed in clinical practice.Infants born with this condition often experience a poor overall health status,and the disease progresses rapidly,leading to severe biliary obstruction,infections,pressure exerted by the enlarged CBD on abdominal organs,disturbances in the internal environment,and multiple organ dysfunction.The treatment of giant CBD using laparoscopy is challenging due to the high degree of variation in the shape of the bile duct and other organs,making it difficult to separate the bile duct wall from adjacent tissues or to control bleeding.CASE SUMMARY Herein,we present the details of an 11-d-old male newborn who was diagnosed with giant CBD.The patient was admitted to the neonatal surgery department of our hospital due to a history of common bile duct cyst that was detected more than 3 mo ago,and also because the patient had been experiencing yellowish skin for the past 9 d.The abnormal echo in the fetal abdomen was first noticed by the patient’s mother during a routine ultrasound examination at a local hospital,when the patient was at 24 wk+6 d of pregnancy.This finding raised concerns about the possibility of congenital biliary dilatation(22 mm×21 mm).Subsequent ultrasound examinations at different hospitals consistently confirmed the presence of a congenital biliary dilatation.No specific treatment was administered for biliary dilatation during this period.A computed tomography scan conducted during the hospitalization revealed a large cystic mass in the right upper quadrant and pelvis,measuring approximately 9.2 cm×7.4 cm×11.3 cm.Based on the CONCLUSION The analysis reveals that prenatal imaging techniques,such as ultrasound and magnetic resonance imaging,play a crucial role in the early diagnosis,fetal prognosis,and treatment plan for giant CBD.Laparoscopic surgery for giant CBD presents certain challenges,including difficulties in separating the cyst wall,anastomosis,and hemostasis,as well as severe biliary system infection and ulceration.Consequently,there is a high likelihood of converting to laparotomy.The choice between surgical methods like hepaticojejunostomy(HJ)or hepaticoduodenostomy has not been standardized yet.However,we have achieved favorable outcomes using HJ.Preoperative management of inflammation,biliary drainage,liver function protection,and supportive treatment are particularly vital in improving children’s prognosis.After discharge,it is essential to conduct timely reexamination and close follow-up to identify potential complications.展开更多
AIM: To clarify the pathogenesis of biliary dilatation associated with anomalous union of the pancreatic and biliary ducts (AUPBD).METHODS: Direct cholangiopancreatograms of 350 adult patients with or with suspicion o...AIM: To clarify the pathogenesis of biliary dilatation associated with anomalous union of the pancreatic and biliary ducts (AUPBD).METHODS: Direct cholangiopancreatograms of 350 adult patients with or with suspicion of hepatobiliary or pancreatic disorders were reviewed. AUPBD was diagnosed cholangiopancreatographically, when the pancreaticobiliary ductal union was located above the narrow distal segment of the bile duct, which represents the action of the sphincter of Oddi. The narrow portion of the terminal choledochus was defined as symmetrical stricture of the common bile duct just above the pancreaticobiliary ductal union.RESULTS: AUPBD was found in 36 patients. Among cholangiopancreatographic features, the narrow portion of the terminal choledochus was the most pathognomonic for AUPBD (accuracy, 98%); it was present in 29 (81%)patients with AUPBD, but was not found in any patients without AUPBD. Among patients with AUPBD, biliary dilatation (>10 mm) was more frequent in those with the narrow portion of the terminal choledochus (23/29)than in those without (2/7; P = 0.018) AUPBD. Among the patients with both AUPBD and the narrow portion of the terminal choledochus, there was a strong negative correlation between the minimum diameter of the narrow portion and the maximum diameter of the choledochus (r = -0.78, P<0.001), suggesting that the degree of biliary narrowing at the narrow portion correlates with that of upstream biliary dilatation.CONCLUSION: The narrow portion of the terminal choledochus, a pathognomonic radiologic feature of AUPBD, may be a cause of biliary dilatation in patients with AUPBD.展开更多
基金Supported by National Natural Science Foundation of China,No.82170676Natural Science Foundation of Shaanxi Provincial Key Industries Innovation Chain(Cluster)-Social Development Project,No.2020ZDLSF02-03Xi’an Talents Plan Project:Clinical Application of Minimally Invasive Treatment of Alimentary Tract Malformation in Children by Combining Medical and Industrial Innovative Technology of Magnetic Surgery,No.XAYC210064.
文摘BACKGROUND Giant congenital biliary dilation(CBD)is a rare condition observed in clinical practice.Infants born with this condition often experience a poor overall health status,and the disease progresses rapidly,leading to severe biliary obstruction,infections,pressure exerted by the enlarged CBD on abdominal organs,disturbances in the internal environment,and multiple organ dysfunction.The treatment of giant CBD using laparoscopy is challenging due to the high degree of variation in the shape of the bile duct and other organs,making it difficult to separate the bile duct wall from adjacent tissues or to control bleeding.CASE SUMMARY Herein,we present the details of an 11-d-old male newborn who was diagnosed with giant CBD.The patient was admitted to the neonatal surgery department of our hospital due to a history of common bile duct cyst that was detected more than 3 mo ago,and also because the patient had been experiencing yellowish skin for the past 9 d.The abnormal echo in the fetal abdomen was first noticed by the patient’s mother during a routine ultrasound examination at a local hospital,when the patient was at 24 wk+6 d of pregnancy.This finding raised concerns about the possibility of congenital biliary dilatation(22 mm×21 mm).Subsequent ultrasound examinations at different hospitals consistently confirmed the presence of a congenital biliary dilatation.No specific treatment was administered for biliary dilatation during this period.A computed tomography scan conducted during the hospitalization revealed a large cystic mass in the right upper quadrant and pelvis,measuring approximately 9.2 cm×7.4 cm×11.3 cm.Based on the CONCLUSION The analysis reveals that prenatal imaging techniques,such as ultrasound and magnetic resonance imaging,play a crucial role in the early diagnosis,fetal prognosis,and treatment plan for giant CBD.Laparoscopic surgery for giant CBD presents certain challenges,including difficulties in separating the cyst wall,anastomosis,and hemostasis,as well as severe biliary system infection and ulceration.Consequently,there is a high likelihood of converting to laparotomy.The choice between surgical methods like hepaticojejunostomy(HJ)or hepaticoduodenostomy has not been standardized yet.However,we have achieved favorable outcomes using HJ.Preoperative management of inflammation,biliary drainage,liver function protection,and supportive treatment are particularly vital in improving children’s prognosis.After discharge,it is essential to conduct timely reexamination and close follow-up to identify potential complications.
文摘AIM: To clarify the pathogenesis of biliary dilatation associated with anomalous union of the pancreatic and biliary ducts (AUPBD).METHODS: Direct cholangiopancreatograms of 350 adult patients with or with suspicion of hepatobiliary or pancreatic disorders were reviewed. AUPBD was diagnosed cholangiopancreatographically, when the pancreaticobiliary ductal union was located above the narrow distal segment of the bile duct, which represents the action of the sphincter of Oddi. The narrow portion of the terminal choledochus was defined as symmetrical stricture of the common bile duct just above the pancreaticobiliary ductal union.RESULTS: AUPBD was found in 36 patients. Among cholangiopancreatographic features, the narrow portion of the terminal choledochus was the most pathognomonic for AUPBD (accuracy, 98%); it was present in 29 (81%)patients with AUPBD, but was not found in any patients without AUPBD. Among patients with AUPBD, biliary dilatation (>10 mm) was more frequent in those with the narrow portion of the terminal choledochus (23/29)than in those without (2/7; P = 0.018) AUPBD. Among the patients with both AUPBD and the narrow portion of the terminal choledochus, there was a strong negative correlation between the minimum diameter of the narrow portion and the maximum diameter of the choledochus (r = -0.78, P<0.001), suggesting that the degree of biliary narrowing at the narrow portion correlates with that of upstream biliary dilatation.CONCLUSION: The narrow portion of the terminal choledochus, a pathognomonic radiologic feature of AUPBD, may be a cause of biliary dilatation in patients with AUPBD.