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Objective To summarize our therapeutic experiences on patients with pediatric spontaneous biliary duct perforation.Methods We retrospectively analyzed the clinical data of patients diagnosed as spontaneous biliary duct perforation who were admitted into the Department of Pediatric General Surgery,the Beijing Children Hospital from January 2008 to December 2014,and summarized the therapeutic experiences.Results There were 7 boys and 18 girls,with a average age of 2.4 years (range 11 months to 10 years).Twenty-one patients (84.0％) were diagnosed by ultrasonography.Two patients were treated with conservative therapy and were discharged home.The remaining 23 patients were treated with emergent surgery.Of these patients,9 were treated with cholecystostomy and abdominal drainage (the cholecystostomy group),and the remaining 14 were treated with choledochal drainage and abdominal drainage (the choledochal drainage group).The mean hospitalization stay for the cholecystostomy group was 25.2 days,and 3 patients developed comphcations (33.3％).The mean hospitalization stay for thecholedochal drainage group was 16.1 day,and 2 patients developed complications (14.2％).Twenty-four patients were diagnosed to suffer from congenital choledochal cysts or pancreaticobiliary maljunction by imaging studies during or after surgery.Elective choledochal cystectomy with hepaticojejunostomy were performed on 23 stable patients who developed no severe complications.Conclusions Pediatric spontaneous bile duct perforation is closely related with congenital choledochal cysts,and the pathological basis in diagnosis is pancreaticobiliary maljunction.Bile duct elastic fiber hypogenesis and specific blood supply are important to the onset of perforation.Abdominal ultrasonography plays an important role in the diagnosis.Patients with peritoneal irritation and non-localized perforation should be operated in time,and choledochal drainage with abdominal drainage is a good treatment choice.All patients diagnosed as congenital choledochal cysts or pancreaticobiliary maljunction should undergo elective choledochal cystectomy with hepaticojejunostomy.
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Objective To investigate the clinical value of 3.0T magnetic resonance cholangiopancreatography (MRCP) in diagnosis of congenital biliary malformation in children.Methods Magnetic resonance imaging (MRI)and MRCP images,including the source image data and maximum intensity projection (MIP) reconstruction images,were analyzed retrospectively in 83 children with congenital biliary malformation diagnosed at Shanghai Children's Hospital,and their operative results and intraoperative cholangiopancreatography images were compared.Two experienced radiologists independently reviewed the images then they reached diagnostic conclusions by consensus.Eighty-three patients included 35 boys and 48 girls,aged from 20 days to 14 years.Results The MRCP indicated that 69 cases with choledochal cysts were consistent with intraoperative cholangiopancreatography findings,and the common bile duct showed different degrees of saccular and fusiform or columnar expansion with different types(Todani classification type Ⅰ 59 cases,type Ⅱ 1 cases,type Ⅳ a 7 cases,and type V 2 cases).The accuracy of localization and quality were 100％.MRCP demonstrated the anomalous junction of pancreaticobiliary duct in 14 cases.MRCP in 19 cases detected by intraoperative cholangiopancreatography with a sensitivity of 79％ and a specificity of 100％.Among 13 cases of biliary atresia,including 1 case accompanied with peritoneal organ inversion proved by MRI,11 cases of the entire extra—hepatic bile duct could not be completed visually,and 2 cases of small or small triangular signals were seen near the hepatic portal area,with the portal vein,and the left or right branches with 100％ accuracy of localization and quality including 1 case accompanied with peritoneal organ inversion proved by MRI.Congenital absence of gallbladder accompanied with duodenal obstruction was seen in 1 case,no gallbladder was found in the gallbladder fossa by MRCP,but the proximal duodenum was obviously dilated.Surgery confirmed that it was complicated and annular pancreas without gallbladder congenitally.Conclusion 3.0T MRCP is a noninvasive and effective method for diagnosis of congenital biliary malformation in children.It can be used as the preferred technique for diagnosis and long-term follow-ups.
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Objective To investigate the clinical value of 3.0T magnetic resonance cholangiopancreatography (MRCP) in diagnosis of congenital biliary malformation in children.Methods Magnetic resonance imaging (MRI)and MRCP images,including the source image data and maximum intensity projection (MIP) reconstruction images,were analyzed retrospectively in 83 children with congenital biliary malformation diagnosed at Shanghai Children's Hospital,and their operative results and intraoperative cholangiopancreatography images were compared.Two experienced radiologists independently reviewed the images then they reached diagnostic conclusions by consensus.Eighty-three patients included 35 boys and 48 girls,aged from 20 days to 14 years.Results The MRCP indicated that 69 cases with choledochal cysts were consistent with intraoperative cholangiopancreatography findings,and the common bile duct showed different degrees of saccular and fusiform or columnar expansion with different types(Todani classification type Ⅰ 59 cases,type Ⅱ 1 cases,type Ⅳ a 7 cases,and type V 2 cases).The accuracy of localization and quality were 100％.MRCP demonstrated the anomalous junction of pancreaticobiliary duct in 14 cases.MRCP in 19 cases detected by intraoperative cholangiopancreatography with a sensitivity of 79％ and a specificity of 100％.Among 13 cases of biliary atresia,including 1 case accompanied with peritoneal organ inversion proved by MRI,11 cases of the entire extra—hepatic bile duct could not be completed visually,and 2 cases of small or small triangular signals were seen near the hepatic portal area,with the portal vein,and the left or right branches with 100％ accuracy of localization and quality including 1 case accompanied with peritoneal organ inversion proved by MRI.Congenital absence of gallbladder accompanied with duodenal obstruction was seen in 1 case,no gallbladder was found in the gallbladder fossa by MRCP,but the proximal duodenum was obviously dilated.Surgery confirmed that it was complicated and annular pancreas without gallbladder congenitally.Conclusion 3.0T MRCP is a noninvasive and effective method for diagnosis of congenital biliary malformation in children.It can be used as the preferred technique for diagnosis and long-term follow-ups.
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Objective To explore the clinical efficacies of open and laparoscopic surgery in the treatment of adult patients with congenital choledochal cyst.Methods The clinical data of 36 adult patients with congenital choledochal cysts who were admitted to the Sir Run Run Shaw Hospital from February 2008 to February 2015 were retrospectively analyzed.Twenty-four patients underwent common bile duct cyst resection + cholecystectomy +biliojejunal Roux-en-Y anastomosis (open surgery group),12 patients underwent laparoscopic common bile duct cyst resection + cholecystectomy + biliojejunal Roux-en-Y anastomosis (laparoscopy group).The intraoperative conditions,detection indexes and postoperative recovery indexes in the 2 groups were analyzed.The follow-up by regular outpatient examination and telephone interview were done up to March 2015.Non-normal distribution data were described as M(P25,P75).Comparison between groups was analyzed using the Mann-Whitney U test and Fisher exact probability.Measurement data with normal distribution were presented as (x) ± s and analyzed using t test.Count data were evaluated by the ratio and proportion.Results The operation time of the open surgery group and laparoscopy group were 238 minutes (191 minutes,283 minutes) and 270 minutes (225 minutes,326 minutes),with a significant difference between the 2 groups (Z =-1.360,P ＞ 0.05).The volume of intraoperative blood loss in the open surgery group and laparoscopy group were 200 mL (113 mL,363 mL) and 75 mL(50 mL,138 mL),with a significant difference between the 2 groups (Z =-3.377,P ＜0.05).The level of C-reactive protein in the open surgery group and laparoscopy group were 94 mg/L (81 mg/L,104 mg/L) and 29 mg/L (21 mg/L,61 mg/L),with a significant difference between the 2 groups (Z =-4.296,P ＜0.05).The pain scores at postoperative day 3,time to anal exsufflation,time for fluid diet intake and duration of hospital stay of the open surgery group and laparoscopy group were 2.5 ± 0.9,(3.7 ± 1.0) days,(5.0 ± 1.6) days,(10.0 ± 2.7) days and 1.9 ±0.3,(2.6 ± 1.0) days,(3.6 ± 1.6) days,(7.8 ± 2.2) days,respectively,showing significant differences between the 2 groups (t =3.146,3.286,2.450,2.523,P ＜ 0.05).Nine patients in the open surgery group and 3 patients in the laparoscopy group had the complications,with improvement of conditions after symptomatic treatment,showing no significant difference between the 2 groups (P ＞ 0.05).All the 36 patients were followed up for a median time of 36 months (range,1-72 months) with good recovery,without long-term postoperative complications and re-operation.Conclusion Laparoscopic surgery in the treatment of adult patients with congenital choledochal cyst is safe and feasible,with the advantages of less intraoperative bleeding,less surgical trauma,fast recovery compared with open surgery.
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Las lesiones quísticas de vías biliares, constituyen una entidad, que rara vez se observa o diagnostica en el adulto. El motivo de este trabajo es mostrar la experiencia de un centro de referencia biliopancreática en el diagnóstico y tratamiento de quistes de colédoco. Usamos la clasificación de Todani para la descripción de los mismos. Fue un estudio Descriptivo y Retrospectivo con 502 colangiopancreatografías retrógradas endoscópicas y de ellas 20 casos de quistes de colédoco identificados mediante colangiografías de alta calidad.
Cystic lesions of the bile ducts constitute an entity that is rarely seen or diagnosed in adults. The purpose of this study is to show the experience of a biliopancreatic referral center in the diagnosis and treatment of choledochus cysts. The Todani classification was used to describe them. It was a retrospective, descriptive study of 502 endoscopic retrograde cholangiopancreatographies and of these 20 cases of choledochal cysts were identified by high-quality cholangiographies.
Subject(s)
Humans , Male , Female , Cholangiography , Clinical Diagnosis , Bile Ducts/anatomy & histology , Bile Ducts/pathology , Choledochal Cyst/surgery , Choledochal Cyst/diagnosis , Choledochal Cyst/etiology , Diagnostic Imaging , GastroenterologyABSTRACT
Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.