BACKGROUND Type Ⅲ choledochal cysts(CCs)are extremely rare,and they present as dilatations and herniations of the end of the common bile duct into the duodenum.Moreover,type Ⅱ CCs may be easily misdiagnosed as intra...BACKGROUND Type Ⅲ choledochal cysts(CCs)are extremely rare,and they present as dilatations and herniations of the end of the common bile duct into the duodenum.Moreover,type Ⅱ CCs may be easily misdiagnosed as intraduodenal polyps or tumors.Thus,adequate differential diagnosis and selection of appropriate treatment are important.CASE SUMMARY A young man with a duodenal mass presented with 3-year intermittent abdominal pain and acute pancreatitis 3 days before hospitalization.After evaluation by magnetic resonance imaging and endoscopic ultrasonography,the duodenal papilla was pressed,and the bile flowed out slowly,which was speculated to be the cause of his symptoms.The lesion was punctured with a submucosal injection needle,and golden clear fluid was aspirated.Laboratory tests of the aspirate after 50-fold dilution revealed significantly elevated total bilirubin,direct bilirubin,amylase and lipase.Taken together,these findings confirmed that the lesion was a type Ⅲ CC.The patient underwent fused surgical procedures.Fenestration plus internal drainage of the lesion was subsequently performed with a DualKnife.After drainage,the incision was sealed with tissue clips.During follow-up,the patient recovered well,and no abdominal pain symptoms or acute pancreatitis recurred.CONCLUSION Laboratory tests of cyst aspirates are beneficial for diagnosis,and endoscopic fenestration plus internal drainage works well to mitigate cysts.展开更多
BACKGROUND Choledochal cyst is a rare biliary tract disorder with five subtypes categorized based on the anatomical location of cystic dilatation.Type IV(which affects the intrahepatic and extrahepatic ducts)is the mo...BACKGROUND Choledochal cyst is a rare biliary tract disorder with five subtypes categorized based on the anatomical location of cystic dilatation.Type IV(which affects the intrahepatic and extrahepatic ducts)is the most common subtype in adults.Its clinical manifestations are diverse and the disease can potentially become malignant.Currently,the mainstay treatment is surgical excision which is used as a definitive treatment to prevent complications and avoid carcinogenesis.However,the surgical operation for giant choledochal cysts is technically challenging.CASE SUMMARY Here,we present a case of a giant choledochal cyst in an 18-year-old female.The diagnosis of patient was confirmed through magnetic resonance cholangiopancreatography.The patient developed acute cholangitis,and was treated with percutaneous transhepatic cholangial drainage to alleviate symptoms.The final treatment approach was cyst excision followed by Roux-en-Y hepaticojejunostomy,which successfully achieved excellent postoperative recovery.CONCLUSION Ultrasonography and magnetic resonance cholangiopancreatography can effectively diagnose choledochal cysts.Combined percutaneous transhepatic cholangial drainage surgery for giant choledochal cysts is safe and effective.展开更多
BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic pati...BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.展开更多
Choledochal cysts(CCs)are rare bile duct dilatations,intra-and/or extrahepatic,and have higher prevalence in the Asian population compared to Western populations.Most of the current literature on CC disease originates...Choledochal cysts(CCs)are rare bile duct dilatations,intra-and/or extrahepatic,and have higher prevalence in the Asian population compared to Western populations.Most of the current literature on CC disease originates from Asia where these entities are most prevalent.They are thought to arise from an anomalous pancreaticobiliary junction,which are congenital anomalies between pancreatic and bile ducts.Some similarities in presentation between Eastern and Western patients exist such as female predominance,however,contemporary studies suggest that Asian patients may be more symptomatic on presentation.Even though CC disease presents with an increased malignant risk reported to be more than 10%after the second decade of life in Asian patients,this risk may be overstated in Western populations.Despite this difference in cancer risk,management guidelines for all patients with CC are based predominantly on observations reported from Asia where it is recommended that all CCs should be excised out of concern for the presence or development of biliary tract cancer.展开更多
AIM: To summarize the experience of diagnosis and treatment of congenital choledochal cyst in the past 20 years (1980-2000). METHODS: The clinical data of 108 patients admitted from 1980 to 2000 were analyzed retrospe...AIM: To summarize the experience of diagnosis and treatment of congenital choledochal cyst in the past 20 years (1980-2000). METHODS: The clinical data of 108 patients admitted from 1980 to 2000 were analyzed retrospectively. RESULTS: Abdominal pain,jaundice and abdominal mass were presented in most child cases. Clinical symptoms in adult cases were non-specific, resulting in delayed diagnosis frequently. Fifty-seven patients (52.7%) had coexistent pancreatiobiliary disease. Carcinoma of the biliary duct occurred in 18 patients (16.6%).Ultrasonic examination was undertaken in 94 cases, ERCP performed in 46 cases and CT in 71 cases. All of the cases were correctly diagnosed before operation. Abnormal pancreatobiliary duct junction was found in 39 patients. Before 1985 the diagnosis and classification of congenital choledochal cyst were established by ultrasonography preoperatively and confirmed during operation, the main procedures were internal drainage by cyst enterostomy. After 1985, the diagnosis was established by ERCP and CT, and cystectomy with Roux-en-Y hepaticojejunostomy was the conventional procedures.In 1994, we reported a new and simplified operative procedure in order to reduce the risk of choledochal cyst malignancy. Postoperative complication was mainly retrograde infection of biliary tract, which could be controlled by the administration of antibiotics, there was no perioperative mortality. CONCLUSION: The concept in diagnosis and treatment of congenital choledochal cyst has obviously been changed greatly.CT and ERCP were of great help in the classification of the disease.Currently, cystectomy with Roux-en-Y hepaticojejunostomy is strongly recommended as the choice for patients with type I and type IV cysts. Piggyback orthotopic liver transplantation is indicated in type V cysts (Caroli's disease) with frequently recurrent cholangitis.展开更多
We report the first case series from Africa and the Middle East on choledochal cyst, a disease which shows significant geographical distribution with high incidence in the Asian population. In this study, the epidemio...We report the first case series from Africa and the Middle East on choledochal cyst, a disease which shows significant geographical distribution with high incidence in the Asian population. In this study, the epidemiological data of the patients are presented and analyzed. Attention was paid to diagnostic imaging and its accuracy in the diagnosis and classification of choledochal cyst. Most cases of choledochal cyst disease have type I and IV-A cysts according to the Todani classification system, which support the etiological theories of choledochal cyst, especially Babbitt’s theory of the anomalous pancreaticobiliary duct junction, which are clearly stated. The difficulties and hazards of surgical management and methods used to avoid operative complications are clarified. Early and late postoperative complications are also included. This study should be followed by multicenter studies throughout Egypt to help assess the incidence of choledochal cysts in one of the largest populations in Africa and the Middle East.展开更多
AIM:To determine the merits of magnetic resonance cholangiopancreatography(MRCP) as the primary diagnostic test for choledochal cysts(CC’s).METHODS:Between 2009 and 2012,patients who underwent MRCP for perioperative ...AIM:To determine the merits of magnetic resonance cholangiopancreatography(MRCP) as the primary diagnostic test for choledochal cysts(CC’s).METHODS:Between 2009 and 2012,patients who underwent MRCP for perioperative diagnosis were identified.Demographic information,clinical characteristics,and radiographic findings were recorded.MRCP results were compared with intraoperative findings.A PubMed search identified studies published between 1996-2012,employing MRCP as the primary preoperative imaging and comparing results with either endoscopic retrograde cholangiopancreatography(ERCP) or operative findings.Detection rates for CC’s and abnormal pancreaticobiliary junction(APBJ) were calculated.In addition detection rates for clinically related biliary pathology like choledocholithiasis and cholangiocarcinomas in patients diagnosed with CC’s were also evaluated.RESULTS:Eight patients were identified with CC’s.Six patients out of them had typeⅣCC’s,1 had type I and 1 had a new variant of choledochal cyst with confluent dilatation of the common bile duct(CBD) and cystic duct.Seven patients had an APBJ and 3 of those had a long common-channel.Gallstones were found in 2 patients,1 had a CBD stone,and 1 pancreatic-duct stone was also detected.In all cases,MRCP successfully identified the type of CC’s,as well as APBJ with ductal stones.From analyzing the literature,we found that MRCP has 96%-100% detection rate for CC’s.Additionally,we found that the range for sensitivity,specificity,and diagnostic accuracy was 53%-100%,90%-100% and 56%-100% in diagnosing APBJ.MRCP’s detection rate was 100% for choledocholithiasis and 87% for cholangiocarcinomas with concurrent CC’s.CONCLUSION:After initial ultrasound and computed tomography scan,MRCP should be the next diagnostic test in both adult and pediatric patients.ERCP should be reserved for patients where therapeutic intervention is needed.展开更多
AIM: To investigate the relationship between pancreatic amylase in bile duct and the clinico-pathological features in adult patients with choledochal cyst and anomalous pancreatico-biliary ductal union (APBDU).METHODS...AIM: To investigate the relationship between pancreatic amylase in bile duct and the clinico-pathological features in adult patients with choledochal cyst and anomalous pancreatico-biliary ductal union (APBDU).METHODS: From 39 patients who underwent surgery for choledochal cyst between March 1995 and March 2003,we selected 15 adult patients who had some symptoms and were radiologically diagnosed as APBDU, and their clinico-pathological features were subsequently evaluated retrospectively. However, we could not obtain biliary amylase in all the patients because of the surgeon's slip.Therefore, we measured the amylase level in gall bladder of 10 patients and in common bile duct of 11 patients.RESULTS: Levels of amylase in common bile duct and gall bladder ranged from 11 500 to 212 000 IU/L, and the younger the patients, the higher the biliary amylase level (r= -0.982, P＜0.01). Pathologically, significant correlation was found between the size of choledochal cyst and the grade of inflammation (r= 0.798,P＜0.01). And, significant correlation was found between the level of amylase in gall bladder and the grade of hyperplasia. On the other hand, there was no correlation to the age of symptomatic onset or inflammatory grade (r = 0.743, P＜0.05). Level of lipase was elevated from 6 000 to 159 000 IU/L in bile duct and from 14 400 to 117 000 IU/L in the gall bladder;however, there was no significant correlation with age or clinico-pathological features.CONCLUSION: The results support the notion that amylase has a particular role in the onset of symptoms, and suggest that a large amount of biliary amylase induces early onset of symptom, thereby making early diagnosis possible.展开更多
Our paper describes the key surgical points of pediatric choledochocystectomy performed completely by Da Vinci robotic system. A choledochocystectomy was safely carried out for a girl at our hospital, and without any ...Our paper describes the key surgical points of pediatric choledochocystectomy performed completely by Da Vinci robotic system. A choledochocystectomy was safely carried out for a girl at our hospital, and without any complication. Then systematic literature review was done to discuss the methods of intestine surgery and intestinal anastomosis, the use of 3 rd robotic arm, the surgical safety and advantages comparing open and laparoscopic surgery. We systematically reviewed choledochocystectomy for children performed by robotic surgery. We included a total of eight domestic and foreign reports and included a total of 86 patients, whose average age was 6.3(0.3-15.9) years; the maleto-female ratio was 1:3.5(19:67). Seven patients experienced conversion to open surgery, and the surgery success rate was 91.9%(79/86). The average total operation time was 426(180-520) min, the operation time on the machine was 302(120-418) min, 11 cases used the number 3 arm, and the remaining mainly used the hitch-stitch technique to suspend the stomach wall and liver. Forty-seven patients underwent pull-through intestine and intestinal anastomosis, and 39 patients underwent complete robotic intestine surgery and intestinal anastomosis. The hospitalization time of roboticassisted choledochocystectomy was 8.8 d. Eight patientshad biliary fistula and were all cured by conservative treatment and continuous observation. One patient had anastomotic stenosis, and one patient had wound dehiscence, both cured by surgery. Choledochocystectomy for children performed by completely robotic surgery and Roux-en-Y hepaticojejunostomy is safe and feasible. The initial experience shows that this surgical approach has a clearer field than the traditional endoscopy, and its operation is more flexible, the surgery is more accurate, and the injury is smaller. With the advancement of technology and the accumulation of surgeons' experience, robotic surgery may become a new trend in this surgical procedure.展开更多
Objective: To retrospectively study the early compli- cations of excision with hepaticoenterostomy for in- fants and children with choledochal cysts. Methods: We analyzed 16 patients with early posto- perative complic...Objective: To retrospectively study the early compli- cations of excision with hepaticoenterostomy for in- fants and children with choledochal cysts. Methods: We analyzed 16 patients with early posto- perative complications out of 173 patients with con- genital choledochal cysts aged 27 days to 14 years (mean 2.4 years) who had undergone excisional pro- cedures and biliary tract reconstruction. Results: The early complications included bile leak- age (10 patients), abdominal wall dehiscence (3), and hepatic failure, pancreatic juice leakage and postoperative intussusception (each in 1) respective- ly. Three patients died from bile leakage and 1 from postoperative hepatic failure. No statistical differen- ces were observed between the procedures of biliary tract reconstruction with jejunal segment interposi- tion hepaticoduodenostomy and Roux-en-Y hepatico- jejunostomy (P>0.75). The morbidity was signifi- cantly higher in infants below 1 year than in children (P<0.005). Prevention and treatment of the com- plications were discussed. Conclusion: Bile leakage and abdominal wall dehis- cence are major early postoperative complications. The morbidity of cholechal cysts is higher in infants than in children. Exploratory laparotomy should not be delayed when biliary leakage with diffuse peritoni- tis appears. The 'tension suture in the fascial space of the abdominal wall' is useful to prevent and treat wound dehiscence.展开更多
Objective: To evaluate the procedures and timing of operation as well as long-term postoperative effect of congenital choledochal cysts (CCC) in adults. Methods: The procedures and timing of operation, effective rate,...Objective: To evaluate the procedures and timing of operation as well as long-term postoperative effect of congenital choledochal cysts (CCC) in adults. Methods: The procedures and timing of operation, effective rate, re-operation rate and incidence of car- cinoma after operation for 70 adult patients with CCC from January 1980 to June 1999 were analyzed retrospectively. Results: The re-operation rate of external drainage was 86% (6/7). The effective rate of internal drain- age was significantly lower than that of cyst resection (3/10 vs 45/49, X^2=20. 94, P<0.001). The re-op- eration rate and incidence of carcinoma of internal drainage were higher than those of cyst resection (5/ 10 vs 3/49, X^2=13. 64, P<0. 001 and 3/10 vs 3/49, X^2=5. 18, P<0. 025). The reoperation rate of e- mergency surgery was higher than that of selective operation (8/10 vs 6/56, X^2=24. 37, P<0.001). Conclusions: External drainage should be the first- aid measure and the therapy of choice on emergency basis. Internal drainage should never be attempted. Cyst resection with Roux-en-Y hepaticojejunostomy is recommended as the treatment of choice in selective operation.展开更多
Choledochal cysts are congenital anomalies of the biliary ducts, characterized by cystic dilatation of the ducts.Prenatal diagnosis of this anomaly using ultrasonography (US) has been well documented. Magnetic resonan...Choledochal cysts are congenital anomalies of the biliary ducts, characterized by cystic dilatation of the ducts.Prenatal diagnosis of this anomaly using ultrasonography (US) has been well documented. Magnetic resonance imaging (MRI) has recently become an important complement to US in prenatal diagnosis of fetal anomalies. We herein report a patient in whom at 24 wk' gestation US suggested a right upper quadrant abdominal cyst and in whom at 26 wk' gestation MRI more clearly delineated the cyst and its surrounding structures and suggested a choledochal cyst, which was confirmed at postnatal surgery and histopathology.展开更多
BACKGROUND:Choledochal cysts in adults are more commonly associated with complications such as cystolithiasis,recurrent cholangitis,portal hypertension and malignancy,than in the pediatric age group.METHOD:We report a...BACKGROUND:Choledochal cysts in adults are more commonly associated with complications such as cystolithiasis,recurrent cholangitis,portal hypertension and malignancy,than in the pediatric age group.METHOD:We report a case of adult choledochal cyst with long-term complication of large stone cast and portal hypertension due to secondary biliary cirrhosis.RESULTS:A 50-year-old patient presented with obstructive jaundice and hepatosplenomegaly.On investigation,she was diagnosed as having a choledochal cyst with large stone cast and portal hypertension.Single stage resection of the choledochal cyst with Roux-en-Y hepaticojejunostomy was done by meticulous dissection and ligation of collaterals.CONCLUSION:Single stage resection of a choledochal cyst is possible in spite of associated portal hypertension,if the portal vein is patent.展开更多
BACKGROUND: Choledochal cysts are congenital cystic dilatations of the extrahepatic or intrahepatic portion of the biliary tree. Complete excision of choledochal cysts is currently regarded as the gold standard treatm...BACKGROUND: Choledochal cysts are congenital cystic dilatations of the extrahepatic or intrahepatic portion of the biliary tree. Complete excision of choledochal cysts is currently regarded as the gold standard treatment, while less extensive procedures including cystoduodenostomy have become obsolete due to the potential for malignant change in the remnant cyst. For type-1 choledochal cysts, which sometimes extend to the main pancreatic duct closely, some surgeons may adopt a less aggressive approach in order to avoid damage to the main pancreatic duct as such damage can lead to serious consequences. However, incomplete excision of choledocha cysts may also cause problems. METHOD: Here we report on a reoperation treating incomplete excision of a choledochal cyst with focus on the technical aspect. RESULTS: In the reoperation, meticulous dissection of the live hilum which had been previously operated on was performed The hepaticojejunostomy was left intact. With the assistance o intraoperative cholangiography, the residual pancreatic portion of the choledochal cyst was completely excised. The pancreatic opening and the lower end of the common bile duct were reconstructed. Whipple operation was avoided. CONCLUSION: Careful planning with the aid of precise imaging before and during the operation largely enhanced the accuracy of the excision of the choledochal cyst.展开更多
BACKGROUND: Choledochal cyst, an isolated defect un- restricted to the bile duct, is more appropriately regarded as the sentinel feature of a constellation of anomalies affect- ing the pancreatobiliary system. This st...BACKGROUND: Choledochal cyst, an isolated defect un- restricted to the bile duct, is more appropriately regarded as the sentinel feature of a constellation of anomalies affect- ing the pancreatobiliary system. This study was to assess the relationship between the expression of inducible nitric oxide synthase (iNOS) and the p53 gene as well as the pathoge- nesis of choledochal cysts. METHODS: iNOS and p53 were detected by immunohisto- chemistry staining in 26 patients with congenital choledo- chal cysts. Histopathologically, hyperplasia of the mucosa of the cysts and the amylase level in the bile were also inve- stigated. RESULTS: Patients with a high level of amylase in the bile had higher expression of iNOS than those with a low level of amylase. p53 protein was expressed neither in fusiform type nor in cystic type. The incidence of mucosal hyperpla- sia was significantly higher in the fusiform type than that in the cystic type. CONCLUSIONS: Higher expression of iNOS may partici- pate in hyperplasia and carcinogenesis of the mucosa of choledochal cysts. The regurgitation of pancreatic juice in- to the biliary system might induce mucosal hyperplasia of the biliary tract and inflammatory reaction. In preventing regurgitation-caused hyperplasia and malignancy of the bi- liary tract, early surgery is important for children with con- genital choledochal cysts.展开更多
Choledochal cyst (CC) is a rare, congenital anomaly of the bile ducts. We describe a 26-year-old male patient who was transferred to our hospital with a reported traumatic rupture of cystic liver lesions following a f...Choledochal cyst (CC) is a rare, congenital anomaly of the bile ducts. We describe a 26-year-old male patient who was transferred to our hospital with a reported traumatic rupture of cystic liver lesions following a fall. At the time of injury, the patient experienced severe abdominal pain. He was found to have peritonitis and abdominal hemorrhage, which is quite rare. Laparot-omy revealed 3000 mL fluid consisting of a mixture of blood, bile and inflammatory effusion in the peritoneal cavity. The liver, gallbladder, spleen, stomach, duo-denum, small intestine, and colon appeared normal. A large cystic mass was discovered near the porta hepatis. This mass, which connected to the hepatic bifurcation and gallbladder had a 5 cm rupture in the right wall with active arterial bleeding. Abdominal com-puted tomography (CT) and emergency laparotomy revealed rupture of a huge type Ⅳa CC. The patient was successfully managed by primary cyst excision, cholecystectomy, and Roux-en-Y end-to-side hepatico-jejunostomy reconstruction. The postoperative course was uneventful and the patient was discharged on the 12 th day of hospitalization. Four weeks after surgery,abdominal CT scan showed pneumatosis in the intra-hepatic bile duct, and intrahepatic dilatation which decreased following adequate biliary drainage. The patient has remained well in the close follow-up period for 9 mo.展开更多
Objective: To determine the causes of choledochal di-latation in patients with obstructive jaundice.Methods: One hundred and sixty-four patients withobstructive jaundice were investigated by endoscopicretrograde chola...Objective: To determine the causes of choledochal di-latation in patients with obstructive jaundice.Methods: One hundred and sixty-four patients withobstructive jaundice were investigated by endoscopicretrograde cholangiopancreatography(ERCP), and pa-tients with choledochal dilatation (group Ⅰ, n=110)were compared with those without choledochal dilata-tion(group Ⅱ, n=54).Results: The causes of common bile duct dilatationwere choledocholith, juxtapapillary duodenal divertic-ula and congenital dilatation of the common bile duct.The distal common bile duct and its surroundings wereabnormal in 104(94.55%) of the 110 patients and in 13(24.08%) of the 54 patients (P【0.01). Juxtapapil-lary duodenal diverticulum accounted for 24.55% ingroup Ⅰ, and only in 9.26% in group Ⅱ (P【0.05).Post-cholecystectomy patients were 13.64% in groupⅠ, and only 5.56% in group Ⅱ.Conclusions: The abnormalities of the distal commonbile duct and its surroundings can usually be detectedas underlying causes of common bile duct dilatation.ERCP is necessary before cholecystectomy, since it isconsidered the "gold standard" for the diagnosis ofdistal common bile duct abnormalities.展开更多
A case of a 24-year-old male with jaundice and epigastric pain is reported. The patient underwent a thorough clinical, laboratory, and imaging investigation. Computerized tomography revealed a 9 cm×10 cm choledoc...A case of a 24-year-old male with jaundice and epigastric pain is reported. The patient underwent a thorough clinical, laboratory, and imaging investigation. Computerized tomography revealed a 9 cm×10 cm choledochal cyst. Magnetic resonance imaging and magnetic cholangiopancreatography were performed, during which he developed an ＂acute abdomen＂ with radiological evidence of bUiary peritoneal leak. Urgent surgery revealed rupture of the distended malformed common bile duct. A peritoneal drain was instilled and a more definitive surgical procedure was accordingly scheduled. Hepatobiliary scintigraphy following surgery verified these findings, as well as confirmed the adequacy of the urgent surgery. A combination of radiological and nuclear medicine techniques substantially contributes to the diagnosis of choledochal cyst rupture and the adequacy of surgical intervention.展开更多
Choledochal cysts are a congenital anomaly, and they show dilatation of the intra- or extrahepatic biliary tree. These cysts are uncommon in Western countries, but are not rare in Asian countries. Choledochal cysts ar...Choledochal cysts are a congenital anomaly, and they show dilatation of the intra- or extrahepatic biliary tree. These cysts are uncommon in Western countries, but are not rare in Asian countries. Choledochal cysts are classified into five groups based on location or shape of the cysts. Types I and IV-A cysts are the most common types, which are associated with anomalous pancreaticobiliary junction (APBJ), but other cysts are not associated with APBJ. Types I and IV-A cysts appear to belong to a different category from other cysts embryologically. Type I and IV-A cysts accompany anomalies of the pancreas. Type I and IV-A cysts might occur when left ventral anlage persists, and with disturbed recanalization of the common bile duct. Endoscopic retrograde cholangiopancreatography is the gold standard for detecting APBJ, but it is an invasive procedure. Magnetic resonance cholangiopancreatography (MRCP) is a non-invasive imaging tool for detecting pancreatic and biliary trees. MRCP is the first-choice modality for diagnosing choledochal cysts and APBJ in pediatric patients. Cystoenterostomy is been performed because of high complication and mortality rates. Complete excision of the cysts with Roux-en-Y hepatojejunostomy is a standard procedure for choledochal cysts to prevent postoperative complications, including development of cancer. In this study, we review classification, pathogenesis, diagnosis, and treatment of types I and IV-A choledochal cysts.展开更多
文摘BACKGROUND Type Ⅲ choledochal cysts(CCs)are extremely rare,and they present as dilatations and herniations of the end of the common bile duct into the duodenum.Moreover,type Ⅱ CCs may be easily misdiagnosed as intraduodenal polyps or tumors.Thus,adequate differential diagnosis and selection of appropriate treatment are important.CASE SUMMARY A young man with a duodenal mass presented with 3-year intermittent abdominal pain and acute pancreatitis 3 days before hospitalization.After evaluation by magnetic resonance imaging and endoscopic ultrasonography,the duodenal papilla was pressed,and the bile flowed out slowly,which was speculated to be the cause of his symptoms.The lesion was punctured with a submucosal injection needle,and golden clear fluid was aspirated.Laboratory tests of the aspirate after 50-fold dilution revealed significantly elevated total bilirubin,direct bilirubin,amylase and lipase.Taken together,these findings confirmed that the lesion was a type Ⅲ CC.The patient underwent fused surgical procedures.Fenestration plus internal drainage of the lesion was subsequently performed with a DualKnife.After drainage,the incision was sealed with tissue clips.During follow-up,the patient recovered well,and no abdominal pain symptoms or acute pancreatitis recurred.CONCLUSION Laboratory tests of cyst aspirates are beneficial for diagnosis,and endoscopic fenestration plus internal drainage works well to mitigate cysts.
基金Supported by Cuiying Scientific and Technological Innovation Program of Lanzhou University Second Hospital,No.CY2024-MS-B04Gansu Provincial Natural Science Foundation Project,No.24JRRA331the Traditional Chinese Medicine Research Project of Gansu Province,No.GZKZ-2024-26.
文摘BACKGROUND Choledochal cyst is a rare biliary tract disorder with five subtypes categorized based on the anatomical location of cystic dilatation.Type IV(which affects the intrahepatic and extrahepatic ducts)is the most common subtype in adults.Its clinical manifestations are diverse and the disease can potentially become malignant.Currently,the mainstay treatment is surgical excision which is used as a definitive treatment to prevent complications and avoid carcinogenesis.However,the surgical operation for giant choledochal cysts is technically challenging.CASE SUMMARY Here,we present a case of a giant choledochal cyst in an 18-year-old female.The diagnosis of patient was confirmed through magnetic resonance cholangiopancreatography.The patient developed acute cholangitis,and was treated with percutaneous transhepatic cholangial drainage to alleviate symptoms.The final treatment approach was cyst excision followed by Roux-en-Y hepaticojejunostomy,which successfully achieved excellent postoperative recovery.CONCLUSION Ultrasonography and magnetic resonance cholangiopancreatography can effectively diagnose choledochal cysts.Combined percutaneous transhepatic cholangial drainage surgery for giant choledochal cysts is safe and effective.
基金Supported by the Beijing Municipal Science and Technology Commission,No.Z191100006619002Haiyou Health High-Caliber Talent Project,No.202412the Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment,Chinese Academy of Medical Sciences,No.2021RU015.
文摘BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.
文摘Choledochal cysts(CCs)are rare bile duct dilatations,intra-and/or extrahepatic,and have higher prevalence in the Asian population compared to Western populations.Most of the current literature on CC disease originates from Asia where these entities are most prevalent.They are thought to arise from an anomalous pancreaticobiliary junction,which are congenital anomalies between pancreatic and bile ducts.Some similarities in presentation between Eastern and Western patients exist such as female predominance,however,contemporary studies suggest that Asian patients may be more symptomatic on presentation.Even though CC disease presents with an increased malignant risk reported to be more than 10%after the second decade of life in Asian patients,this risk may be overstated in Western populations.Despite this difference in cancer risk,management guidelines for all patients with CC are based predominantly on observations reported from Asia where it is recommended that all CCs should be excised out of concern for the presence or development of biliary tract cancer.
文摘AIM: To summarize the experience of diagnosis and treatment of congenital choledochal cyst in the past 20 years (1980-2000). METHODS: The clinical data of 108 patients admitted from 1980 to 2000 were analyzed retrospectively. RESULTS: Abdominal pain,jaundice and abdominal mass were presented in most child cases. Clinical symptoms in adult cases were non-specific, resulting in delayed diagnosis frequently. Fifty-seven patients (52.7%) had coexistent pancreatiobiliary disease. Carcinoma of the biliary duct occurred in 18 patients (16.6%).Ultrasonic examination was undertaken in 94 cases, ERCP performed in 46 cases and CT in 71 cases. All of the cases were correctly diagnosed before operation. Abnormal pancreatobiliary duct junction was found in 39 patients. Before 1985 the diagnosis and classification of congenital choledochal cyst were established by ultrasonography preoperatively and confirmed during operation, the main procedures were internal drainage by cyst enterostomy. After 1985, the diagnosis was established by ERCP and CT, and cystectomy with Roux-en-Y hepaticojejunostomy was the conventional procedures.In 1994, we reported a new and simplified operative procedure in order to reduce the risk of choledochal cyst malignancy. Postoperative complication was mainly retrograde infection of biliary tract, which could be controlled by the administration of antibiotics, there was no perioperative mortality. CONCLUSION: The concept in diagnosis and treatment of congenital choledochal cyst has obviously been changed greatly.CT and ERCP were of great help in the classification of the disease.Currently, cystectomy with Roux-en-Y hepaticojejunostomy is strongly recommended as the choice for patients with type I and type IV cysts. Piggyback orthotopic liver transplantation is indicated in type V cysts (Caroli's disease) with frequently recurrent cholangitis.
文摘We report the first case series from Africa and the Middle East on choledochal cyst, a disease which shows significant geographical distribution with high incidence in the Asian population. In this study, the epidemiological data of the patients are presented and analyzed. Attention was paid to diagnostic imaging and its accuracy in the diagnosis and classification of choledochal cyst. Most cases of choledochal cyst disease have type I and IV-A cysts according to the Todani classification system, which support the etiological theories of choledochal cyst, especially Babbitt’s theory of the anomalous pancreaticobiliary duct junction, which are clearly stated. The difficulties and hazards of surgical management and methods used to avoid operative complications are clarified. Early and late postoperative complications are also included. This study should be followed by multicenter studies throughout Egypt to help assess the incidence of choledochal cysts in one of the largest populations in Africa and the Middle East.
文摘AIM:To determine the merits of magnetic resonance cholangiopancreatography(MRCP) as the primary diagnostic test for choledochal cysts(CC’s).METHODS:Between 2009 and 2012,patients who underwent MRCP for perioperative diagnosis were identified.Demographic information,clinical characteristics,and radiographic findings were recorded.MRCP results were compared with intraoperative findings.A PubMed search identified studies published between 1996-2012,employing MRCP as the primary preoperative imaging and comparing results with either endoscopic retrograde cholangiopancreatography(ERCP) or operative findings.Detection rates for CC’s and abnormal pancreaticobiliary junction(APBJ) were calculated.In addition detection rates for clinically related biliary pathology like choledocholithiasis and cholangiocarcinomas in patients diagnosed with CC’s were also evaluated.RESULTS:Eight patients were identified with CC’s.Six patients out of them had typeⅣCC’s,1 had type I and 1 had a new variant of choledochal cyst with confluent dilatation of the common bile duct(CBD) and cystic duct.Seven patients had an APBJ and 3 of those had a long common-channel.Gallstones were found in 2 patients,1 had a CBD stone,and 1 pancreatic-duct stone was also detected.In all cases,MRCP successfully identified the type of CC’s,as well as APBJ with ductal stones.From analyzing the literature,we found that MRCP has 96%-100% detection rate for CC’s.Additionally,we found that the range for sensitivity,specificity,and diagnostic accuracy was 53%-100%,90%-100% and 56%-100% in diagnosing APBJ.MRCP’s detection rate was 100% for choledocholithiasis and 87% for cholangiocarcinomas with concurrent CC’s.CONCLUSION:After initial ultrasound and computed tomography scan,MRCP should be the next diagnostic test in both adult and pediatric patients.ERCP should be reserved for patients where therapeutic intervention is needed.
文摘AIM: To investigate the relationship between pancreatic amylase in bile duct and the clinico-pathological features in adult patients with choledochal cyst and anomalous pancreatico-biliary ductal union (APBDU).METHODS: From 39 patients who underwent surgery for choledochal cyst between March 1995 and March 2003,we selected 15 adult patients who had some symptoms and were radiologically diagnosed as APBDU, and their clinico-pathological features were subsequently evaluated retrospectively. However, we could not obtain biliary amylase in all the patients because of the surgeon's slip.Therefore, we measured the amylase level in gall bladder of 10 patients and in common bile duct of 11 patients.RESULTS: Levels of amylase in common bile duct and gall bladder ranged from 11 500 to 212 000 IU/L, and the younger the patients, the higher the biliary amylase level (r= -0.982, P＜0.01). Pathologically, significant correlation was found between the size of choledochal cyst and the grade of inflammation (r= 0.798,P＜0.01). And, significant correlation was found between the level of amylase in gall bladder and the grade of hyperplasia. On the other hand, there was no correlation to the age of symptomatic onset or inflammatory grade (r = 0.743, P＜0.05). Level of lipase was elevated from 6 000 to 159 000 IU/L in bile duct and from 14 400 to 117 000 IU/L in the gall bladder;however, there was no significant correlation with age or clinico-pathological features.CONCLUSION: The results support the notion that amylase has a particular role in the onset of symptoms, and suggest that a large amount of biliary amylase induces early onset of symptom, thereby making early diagnosis possible.
基金Supported by the PLA general hospital Clinical Support Grant,No.2017FC-TSYS-3010
文摘Our paper describes the key surgical points of pediatric choledochocystectomy performed completely by Da Vinci robotic system. A choledochocystectomy was safely carried out for a girl at our hospital, and without any complication. Then systematic literature review was done to discuss the methods of intestine surgery and intestinal anastomosis, the use of 3 rd robotic arm, the surgical safety and advantages comparing open and laparoscopic surgery. We systematically reviewed choledochocystectomy for children performed by robotic surgery. We included a total of eight domestic and foreign reports and included a total of 86 patients, whose average age was 6.3(0.3-15.9) years; the maleto-female ratio was 1:3.5(19:67). Seven patients experienced conversion to open surgery, and the surgery success rate was 91.9%(79/86). The average total operation time was 426(180-520) min, the operation time on the machine was 302(120-418) min, 11 cases used the number 3 arm, and the remaining mainly used the hitch-stitch technique to suspend the stomach wall and liver. Forty-seven patients underwent pull-through intestine and intestinal anastomosis, and 39 patients underwent complete robotic intestine surgery and intestinal anastomosis. The hospitalization time of roboticassisted choledochocystectomy was 8.8 d. Eight patientshad biliary fistula and were all cured by conservative treatment and continuous observation. One patient had anastomotic stenosis, and one patient had wound dehiscence, both cured by surgery. Choledochocystectomy for children performed by completely robotic surgery and Roux-en-Y hepaticojejunostomy is safe and feasible. The initial experience shows that this surgical approach has a clearer field than the traditional endoscopy, and its operation is more flexible, the surgery is more accurate, and the injury is smaller. With the advancement of technology and the accumulation of surgeons' experience, robotic surgery may become a new trend in this surgical procedure.
文摘Objective: To retrospectively study the early compli- cations of excision with hepaticoenterostomy for in- fants and children with choledochal cysts. Methods: We analyzed 16 patients with early posto- perative complications out of 173 patients with con- genital choledochal cysts aged 27 days to 14 years (mean 2.4 years) who had undergone excisional pro- cedures and biliary tract reconstruction. Results: The early complications included bile leak- age (10 patients), abdominal wall dehiscence (3), and hepatic failure, pancreatic juice leakage and postoperative intussusception (each in 1) respective- ly. Three patients died from bile leakage and 1 from postoperative hepatic failure. No statistical differen- ces were observed between the procedures of biliary tract reconstruction with jejunal segment interposi- tion hepaticoduodenostomy and Roux-en-Y hepatico- jejunostomy (P>0.75). The morbidity was signifi- cantly higher in infants below 1 year than in children (P<0.005). Prevention and treatment of the com- plications were discussed. Conclusion: Bile leakage and abdominal wall dehis- cence are major early postoperative complications. The morbidity of cholechal cysts is higher in infants than in children. Exploratory laparotomy should not be delayed when biliary leakage with diffuse peritoni- tis appears. The 'tension suture in the fascial space of the abdominal wall' is useful to prevent and treat wound dehiscence.
文摘Objective: To evaluate the procedures and timing of operation as well as long-term postoperative effect of congenital choledochal cysts (CCC) in adults. Methods: The procedures and timing of operation, effective rate, re-operation rate and incidence of car- cinoma after operation for 70 adult patients with CCC from January 1980 to June 1999 were analyzed retrospectively. Results: The re-operation rate of external drainage was 86% (6/7). The effective rate of internal drain- age was significantly lower than that of cyst resection (3/10 vs 45/49, X^2=20. 94, P<0.001). The re-op- eration rate and incidence of carcinoma of internal drainage were higher than those of cyst resection (5/ 10 vs 3/49, X^2=13. 64, P<0. 001 and 3/10 vs 3/49, X^2=5. 18, P<0. 025). The reoperation rate of e- mergency surgery was higher than that of selective operation (8/10 vs 6/56, X^2=24. 37, P<0.001). Conclusions: External drainage should be the first- aid measure and the therapy of choice on emergency basis. Internal drainage should never be attempted. Cyst resection with Roux-en-Y hepaticojejunostomy is recommended as the treatment of choice in selective operation.
文摘Choledochal cysts are congenital anomalies of the biliary ducts, characterized by cystic dilatation of the ducts.Prenatal diagnosis of this anomaly using ultrasonography (US) has been well documented. Magnetic resonance imaging (MRI) has recently become an important complement to US in prenatal diagnosis of fetal anomalies. We herein report a patient in whom at 24 wk' gestation US suggested a right upper quadrant abdominal cyst and in whom at 26 wk' gestation MRI more clearly delineated the cyst and its surrounding structures and suggested a choledochal cyst, which was confirmed at postnatal surgery and histopathology.
文摘BACKGROUND:Choledochal cysts in adults are more commonly associated with complications such as cystolithiasis,recurrent cholangitis,portal hypertension and malignancy,than in the pediatric age group.METHOD:We report a case of adult choledochal cyst with long-term complication of large stone cast and portal hypertension due to secondary biliary cirrhosis.RESULTS:A 50-year-old patient presented with obstructive jaundice and hepatosplenomegaly.On investigation,she was diagnosed as having a choledochal cyst with large stone cast and portal hypertension.Single stage resection of the choledochal cyst with Roux-en-Y hepaticojejunostomy was done by meticulous dissection and ligation of collaterals.CONCLUSION:Single stage resection of a choledochal cyst is possible in spite of associated portal hypertension,if the portal vein is patent.
文摘BACKGROUND: Choledochal cysts are congenital cystic dilatations of the extrahepatic or intrahepatic portion of the biliary tree. Complete excision of choledochal cysts is currently regarded as the gold standard treatment, while less extensive procedures including cystoduodenostomy have become obsolete due to the potential for malignant change in the remnant cyst. For type-1 choledochal cysts, which sometimes extend to the main pancreatic duct closely, some surgeons may adopt a less aggressive approach in order to avoid damage to the main pancreatic duct as such damage can lead to serious consequences. However, incomplete excision of choledocha cysts may also cause problems. METHOD: Here we report on a reoperation treating incomplete excision of a choledochal cyst with focus on the technical aspect. RESULTS: In the reoperation, meticulous dissection of the live hilum which had been previously operated on was performed The hepaticojejunostomy was left intact. With the assistance o intraoperative cholangiography, the residual pancreatic portion of the choledochal cyst was completely excised. The pancreatic opening and the lower end of the common bile duct were reconstructed. Whipple operation was avoided. CONCLUSION: Careful planning with the aid of precise imaging before and during the operation largely enhanced the accuracy of the excision of the choledochal cyst.
文摘BACKGROUND: Choledochal cyst, an isolated defect un- restricted to the bile duct, is more appropriately regarded as the sentinel feature of a constellation of anomalies affect- ing the pancreatobiliary system. This study was to assess the relationship between the expression of inducible nitric oxide synthase (iNOS) and the p53 gene as well as the pathoge- nesis of choledochal cysts. METHODS: iNOS and p53 were detected by immunohisto- chemistry staining in 26 patients with congenital choledo- chal cysts. Histopathologically, hyperplasia of the mucosa of the cysts and the amylase level in the bile were also inve- stigated. RESULTS: Patients with a high level of amylase in the bile had higher expression of iNOS than those with a low level of amylase. p53 protein was expressed neither in fusiform type nor in cystic type. The incidence of mucosal hyperpla- sia was significantly higher in the fusiform type than that in the cystic type. CONCLUSIONS: Higher expression of iNOS may partici- pate in hyperplasia and carcinogenesis of the mucosa of choledochal cysts. The regurgitation of pancreatic juice in- to the biliary system might induce mucosal hyperplasia of the biliary tract and inflammatory reaction. In preventing regurgitation-caused hyperplasia and malignancy of the bi- liary tract, early surgery is important for children with con- genital choledochal cysts.
文摘Choledochal cyst (CC) is a rare, congenital anomaly of the bile ducts. We describe a 26-year-old male patient who was transferred to our hospital with a reported traumatic rupture of cystic liver lesions following a fall. At the time of injury, the patient experienced severe abdominal pain. He was found to have peritonitis and abdominal hemorrhage, which is quite rare. Laparot-omy revealed 3000 mL fluid consisting of a mixture of blood, bile and inflammatory effusion in the peritoneal cavity. The liver, gallbladder, spleen, stomach, duo-denum, small intestine, and colon appeared normal. A large cystic mass was discovered near the porta hepatis. This mass, which connected to the hepatic bifurcation and gallbladder had a 5 cm rupture in the right wall with active arterial bleeding. Abdominal com-puted tomography (CT) and emergency laparotomy revealed rupture of a huge type Ⅳa CC. The patient was successfully managed by primary cyst excision, cholecystectomy, and Roux-en-Y end-to-side hepatico-jejunostomy reconstruction. The postoperative course was uneventful and the patient was discharged on the 12 th day of hospitalization. Four weeks after surgery,abdominal CT scan showed pneumatosis in the intra-hepatic bile duct, and intrahepatic dilatation which decreased following adequate biliary drainage. The patient has remained well in the close follow-up period for 9 mo.
文摘Objective: To determine the causes of choledochal di-latation in patients with obstructive jaundice.Methods: One hundred and sixty-four patients withobstructive jaundice were investigated by endoscopicretrograde cholangiopancreatography(ERCP), and pa-tients with choledochal dilatation (group Ⅰ, n=110)were compared with those without choledochal dilata-tion(group Ⅱ, n=54).Results: The causes of common bile duct dilatationwere choledocholith, juxtapapillary duodenal divertic-ula and congenital dilatation of the common bile duct.The distal common bile duct and its surroundings wereabnormal in 104(94.55%) of the 110 patients and in 13(24.08%) of the 54 patients (P【0.01). Juxtapapil-lary duodenal diverticulum accounted for 24.55% ingroup Ⅰ, and only in 9.26% in group Ⅱ (P【0.05).Post-cholecystectomy patients were 13.64% in groupⅠ, and only 5.56% in group Ⅱ.Conclusions: The abnormalities of the distal commonbile duct and its surroundings can usually be detectedas underlying causes of common bile duct dilatation.ERCP is necessary before cholecystectomy, since it isconsidered the "gold standard" for the diagnosis ofdistal common bile duct abnormalities.
文摘A case of a 24-year-old male with jaundice and epigastric pain is reported. The patient underwent a thorough clinical, laboratory, and imaging investigation. Computerized tomography revealed a 9 cm×10 cm choledochal cyst. Magnetic resonance imaging and magnetic cholangiopancreatography were performed, during which he developed an ＂acute abdomen＂ with radiological evidence of bUiary peritoneal leak. Urgent surgery revealed rupture of the distended malformed common bile duct. A peritoneal drain was instilled and a more definitive surgical procedure was accordingly scheduled. Hepatobiliary scintigraphy following surgery verified these findings, as well as confirmed the adequacy of the urgent surgery. A combination of radiological and nuclear medicine techniques substantially contributes to the diagnosis of choledochal cyst rupture and the adequacy of surgical intervention.
文摘Choledochal cysts are a congenital anomaly, and they show dilatation of the intra- or extrahepatic biliary tree. These cysts are uncommon in Western countries, but are not rare in Asian countries. Choledochal cysts are classified into five groups based on location or shape of the cysts. Types I and IV-A cysts are the most common types, which are associated with anomalous pancreaticobiliary junction (APBJ), but other cysts are not associated with APBJ. Types I and IV-A cysts appear to belong to a different category from other cysts embryologically. Type I and IV-A cysts accompany anomalies of the pancreas. Type I and IV-A cysts might occur when left ventral anlage persists, and with disturbed recanalization of the common bile duct. Endoscopic retrograde cholangiopancreatography is the gold standard for detecting APBJ, but it is an invasive procedure. Magnetic resonance cholangiopancreatography (MRCP) is a non-invasive imaging tool for detecting pancreatic and biliary trees. MRCP is the first-choice modality for diagnosing choledochal cysts and APBJ in pediatric patients. Cystoenterostomy is been performed because of high complication and mortality rates. Complete excision of the cysts with Roux-en-Y hepatojejunostomy is a standard procedure for choledochal cysts to prevent postoperative complications, including development of cancer. In this study, we review classification, pathogenesis, diagnosis, and treatment of types I and IV-A choledochal cysts.
