Preliminary exploration of animal models of congenital choledochal cysts

BACKGROUND Various animal models have been used to explore the pathogenesis of choledochal cysts(CCs),but with little convincing results.Current surgical techniques can achieve satisfactory outcomes for treatment of CCs.Consequently,recent studies have focused more on clinical issues rather than basic research.Therefore,we need appropriate animal models to further basic research.AIM To establish an appropriate animal model that may contribute to the investigation of the pathogenesis of CCs.METHODS Eighty-four specific pathogen-free female Sprague-Dawley rats were randomly allocated to a surgical group,sham surgical group,or control group.A rat model of CC was established by partial ligation of the bile duct.The reliability of the model was confirmed by measurements of serum biochemical indices,morpho-logy of common bile ducts of the rats as well as molecular biology experiments in rat and human tissues.RESULTS Dilation classified as mild(diameter,≥1 mm to<3 mm),moderate(≥3 mm to<10 mm),and severe(≥10 mm)was observed in 17,17,and 2 rats in the surgical group,respectively,while no dilation was observed in the control and sham surgical groups.Serum levels of alanine aminotransferase,aspartate aminotrans-ferase,total bilirubin,direct bilirubin,and total bile acids were significantly elevated in the surgical group as compared to the control group 7 d after surgery,while direct bilirubin,total bilirubin,and gamma-glutamyltransferase were further increased 14 d after surgery.Most of the biochemical indices gradually decreased to normal ranges 28 d after surgery.The protein expression trend of signal transducer and activator of transcription 3 in rat model was consistent with the human CC tissues.CONCLUSION The model of partial ligation of the bile duct of juvenile rats could morphologically simulate the cystic or fusiform CC,which may contribute to investigating the pathogenesis of CC.

Maternal choledochal cysts in pregnancy:A systematic review of case reports and case series

BACKGROUND Choledochal cysts(CC)are cystic dilatations of the biliary tract,usually diagnosed during childhood,with an estimated incidence in the general population of 1:100000.Complications related to CC include rupture,biliary obstruction,and cholangitis.Maternal CC in pregnancy are rarely reported,and there are no guidelines on optimal management.AIM To systematically review maternal CC diagnosed during pregnancy or postpartum with regard to the clinical presentation of CC,the mode of treatment and delivery,and maternal outcomes.METHODS A literature search of cases and case series of maternal CC in pregnancy and postpartum was conducted using MEDLINE/PubMed,Web of Science,Google Scholar,and Embase.There were no restrictions on language or publication year.Databases were lastly accessed on September 1,2022.RESULTS Overall,71 publications met the inclusion criteria,reporting 97 cases.Eighty-eight cases were diagnosed during pregnancy and nine in the puerperium.The most common symptoms were abdominal pain(81.2%)and jaundice(60.4%).Interventions for CC complications were required in 52.5%of the cases,and 34%of pregnancies were induced.Urgent cesarean section(CS)was done in 24.7%.The maternal mortality was 7.2%,while fetal mortality was inconsistently reported.Cholangitis,CC>15 cm,and bilirubin levels>80 mmol/L were associated with a higher likelihood of urgent CS and surgical intervention for CC.Bilirubin levels positively correlated with CC size.There was no correlation between age and cyst dimension,gestational age at cyst discovery,and CC size.CONCLUSION Although rare,maternal CC in pregnancy should be included in the evaluation of jaundice with upper abdominal pain.Symptomatology and clinical course are variable,and treatment may range from an expectative approach to emergent surgical CC treatment and urgent CS.While most cases were managed by conservative measures or drainage procedures,CC>15 cm and progressive cholangitis carry the risk of CC rupture and septic complications,which may increase the rates of unfavorable maternal and fetal outcomes.Therefore,such cases require specific surgical and obstetric interventions.

Early complications after excision with hepaticoenterostomy for infants and children with choledochal cysts

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.

Procedures for congenital choledochal cysts and curative effect analysis in adults

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.

Expressions of p53 and inducible nitric oxid synthase in congenital choledochal cysts

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.

Technical note on complete excision of choledochal cysts

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.

Congenital choledochal cysts in adults:twenty-five-year experience

Background Choledochal cyst is rare in western countries. The relatively high incidence of coexistent hepatobiliary disease increases the difficulty of the surgical management of choledochal cyst. Here we analyze the diagnosis and treatment of congenital bile duct cyst in 122 Chinese adults. Methods The clinical data of 122 patients with congenital choledochal cysts admitted from 1981 to 2006 were analyzed. Results Clinical symptoms in most cases were nonspecific, resulting in delayed diagnosis. Sixty-one patients （50%） had coexistent pancreatobiliary disease. Among the 122 patients, 119 patients underwent ultrasonic examination; ERCP/MRCP was performed in 63 cases and CT in 102 cases. Abnormal pancreatobiliary duct junction was found in 48 patients. Sixteen patients had malignant lesions in the bile duct, arising in 11 of them from incomplete choledochal cyst that underwent various operations including cystenterostomy or cystojejunostomy There was significant difference between the patients who underwent incomplete cyst resection and complete cyst resection in malignancy rate of bile duct （Chi square test, P= 0.000; odds ratio, 7.800; 95 % confidence interval, 2.450 to 24.836）. Conclusions ERCP, CT and MRCP had proved their great values in the classification of the disease. Cyst excision with Roux-en-Y hepaticojejunostomy is recommended as the treatment of choice for patients with type Ⅰ or type Ⅳ cysts. For type Ⅴ cyst （Caroli＇s disease） with recurrent cholangitis, liver transplantation should be considered.

Coagulopathy in a subtype of choledochal cyst and management strategy

AIM: To evaluated our management algorithm of the coagulopathy. We evaluated our management algorithm of the coagulopathy.

Diseases of bile duct in children

Several diseases originate from bile duct pathology.Despite studies on these diseases,certain etiologies of some of them still cannot be concluded.The most common disease of the bile duct in newborns is biliary atresia,whose prognosis varies according to the age of surgical correction.Other diseases such as Alagille syndrome,inspissated bile duct syndrome,and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction.The majority of these diseases present with cholestatic jaundice in the newborn or infant period,which is quite difficult to differentiate regarding clinical acumen and initial investigations.Intraoperative cholangiography is potentially necessary to make an accurate diagnosis,and further treatment will be performed synchronously or planned as findings suggest.This article provides a concise review of bile duct diseases,with interesting cases.

Current status of the biliary tract malformation

The choledochal cyst(CC)can be better termed as biliary tract malformation because of the close association of embryology and etiology in the causation of CC.Contrary to Babbitt's postulation of reflux,damage and dilatation,reflux was not demonstrable as the causative factor in all varieties of CC.High pressure in the biliary system,otherwise termed ductal hypertension,is put forth as an alternative to explain the evolution of CC.The forme fruste type,which does not find a place in the standard classification,typifies the ductal hypertension hypothesis.Hence a closer,in-depth review would be able to highlight this apt terminology of biliary tract malformation.

Diagnosis and treatment of congenital choledochal cyst: 20 years' experience in China

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.

Diagnosis and management of choledochal cyst: 20 years of single center experience

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&#x02005;I&#x02005;and IV-A cysts according to the Todani classification system, which support the etiological theories of choledochal cyst, especially Babbitt&#x02019;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.

Amylase level in extra hepatic bile duct in adult patients with choledochal cyst plus anomalous pancreatico-biliary ductal union

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.

Prenatal diagnosis of choledochal cyst using magnetic resonance imaging: A case report

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.

Choledochal cyst with large stone cast and portal hypertension

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.

Spontaneous rupture of a type IVA choledochal cyst in a young adult during radiological imaging

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.

Cystic metastasis from a mucinous adenocarcinoma of duodenum mimicking type Ⅱ choledochal cyst: A case report

A 51-year-old male patient was referred to our hospital because of an incidentally detected cystic mass near the common bile duct(CBD). Imaging studies demonstrated a cystic mass that was suspected to communicate with the CBD. Gastroscopy showed irregular mucosal thickening with hyperemic change in the second portion of the duodenum. A type Ⅱ choledochal cyst combined with duodenal malignancy was suspected. The patient underwent surgical resection and the histological diagnosis was mucinous adenocarcinoma of the duodenum with cystic metastasis. Although its incidence is extremely rare, care should be taken to check for other sites of malignancy when a pericholedochal cystic mass is detected.

Computed tomography virtual endoscopy with angiographic imaging for the treatment of type Ⅳ-A choledochal cyst

Type Ⅳ-A choledochal cysts (CCs) are a congenital biliary anomaly which involve dilatation of the extrahepatic and intrahepatic bile ducts. We present the case of a 30-year-old woman with type Ⅳ-A CC, on whom threedimensional computed tomography (3D CT) and virtual endoscopy were performed. 3D CT revealed partial dilatation in the posterior branch of the intrahepatic bile duct and a relative stricture between it and the extrahepatic bile duct. Virtual endoscopy showed that this stricture was membrane-like and separated from the surrounding blood vessels. Based on these image findings, complete cyst resection, bile duct plasty for the stricture, and hepaticojejunostomy were safely performed. To the best of our knowledge, there are no reports of imaging by virtual endoscopy of the biliary tract which show the surrounding blood vessels running along the bile duct.

Risk factors for preoperative carcinogenesis of bile duct cysts in adults

BACKGROUND Bile duct cyst(BDC)is a rare congenital bile duct malformation.The incidence of bile duct malignancy in BDC patients is markedly higher than that in the general population.However,few studies have been conducted on the risk factors for preoperative carcinogenesis in BDC patients.AIM To analyze the risk factors associated with preoperative carcinogenesis in BDC patients.METHODS The medical records of BDC patients treated at our hospital between January 2012 and December 2018 were retrospectively reviewed.We constructed a database and compared the characteristics of BDC patients with dysplasia and carcinoma against those with benign cysts.The risk factors for preoperative carcinogenesis were identified using univariate and multivariate analyses.RESULTS The cohort comprised 109 BDC patients.Ten patients had preoperative dysplasia or adenocarcinoma.Univariate and multivariate analyses showed that gallbladder wall thickness>0.3 cm[odds ratio(OR),6.551;95%confidence interval(CI),1.351 to 31.763;P=0.020]and Todani type IV(OR,7.675;95%CI,1.584 to 37.192;P=0.011)were independent factors associated with preoperative carcinogenesis.CONCLUSION BDC is a premalignant condition.Our findings show that gallbladder wall thickness>0.3 cm and Todani type IV are independent risk factors for preoperative carcinogenesis of BDC.They are therefore useful for deciding on the appropriate treatment strategy,especially in asymptomatic patients.

Mixed type Ⅰ and Ⅱ choledochal cyst in an adult

BACKGROUND:Choledochal cysts are classified into five types based on the location of the cyst.Mixed types of choledochal cysts are extremely rare.Only five cases of mixed typeⅠandⅡcholedochal cysts have been reported, of which one was an adult case.We report a mixed typeⅠ andⅡcholedochal cyst in a 25-year-old man. METHODS:The unusual nature of the choledochal cyst, suspected on magnetic resonance cholangiopancreatography RCP to be typeⅠinitially,was confirmed by laparotomy to be a mixed typeⅠ+Ⅱcyst.Excision of the cyst and hepaticojejunostomy were performed. RESULT:The operation was uneventful,and the patient recovered well. CONCLUSIONS:Mixed type choledochal cysts are rare, and may be missed on imaging,unless careful evaluation is done.The operative method may not need to be modified significantly,as in the management of our case.