Bile duct cyst in adults:Interventional treatment,resection,or transplantation?

Cystic dilatations of the bile ducts may be found along the extrahepatic biliary tree,within the liver,or in both of these locations simultaneously.Presentation in adults is often associated with complications.The therapeutic possibilities have changed considerably over the last few decades.If possible,complete resection of the cyst(s)can cure the symptoms and avoid the risk of malignancy.According to the type of bile duct cyst,surgical procedures include the Roux-en-Y hepaticojejunostomy and variable types of hepatic resection.However,the diffuse forms of Todani type Ⅴ cysts(Caroli disease and Caroli syndrome)in particular remain a therapeutic problem,and liver transplantation has become an important option.The mainstay of interventional treatment for Todani typeⅢbile duct cysts is via endoscopic retrograde cholangiopancreatography.The diagnostic term"bile duct cyst"comprises quite different pathological and clinical entities.Interventional therapy,hepatic resection,and liver transplantation all have their place in the treatment of this heterogeneous disease group.They should not be seen as competitive treatment modalities,but as complementary options.Each patient should receive individualized treatment after all of the clinical findings have been considered by an interdisciplinary team.

A rare case of bile duct cyst

Choledochal cyst is an uncommon disease usually seen in young women and can be divided into five types. We report a 66-year-old woman who was diagnosed with types and bile duct cyst simultaneously after surgery, which is a rare type of bile duct cyst.

A case of peribiliary cysts accompanying bile duct carcinoma

A rare case of peribiliary cysts accompaying bile duct carcinoma is presented. A 54-year-old man was diagnosed as having lower bile duct carcinoma and peribiliary cysts by diagnostic imaging. He underwent pylorus preserving pancreatoduodenectomy. As for the peribiliary cysts, a course of observation was taken. Over surgery due to misdiagnosis of patients with biliary malignancy accompanied by peribiliary cysts should be avoided.

The Extra-and Intra-Hepatic Ducts of Congenital Choledochal Cyst in Infants and Children

Congenital choledochal cyst is frequently seen ininfants and children in China. The extra-hepatic ductalchanges have been well described, but the changes in theintra-hepatic ducts in choledochal cyst are less well known.This report is composed of 2 groups of patients: thirty-four cases who were operated upon between 1971 and1982 were followed post-operatively by plain abdominalfilm and barium meal examination and a group of 26cases receiving PTC between 1982 and 1984. In this lattergroup, both extra-and intra-hepatic ducts were shownsatisfactorily by PTC. In 23 cases (88.5%) besides thedilatation of the extra-hepatic duct, there were differentdegree and different forms of dilatation of the intra-hepatic ducts. In 10 cases(38.5%)the branches peripheralto the dilated main intra-hepatic ducts were found to benormal, in the other 10 cases (38.5%) the more peripheralbranches abruptly narrowed or were obliterated. In one case(3.8%) the entire intra-hepatic ductal system were dilatedwith a bead-like appearance. In only 3 cases were theintra-hepatic ducts normal (11.5%). Moreover the dilata-tion of the intra-hepatic ducts were found to persist longafter the obstruction was relieved in 28 cases (82.3%) ofthe 34 post-operative cases. Due to the frequent associationof intra-hepatic ductal dilatation with choledochal cyst,the various forms of dilatation and persistance of dilata-tion after relief of the obstruction, the term congenital biliary dilatation is suggested for this disease.

Common Bile Duct Cysts Leading to Cholestasis and Jaundice: A Rare Case Report

Cystic dilatation of the common bile duct constitutes a rare congenital malformation prevalent in people of Asian origin with a female predominance. Its classification follows that of Todani. The commonest form, found in 80% of cases, is type I. Common bile duct cysts are often diagnosed in 2 out of 3 cases, during childhood with adult forms often rare. We hereby report the case of a cystic dilatation of the bile duct in a 75-year-old patient without significant history of disease.

Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts

AIM:To analyze the impact of previous cyst-enterostomy of patients underwent congenital bile duct cysts(BDC)resection.METHODS:A multicenter European retrospective study between 1974 and 2011 were conducted by the French Surgical Association.Only Todani subtypes I and IVb were included.Diagnostic imaging studies and operative and pathology reports underwent central revision.Patients with and without a previous history of cystenterostomy(CE)were compared.RESULTS:Among 243 patients with Todani types I and IVb BDC,16 had undergone previous CE(6.5%).Patients with a prior history of CE experienced a greater incidence of preoperative cholangitis(75%vs 22.9%,P<0.0001),had more complicated presentations(75%vs 40.5%,P=0.007),and were more likely to have synchronous biliary cancer(31.3%vs 6.2%,P=0.004)than patients without a prior CE.Overall morbidity(75%vs 33.5%;P<0.0008),severe complications(43.8%vs 11.9%;P=0.0026)and reoperation rates(37.5%vs 8.8%;P=0.0032)were also significantly greater in patients with previous CE,and their Mayo Risk Score,during a median follow-up of 37.5 mo(range:4-372 mo)indicated significantly more patients with fair and poor results(46.1%vs 15.6%;P=0.0136).CONCLUSION:This is the large series to show that previous CE is associated with poorer short-and longterm results after Todani types I and IVb BDC resection.

An Unusual and Previously Unreported Association between Tyrosinemia Type 1 and an Extremely Rare Variation of Congenital Cystic Dilatation: TODANI’s VI Cystic Duct Cyst: Report of a Case

Cystic duct cysts are rare lesions, and type VI of TODANI’s modified classification is the rarest subtype with only sporadic case reports in the literature. The following report describes the coexistence of this entity and type 1 tyrosinemia in a one month-old infant referred to our department for etiological investigations of prolonged neonatal cholestasis. To the best of our knowledge, we report herein the first case in the literature describing this association. Cystic duct abnormalities should be considered in the differential diagnosis of neonatal cholestases, however further investigations should be performed to exclude associated life-threatening conditions such as metabolic disorders including tyrosinemia.

Cystic low-grade collecting duct renal carcinoma with liver compression—A challenging diagnosis and therapy: A case report

BACKGROUND A collecting duct carcinoma is a very rare, malignant renal epithelial tumor.Distant metastases are present in one third of cases at the time of diagnosis. It is known to have a poor prognosis.CASE SUMMARY A 42-year-old male was sent to our surgery clinic for removal of a 119.2 mm ×108.3 mm encapsulated cystic mass, which was localized in the 8th segment of the right liver lobe. The lesion was first identified on ultrasonography. A computed tomography scan confirmed the presence of a Bosniak type Ⅲ cystic lesion,which affected the liver and convexity of the right kidney. Surgical intervention involved a right nephrectomy, with removal of the cystic mass. The patient was mobilized on the first postoperative day and was discharged after 7 d. The histological and immunohistochemical examination revealed a low-grade collecting duct renal carcinoma, which is a rare variant of papillary carcinoma,with low malignant potential. The patient did not receive chemotherapy and after 21 mo of follow-up, a radiological examination and laboratory analyses showed normal aspects. No relapse or other complications were reported.CONCLUSION To manage renal tumors properly, a correct histopathological diagnosis is crucial,as is early diagnosis and correct surgical treatment.

Simultaneous thyroglossal duct cyst with parathyroid cyst: A case report

BACKGROUND Thyroglossal duct cysts(TDC)are common congenital deformities.Most of them are cysts formed by the thyroglossal ducts that do not disappear and degenerate in the early embryonic stage.TDC exists alone and is rarely complicated by other congenital embryonic malformations.Only a few reports of TDC with branchial cleft cysts,thyroid cancer,thyroid hematoma,and epidermoid cysts have been reported.Therefore,we report a patient with TDC and parathyroid cyst(PC),a rare disease that has never been reported.CASE SUMMARY A 47-year-old woman presented to clinic in April 2021 with a neck tumor which she had noticed 5 d earlier.We perfected the relevant examinations,such as ultrasound and computed tomography,and resected the tumor.After surgical treatment,the pathology revealed a cervical thyroglossal duct cyst and a left lobe parathyroid cyst.The patient was followed up for 1 year without significant recurrence.CONCLUSION We report a patient with a simultaneous TDC and a PC to explore the correlation between the two congenital anomalies.

Thoracic Duct Cyst of the Anterior Mediastinum

Mediastinal thoracic duct cyst is a rare benign cystic disease. The lesion is generally in the post-erior or superior mediastinum, where the thoracic duct passes. We herein report an extremely rare case of surgically resected anterior mediastinal thoracic duct cyst. A thoracic duct cyst should be considered as an uncommon differential diagnosis of an anterior mediastinal lesion.

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.

Pathological features and diagnosis of intraductal papillary mucinous neoplasm of the pancreas

Intraductal papillary mucinous neoplasm(IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucinproducing cells arising in the main duct(MD) and/or branch ducts(BD) of the pancreas.Involved ducts are dilated and filled with neoplastic papillae and mucus in variable intensity.IPMN lacks ovarian-type stroma,unlike mucinous cystic neoplasm,and is defined as a grossly visible entity(≥ 5 mm),unlike pancreatic intraepithelial neoplasm.With the use of high-resolution imaging techniques,very small IPMNs are increasingly being identified.Most IPMNs are solitary and located in the pancreatic head,although 20%-40% are multifocal.Macroscopic classification in MD type,BD type and mixed or combined type reflects biological differences with important prognostic and preoperative clinical management implications.Based on cytoarchitectural atypia,IPMN is classified into low-grade,intermediategrade and high-grade dysplasia.Based on histological features and mucin(MUC) immunophenotype,IPMNs are classified into gastric,intestinal,pancreatobiliary and oncocytic types.These different phenotypes can be observed together,with the IPMN classified according to the predominant type.Two pathways have been suggested:gastric phenotype corresponds to less aggressive uncommitted cells(MUC1-,MUC2-,MUC5 AC +,MUC6 +) with the capacity to evolve to intestinal phenotype(intestinal pathway)(MUC1-,MUC2 +,MUC5 AC +,MUC6- or weak +) or pancreatobiliary /oncocytic phenotypes(pyloropancreatic pathway)(MUC1 +,MUC 2-,MUC5 AC +,MUC 6 +) becoming more aggressive.Prognosis of IPMN is excellent but critically worsens when invasive carcinoma arises(about 40% of IPMNs),except in some cases of minimal invasion.The clinical challenge is to establish which IPMNs should be removed because of their higher risk of developing invasive cancer.Once resected,they must be extensively sampled or,much better,submitted in its entirety for microscopic study to completely rule out associated invasive carcinoma.

Biliary cystadenoma

The diagnosis of cystadenoma is rare, even more so when located in the extrahepatic bile duct. Unspecific clinical signs may lead this pathology to be misdiagnosed. The need for pathological anatomy in order to distinguish cystadenomas from simple biliary cysts is crucial. The most usual treatment nowadays is resection of the bile duct, together with cholecystectomy and Roux-en-Y reconstruction.

Bile duct hamartomas-the von Meyenburg complex

: Hamartomas of the bile duct (von Meyenburg complex) are benign neoplasms of the liver, constituted histologically cystic dilatations of the bile duct, encompassed by fibrous stroma. We report a 42-year-old female patient with symptomatic cholecystitis, whose gross and ultrasonic appearance suggestive of multiple liver metastases. Magnetic resonance imaging and liver biopsy are the gold standards for diagnosis of this rare hepatobiliary condition.

Cholangiographic characteristics of common bile duct dilatation in children

AIM: To investigate whether children with congenital common bile duct dilatation(CBDD) differ from children with obstructive CBDD in cholangiographic characteristics.METHODS: In this retrospective cohort study, the baseline data and the results of imaging analyses were reviewed among children who had endoscopic retrograde cholangiopancreatography(ERCP) due to CBDD. ERCP was performed on all pediatric patients by experienced pediatric endoscopists. The maximal transverse diameter of the common bile duct(CBD) was measured on ERCP. To assess whether ageadjusted CBDD could be used for differential diagnosis, a CBDD severity index(SI) was calculated by dividing the measured CBD diameter by the age-corrected maximal diameter of a normal CBD. RESULTS: A retrospective medical chart review revealed that 85 consecutive children under 16 years of age with hepatobiliary disease and CBDD were referred to Seoul Asan Medical Center. Fiftyfive(64.7%) children had congenital CBDD and 30(35.3%) had obstructive CBDD. The two groups did not differ significantly in terms of clinical characteristics except for sex. The congenital and obstructive CBDD groups did not differ significantly in terms of mean CBD diameter(19.3 ± 9.6 mm vs 12.2 ± 4.1 mm, P > 0.05). However, congenital CBDD cases had a significantly higher mean SI than obstructive CBDD cases(3.62 ± 1.64 vs 1.98 ± 0.71, P = 0.01). In multivariate analysis, an SI value ≥ 2.32 and comorbidity with anomalous union of pancreaticobiliary duct(APBDU) in ERCP independently predicted congenital CBDD.CONCLUSION: Measuring the CBD may aid the differential diagnosis of both CBDD and APBDU in children.

Extrahepatic right hepatic duct diverticulum: a rare entity

BACKGROUND:Douglas described choledochal cysts in 1852 and Todani proposed an anatomy-based classification in 1977. The classification is the most extensively used,but does not include some rare variations.We present a case of hepatic duct diverticulum,one of the variations,and discuss its diagnosis and treatment. METHODS:A 57-year-old woman presented with upper abdominal pain and discomfort associated with nausea and vomiting.She was finally diagnosed with cholelithiasis and right hepatic duct diverticulum. RESULT:The patient underwent resection of the hepatic duct diverticulum and cholecystectomy,and was asymptomatic 26 months after surgery. CONCLUSIONS:Hepatic duct diverticulum is a rare form of choledochal cyst,not included in Todani’s classification. Todani’s classification including this and other uncommon variations of choledochal cysts must be reviewed.The best diagnostic imaging methods and treatment for choledochal cysts must be defined.

Similarities and differences in guidelines for the management of pancreatic cysts

Accurate diagnosis of Pancreatic cysts(PC) is key in the management. The knowledge of indications for surgery, the role of endoscopic ultrasound-guided fine needle aspiration, cyst fluid analysis, imaging, and surveillance of PC are all important in the diagnosis and management of PC. Currently, there are many guidelines for the management of PC. The optimal use of these guidelines with a patient-centered approach helps diagnose early cancer and prevent the spread of cancer.

Preoperative Diagnosis of Thyroglossal Duct Cancer: A Case Report and Literature Review

Objective: To clarify the preoperative diagnostic rate and elucidate the morphological features of thyroglossal duct cancer through a literature search on cases reported in Japan. Methods: A search of a medical database (Japan Medical Abstracts Society) identified 40 studies on thyroglossal duct cancer in Japanese patients between 1976 and 2014. A total of 47 cases, including the present case, are summarized herein. Patient characteristics, preoperative diagnosis, and morphological features were reviewed and analyzed. Morphological features of the internal portions in the cystic lesions were classified using the previously reported Yokosuka Kyosai Hospital criteria for ultrasonography findings of thyroid cystic tumors. Results: Preoperative diagnosis was described for 43 of the 47 cases. Malignancy was suspected in 18 (41.9%) of the 43 cases on the basis of fine needle aspiration (FNA) cytology (presence of suspected papillary carcinoma cells) and imaging studies (presence of calcifications), 12 and 6 cases, respectively. Preoperative FNA was performed in 24 cases with a correct diagnosis obtained in only 12 (50%) cases. Morphological features were evaluated by preoperative imaging studies and/or postoperative histopathology. We found 6 cases (15%) with solid lesions, 32 cases (80%) with cystic lesions containing a solid part, and 2 cases (5%) with solo cystic lesions, respectively. Calcification was observed in 28 (72.5%) cases. We further examined the internal morphology of 32 cases with cystic lesions according to the criteria for ultrasonography findings of thyroid cystic tumors described in Methods. Of the 32 cases, 25 (62.5%) and 7 (17.5%) were classified as “eccentric acute angle type (Ea)” and “multiseptate type (M)”, respectively. The boundary between the solid part and the cystic part was irregular in all 7 “M” cases. No “eccentric and blunt angle type (Eb)” or “concentric type (C)” lesions were observed. Conclusions: The preoperative diagnostic rate for thyroglossal duct cancer using FNA is low, and it is important that diagnosis be performed in conjunction with imaging findings. The presence of solid parts or calcified lesions classified as “Ea” or “M with irregular boundaries” on the basis of imaging findings is suggestive of malignancy.

Familial occurrence of congenital bile duct dilatation

Congenital bile duct dilatation (CBD) that developed in a parent and son is presented. Familial occurrence of CBD is rare, with only a few male cases having been reported. Since the initial report of CBD occurring in siblings in 1981, a total of 20 cases (10 pairs) have been published as of 2007. Clinical and genetic features of CBD are discussed.

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.