Single-Center Results of Choledochal Cysts in Turkish Population.

BACKGROUND: Choledochal cysts are seen commonly in Asian populations, but rarely in Western populations. The pathogenesis of these premalignant lesions is not fully understood yet and the risk of malignant transformation increases with age. The overall malignancy risk is 10%-15% in East Asian countries. In this study, we aimed to present our surgical experience as a hepatobiliary center to the literature. METHODS: We retrospectively analyzed the data from the medical records of 70 patients operated for choledochal cyst between 2008-2019. RESULTS: Sixty-two of the 70 (89%) patients were female and 8 (11%) were male, the mean age was 45.89 ± 15.32 years. Overall, 44 (63%) patients had type I (a+b+c), 20 (28%) type V (Caroli), 2 (3%) type II, 2 (3%) type III and 2 (3%) type IVb cysts. The most common operation was cyst excision combined with hepaticojejunostomy (n: 26, 37%). The median diameter of the resected cysts was 3 cm (min- max: 1-11 cm). Malignancy was observed only in three (4%) patients with type III, type Ib, and type V cyts, who were 19, 38, and 72 years old, respectively. Mortality was not observed, morbidity was determined totally in 30 (43%) cases during early and late postoperative periods. CONCLUSION: Type of surgery in choledochal cysts differs according to the type of the cyst. Malignancy was observed at a rate of 4% in all age groups. Although the frequency of malignancy varies, the main treatment of choice should be surgery because malignancy can be seen at a young age.

Choledochal cysts in children: How to Diagnose and Operate on.

OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.

Choledochal cysts in children: How to Diagnose and Operate on

OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.

Neonatal Jaundice.

Hyperbilirubinemia is a common occurrence in neonates; it may be physiological or pathological. Conjugated hyperbilirubinemia may result from medical or surgical causes, and can result in irreversible liver damage if untreated. The aim of imaging is the timely diagnosis of surgical conditions like biliary atresia and choledochal cysts. Abdominal ultrasound is the first line imaging modality, and Magnetic resonance cholangiopancreatography (MRCP) also has a role, especially in pre-operative assessment of choledochal cysts (CDCs). For biliary atresia, the triangular cord sign and gallbladder abnormalities are the two most useful ultrasound features, with a combined sensitivity of 95%. Liver biopsy has an important role in pre-operative evaluation; however, the gold standard for diagnosis of biliary atresia remains an intra-operative cholangiogram. Choledochal cysts are classified into types according to the number, location, extent and morphology of the areas of cystic dilatation. They are often associated with an abnormal pancreaticobiliary junction, which is best assessed on MRCP. Caroli's disease or type 5 CDC comprises of multiple intrahepatic cysts. CDCs, though benign, require surgery as they may be associated with complications like cholelithiasis, cholangitis and development of malignancy. Severe unconjugated hyperbilirubinemia puts neonates at high risk of developing bilirubin induced brain injury, which may be acute or chronic. Magnetic resonance imaging of the brain is the preferred modality for evaluation, and shows characteristic involvement of the globus pallidi, subthalamic nuclei and cerebellum - in acute cases, these areas show T1 hyperintensity, while chronic cases typically show hyperintensity on T2 weighted images.

Type VI choledochal cyst with gall bladder carcinoma.

An isolated dilatation of the cystic duct (type VI choledochal cyst (CDC)) is extremely rare with only 21 cases reported in the world literature until now. There is only one case of in situ gall bladder cancer (GBC) reported in association with type VI CDC in the literature. Here we are reporting a case of type VI CDC with papillary GBC.

Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of bile duct cysts.

BACKGROUND: The purpose of this study was to compare the impact of the extent of excision and the patent bile duct flow on treatment outcomes of bile duct cysts (BDCs). METHODS: We retrospectively analyzed the records of 382 patients who received surgery for BDCs from January 2005 to December 2014. RESULTS: For Type Ia cysts, proper bile flow was associated with good long-term treatment outcomes with a greater level of significance (p < 0.001) than complete excision (p = 0.012). For Type IVa cysts, proper bile flow, but not complete excision, was associated with good long-term outcomes (p < 0.00001). In addition, 96.3% (104/108) of Type IVa patients with proper bile flow had no late complications and good biliary function, while no patient without patent bile flow had a good clinical outcome. For Type Ic cysts, 92 patients who received partial excisions had good outcomes when proper bile flow was restored. Regression analysis revealed that the absence of proper bile flow, in comparison to incomplete excision, is a greater risk factor for poor long-term treatment effects for Type Ia and Type IVa cysts. CONCLUSIONS: Compared to complete excision, the establishment of proper bile flow exerted a greater impact on improving long-term clinical outcomes after BDC surgery.

Pediatric types I and VI choledochal cysts complicated with acute pancreatitis and spontaneous perforation: A case report and literature review.

RATIONALE: Choledochal cysts are a congenital disorder of the common bile duct that can cause progressive biliary obstruction and biliary cirrhosis. They were classified by Todani into five types. Of these, type VI choledochal cysts are rarely reported in the literature. PATIENT CONCERNS: A 22-month-old girl presented with intermittent epigastralgia for approximately 10 days and fever for three days. Fasting and total parenteral nutrition were administered after admission. However, sudden onset of severe epigastric pain occurred. An abdominal sonogram showed turbid ascites and peritonitis was impressed. DIAGNOSES: An emergent exploratory laparotomy was performed, and perforation of the posterior wall of types I and VI choledochal cysts was observed. INTERVENTIONS: Intraoperative cholangiography revealed concomitant types I and VI choledochal cysts with stricture of the distal common bile duct. Definite surgery for resection of the choledochal cysts and gallbladder was performed with Roux-en-Y choledochojejunostomy. OUTCOMES: The patient had no evidence of ascending cholangitis at three years after the operation. LESSONS: Type VI choledochal cysts are rarely reported in the literature. To our knowledge, this is the first reported pediatric case of concomitant types I and VI choledochal cysts complicated with acute pancreatitis and spontaneous perforation.

Pediatric choledochal cysts: diagnosis and current management.

Choledochal cysts are rare congenital disorders first described by Vater and Ezler in 1723. Their exact etiology remains incompletely understood; however, an anomalous pancreaticobiliary union (APBDU) and subsequent reflux of biliary contents into the biliary tree are thought to play a role. Accordingly, APBDU-associated choledochal cyst patients are significantly more likely to have evidence of hepatitis, cholangitis or pancreatitis and pathologically confirmed inflammation. In 1977, Todani and colleagues modified the original Alonso-Lej classification to include five types of CC. Type I and IV are the most common and most likely to be associated with malignancy. The majority of choledochal cysts are diagnosed in childhood. Clinical presentation varies and most often consists of nonspecific abdominal pain. Diagnosis is typically accomplished using multimodality imaging techniques including computed tomography, magnetic resonance imaging, ultrasound and MRCP. The use of diagnostic PTC and ERCP in CC has been largely replaced by MRCP. Appropriate management consists of prompt, complete cyst excision followed by restoration of biliary enteric continuity when necessary. Minimally invasive CC resection in the pediatric population has demonstrated acceptable outcomes. Prognosis is generally excellent; however, malignancy risk remains higher than the general population even after complete surgical excision.

The distal classification and management of choledochal cyst in adults: Based on the relation between cyst and pancreatic duct.

Todani classification is extensively used to guide the surgical strategy of choledochal cysts, but no systematic investigations on the distal management of intrapancreatic choledochal cysts have been conducted. This study reports the distal classification and management of choledochal cysts in adults based on the relation between the cyst and pancreatic duct. Patients with choledochal cyst who underwent operation, including distal management, in our department from January 2009 to December 2014 were retrospectively reviewed. Patients presenting symptoms, coexisting diseases, surgical treatment, perioperative complications, and long-term follow-up according to the distal classification of choledochal cyst were analyzed. A total of 54 patients with choledochal cyst were included in the present retrospective study. Based on the distal classification of choledochal cyst, 39 patients (72.22%) were type 1, 13 patients (24.07%) were type 2, and 2 patients (3.70%) were type 3. Thirty-nine type 1 patients and 10 type 2 patients underwent excision of intrapancreatic choledochal cyst or bile duct. Three type 2 patients received excision of distal cylindrical cyst and papilla, followed by pancreatic duct plasty with duodenum mucosa. One type 3 patient underwent endoscopic sphincteroplasty, and another type 3 patient underwent transduodenal sphincteroplasty. After the operation, 11 patients (20.37%, 11/54) had short-term perioperative complications. The long-term follow-up results showed that the satisfactory rate (excellent and good outcomes) was 95.83%. Current distal classification of choledochal cysts could provide a more targeted strategy for complete excision to eliminate potential dead space within the pancreas, protect the pancreatic duct, and prevent reoperation.

Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings.

Approximately 20% of choledochal cysts (CC) present in adult patients and they are commonly associated with a high risk of complications, including malignancy. Additionally, children who underwent internal drainage procedures for CCs can develop complications during adulthood despite treatment. Concepts regarding classification and pathogenesis of the CCs have been evolving. While new subtypes are being added to the widely accepted Todani classification system, simplified classification schemes have also been proposed to guide appropriate management. The exact etiology of CCs is currently unknown. The two leading theories involve either the presence of an anomalous pancreatico-biliary junction with associated reflux of pancreatic juice into the biliary system or, more recently, some form of antenatal biliary obstruction with resulting proximal bile duct dilation. Imaging studies play an important role in the initial diagnosis, surgical planning, and long-term surveillance of CCs.

Evaluation and screening ultrasonic signs in the diagnosis of fetal biliary cystic malformation.

OBJECTIVE: To evaluate and screen for fetal biliary cystic malformation (BCM) associated-ultrasonic key signs or sign-combinations. METHODS: Thirty cases of fetal abdominal cysts were investigated, followed up and divided into BCM and non-BCM groups. Expression rates of seven fetal BCM-associated ultrasonic signs in the two groups (A: the cyst was located in the right upper quadrant of the abdomen, B: located beneath the porta hepatis, C: having no septum, D: not the gallbladder, E: connected to the gallbladder, F: connected to the hepatic ducts, G: its inferior portion ended in the epigastric region, anterior to the spinal column) were compared. The diagnostic efficacy of single signs and sign-combinations was evaluated by diagnostic test. RESULTS: The expressions of Sign A and Sign D had no statistically significant differences between the two groups. Single sign of B, F and G had diagnostic efficacy. The diagnostic index of B reached 1.8571. The diagnostic index of the sign-combinations B and D, F or G, and E or F or G reached 2.0000. CONCLUSION: When the fetal cyst was located beneath the porta hepatis and was not the gallbladder, BCM could be diagnosed. Sign G might be another significant ultrasonic sign in BCM prenatal diagnosis.

Selection of the surgical approach for reoperation of adult choledochal cysts.

BACKGROUND/PURPOSE: Our purpose was to evaluate the reasons for reoperations and outcomes in patients with choledochal cysts (CCs). METHODS: The records of patients with CCs who underwent reoperations from 1995 to 2012 were retrospectively reviewed. RESULTS: Of 165 patients with a mean age of 42.54 ± 14.05 years, 62 had Todani type I (37.6 %), 84 type IV-A (50.9 %), and 19 had unknown type CCs (11.5 %). Previous surgery was internal or external drainage alone in 66.1 % of patients with type I and 23.8 % of patients with type IV-A CCs. Partial cyst excision and Roux-en-Y cyst-jejunostomy or cyst excision and choledochoplasty by jejunal interposition were performed in 16.1 and 11.3 % of patients with type I and IV-A CCs, respectively. Reoperations at our hospital were maximal cyst excision and Roux-en-Y hepaticojejunostomy. Radical cyst excision was achieved in 93.5 % of patients with type I and 44.0 % of patients with type IV-A CCs. With an average follow-up of 48.23 ± 12.30 months, recurrent cholangitis and biliary-enteric anastomotic stenosis occurred in 18 (13.2 %) and 9 patients (6.6 %), respectively. Long-term biliary function was excellent or good in 83.8 % of patients. CONCLUSIONS: Radical cyst excision and Roux-en-Y hepaticojejunostomy provide good outcomes in patients with CCs.

An unclassified congenital bile duct cyst.

Congenital bile duct cysts are rare in adulthood. The most frequently used classification was proposed by Todani in 1977. However, in rare cases, not all the bile duct cysts are suitable to this classification. Hereby, we describe the case of an unclassified and very rare form of congenital bile duct cyst--isolated cystic duct cyst. En-block resection of the cyst, along with gallbladder, is the treatment of choice. Although exceptional, cystic duct cysts should be included in Todani classification so that the surgeons to be aware for this variation.

Biliary cysts: a review and simplified classification scheme.

Biliary cystic disease has been known of for centuries. It has traditionally been classified as 5 major types of disease, each with different clinical profiles and attributes. In this article, the basis for the existing classification schemes is reviewed and a simplified classification scheme and treatment regimen are suggested.