Bardet-Biedl syndrome is an autosomal-recessive ciliopathic disorder affecting multiple organ systems. Characteristic features include progressive retinal dystrophy, obesity, polydactyly hypogonadism, mental retardation, and renal disorders. Other manifestations include congenital heart diseases, hepatic fibrosis, ataxia, and diabetes. Approximately 30% of patients with Biedl-Bardet syndrome (BBS) have hepatobiliary disorders such as periportal fibrosis, nonalcoholic fatty liver disease, and cystic dilation of the bile ducts. The association of BBS with choledochal cysts (CDC) is extremely rare. Here, we report a case of a 14-year-old boy with a novel variant of BBS and associated type IV CDC. The patient was managed surgically with CDC excision and Roux-en-Y hepaticojejunostomy.

UNASSIGNED: Ductal plate malformations (DPMs) are poorly documented in the veterinary literature, particularly those of the polycystic liver disease (PCLD) phenotype. A 13-year-old female spayed cat presented with progressive icterus, abdominal distension, weight loss and elevated liver enzymes. Initial empirical treatment consisting of amoxicillin/clavulanate, ursodiol and later prednisolone was attempted; however, clinical signs progressed. On abdominal ultrasound, numerous large hepatic cystic masses were noted, characterized by an anechoic center with a heterogeneous, hyperechoic wall. A post-mortem examination confirmed numerous hepatic cysts, the larger of which resulted in hemorrhage and subsequent hemoabdomen. Histologically, these cysts were determined to be of biliary origin, and a diagnosis of PCLD was assigned.
UNASSIGNED: Herein, we present a detailed report of clinical, gross and histologic findings in a cat clinically affected by PCLD. This case demonstrates that cysts present in this congenital disease can ultimately lead to hepatobiliary malfunction and clinical decline via marked expansion of cysts, compression of the liver and hemoabdomen from cyst rupture. DPMs, specifically PCLD, should be considered in cats presenting with multifocal large hepatic cysts.

BACKGROUND: A choledochal cyst is a cystic dilatation of the biliary tree, also termed a biliary cyst, including an intrahepatic cyst as well. Magnetic resonance cholangiopancreatography (MRCP) is the gold standard investigation of choice for this pathology. Todani classification is most commonly used to classify choledochal cysts.
METHODS: A total of 30 adult patients with choledochal cysts presenting at our center from December 1, 2009, to October 31, 2019, were studied retrospectively.
RESULTS: The mean age was 35.13 years ranging from 18 to 62 years with a male-to-female ratio of 1:3.29. Of the patients, 86.6% presented with abdominal pain. Total serum bilirubin was raised in six patients with a mean of 1.84 mg/dL. MRCP was done in all patients, which had almost 100% sensitivity. Two cases had anomalous pancreaticobiliary duct union. In our study, we found only type I and type IVA cysts according to the Todani classification (type IA = 56.3%, IB = 11%, 1C = 16%, and IVA = 17%). The mean size of the cyst was 2.37 cm. Complete cyst excision with Roux-en-Y hepaticojejunostomy was performed in all patients. Four patients had surgical site infections and two had bile leaks. One patient developed hepatic artery thrombosis. All complications were eventually managed conservatively. Mortality was nil in our study with the mean postoperative stay being 7.97 days.
CONCLUSIONS: Adult presentation of biliary cysts is not an uncommon entity in the Indian population and should be considered as a differential diagnosis of biliary pathology in adult patients. Complete excision of cysts with bilioenteric anastomosis is the current treatment of choice.

Highlight With less than 20 cases reported worldwide, cystic dilation of the cystic duct (type 6 biliary cyst) is a very rare condition which can commonly present as right upper quadrant abdominal pain. Dharan presents imaging of this rare entity and briefly discusses the available literature.

Biliary cysts are rare cystic dilatations of the biliary tree. Biliary cysts are positively associated with several significant complications, amongst them, cholangiocarcinoma befalls the most dreadful one. The elevated incidence is 20-30% in the unresected cyst and 0.7% in resected cysts. Magnetic resonance imaging (MRI) scan, magnetic resonance cholangiopancreatography (MRCP) or a contrast-enhanced computed tomography (CECT) is applied for the initial diagnostic study but the ultimate diagnosis ordinarily requires the tissue biopsy. Currently, the sole curative option involves the complete surgical resection of the lesion, with standard chemotherapy and active radiation applied as an alternative for the unresectable tumors. Despite the curative surgery the percentage of eternal recurrence of the tumor indefinitely persists, and effective post-surgical surveillance is reasonably demanded. We report a case of 29-year-old female with local recurrence of cholangiocarcinoma in a previously resected biliary cyst type I. The curative resection of the choledochal cyst only minimizes the considerable risk of the possible development of future cholangiocarcinoma but it does not completely prevent it. The appropriate follow-up for potential patients who have been typically treated for a biliary cyst is unclear. The lethal course of cholangiocarcinoma is believed due to its slow asymptomatic growing phase. Therefore, to adequately screen for malignancy, periodic imaging along with annual liver tests represents a reasonable approach to prevent the possible development of this appalling complication.

HCC is a rapidly increasing cancer worldwide. Most HCC rises in the setting of chronic and advanced liver disease caused by viral hepatitis, alcohol use, non-alcoholic liver disease or their combination. We found that in the mouse model, alcohol alone does not induce HCC, however, it can promote HCC development after a carcinogen exposure. Multiple mechanisms are involved in carcinogenesis and alcohol affects many of those including epithelial-mesenchymal transition, cancer stem marker expression and inflammation as evidenced in our HCC model.

In biliary atresia, the disease process of obliterative cholangiopathy may begin in the perinatal period; however, no chronological evidence exists on how the cholangiopathy progresses to biliary obliteration. This is the first acquired case with the final diagnosis of type III cystic biliary atresia with an extrahepatic biliary cyst which showed the progression of obliterative cholangiopathy in chronological order after birth.
An 81-day-old girl presented with acute abdominal distress due to bilious peritonitis caused by biliary cyst perforation, for which she underwent emergency biliary drainage. Postoperative images showed a dilated common bile duct and hepatic ducts bilaterally, with flow of the contrast medium to the duodenum through the dilated common bile duct. Biochemistry of the bile collected during and after the operation revealed elevated levels of pancreatic enzymes in the bile from the gallbladder. The patient was diagnosed as having a congenital choledochal cyst and underwent laparotomy at 120 days of age which revealed that she had pancreaticobiliary maljunction. The biliary cyst was resected at the narrow portion just above the junction with the main pancreatic duct. During dissection up to the hepatic hilum, we found that the hilar hepatic ducts were bilaterally replaced by fibrous tissue and were obstructed, leading to a diagnosis of type III a1, μ biliary atresia. The fibrous tissue was excised, and hepatic portoenterostomy was performed according to the Kasai procedure. The patient\'s postoperative course was uneventful and the jaundice resolved within 1 month. She has had normal liver function tests with no episode of cholangitis for 3 years after discharge.
We demonstrated the process of acquired type III biliary atresia in a patient with cystic biliary atresia and biliary cyst perforation. To the best of our knowledge, this is the first case of acquired cystic biliary atresia showing chronological progression of the course of obliterative cholangiopathy, providing a better understanding of the development of type III biliary atresia as an acquired disease.

OBJECTIVE: Biliary cysts in pregnant women are a complex medical issue, especially when complicated with cholangitis. It is a serious and life-threatening diagnosis that can seriously endanger both the expectant mother and the fetus. However, during pregnancy, surgical treatment would lead to further complications and higher fetal mortality. Here, we propose a novel therapeutic approach that would be safe for both mother and child during pregnancy, with a definitive treatment postponed until after delivery.
METHODS: In this retrospective study we have summarized the clinical course of six adult patients diagnosed with choledochal cysts during pregnancy. Treatment was conducted in two stages, firstly, percutaneous cholecystostomy under ultrasound guidance and sustained negative pressure suction until delivery, and secondly, selective choledochal cyst excision when the patients recovered from delivery.
RESULTS: All the six patients gave birth to healthy babies. Four patients had Type-I choledochal cysts, and underwent Roux-en-Y hepaticojejunostomy surgery. Two patients had a Type-IV choledochal cyst. The first patient with Type-IV choledochal cyst underwent anastomosis between the secondary hepatic bile duct and jejunum and the second patient underwent laparoscopic cyst internal drainage. No serious complications were recorded after gallbladder drainage or during the perioperative period.
CONCLUSIONS: Based on our single-centre experience we can conclude that treatment of choledochal cyst with cholangitis during pregnancy can be conducted safely and efficiently through the two stages strategy that we proposed in this paper. The first stage should be percutaneous cholecystostomy followed by elective surgical treatment following delivery.

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.

BACKGROUND: Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly.
METHODS: PubMed and Cochrane databases were accessed 1975-2015 using terms \"choledochocele\" or \"choledochal cyst\". Studies reviewed that met the following criteria: English language, published 1975-2015 with human subjects.
RESULTS: 325 patients with a choledochocele were identified, including 71 case reports and 254 cases within institutional reviews. 13 pediatric case reports of choledochocele exist, with abdominal pain being the most common symptom (n=11). The most frequent diagnostic and treatment modalities were ultrasound (n=10), and endoscopic sphincterotomy (n=5). No malignancies were reported. 58 adult case reports exist, with the most common presenting symptom being abdominal pain (n=54). Ultrasound was the frequently employed diagnostic modality (n=32). Open procedures were performed more often (n=30). Malignant lesions were identified in 5. In 42 institutional reviews, the frequency of choledochocele was 0.7%. Of those for whom treatment was reported, 69% underwent endoscopic sphincterotomy.
CONCLUSIONS: Choledochocele is a rare malformation. Similarities exist between pediatric and adult patients, but malignancy has only been reported in adults. An algorithm based on patient age, cyst size, lining and amenability to endoscopic resection may be considered as a treatment strategy for this uncommon condition.