Duct Dilatation Research Articles

Domestic ferrets (Mustela putorius furo) with cholecystitis commonly show hyperbilirubinemia and common bile duct dilation, gallbladder sludge, and gallbladder wall thickening on ultrasound.

To report clinical signs, biological anomalies, treatment, and outcome in domestic ferrets with cholecystitis, describe the ultrasonographic features of cholecystitis, and compare the ultrasonographic appearance of the hepatobiliary system between diseased and healthy ferrets. 11 ferrets with confirmed cholecystitis and abdominal ultrasonography were retrospectively included. Ten healthy ferrets were prospectively recruited to undergo hepatobiliary abdominal ultrasonography. Comparisons of explanatory variables (group, age, body weight, and sex) were performed. The most common clinical signs were nonspecific, and icterus was observed in 1/4 of diseased ferrets. Compared with healthy individuals, diseased ferrets showed a larger common bile duct (CBD; ≥ 2.8 mm), a thicker CBD wall (≥ 0.7 mm), a thicker gallbladder (GB) wall, more frequent echoic GB luminal content, visible intrahepatic bile ducts, and a greater GB volume. Bile culture was positive in 9/11 mostly for Escherichia coli (8/9). Bile cytology indicated septic purulent cholecystitis in 7/7 ferrets. Survival time after antibiotic treatment ranged from 6 to 104 weeks, with a median of 36 weeks. Ferrets with cholecystitis presented with nonspecific clinical signs and frequently showed hyperbilirubinemia. Ultrasonography findings such as enlarged CBD, thickened CBD wall, thicker GB wall, GB sludge, visible intrahepatic bile ducts, and enlarged GB may suggest cholecystitis. Escherichia coli was commonly isolated from bile. The medium-term mortality rate was high despite targeted antibiotherapy. This is the first study describing the clinical signs, biological anomalies, ultrasonographic findings, and outcomes after treatment in a series of domestic ferrets with cholecystitis.

041. A Case of Cholangiocarcinoma in A Young Adolescent

Background: Cholangiocarcinoma usually occurs in 50-70 years age group. Recent study showed an increased incidence in younger age group. We describe a case of Cholangiocarcinoma in an adolescent girl in order to understand its natural history. Case: A 16 year old girl presented with 2 months history of jaundice, itchyness, right upper quadrant abdominal pain, pale stool and dark urine. Initial investigation showed : Total Bilirubin 8.87, (Direct 3.53, Indirect 12.40), SGOT 75, SGPT 56, ALP 240, GGT 59, Hepatitis B and C negative, AFP 1.96, CA19-9 3.1, and CEA 5.1. Imaging showed dilated hepatic duct with 1.5cm mass in mid-Common Bile Duct (CBD). Due to unforeseen personal circumstances, surgery was done 16 months from onset of complaints. Tumor was found at junction of cystic duct + CBD. The CBD and Gallbladder was resected (with frozen section), lymph node dissected along hepatic artery, Roux-en-Y hepaticojejunostomy anastomosis and Jejuno-Jejunostomy anastomosis was performed. Histopathology showed Cholangiocarcinoma grade 2 with perineural + lymphovascular invasion, and malignant cells’ perineural invasion at proximal margin. She underwent adjuvant chemotherapy (Gemcitabine + Cisplatin). CT scan 6 months post Chemotherapy showed 3cm lesion in CBD. After a further 2 months of chemotherapy, CT evaluation showed no residual mass left. Conclusion: This highlight that despite significant time lapse from onset of symptoms, surgery with curative intent may still be considered. Secondly, chemotherapy is an extremely important part of the treatment, especially in presence of malignant cells at resection margin.

Perihilar cholangiocarcinoma masquerading as intrahepatic cholestasis of pregnancy: a case report and review of the literature

Cholangiocarcinoma (CCA) is a tumor that arises from the epithelium of the intrahepatic bile ducts. It is rarely diagnosed in individuals under 40 years of age and has a very aggressive nature: 95% of patients die within 5 years. We present the first detailed report of a case of perihilar CCA (pCCA) presenting during pregnancy masquerading as intrahepatic cholestasis of pregnancy (ICP). First, the patient exhibited typical pruritus, particularly in her limbs; second, a raised biomarker of total bile acid (TBA) was noted; third, the onset occurred in the second trimester, aligning with the epidemiological profile; and finally, importantly, there was no mass detected in her liver. First-line drugs were given to treat ICP, but they failed, and ultimately, the condition was identified as pCCA. Following an inadequate excision, traditional Chinese medicine was administered. After 26 months, she succumbed to cachexia. As gestational symptoms are sometimes associated with pregnancy-related disorders, pCCA in pregnant women is frequently misdiagnosed. Symptoms such as jaundice, pruritus, and dilated bile ducts in pregnant women may indicate pCCA. In addition, the appropriate treatment for pCCA in pregnant women may be surgery or chemotherapy; if surgery is not an option, chemotherapy may also help extend the gestational week. Our work is important and can educate on the diagnosis and treatment of pregnancy-related diseases, such as ICP and pCCA.

A case of hepatocellular carcinoma arising from the intraductal hepatic bile duct without parenchymal lesion.

Hepatocellular carcinoma (HCC) rarely presents as an intraductal tumor with no parenchymal lesions. Here, we present a case of HCC arising from an intrahepatic bile duct. A 74-year-old man who had been treated with direct-acting antiviral therapy for hepatitis C virus infection was referred to our hospital because of elevated serum prothrombin levels induced by vitamin K absence II (PIVKA-II). Initial imaging revealed no findings suspicious of HCC; however, repeat CT six months later revealed dilation of the intrahepatic bile ducts in the lateral segment of the liver. Cholangiography revealed a significant stricture of B3, and biopsy suggested adenocarcinoma. A left hepatectomy was performed with a preoperative diagnosis of intrahepatic cholangiocarcinoma. Although a surface nodule on the lateral segment of the liver, suspicious for intrahepatic metastasis, was found intraoperatively, radical resection was performed. Histopathological examination revealed a tumor filling the intrahepatic bile duct from the stump to the peripheral bile duct, with no infiltration into the liver parenchyma. The surface nodule is a dilated peripheral bile duct filled with a tumor with no extrabiliary invasion. Immunohistochemistry suggested HCC. No tumor lesions were observed in the hepatic parenchyma, and the tumor was finally diagnosed as HCC stemming from the biliary epithelium.

Development of an intraductal papillary mucinous neoplasm malignancy prediction scoring system.

Despite the presence of various guidelines, diagnosing malignant intraductal papillary mucinous neoplasm (IPMN) continues to pose challenges. Furthermore, although endoscopic ultrasonography (EUS) offers high-resolution images, it has not yet recognized as the primary tool for malignancy diagnosis. The study objective was to develop a simplified and user-friendly scoring system to improve the diagnostic accuracy of malignant IPMNs. Additionally, the utility of EUS and its effect on diagnostic accuracy were assessed. We retrospectively collected the clinical data on 160 cases of resected IPMN at Tokyo Medical and Dental University Hospital from January 2008 to December 2022. We examined clinical features, computed tomography (CT) and magnetic resonance imaging (MRI) findings, and EUS results if available. We then calculated the odds ratio of malignancy for these factors and developed an IPMN malignancy prediction (IMAP) scoring system. There were 89 (55.6%) cases of benign IPMNs and 71 (44.4%) of malignant IPMNs. Eight clinical and imaging findings, including age, diabetes mellitus status, jaundice, CA19-9 level, enhancing mural nodules ≥5mm, thickened wall, and main duct dilatation, were significantly associated with malignancy. The IMAP score was calculated by assigning 0 to 2 points to these factors based on the odds ratio. The area under the receiver operating characteristic curve for the IMAP score was 0.78 [95% confidence interval (CI): 0.71-0.85] based on CT/MRI alone and improved to 0.81 (95% CI: 0.74-0.87) when EUS was added. When the total exceeds 5 points, the positive predictive value becomes 100% (95% CI: 95.9-100). In conclusion, the IMAP scoring system has demonstrated promise as a clinically useful tool, offering both simplicity and sufficient accuracy. It holds potential as an important decision criterion for determining the treatment approach for IPMN. Additionally, EUS contributes to enhancing the diagnostic accuracy of the IMAP scoring system, thereby enabling more precise decision-making.

Comprehensive Next Generation Sequencing Reveals that Purported Primary Squamous Cell Carcinomas of the Parotid Gland are Genetically Heterogeneous.

Squamous cell carcinoma (SCC) is one of the most common malignancies involving the parotid gland, but it has been recognized that the vast majority of parotid SCC represents metastases, especially from the ipsilateral facial skin. Bona fide primary SCC of the parotid is so rare that it is unclear whether it truly exists at all. We sought to molecularly characterize cases diagnosed as primary parotid gland SCC to see if they possess a unique genetic makeup.We identified cases in our archives which had been diagnosed as primary SCC of the parotid gland. In all cases, metastatic disease was excluded by a thorough history and physical examination. Cases with histologic evidence of a precursor neoplasm (e.g., carcinoma ex-pleomorphic adenoma) were also excluded. Targeted next-generation sequencing (NGS) was attempted on all cases.Six cases diagnosed as primary parotid SCC were identified, arising in 4 males and 2 females ranging from 8 to 73 years (mean, 51.8 years). All cases exhibited keratinization and unequivocal invasion. Four of 6 appeared to be arising from cystically dilated ducts. Five of 6 exhibited well-developed cellular atypia; the remaining case, while cytologically bland, demonstrated perineural invasion. Targeted NGS was successful in 5 of 6 cases. Two SCC harbored several mutations in a mutational profile reminiscent of SCCs seen in other organs. One case harbored YAP1::MAML2, a fusion previously reported in porocarcinoma and other neoplasms. One case harbored IRF2BP2::RUNX2, and presumably represents keratocystoma or SCC ex-keratocystoma. Finally, one case an increase of C > T mutations consistent with ultraviolet damage, suggesting that this case represented a cryptic metastasis from cutaneous SCC.Our analysis did not confirm a unifying genetic signature for purported primary parotid SCC. Indeed, our findings suggest that true primary parotid gland SCC is even rarer than already believed. In our 5 cases with results, NGS findings demonstrated that one was likely a keratocystoma, one a cryptic metastasis from a cutaneous SCC, and one a porocarcinoma, either metastatic or primary. The two remaining cases had complex genotypes reminiscent of SCCs from other sites. This may be the signature of genuine parotid primary SCC, but metastasis from an SCC from another organ cannot be excluded. Accordingly, a diagnosis of primary parotid gland SCC should be viewed with skepticism.

Clinical Implications and Risk Factors of Dilatation of Remnant Pancreatic Duct at 1 Year after Pancreatoduodenectomy: A Prospective, Japanese, Multicenter, Observational Cohort Study (DAIMONJI Study).

To determine the precise frequency of main pancreatic duct (MPD) dilatation within the remnant pancreas at 1 year after pancreatoduodenectomy (PD) and its clinical implications, a prospective multicenter cohort study was performed in patients without MPD dilatation before PD (registry number: UMIN000029662). Between October 2017 and July 2020, patients with an MPD diameter less than 3 mm who were planned to undergo PD for periampullary lesions at 21 hospitals were enrolled. The primary endpoints were frequency of MPD dilatation at 1 year after PD, and the relationship between MPD dilatation and pancreatic endo- and exocrine function, nutritional status, and fatty liver. Secondary endpoints were risk factors for MPD dilatation at 1 year after PD and time-course change in MPD diameter. Of 200 registered patients, 161 patients were finally analyzed. Pancreatic fistula was the most frequent complication (76; 47.2%). MPD dilatation (MPD > 3 mm) at 1 year after PD was seen in 35 patients (21.7%). Pancreatic exocrine function, assessed by steatorrhea, was significantly impaired in patients with MPD dilatation. However, endocrine function, nutrition status, and fatty liver development were comparable between the 2 groups. In multivariate analysis, the serum total protein level 7.3 g/dL or more was an independent predictor for MPD dilatation at 1 year after PD (odds ratio 3.12, 95% CI 1.31 to 7.15). A mean MPD diameter significantly increased at 6 months after PD and kept plateau thereafter. MPD dilatation at 1 year after PD was seen in 21.7% of patients and significantly associated with exocrine function impairment but not with endocrine function, nutrition status, or development of fatty liver.

Comparison of the Inadequacies of Ultrasonography and Computed Tomography in the Diagnosis of Sialolithiasis.

This study aimed to determine the value of either ultrasonography and computed tomography (CT) alone or both for diagnosing salivary gland stones. Based on their clinical findings and physical examination, 188 patients with salivary stones were included. Initially, an ultrasound was performed, and then a non-contrast-enhanced thin-sliced CT scan was done. The study included 161 patients with suspected submandibular gland (SMG) sialolithiasis and 27 with parotid gland (PG) sialolithiasis. Among the SMG cases, stones were confirmed in 130 patients through interventional sialoendoscopy. Sixteen of the stones were detected by CT scan only and were not seen in the previous ultrasound examination. After the second follow-up ultrasound, which was conducted after reviewing the CT scans, 9 of them were identified. Five patients with distal stones and ductal dilation on ultrasound were confirmed to have sialolithiasis. In 5 patients with stones detected on ultrasound but not on CT, dental filling artifacts were identified as the cause. For PG cases, stones were observed in 18 out of 19 patients with suspected sialolithiasis based on ultrasound and CT findings. Three patients with positive CT findings but negative ultrasound showed stones during sialendoscopy. During an ultrasound examination, palpating the floor of the mouth with the other hand's index finger can help identify stones obscured by the shadow of the mandible, thereby enhancing the test's accuracy. Patients with dental fillings, implants, and permanent dentures should not be referred for a CT scan to avoid unnecessary exposure to ionizing radiation. They also cause significant metallic artifacts in the field of interest. In these cases, ultrasound should be considered. To minimize the effects of ionizing radiation, it is recommended to create limited field requests for the target, focusing solely on the submandibular and PGs.

Proximity predicament: a rare case of hydatid cyst in the porta hepatis region

Hydatid cysts near the porta hepatis present notable medical challenges due to their intricate positioning and potential influence on crucial structures within the liver and its vicinity. While relatively rare, their presence can lead to intricate medical scenarios because of their proximity to vital liver and bile duct structures, but their occurrence can present complex medical situations due to their proximity to critical structures in the liver and bile ducts. We present an unusual case of a 33-year-old male with no known comorbidities presented to the outpatient department of general surgery with pain, insidious in onset and gradually progressive, in the right hypochondrial region for the past 3 months, along with mild fever, reduced appetite, and weight loss of 3 kg. Managing hepatic cysts, especially when near the porta hepatis, poses significant diagnostic challenges. Imaging scans revealed multiple hypodense lesions suggestive of hepatic cysts, with the largest one measuring 5 cm × 8 cm in segment VI. Native post-contrast lesions were also observed in the right lower lobe. Although hepatic duct dilation was not observed, the presence of a cyst near the porta hepatis an atypical site for hydatid cyst, complicated potential PAIR procedures (puncture, aspirate, injection, re-aspirate), raising concerns about the risk of damaging vital structures like the hepatic artery or major bile duct. A multidisciplinary approach engaged interventional radiology to evaluate PAIRS feasibility. PAIRS was performed successfully, except for the cyst near the porta hepatis to avoid complications. Despite this, the patient continued to experience abdominal discomfort post-operation, although liver function tests improved in the postop period. This case underscores the uncommon occurrence of hydatid cysts near the porta hepatis and highlights the need for additional research to comprehend its prevalence and causes. Further investigation is crucial to enhance our understanding of the epidemiology and optimal management strategies involving PAIRS in such challenging cases.

Clinically oriented look into diagnostics and therapy of primary sclerosing cholangitis. Review of literature

Introduction: Primary sclerosing cholangitis (PSC) is a complex liver disorder marked by multifocal bile duct strictures and dilatations, leading to cholestasis and liver fibrosis. Although the exact cause is unknown, autoimmune mechanisms are strongly suspected. Aim of the Study: This paper reviews current knowledge on PSC, covering its etiology, prevalence, treatment options, and practical management strategies. Current State of Knowledge: PSC management focuses on symptom relief and liver transplantation, with limited success in pharmacological treatments. New therapies, including immune modulators and anti-fibrotic agents, show promise but require further research. Liver transplantation remains the most effective treatment, though recurrence risks necessitate ongoing monitoring. Materials and Methods: This article reviews the current understanding of PSC, including its pathophysiology, diagnostic approaches, clinical features, and management strategies. A comprehensive literature search was conducted using PubMed and Google Scholar, with articles selected based on relevant keywords and evaluated for inclusion in this review. Conclusions: Effective management of PSC necessitates a multidisciplinary approach involving hepatologists, gastroenterologists, radiologists, and transplant surgeons. Ongoing efforts to better understand PSC’s pathogenesis, refine diagnostic methods, and develop more effective therapies are crucial for improving patient outcomes.

6981 Atypical hypoglycemia in a teenage boy with prediabetes

Abstract Disclosure: N. Shahid: None. C. salgado: None. N. Gurtunca: None. Background: Hypoglycemia in teenagers is seen less commonly than in younger children. Overweight children with prediabetes presenting with hypoglycemia symptoms are often considered to have reactive post-prandial hypoglycemia due to the degree of hyperinsulinemia with insulin resistance seen in these children. We present a 13-year-old boy with prediabetes, who presented with episodes of altered mental status and was found to have hypoglycemia due to a solitary insulin secreting tumor. Case presentation: A 13-yr old boy with pre-diabetes presented to the ER with altered consciousness and non-ketotic, non-acidotic hypoglycemia [25 mg/dl] & trace ketonuria in the setting of Influenza A infection. Weight and BMI above the 99th%ile and acanthosis nigricans in the skin creases. No access to oral hypoglycemic agents. Urine toxicology was negative. EEG was normal. Euglycemia and level of consciousness restored after IV dextrose & this was considered a possible hypoglycemic seizure episode secondary to poor oral intake. 4 months later he had another episode of altered mental status, jittery movements and was found to be hypoglycemic [41 mg/dl]. He was admitted for further workup of recurrent hypoglycemia. Adrenal insufficiency was ruled out and a diagnostic fast was started. After 15 hours, he developed non-ketotic hypoglycemia [<50 mg/dl]. Critical sample [Table 1] and Glucagon challenge [Table 2] at the termination of the fast confirmed hyperinsulinemic hypoglycemia. Given the unusual age and presentation, an MRI abdomen was done which showed a 1.6 cm lesion in the pancreatic body [Fig 1]. Dotatate whole body PET scan confirmed the insulinoma with no evidence of nodal or distant metastasis [Fig 2]. He underwent surgical enucleation of the insulinoma. Intraoperative secretin showed no evidence of pancreatic duct dilatation or leakage. Hereditary endocrine tumor panel including multiple endocrine neoplasia [MEN] gene analysis was negative. The postoperative course was complicated by a persistent pancreatic leak and pancreatitis eventually requiring an ERCP and stent placement 2 months post operatively. Conclusion: Insulinomas are very rare neuroendocrine tumors in pediatric population, with a prevalence of one in 250,000 and median age at presentation of 45-50 years. About 90% insulinomas are benign and less than 10% are associated with MEN-1 syndrome. Chronic hypoglycemia irrespective of the underlying insulin resistance of prediabetes can be seen with insulinoma and can cause hypoglycemia unawareness as seen in our patient. This has also been reported in literature to be misdiagnosed as refractory epilepsy or psychiatric disorders. Hypoglycemic symptoms at any age should be promptly worked up. Ruling out associated neuroendocrine hereditary tumor syndromes is crucial and prognosis of benign solitary insulinomas after surgical removal is mostly favorable. Presentation: 6/3/2024

Incidentally detected pancreatic duct dilatation on ultrasound and role of MRI.

The clinical significance of incidentally detected pancreatic duct (PD) dilatation at ultrasound (US) without a visualized underlying cause is unclear. We aimed to assess the role of subsequent MRI (including MRCP) and to identify US imaging and laboratory findings predictive of underlying pancreaticobiliary malignancy at the time of initial US. Patients with incidentally detected PD dilatation at ultrasound from 2011 to 2019 that had an ensuing MRI were included. Based on MRI results patients were divided into three groups: malignant pancreaticobiliary causes, benign causes and idiopathic PD dilatation. Subsequently the diagnostic ability of MRI was assessed. Initial ultrasound findings and laboratory results were compared between groups to identify predictors of underlying pancreaticobiliary pathology. A p-value < .05 was considered statistically significant. In 37/59 (63%) patients PD dilatation was confirmed on MRI. MRI demonstrated malignant 7/59 (12%) and benign 10/59 (17%) causes of PD dilatation detected at initial ultrasound. Sensitivity, specificity, negative predictive value, positive predictive value and accuracy of MRI to ascertain the cause of PD dilatation was 89%, 100%, 95%, 100% and 97% respectively. Patients with a larger magnitude of PD dilatation, concomitant CBD dilatation and elevated lipase values were more likely to have underlying pancreaticobiliary malignancy (p < 0.05). No patient with initial negative MRI had pancreaticobiliary malignancy on subsequent work-up. Incidentally detected PD dilatation on ultrasound is an important finding and should prompt referral to MRI. MRI is an accurate, noninvasive method for identifying the underlying cause of PD dilatation in these patients and in excluding pancreaticobiliary malignancy.

Case report : lemmel syndrome-aggravated by consumption of fatty food

Introduction and Importance: One of the very rare causes of obstructive jaundice, Lemmel’s syndrome is caused due to biliary mechanical compression by a juxtapapillary diverticulum. It is defined as obstructive jaundice due to juxtapapillary diverticulum in the absence of cholelithiasis, periampullary tumours or other detectable obstacles. It is extremely rare syndrome therefore studies need to be done for proper diagnosis and management. Case Presentation: Our patient, a 73-year-old woman, gave a history of recent acute right hypochondriac pain that had subsided the day before. Lab investigations were performed and liver enzymes were found to be significantly raised. An endoscopy followed by an endoscopic ultrasound was performed for further diagnosis which showed the diverticulum outpouching from the duodenum. MRCP was then performed which revealed a dilated common bile duct and common hepatic duct, with tapering at the distal end, most likely to be a stricture. Lemmel syndrome was identified based on the clinical presentation and imaging results. A dilated common bile duct, and two large duodenal diverticula with interdiverticular papilla was discovered during the patient’s endoscopic ultrasound which was performed by the gastrointestinal team. Clinical Discussion: Duodenal diverticula are the second most common site of diverticula after colon with an incidence of 1-5% in radiological series3. Lemmel’s syndrome is a rare cause of obstructive jaundice associated with juxtapapillary diverticulum. With a low incidence rate, only a few cases of Lemmel’s syndrome are reported with the first case reported in 1934 by Lemmel.4 Imaging techniques such as barium meal, EUS, ERCP, or magnetic resonance cholangiopancreatography are used to confirm the diagnosis. Our observations demonstrate that the diverticulum which is often found on the medial wall of the second half of the duodenum and contains bezoar, compresses the distal common bile duct laterally. Nowadays, ERCP and EUS are used to confirm the diagnosis.5 Only symptomatic periampullary duodenal diverticulum(PDD) requires conservative or surgical therapy. The first line of treatment for biliopancreatic PDD problems is endoscopic sphincterotomy, which is 95% effective.6 Conclusion: Lemmel syndrome occurs due to the compression of the common bile duct by a duodenal diverticulum. Imaging including CT scan and MRCP are important for the diagnosis of this syndrome. Treatment modalities are not the same for each patient, however.

Role of sonography in detection and evaluation of post liver transplant complications.

To evaluate the role of sonography in revealing and characterizing liver transplant complications based on gray scale and color Doppler, describe the normal Doppler findings, and discuss the significance of distinguishing normal transient changes in the spectral waveform from findings that may suggests ominous complications. We carried out a retrospective cross-sectional study at Prince Sultan Military Medical City, Riyadh, Saudi Arabia. The medical records and imaging studies of a total of 122 candidates who underwent transplantation between January 2016 to February 2022 were reviewed. Our results showed that most patients were males with the most frequent age group being those between 54-71 years. Hepatitis B virus and hepatic cellular carcinoma were the most common indications for transplants. A total of 95 patients received a graft from a living related donor. Regarding complications, biliary issues (including leaks and ducts dilation) were the second most frequent complication after collections. Vascular complications represented 7.4% of all complications and was the leading cause of death in 4.8% of cases. Among all vascular issues encountered during liver transplant, portal vein thrombosis was the most predominant. In respect to Doppler findings, portal vein velocities and resistive index of hepatic artery had re-averaged within 7-10 post-operative days in most patients. Ultrasound plays crucial role in the post-operative management of compilations, facilitating early detection, which is substantial for the graft survival.

Meibomian gland lipid alterations and ocular surface sequela in Awat2 knockout murine model of meibomian gland dysfunction and evaporative dry eye disease

PurposeThere is an urgent need for animal models of meibomian gland dysfunction (MGD) and evaporative dry eye disease (EDED) to understand their pathophysiology and investigate novel therapeutics. This study sought to further define the acyl-CoA: wax alcohol acyltransferase 2 knockout (Awat2 KO) mouse as a model of EDED using a combination of novel clinical, biochemical, and biophysical endpoints. MethodsWildtype and Awat2 KO mice between 1 and 18 months of age were used. Ocular examinations and advanced imaging were performed. The lipidomic composition and in situ melting temperature of meibum were determined. qPCR was performed to define ocular surface gene and pro-inflammatory transcript expression. Dynamic contact angle goniometry was performed to assess the adherence capability of the ocular surface. ResultsAwat2 KO mice have mild, white, hyperreflective corneal opacities of the anterior stroma and significantly enlarged apical epithelial cells (P=0.0004). In Awat2 KO meibum, wax esters were 9-10 times lower than in wildtype meibum. Additionally, meibum melting temperature increased from 32° to 47°C (P<0.0001), leading to impaired meibum secretion and dilation of the central duct. Awat2 KO corneal epithelia had significantly decreased mucin expression (Muc1 and Muc4, P=0.0043) and increased interferon-γ production (P=0.0303). Awat2 KO globes have a significantly shortened time of droplet adherence to their ocular surface (P=0.0053), indicating a decreased tear film adherence capacity. Wildtype corneal epithelia does not express Awat2, indicating that the EDED phenotype is secondary to the loss of Awat2 from the meibomian glands. ConclusionsAwat2 KO mice recapitulate many of features of human MGD and EDED, representing a model to test novel therapeutics.

Deletion of Notch Signaling in the Developing Mouse Kidney Results in Expanded Expression of Arf6, Reduced Expression of E-cadherin, and Dilation of Collecting Ducts

Deletion of Notch Signaling in the Developing Mouse Kidney Results in Expanded Expression of Arf6, Reduced Expression of E-cadherin, and Dilation of Collecting Ducts

S2362 Mass Effect Due to Extensive Choledocholithiasis and Biliary Ductal Dilation: An Unusual Cause of Portal Vein Thrombosis

S2362 Mass Effect Due to Extensive Choledocholithiasis and Biliary Ductal Dilation: An Unusual Cause of Portal Vein Thrombosis

Optimizing EUS-guided choledochoduodenostomy with lumen-apposing metal stents for primary drainage of malignant distal biliary obstruction (SCORPION-IIp): a prospective pilot study

Background and AimsBiliary drainage by endoscopic retrograde cholangiography (ERCP) in patients with malignant distal bile duct obstruction (MBO) is frequently associated with complications, such as pancreatitis, hampering patient outcome. EUS-guided choledochoduodenostomy (EUS-CDS) with a lumen-apposing metal stent (LAMS) is a promising alternative in patients with MBO but is associated with a worrisome risk of stent dysfunction. Placement of a fully covered self-expandable metal stent (FCSEMS) through the LAMS, thereby changing the axis of biliary drainage towards the descending duodenum, may decrease the risk of stent dysfunction while maintaining high technical success and low adverse event rates. MethodsProspective single center pilot study in patients with a pathology confirmed MBO without gastric-outlet obstruction. Primary outcome was stent dysfunction, defined as recurrent jaundice after initial clinical success, ongoing jaundice in combination with persistent bile duct dilatation, or cholangitis. The study was registered in clinicaltrials.gov (registry number NCT05595122). ResultsOverall, 27 consecutive patients eligible for EUS-CDS were enrolled. The placement of a LAMS was successful in 24/27 patients (89%), and placement of FCSEMS through the LAMS was successful in 20/24 (83%), in the remaining 4 patients a coaxial double-pigtail plastic stent was placed. In 2 of these 20 patients there was persistent jaundice requiring stent revision (10%), leading to a clinical success rate of 90%. No patients developed stent dysfunction after initial clinical success. ConclusionsThis study showed a stent dysfunction rate of 10% following technically successful EUS-CDS with placement of a FCSEMS through the LAMS. Improving the design of LAMS may further reduce the rate of stent dysfunction.

Management of a Todani Type 1b Choledochal Cyst: Case report

This case report describes the management of a Todani Type 1b choledochal cyst in a 26-year-old woman who presented with chronic epigastric dyspepsia and acute epigastric pain. MRI revealed a cystic dilatation of the distal common bile duct. The patient underwent successful surgical excision of the cyst, cystic duct, and gallbladder, followed by reconstruction with a Roux-en-Y hepaticojejunostomy. The postoperative course was uneventful, with discharge on the sixth day. This case highlights the importance of timely diagnosis and surgical intervention to prevent complications such as biliary cirrhosis and malignancy.

Reconstructive interventions on bile ducts in patients with benign obstructive jaundice

Aim. To evaluate immediate and long-term results of surgical reconstruction of the biliary system in various types of benign obstructive jaundice.Materials and methods. The study enrolled 110 patients with choledocholithiasis, iatrogenic biliary strictures, common bile duct strictures, as well as strictures of biliodigestive and biliobiliary anastomoses. Patients were divided into 3 groups. Group 1 included 43 patients with multiple choledocholithiasis and bile duct dilatation &gt;15 mm. Group 2 enrolled 35 patients with multiple choledocholithiasis and hepaticolithiasis who were intraoperatively diagnosed with stricture, injury, or pressure ulcers of the common bile duct wall. Group 3 included 32 patients with cicatricial and iatrogenic biliary strictures. The immediate and long-term results of surgical treatment were compared.Results. In group 1, the survival rate comprised 89.4%, the average duration of surgery amounted to 137 minutes, complications involved wound infection, bile leakage, bile peritonitis. In group 2, the survival rate accounted for 91.4%, the average duration of surgery comprised 192 minutes; strictures of biliodigestive anastomosis were detected in the remote period, and reconstructive surgeries were performed. Group 3 revealed the survival rate of 100%, the average duration of surgery of 215 minutes, and no complications in the long term.Conclusion. The diameter of the common bile duct, its wall changes, size of gallstones, signs of cholangitis, patency of the bile papilla, and localization of biliary stricture determine the surgical tactics, when performing biliary reconstruction in patients with non-cancerous obstructive jaundice. In case minimally invasive surgery is found impossible under conditions of multiple choledocholithiasis, common bile duct diameter &gt;15 mm, and preservation of its wall, choledocholithotomy with Kerr drainage is considered to be optimal. In case of damage to the CBD wall and patency of the bile papilla, preference shall be given to hepaticoenterostomy. Bile duct reconstruction with stent drainage is indicated for treatment of cicatricial and iatrogenic biliary strictures.