Tuberculosis of the bone marrow with secondary hemophagocytic lympho-histiocytosis presenting as obstructive jaundice: A clinician's challenge for the ages.

Tuberculosis. a disease of great public concern, is spread through inhalation of micro-droplets from an infected person. Despite lungs being the primary site, there may be multisystemic involvement, very rarely involving bone marrow, a dreaded manifestation of disseminated tuberculosis, associated with high mortality and morbidity. We report a case of tuberculosis of bone marrow with concomitant secondary hemophagocytic lympho-histiocytosis, bringing into light the importance of clinical suspicion and evaluation of bone marrow being a primary site of involvement in patients of disseminated tuberculosis.

Diagnostic Challenge in Heterotopic Pancreas in the Ampulla of Vater with Obstructive Jaundice - A Case Report and Literature Review.

Heterotopic pancreas is a rare congenital abnormality. The most common location is the stomach, duodenum and proximal jejunum. Rare locations are represented by the ampulla of Vater, esophagus, ileum, Meckel diverticulum, biliary tract, mesentery and spleen. We present the case of a 49 year old patient investigated for obstructive jaundice and diagnosed with an ampullar heterotopy of pancreas parenchyma, initially considered to be a malignant tumor. A Whipple pancreatoduodenectomy was performed with good postoperative evolution, the serum levels of bilirubin being normal after the first postoperative week.

Case Report: An Unreported Presentation of Lemmel Syndrome Highlighting Diagnostic Challenges of Duodenal Masses.

We present a case involving a 67-year-old patient with a medical history of gastric bypass who was recently diagnosed with a 6-centimeter duodenal mass causing biliary duct stenosis. Despite our best efforts, we were unable to access this tumor endoscopically, necessitating surgical intervention. During the surgical exploration, we discovered a duodenal diverticulum filled with stones, leading to the obstruction of the biliary ductâ?"a manifestation of Lemmel syndrome. This rare condition is characterized by obstructive jaundice in the absence of choledocholithiasis or tumors and is secondary to dilatation of peri-ampullary diverticula. While it is typically managed through endoscopy, our diagnostic and therapeutic approach was complicated by the patient's history of bariatric surgery (gastric bypass), making endoscopic access impossible despite our multiple attempts. This case report sheds light on the challenges posed by the concurrence of a rare pathology and surgically modified anatomy, which is increasingly encountered in daily surgical practice. In such situations, exploratory surgery continues to play a significant role.

Malignancy and mass-forming phenotypes of IgG4-related disease: a challenging diagnosis.

Mass-forming phenotypes of IgG4-related disease (IgG4-RD) mimic malignancy and histological confirmation can be challenging. A woman in her 70s with HIV infection presented with painless obstructive jaundice and weight loss. Magnetic resonance imaging was suggestive of unresectable cholangiocarcinoma. Tumour markers and serum IgG4 were normal. Percutaneous liver biopsy was consistent with IgG4-RD inflammatory pseudotumour, with complete response to glucocorticoid therapy. Two years later, a new episode of obstructive jaundice occurred, with CT showing a solid lesion in the head of the pancreas with double duct sign and encasement of the portal vein. Re-induction therapy was tried without response. Fine-needle biopsy was consistent with pancreatic cancer. Supportive care was offered and the patient died 8 months later, with no signs of disease progression on subsequent imaging. We discuss the challenges of IgG4-RD diagnosis and treatment and the differential diagnosis between mass-forming phenotypes and malignancy, highlighting the difficulties in managing such patients.

A Rare Case of Primary B-Cell Lymphoma of the Pancreas.

Primary pancreatic lymphomas (PPLs) are a subgroup of gastrointestinal (GI) lymphomas. They are an exceedingly rare entity, both in terms of pancreatic malignancies and also extranodal lymphomas. Epidemiological investigations have been challenging to do because of their rarity. This has resulted in a lack of clarity on the clinicopathological characteristics, differential diagnosis, best course of treatment, and prognosis of PPL. Because the clinical signs are frequently non-specific, it can lead to a diagnostic hazard for the unwary physician. Preoperatively, it is imperative to distinguish between adenocarcinoma and PPL, as they present similarly, but have vastly different treatment modalities and prognosis. We herein present a case of an elderly man who presented with obstructive jaundice and was found to have PPL.

Outcomes of jaundice in advanced hepatocellular carcinoma - a sub-Saharan perspective.

BACKGROUND: Jaundice is a marker of advanced disease and poor outcomes in hepatocellular carcinoma (HCC). The aim of this study was to describe and analyse the management and outcomes of jaundiced HCC patients at a large academic referral centre in sub-Saharan Africa (SSA). METHODS: Treatment-naïve adult HCC patients who presented with jaundice between 1990 and 2023 were analysed. RESULTS: During the inclusion period, 676 HCC patients were treated at Groote Schuur Hospital. The mean age of the 126 (18.6%) who were jaundiced was 48.8 (± 13.2) years. Eighty-nine (70.6%) were male. Ninety-four (74.6%) patients with jaundice secondary to diffuse tumour infiltration had best supportive care (BSC) only. Thirty-two had obstructive jaundice (OJ); four were excluded because of missing hospital records. In 28 of these patients, 16 underwent biliary drainage (BD) and 12 received BSC only. The mean overall survival (OS) of the 126 patients was 100.5 (± 242.3) days. The patients with diffuse tumour infiltration had an OS of 105.9 (± 273.3) days. The patients with OJ survived 86.5 (± 135.0) days. There was no significant difference in OS between the three patient groups (p = 0.941). In the OJ group, patients who underwent BD survived longer than the BSC group (117.9 ± 166.4 vs. 29.2 ± 34.7 days, p = 0.015).

Effect of preoperative biliary drainage on intraoperative biliary cultures and surgical outcomes after pancreatic resection.

BACKGROUND: Prolonged obstructive jaundice (OJ), associated with resectable pancreatic pathology, has many deleterious effects that are potentially rectifiable by preoperative biliary drainage (POBD) at the cost of increased postoperative infective complications. The aim of this study is to assess the impact of POBD on intraoperative biliary cultures (IBCs) and surgical outcomes in patients undergoing pancreatic resection. METHODS: Data from patients at Groote Schuur Hospital, Cape Town, between October 2008 and May 2019 were analysed. Demographic, clinical, and outcome variables were evaluated, including perioperative morbidity, mortality, and 5-year survival. RESULTS: Among 128 patients, 69.5% underwent POBD. The overall perioperative mortality in this study was 8.8%. The POBD group had a significantly lower perioperative mortality rate compared to the non-drainage group (5.6% vs. 25.6%). POBD patients had a higher incidence of surgical site infections (55.1% vs. 23.1%), polymicrobial growth from IBCs and were more likely to culture resistant organisms. Five-year survival was similar in the two groups. CONCLUSION: POBD was associated with a high incidence of resistant organisms on the IBCs, a high incidence of surgical site infections and a high correlation between cultures from the surgical site infection and the IBCs.

Risk prediction of cholangitis after stent implantation based on machine learning.

The risk of cholangitis after ERCP implantation in malignant obstructive jaundice patients remains unknown. To develop models based on artificial intelligence methods to predict cholangitis risk more accurately, according to patients after stent implantation in patients' MOJ clinical data. This retrospective study included 218 patients with MOJ undergoing ERCP surgery. A total of 27 clinical variables were collected as input variables. Seven models (including univariate analysis and six machine learning models) were trained and tested for classified prediction. The model' performance was measured by AUROC. The RFT model demonstrated excellent performances with accuracies up to 0.86 and AUROC up to 0.87. Feature selection in RF and SHAP was similar, and the choice of the best variable subset produced a high performance with an AUROC up to 0.89. We have developed a hybrid machine learning model with better predictive performance than traditional LR prediction models, as well as other machine learning models for cholangitis based on simple clinical data. The model can assist doctors in clinical diagnosis, adopt reasonable treatment plans, and improve the survival rate of patients.

Comparison of the clinical effectiveness of hepaticojejunostomy and self-expanding metal stents for bypassing the bile ducts in patients with unresectable pancreatic head cancer complicated by obstructive jaundice.

OBJECTIVE: Aim: To improve treatment outcomes of patients with unresectable pancreatic head cancer complicated by obstructive jaundice by improving the tactics and techniques of surgical interventions. PATIENTS AND METHODS: Materials and Methods: Depending on the treatment tactics, patients were randomised to the main group (53 people) or the comparison group (54 people). The results of correction of obstructive jaundice by Roux-en-Y end to side hepaticojejunostomy (main group) and common bile duct prosthetics with self-expanding metal stents (comparison group) were compared. RESULTS: Results: The use of self-expanding metal stents for internal drainage of the biliary system compared to hepaticojejunostomy operations reduced the incidence of postoperative complications by 29.9% (χ2=13.7, 95% CI 14.38-44.08, p=0.0002) and mortality by 7.5% (χ2=4.16, 95% CI -0.05-17.79, p=0.04). Within 8-10 months after biliary stenting, 11.1% (6/54) of patients developed recurrent jaundice and cholangitis, and another 7.4% (4/54) of patients developed duodenal stenosis with a tumour. These complications led to repeated hospitalisation and biliary restentation in 4 (7.4%) cases, and duodenal stenting by self-expanding metal stents in 4 (7.4%) patients. CONCLUSION: Conclusions: The choice of biliodigestive shunting method should be selected depending on the expected survival time of patients. If the prognosis of survival is up to 8 months, it is advisable to perform prosthetics of the common bile duct with self-expanding metal stents, if more than 8 months, it is advisable to perform hepaticojejunal anastomosis with prophylactic gastrojejunal anastomosis.

Ultrasound-guided percutaneous transhepatic biliary drainage for distal biliary malignant obstructive jaundice.

The main cause of distal biliary malignant obstructive jaundice (DBMOJ) is the stricture of the extrahepatic biliary tract by malignant tumors, including pancreatic head and uncinate process cancer, low-grade cholangiocarcinoma, duodenal cancer, papillary duodenal cancer and other malignant tumors. The most effective treatment is radical pancreaticoduodenectomy. However, preoperative obstructive jaundice can affect the patient's liver function and blood coagulation function, increase local inflammation and oedema, and make surgery more difficult. Patients with severe obstructive jaundice require preoperative biliary drainage, which can be achieved by various methods, including ultrasound endoscopic biliary drainage (EUS-EBD) and endoscopic retrograde biliary drainage (ERBD). The latter is mainly divided into endoscopic nasobiliary drainage and endoscopic biliary stent. Some patients underwent percutaneous transhepatic biliary drainage (PTBD) when ERBD and EUS-EBD failed. In this study, we aimed to identify PTBD in DBMOJ and to further investigate the role of the puncture pathway in DBMOJ. The relationship between PTBD and bile duct internal diameter was confirmed by analysing and collating clinical data. In this study, DBMOJ was grouped according to bile duct internal diameter and liver function was used as an indicator to examine the improvement in liver function with PTBD in patients undergoing DBMOJ. Analysis of puncture complications showed that PTBD puncture was safe. DBMOJ with different bile duct internal diameters had different rates of liver function improvement after PTBD. The right-side approaches had significantly lower alanine aminotransferase (ALT) and alanine transaminase (AST) than the left-side approaches. This study showed that PTBD for DBMOJ is associated with a low complication rate and good reduction of jaundice. Liver function recovery was faster in patients with DBMOJ treated with PTBD in the right-sided approach compared with the left-sided approach. PTBD is an effective tool to be used in patients who have failed ERBD and EUS-EBD.

Pancreaticoduodenectomy after bilirubin adsorption for distal cholangiocarcinoma with severe obstructive jaundice refractory to repeat preoperative endoscopic biliary drainage: a case report.

The necessity of biliary drainage before pancreaticoduodenectomy remains controversial in cases involving malignant obstructive jaundice; however, the benefits of biliary drainage have been reported in cases with severe hyperbilirubinemia. Herein, we present the case of a 61-year-old man suffering from jaundice due to distal cholangiocarcinoma. In this case, obstructive jaundice was refractory to repeat endoscopic drainage and bilirubin adsorption. Hyperbilirubinemia persisted despite successful implementation of biliary endoscopic sphincterotomy and two rounds of plastic stent placements. Stent occlusion and migration were unlikely and oral cholagogues proved ineffective. Owing to the patient's surgical candidacy and his aversion to nasobiliary drainage due to discomfort, bilirubin adsorption was introduced as an alternative therapeutic intervention. Following repeated adsorption sessions, a gradual decline in serum total bilirubin levels was observed and pancreaticoduodenectomy was scheduled. The patient successfully underwent pancreaticoduodenectomy with portal vein resection and reconstruction and D2 lymph node dissection. After the surgery, the serum bilirubin levels gradually decreased and the patient remained alive, with no recurrence at 26 months postoperatively. Therefore, this case highlights the feasibility and safety of performing pancreaticoduodenectomy in patients with severe, refractory jaundice who have not responded to repeated endoscopic interventions and have partially responded to bilirubin adsorption.

A Pilot Study for Biliary Atresia Diagnosis: Fluorescent Imaging of Indocyanine Green in Stool.

BACKGROUND: Biliary atresia is the most common cause of obstructive jaundice in infants and conventional cholangiography is the current diagnostic gold standard. Fluorescent cholangiography with indocyanine green can enhance biliary tree visualization during surgery because it is exclusively excreted into the bile ducts and eventually into the intestine. Therefore, we hypothesized that indocyanine green presence in stool could confirm bile duct patency in infants. METHODS: A prospective single center cohort study was performed on infants (age ≤ 12 months) with and without jaundice after obtaining IRB approval. Indocyanine green was administered intravenously (0.1 mg/kg). Soiled diapers collected post-injection were imaged for fluorescence. RESULTS: After indocyanine green administration, fluorescence was detected in soiled diapers for control patients (n = 4, x = 14 h22 m post-injection) and jaundiced patients without biliary atresia (n = 11, x = 13 h28 m post-injection). For biliary atresia patients (n = 7), post-injection soiled diapers before and after Kasai portoenterostomy were collected. Fluorescence was not detected in stool from 6 of 7 biliary atresia patients. As a test, indocyanine green detection in stool was 97% accurate for assessing biliary patency. CONCLUSION: Fluorescent Imaging for Indocyanine Green (FIInd Green) in stool is a fast and accurate approach to assess biliary patency non-invasively in infants. LEVEL OF EVIDENCE: Level III.

A therapeutic ERCP in an only 5.9 kg infant with obstruction jaundice using JF-260V duodenoscope.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) has found extensive use in pediatric patients; however, challenges persist in the application of therapeutic ERCP in infants. CASE PRESENTATION: This case report details the presentation of a 5.9-kilogram infant with obstructive jaundice and suspected hemolytic anemia who underwent ERCP to alleviate biliary obstruction. The infant was admitted due to clay-colored stools, jaundice, and liver injury. Ultrasound and magnetic resonance cholangiopancreatography (MRCP) revealed dilation of the common bile duct (CBD) accompanied by the presence of stones. ERCP was conducted using a JF-260V duodenoscope under general anesthesia. Successful stone extraction and biliary drainage were achieved. CONCLUSIONS: In centers with considerable expertise in ERCP and pediatric anesthesia, the use of a conventional adult duodenoscope for therapeutic ERCP in infants can be considered safe and feasible, provided careful and stringent patient selection criteria are applied. In the future, clear guidelines and standardized protocols for the indications and procedures of pediatric ERCP should be established.

Perioperative outcomes after open biliary bypass for malignant biliary obstruction (MBO) in resource-limited setups; a multicenter prospective cohort study, 2023.

INTRODUCTION: Obstructive jaundice is the most common symptom of malignant diseases of the extrahepatic biliary system and necessitates either non-operative or operative biliary bypass. Because of percutaneous and endoscopic approaches, the use of palliative surgical procedures has decreased in recent years. However, in resource-limited situations, open biliary bypasses remain a viable option. This study aimed to identify factors associated with adverse perioperative outcomes following open biliary bypass. METHODS: From June 2022 to May 2023, 69 patients underwent open biliary bypass for malignant biliary obstruction. Postoperative morbidity and mortality within 30 days of surgery were assessed. A Kaplan-Meier was used for categorical variables, and a log-rank test was used to determine the statistically significant difference between variables. A Cox regression analysis was conducted to identify factors associated to time to develop complications. RESULTS: The hazard of developing complications among those with preoperative cholangitis was 2.49 times higher than those without preoperative cholangitis (HR 2.49, 95% CI [1.06, 5.84]). For every hour increment in the length of surgery, the hazard of getting complications increased by 2.47 times (HR 2.47, 95% CI [1.28, 4.77]). As serum bilirubin increased by 1 mg/dl, the hazard of developing complications increased by 14% (HR 1.14, 95% CI [1.03, 1.17]). CONCLUSION: Patients who had long operation times, preoperative cholangitis, and elevated total bilirubin levels are at increased risk for poor perioperative outcomes. Clinicians may use these results to optimize these patients to decrease their elevated risk of serious morbidity and mortality.

Safety of biliary drainage with 6-mm metallic stent for preoperative obstructive jaundice in pancreatic cancer: PURPLE SIX STUDY.

BACKGROUND AND AIM: The 10-mm self-expandable metal stent (SEMS) is the standard for endoscopic transpapillary biliary drainage before pancreatic cancer surgery. However, the efficacy of stents thinner than 10 mm has not been adequately validated. Therefore, we aimed to evaluate the safety of a 6-mm fully covered SEMS (FCSEMS) for distal malignant biliary obstruction (DMBO) during preoperative chemotherapy for pancreatic cancer. METHODS: This was a single-arm, multicenter, prospective phase II study of endoscopic transpapillary initial biliary drainage for DMBO before pancreatic cancer surgery. The primary endpoint was stent-related adverse events, and the key secondary endpoint was the non-recurrent biliary obstruction (non-RBO) rate during the observation period for both resectable (R) and borderline resectable (BR) pancreatic cancers. RESULTS: The study enrolled 33 patients, among whom 32 received the study treatment. There were 23 and 9 cases of R and BR pancreatic cancers, respectively. The technical and clinical success rates were 97.0% and 90.1%, respectively. The stent-related adverse event rate was 3.1% (n = 1, acute pancreatitis) (95% confidential interval, 0.00-16.2), which met the criteria to be considered safe. The overall non-RBO rate during the observation period (median 96 days) was 78.1% (82.6% and 66.7% for R and BR pancreatic cancer cases, respectively). CONCLUSIONS: The 6-mm FCSEMS is an extremely safe metallic stent with a low stent-related adverse event rate of 3.1% for preoperative biliary drainage in pancreatic cancer. It is considered the optimal stent for preoperative biliary drainage in terms of the non-RBO rate. UMIN Clinical Trial Registry (UMIN-CTR 000041704).

Utility of enteral nutrition via percutaneous transhepatic cholangiography drainage catheterization in late-stage malignant obstructive jaundice.

Objective: The purpose of this study was to explore the clinical benefits of establishing an enteral nutrition (EN) pathway via percutaneous transhepatic cholangiography drainage (PTCD) catheterization in patients with late-stage malignant obstructive jaundice (MOJ).Methods: We selected 30 patients diagnosed as having late-stage MOJ with malnutrition. A dual-lumen biliary-enteral nutrition tube was placed via PTCD along with a biliary stent implantation. Postoperative EN was provided, and we observed the time taken for tube placement, its success rate, complications, and therapeutic efficacy.Results: Tube placement was successful in all 30 patients with an average procedural time of 5.7 ± 1.4 min with no tube placement complications. Compared to preoperative measures, there was a significant improvement in postoperative jaundice reduction and nutritional indicators one month after the procedure (p < 0.05). Post-placement complications included tube perileakage in 5 cases, entero-biliary reflux in 4 cases, tube blockage in 6 cases, tube displacement in 4 cases, accidental tube removal in 3 cases, and tube replacement due to degradation in 8 cases, with tube retention time ranging from 42 to 314 days, averaging 124.7 ± 37.5 days. All patients achieved the parameters for effective home-based enteral nutrition with a noticeable improvement in their quality of life.Conclusion: In this study, we found that the technique of establishing an EN pathway via PTCD catheterization was minimally invasive, safe, and effective; the tube was easy to maintain; and patient compliance was high. It is, thus, suitable for long-term tube retention in patients with late-stage MOJ.

[Endoscopic stenting for malignant pancreatobiliary strictures]. / Endoskopicheskoe stentirovanie pri opukholevykh strikturakh pankreatobiliarnoi zony.

OBJECTIVE: To improve the outcomes in patients with malignant obstructive jaundice using intraluminal stenting. MATERIAL AND METHODS: The present study included 62 patients with clinical symptoms of malignant obstructive jaundice. In the main group, we performed biliary stenting with self-expanding multi-perforated stents (Hanarostent Multi-hole Biliary). Microscopic perforations of these stents prevent migration and reduce the risk of blocking the cystic and main pancreatic ducts. In the control group, stenting was performed with fully and partially covered self-expanding stents. RESULTS: Lower incidence of obstructive cholecystitis and acute pancreatitis in the main group was associated with multiperforated stents reducing the risk of blocking the main pancreatic and cystic ducts. CONCLUSION: In our study, multiperforated stents excluded migration and reduced the incidence of complications (acute cholecystitis from 11.5 to 3.8%, acute pancreatitis from 15.3 to 7.7%).