A prospective multicentre study evaluating the performance of the modified simple biliary atresia scoring system in predicting biliary atresia.

PURPOSE: Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates. We evaluate the performance of the modified Simple BA Scoring System (SBASS) in diagnosing BA. METHODS: We performed a prospective, cross-sectional study on infants with cholestatic jaundice (June 2021-December 2022). Modified SBASS scoring was applied and compared to the eventual diagnosis (as per intraoperative cholangiogram (IOC) and liver histopathology). The score (0-6), consists of gall bladder length < 1.6 cm (+ 1), presence of triangular cord sign (+ 1), conjugated bilirubin:total bilirubin ratio > 0.7(+ 2), gamma-glutamyl transferase (GGT) ≥ 200 U/L (+ 2). RESULTS: 73 were included: Fifty-two (71%) had BA. In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15 (72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the modified SBASS showed sensitivity of 96.2%, specificity of 61.9% and overall accuracy of 86.3% in diagnosing BA. Area under receiver operating characteristic curve was 0.901. GGT had the highest sensitivity (94.2%), while triangular cord sign showed the highest specificity at 95.2%. CONCLUSION: The SBASS provides a bedside, non-invasive scoring system for exclusion of BA in infantile cholestatic jaundice and reduces the likelihood of negative surgical explorations.

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.

[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%).

[Percutaneous cholecystostomy in multi-field hospital]. / Chreskozhnaya kholetsistostomiya v mnogoprofil'nom statsionare.

OBJECTIVE: To analyze the indications, technical features and results of percutaneous cholecystostomy. MATERIAL AND METHODS: A retrospective single-center study of the results of percutaneous cholecystostomy over 13-year period was carried out. The indications for surgery, technical features and outcomes were studied. RESULTS: The indications for percutaneous cholecystostomy were acute cholecystitis in 40 (63.5%) cases and obstructive jaundice in 23 (36.5%) cases. In acute cholecystitis, cholecystectomy was denied due to severe acute and decompensated chronic diseases. In case of obstructive jaundice, cholecystostomy was preferred if other methods of biliary decompression were impossible. Drainage with locking thread was used in 44 (69.8%) patients. A total of 13 (21.3%) cases of drainage migration were noted. Incidence of migration of catheters with locking threads was 13.6%, without locking threads - 41.2% (p=0.033). Subsequent cholecystectomy was performed in 10 (15.9%) patients. In case of obstructive jaundice, cholecystostomy did not lead to destructive cholecystitis in any case. Six patients with acute cholecystitis had progressive gallbladder destruction. Overall postoperative in-hospital mortality was 36.5% (n=23). Mortality in the group of acute cholecystitis was 32.5% (n=13), in the group of obstructive jaundice - 43.5% (n=10). Mortality was higher in acute cholecystitis Grade III (75.0%) compared to Grade II (21.9%; p=0.008). CONCLUSION: Cholecystostomy is a rare (reserve) intervention. Locking thread significantly reduces the incidence of migration of cholecystostomy catheter. Progressive gallbladder destruction required cholecystectomy in 15% of cases. Treatment of patients with acute cholecystitis depends on physical status and comorbidities.

[Hemobilia as a sign of arterio-biliary fistula]. / Gemobiliya kak proyavlenie arteriobiliarnoi fistuly.

Hepatic artery aneurysms (HAA) are rare (20% of all visceral arteries). Most often, HAAs are asymptomatic and detected at autopsy. However, their ruptures and/or bleeding following pressure ulcers in visceral gastrointestinal organs are a significant clinical and diagnostic problem. We present 2 patients with obstructive jaundice and hemobilia. Diagnostics revealed aneurysm of the right hepatic artery with arterio-biliary fistula. Life-threatening hemobilia is a consequence of HAA rupture into biliary system. Endovascular approach is preferable for HAA without clinical manifestations. Awareness of this disease is important for early detection and active surgical intervention before possible complications.

[Endoscopic treatment of obstructive jaundice in patients with Klatskin tumor]. / Endoskopicheskoe lechenie patsientov s mekhanicheskoi zheltukhoi pri opukholi Klatskina.

OBJECTIVE: To improve treatment outcomes in patients with Klatskin tumor and obstructive jaundice by using of endoscopic bilioduodenal stenting. MATERIAL AND METHODS: There were 1904 transpapillary interventions between August 2017 and February 2022. Endoscopic bilioduodenal stenting was performed in 250 patients including 25 (10%) ones with Klatskin tumor. RESULTS: Bilioduodenal plastic and self-expanding stents were installed in 19 (76%) and 6 (24%) patients, respectively. In Klatskin tumor type I, 11 patients (44%) underwent bilioduodenal stenting of common hepatic duct with plastic stent; 5 (20%) patients with Klatskin tumor type II received self-expanding stents. In case of tumor type IIIA, 3 (12%) patients underwent stenting of the right lobar duct with plastic stent. Four (16%) patients with Klatskin tumor type III B underwent stenting of the left lobar duct. Two 2 (8%) patients with Klatskin tumor type IV underwent bilateral bilioduodenal stenting with plastic and bifurcation self-expanding stents. Peroral cholangioscopy using the SpyGlass DS system was performed in 4 (16%) patients. No intraoperative complications were identified. One (4%) patient developed gastrointestinal bleeding in 2 postoperative days after retrograde intervention that did not require surgery. Moreover, 1 (4%) patient with distal dislocation of plastic bilioduodenal stent required redo bilioduodenal stenting. Three (12%) patients died from multiple organ failure despite adequate biliary decompression, and 22 (88%) patients were discharged in 8±5 days after retrograde intervention. CONCLUSION: Bilioduodenal stenting as minimally invasive and physiological method was highly effective for obstructive jaundice in patients with Klatskin tumor. Peroral cholangioscopy using the SpyGlass system provides effective and safe direct visualization of the biliary tract, as well as biopsy for morphological verification and prescription of chemotherapy in patients with intraductal growth of tumor.

An unusual cause of obstructive jaundice in childhood: Intra-choledochal malignant neuroendocrine tumour.

The extrahepatic biliary apparatus is a rare site for neuroendocrine tumours. A 13-year-old child presented with cholestatic symptoms of jaundice and pruritus with soft hepatomegaly and mild ascites. Magnetic resonance imaging and endoscopic ultrasound revealed a mid-common bile duct mass, and dilated intrahepatic biliary system. An en-bloc resection of the extrahepatic biliary apparatus, showed malignant cells disposed in lobules in a desmoplastic stroma with intramural invasion, staining positive for cytokeratin, chromogranin, synaptophysin and negative for CD56. At 3 months post-resection, whole body positron emission tomography scan was normal with no recurrence at 24 months.

Percutaneous transluminal forceps biopsy with an adjustable curved sheath for obstructive jaundice.

PURPOSE: To evaluate the feasibility and safety of percutaneous transluminal forceps biopsy (PTFB) with an adjustable curved sheath in patients with obstructive jaundice. MATERIAL AND METHODS: Forty-two patients who underwent PTFB with an adjustable curved sheath were analyzed retrospectively. Sensitivity, specificity, negative predictive value (NPV), positive predictive value (PPV) and accuracy were calculated for all populations and in different situations. Technical success and safety were evaluated. RESULTS: The technical success rate was 100%. Thirty-five of 42 cases were diagnosed malignant diseases, the sensitivity of PTFB with an adjustable curved sheath was 74.29% (26/35), the specificity was 100%, the positive predictive value was 100%, the negative predictive value was 43.75% (7/16), and the accuracy rate was 78.57% (33/42). There was a better sensitivity for bile duct malignancies when compared with non-bile duct malignancies (p = 0.012). No statistical difference was found in the sensitivity of the upper part of the biliary tree and the lower part of the biliary tree, and none in the sensitivity of different approaches (left vs. right). The complication rate was 11.90%, and no serious complications were observed. CONCLUSIONS: PTFB with an adjustable curved sheath is an effective and safe technique, without being limited by approaches and obstruction sites.

[Oral cholangioscopy for biliary system diseases]. / Vozmozhnosti peroral'noi videokholangioskopii pri zabolevaniyakh zhelchevydelitel'noi sistemy.

OBJECTIVE: To improve the results of treatment of obstructive jaundice by using of oral cholangioscopy. MATERIAL AND METHODS: There were 321 patients with obstructive jaundice between October 2020 and November 2021. Of these, cholangioscopy (SpyGlass video system) was used in 18 patients. Patients were divided into two groups: group 1 (n=9) - malignant biliary strictures; group 2 (n=9) - choledocholithiasis with large calculi (≥1.2 cm). At admission, all patients underwent laboratory and instrumental examination. In the first group, bilioduodenal stenting with plastic stents 7 and 10 Fr in diameter, 7 to 12 cm long or self-expanding nitinol stents 0.8-1.0 cm in diameter, 6 to 10 cm long was carried out. Patients with large calculi underwent targeted laser lithotripsy under endoscopic control until formation of 1-cm fragments. These fragments were removed using a lithoextraction balloon and Dormia basket. RESULTS: Cholangioscopy (SpyGlass system) was performed within 2 days after admission. In group 1, tumor tissue overgrowths were found during cholangioscopy. Five out of 9 (55.6%) patients underwent bilioduodenal stenting for adequate biliary drainage. Of these, 4 (44.5%) patients developed acute edematous pancreatitis on the first postoperative day. This complication regressed after 3-4 days under therapy. Four (44.5%) patients died from cancer-related multiple organ failure. In group 2, cholangioscopy effectively visualized the calculus and ensured its destruction by laser contact lithotripsy. Intraoperative and postoperative complications were not revealed in both groups. CONCLUSION: SpyGlass system is effective and safe for diagnosis and treatment in 100% of patients with extrahepatic biliary strictures and/or large calculi.

Prediction Efficacy for Clinical Outcome of Prognostic Nutritional Index in Patients with Resectable Biliary Tract Cancer Depends on Sex and Obstructive Jaundice Status.

BACKGROUND: The Prognostic Nutritional Index (PNI), a marker of nutritional status and systemic inflammation, is a proven prognostic biomarker in some cancers. The predictive value of PNI in biliary tract cancer (BTC) has not been established. OBJECTIVE: The aim of this study was to determine the relationship between the PNI and outcomes of resectable BTC. METHODS: In total, 430 patients with stage I-III resectable BTC [gallbladder cancer (GBC), n = 212; cholangiocarcinoma (CHO), n = 218] who had attended Fudan University Zhongshan Hospital were enrolled. The relationship between the PNI and clinical outcomes was evaluated both in the whole cohort and in selected subgroups. RESULTS: Eligible patients were classified into PNI-low (PNI < 45) and PNI-high (PNI ≥ 45) groups. The PNI-low group had significantly worse overall survival (OS) in both the whole cohort (p = 0.002) and in the GBC subgroup (p = 0.001), but had similar OS as the PNI-high group in the CHO subgroup (p = 0.328). Multivariate analysis revealed that low PNI is an independent risk factor for worse survival in GBC (hazard ratio 1.623, 95% confidence interval 1.063-2.480, p = 0.026). PNI was found to predict clinical outcome in women (p < 0.001) and patients without obstructive jaundice (p = 0.017) with GBC, but was not a prognostic factor in any subgroup with CHO. The estimated area under the time-dependent receiver operating characteristic curve was significantly greater when TNM stage was combined with PNI in women with GBC. CONCLUSIONS: PNI is an independent predictor of OS in GBC, but not in CHO. It has no prognostic value in men with GBC or patients with obstructive jaundice.

Cannabinoid Receptor-2 Activation in Keratinocytes Contributes to Elevated Peripheral ß-Endorphin Levels in Patients With Obstructive Jaundice.

BACKGROUND: Cholestatic diseases are often accompanied by elevated plasma levels of endogenous opioid peptides, but it is still unclear whether central or peripheral mechanisms are involved in this process, and little is known about the change of pain threshold in these patients. The purpose of this study was to determine the preoperative pain threshold, postoperative morphine consumption, and central and peripheral ß-endorphin levels in patients with obstructive jaundice. This study also tests the hypothesis that activation of the cannabinoid receptor-2 (CB2R) in skin keratinocytes by endocannabinoids is the mechanism underlying circulating ß-endorphin elevation in patients with obstructive jaundice. METHODS: The electrical pain thresholds, 48-hour postoperative morphine consumption, concentrations of ß-endorphin in plasma and cerebrospinal fluid, skin and liver ß-endorphin expression, and plasma levels of endocannabinoids were measured in jaundiced (n = 32) and control (n = 32) patients. Male Sprague-Dawley rats and human keratinocytes (human immortalized keratinocyte cell line [HaCaT]) were used for the in vivo and in vitro experiments, respectively. Mechanical and thermal withdrawal latency, plasma level, and skin expression of ß-endorphin were measured in CB2R-antagonist-treated and control bile duct-ligated (BDL) rats. In cultured keratinocytes, the effect of CB2R agonist AM1241-induced ß-endorphin expression was observed and the phosphorylation of extracellular-regulated protein kinases 1/2, p38, and signal transducer and activator of transcription (STAT) pathways were investigated. RESULTS: This study found (1) the plasma level of ß-endorphin (mean ± standard error of the mean [SEM]) was 193.9 ± 9.6 pg/mL in control patients, while it was significantly increased in jaundiced patients (286.6 ± 14.5 pg/mL); (2) the electrical pain perception threshold and the electrical pain tolerance threshold were higher in patients with obstructive jaundice compared with controls, while the 48-hour postoperative morphine consumption was lower in the jaundiced patients; (3) there was no correlation between plasma ß-endorphin levels, electrical pain thresholds, and 48-hour postoperative morphine consumption in patients with obstructive jaundice; (4) the plasma level of the endogenous cannabinoid anandamide was increased in the jaundiced patients; (5) CB2R antagonist treatment of the BDL rats reduced ß-endorphin levels in plasma and skin keratinocytes, while it did not alter the nociceptive thresholds in BDL and control rats; (6) the endocannabinoid anandamide-induced ß-endorphin synthesis and release via CB2R in cultured keratinocytes; and (7) phosphorylation of extracellular-regulated protein kinases 1/2 is involved in the CB2R-agonist-induced ß-endorphin expression in keratinocytes. CONCLUSIONS: CB2R activation in keratinocytes by the endocannabinoid anandamide may play an important role in the peripheral elevation of ß-endorphin during obstructive jaundice.

Hemosuccus Pancreaticus and Obstructive Jaundice: Two Rare Complications of Chronic Pancreatitis Treated by Arterial Embolization.

The formation of a peripancreatic pseudoaneurysm is a rare and serious complication of chronic pancreatitis. In this report, we present the case of a 56-year-old male with a history of alcohol-related chronic pancreatitis resulting in both hemosuccus pancreaticus and obstructive jaundice due to a pseudoaneurysm of the gastroduodenal artery that was treated successfully by angiographic coiling.

[Neurofibroma of the bile duct: a rare cause of cholestatic jaundice that mimics Klatskin’s tumor]. / Neurofibroma de la vía biliar: una causa rara de ictericia colestásica que imita el tumor de Klatskin.

The neurofibroma of the bile duct is an extremely rare and generally asymptomatic disease, however, it maybe cause obstructive jaundice and mimic a Klatskin tumor, leading to radical surgical treatment and increased patient morbidity. We present the case of a 62-year-old woman who underwent cholecystectomy 10 years ago, with jaundice and pain in the right upper quadrant, as well as auxiliary tests compatible with cholestasis. Dilation of the bile duct and the presence of a tumor in the left hepatic duct were observed by magnetic cholangioresonance. The preoperative clinical diagnosis was hilar cholangiocarcinoma, but the histopathological study revealed a neoplasm composed of spindle cells without atypia or mitotic activity, whose neurogenic lineage supported by its positivity to protein S100 in immunohistochemistry. We report the case given its infrequency in the literature and its relevance, as it is a benign entity, as a differential diagnosis of cancer.

[Management of obstructive jaundice in patients with neoplasms of the major duodenal papilla]. / Taktika lecheniya mekhanicheskoi zheltukhi u patsientov s novoobrazovaniyami bol'shogo sosochka dvenadtsatiperstnoi kishki.

OBJECTIVE: To develop the indications and assess an effectiveness of treatment of patients with ampullary tumors followed by mechanical jaundice. MATERIAL AND METHODS: There were 26 patients with major duodenal papilla neoplasms for the period 2015-2020 at the Sklifosovsky Research Institute for Emergency Care. RESULTS: Twenty patients underwent transpapillary interventions: papillosphincterotomy followed by lithoextraction and bilio-duodenal stenting in 4 (15.3%) patients, bilio-duodenal stenting in 12 (46.1%) patients, nasobiliary drainage in 2 (7.6%) patients, pancreaticoduodenal stenting in 2 (7.6%) patients. Percutaneous transhepatic microcholecystostomy was performed in 6 (23.4%) patients. In all cases, laboratory values decreased in 5-7 days after drainage. Eight (30.7%) patients refused further surgical treatment and were discharged in satisfactory condition. Another 8 (44.5%) patients underwent endoscopic submucosal papillectomy. There were no postoperative complications. Patients were discharged after 5-7 days. Four (22.2%) patients underwent tumor resection via laparotomy. One of these patients required redo laparotomy in postoperative period due to acute perforated duodenal ulcer. Six (33.3%) patients underwent palliative bilio-duodenal stenting. CONCLUSION: Invasion of muscle layer or distal parts of the common bile duct, as well as abnormal vascularization are indications for open surgery or palliative endoscopic treatment. Tumor location within mucous and muscle layers without invasion of distal third of the common bile duct and no abnormal vascularization of tumor justify endoscopic papillectomy. Endoscopic approach can be considered as a final minimally invasive method with minimal risk of postoperative complications in case of benign ampullary tumor.

Presentation of Severe Acute Respiratory Syndrome-Coronavirus 2 Infection as Cholestatic Jaundice in Two Healthy Adolescents.

Liver abnormalities in severe acute respiratory syndrome-coronavirus 2 infection, including hepatitis and cholestasis, have been observed in adults and are associated with worse outcomes. We describe 2 adolescents with cholestasis and hepatitis with mild presentation of severe acute respiratory syndrome-coronavirus 2 lacking typical symptoms. Our intention is to raise index of suspicion for testing and protective equipment use.

Obstructive jaundice caused by ulcerative duodenal stenosis: A case report.

A peptic ulcer is a rare cause of distal common bile duct stricture, Obstructive jaundice as a complication of ulcerative duodenal stenosis is quite difficult to differentiate from malignant disease, especially in those in which esophagogastroduodenoscopy examination does not reveal an ulcer. In this case report, a 61-year-old male suffered from right upper quadrant pain, chills and fever caused by duodenal and distal common bile duct stenosis originating from ulcer and was treated surgically.

[Russian consensus on current issues in the diagnosis and treatment of obstructive jaundice syndrome]. / Rossiiskii konsensus po aktual'nym voprosam diagnostiki i lecheniya sindroma mekhanicheskoi zheltukhi.

The Russian consensus document on topical issues of the diagnosis and treatment of obstructive jaundice syndrome was prepared by a group of experts in various fields of surgery, endoscopy, interventional radiology, radiological diagnosis and intensive care. The goal of this document is to clarify and consolidate the opinions of national experts on the following issues: timing of diagnosis of obstructive jaundice, features of diagnostic measures, the need and possibility of conservative measures for obstructive jaundice, and strategy of biliary decompression depending on the cause and level of biliary block.

[Rhabdomyosarcoma of the common bile duct mimicking choledochal cyst: a rare cause of obstructive jaundice].

Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.