[Ectopic biliary varices as a cause of portal biliopathy and mechanical jaundice syndrome]. / Ektopicheskie biliarnye varikoznye veny kak prichina portal'noi biliopatii i sindroma mekhanicheskoi zheltukhi.

The authors consider ectopic biliary varices as a possible cause of portal biliopathy in extrahepatic portal hypertension. The main diagnostic methods including ultrasound CT, MRI, endoscopic ultrasonography, cholangioscopy, difficulties of differential diagnosis and clinical manifestations of portal biliopathy are presented. Various treatment options including portosystemic shunting and endoscopic biliary decompression are discussed. The authors emphasize the role of differentiated treatment depending on bile duct lesions. The study not only reviews this problem, but also highlights the issues requiring further analysis for selection of optimal diagnostic and treatment methods.

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.

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.

[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%).

Obstructive Jaundice with skin involvement - An unusual presentation of Myeloid Sarcoma.

Biliary obstruction secondary to malignancy is a common clinical problem. Rarely, biliary obstruction is due to leukemia, and obstructive jaundice in these patients usually presents late in the course of the disease. We present a rare case of a patient who presented with fever, jaundice, and pruritus with multiple nodular swellings in the left shoulder, left thigh, and lower back. Magnetic resonance cholangiopancreatography (MRCP) revealed periampullary mass lesion causing dilated common bile duct (CBD) and intrahepatic bile ducts; hence, endoscopic retrograde cholangiography with plastic stenting was done. Biopsy from the shoulder lesion revealed a mesenchymal tumor, and immunohistochemistry (IHC) confirmed the lesion as myeloid sarcoma. Myeloid sarcoma is an extramedullary tumor, a subtype of acute myeloid leukemia, and presentation as biliary lesions with multiple anatomical sites is very rare. The patient was started on chemotherapy after the normalization of bilirubin. The patient showed improvement of skin lesions and normalization of liver function test (LFT) after 3 weeks of chemotherapy.

Hepatocellular carcinoma (HCC) masquerading as hilar cholangiocarcinoma: An unusual presentation of jaundice.

Jaundice usually occurs in the late stages of hepatocellular carcinoma (HCC). Obstructive jaundice is rarely seen as an initial presentation of HCC, as opposed to cholangiocarcinoma. Various causes of obstructive jaundice in these cases also known as "Icteric HCC" have been described such as tumour thrombi, compression, infiltration or tumours arising from native hepatocytes in the bile duct. We present a case of 74-year-old gentleman with "Icteric HCC" that clinically and radiologically mimicked cholangiocarcinoma for which the patient underwent left hepatectomy with Roux-en-Y hepaticojejunostomy. Histopathology revealed dilated large duct with polygonal sheets of cells of hepatoid morphology which stained diffusely positive for both glypican 3 and Hep-par 1. The epicentre was in the left hepatic duct with no discernible liver lesion and the tumour probably originated from the ectopic hepatocytes within the biliary duct The patient was disease free at 1.5 years of follow up. In conclusion, HCC should be a differential for obstructive jaundice. Patients with such "Icteric HCC" benefit from surgical resection with favourable outcomes. The prognosis in such patients is better than in patients of HCC with jaundice due to hepatic insufficiency.

[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.

[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.

Síndrome colestásico como forma de presentación de linfoma no Hodgkin. Reporte de un caso pediátrico / Cholestatic syndrome as a presentation of non-Hodgkin lymphoma. A pediatric case report

La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica

Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an altered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical manifestations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pediatric age is uncommon

Salmonella enteritidis abdominal aorta mycotic aneurysm presented with acute cholestatic jaundice: A case report and literature review.

Mycotic aneurysm is one of the extra-intestinal manifestations of Salmonella Enteritidis infection. The diagnosis of this condition is challenging owed to its variation in clinical presentations. We presented a case of a 54-year-old man with underlying diabetes mellitus and chronic smokers presented with acute right flank pain and fever associated with mild jaundice. The initial laboratory investigations suggested features of obstructive jaundice and urinary tract infection. The contrast enhancing computed tomography of the abdomen revealed the presence of saccular mycotic aneurysm located at the infrarenal abdominal aorta. The blood culture grew Salmonella Enteritidis which was susceptible to ceftriaxone, trimethoprim-sulfamethoxazole, ciprofloxacin, ampicillin, and amoxicillin-clavulanic acid. Intravenous ceftriaxone was initiated, and he underwent open surgery and artery repair at day 8 of admission. He responded well to the treatment given and subsequently discharged home after completed three weeks of intravenous ceftriaxone.

Arthrogryposis, renal dysfunction, cholestasis syndrome in a neonate: an uncommon association of common problems.

A male infant born out of non-consanguineous marriage to a primigravida presented to us as his third hospitalisation with ichthyotic lesions all over the body, cholestatic jaundice, multiple joint contractures and a history of recurrent sepsis. Blood and urine investigations revealed Fanconi syndrome, hypothyroidism and direct hyperbilirubinaemia with elevated liver enzymes and normal gamma glutamyl transpeptidase levels. The combination of arthrogryposis, renal dysfunction and cholestasis led to the suspicion of arthrogryposis, renal tubular dysfunction, cholestasis (ARC) syndrome, which was then proved by genetic testing. The baby was managed conservatively with respiratory support, antibiotics, multivitamins, levothyroxine and other supportive measures but succumbed to the illness on day 15 of hospitalisation. Genetic analysis using next-generation sequencing was confirmatory of a homozygous mutation in VIPAS39 gene leading to ARC syndrome type 2 in the present case. Genetic counselling was provided and prenatal testing was advised to the parents for future pregnancies.

Fibrolamellar hepatocellular carcinoma presenting as cholestatic jaundice: An unusual presentation of a rare disease.

Fibrolamellar hepatocellular carcinoma is a rare primary hepatic tumor that usually occurs in youth. The common presenting features are vague abdominal pain, nausea, vomiting and weight loss. We present a case report of a young male who presented with cholestatic jaundice and on evaluation was diagnosed to have fibrolamellar hepatocellular carcinoma. He underwent successful surgical resection of the tumor. In young individuals presenting with unexplained cholestasis, fibrolamellar hepatocellular carcinoma should be considered.

Primary Sclerosing Cholangitis- A Rare Cause of Obstructive Jaundice: A Case Report.

Diagnosis of primary sclerosing cholangitis (PSC) is often very difficult and may have a suspicion with altered liver functions. PSC is known to be associated with inflammatory bowel disease. This article presents a case study of a 70 years old male patient who presented with obstructive jaundice with recurrent episode of cholangitis in June 2019 at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. Evaluation revealed beaded appearance in MRCP with positive relevant markers which raised suspicion of PSC and it was confirmed by biopsy and histopathology of the affected segment in biliary tree. The importance of early detection of primary sclerosing cholangitis in an effort to decrease the morbidity and mortality from cholangiocarcinoma will also be emphasized and our management according to local protocol and outcome of this patient.

Cholestatic syndrome as a presentation of non-Hodgkin lymphoma. A pediatric case report. / Síndrome colestásico como forma de presentación de linfoma no Hodgkin. Reporte de un caso pediátrico.

Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an al- tered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical mani- festations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pedia- tric age is uncommon.

La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica.

[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.