Cause Of Obstructive Jaundice Research Articles

Management Outcome Assessment of Obstructive Jaundice and Associated Factor from Yekatit 12 Hospital Medical College Addis Ababa, Ethiopia

Background: Obstructive jaundice is common problem in daily clinical practice and among the most challenging conditions for planning current and future management. The causes are varied, but it is most commonly due to choledocholithiasis; benign strictures of the biliary tract, pancreaticobiliary malignancies, and metastatic disease. Surgery in patients with obstructive jaundice is generally considered to be associated with a higher incidence of complication and mortality. Objective: The purpose of the study was to assess outcome of obstructive jaundice and associated factors at Yekatit 12 Hospital Medical College, Addis Ababa, Ethiopia. Methods: A longitudinal study design was used to conduct the study and all patients admitted to the surgical wards for whom surgery was done for obstructive jaundice from May 1, 2022 to April 30, 2023 G.C were included. Chi square fisher’s exact test was used to see the association between obstructive jaundice outcome and independent variables, significance was determined by p-value <0.05. Results: In these study 31 patients were enrolled and the mean age was 49.03 SD±16.16 years. Of patients diagnosed to have obstructive jaundice majority are due to benign causes. Common bile duct stones are the commonest benign condition accounting 88.2% and peri-ampullary cancers are the most frequent (48.4%) cause of obstructive jaundice from malignant condition. Among patients operated 38.7% were underwent bypass procedures. Post-operative complications occurred in 9 (24.3. %) of patients, with surgical site infection being commonest (55.5%). Significant association was identified between types of post-operative complication and management outcome (P=0.034). Conclusion: The most common cause of obstructive jaundice was bile duct stone. Post-operative complications were significantly associated with management outcome of obstructive jaundice.

Exploring the causes of obstructive jaundice: a single-centre retrospective analysis

Background: Obstructive jaundice is a condition caused by bile flow blockage and has various benign and malignant etiologies. Accurate and timely diagnosis is essential for effective treatment, particularly in cases of possible malignancy. Advances in diagnostic methods have improved our understanding and management of diseases. This study aimed to investigate the causes and clinical presentation of obstructive jaundice in Saudi Arabian population. Methods: This study included 193 patients diagnosed with obstructive jaundice admitted to the surgical wards from January 2019 to December 2022. Methods involved comprehensive clinical evaluations, medical history reviews, physical examinations, laboratory tests, and diagnostic imaging to identify the underlying disease. Data were collected systematically and statistically analyzed. Results: The study included 193 patients with a male-to-female ratio of 1:1.4, and an average age of 54.10 years. Malignant causes, primarily carcinoma of the pancreatic head, accounted for 59.4% of cases, while benign causes, notably choledocholithiasis, accounted for 41.6%. Symptoms included jaundice, abdominal pain, and loss of appetite, with treatment varying according to the identified cause. Conclusions: The study highlights that obstructive jaundice is more commonly found in females and tends to be caused by benign conditions in younger people, while malignant causes are more prevalent in older individuals. The most common malignant cause is carcinoma of the pancreatic head, and leading benign cause is choledocholithiasis. For diagnosing obstructive jaundice, key imaging techniques include magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), and computed tomography (CT).

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

BackgroundBiliary 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. MethodsA 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. ResultsAfter 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. ConclusionFluorescent Imaging for Indocyanine Green (FIInd Green) in stool is a fast and accurate approach to assess biliary patency non-invasively in infants. Level of EvidenceLevel III.

OBSTRUCTIVE JAUNDICE: ETIOLOGY AND PATTERN OF PRESENTATION

Surgical jaundice mainly occurs due to obstruction in any duct from the liver to the gall bladder and then the small intestine. It can be divided into intra-hepatic or extra-hepatic causes. Biliary stones, iatrogenic or ischemic biliary stricture, pseudo-pancreatic cysts, carcinoma of the head of the pancreas, and periampullary carcinomas are common pathologies of obstructive jaundice. Common bile duct stones account for 10-15% of biliary stone disease and are the most common cause of obstructive jaundice. Obstructive jaundice presents with a wide range of associated features. Ultrasonography, MRCP, and ERCP are the primary diagnostic tools for diagnosing and managing obstructive jaundice, in addition to blood tests. This study aims to determine the pattern of presentation and causes of obstructive jaundice. This observational, retrospective study was conducted in general surgery and surgical oncology unit 2 of Sheikh Zayed Hospital, Lahore. The study lasted from January 2022 to April 2023. A non-probability convenience sampling technique was used. A total of 55 patients with obstructive jaundice were presented in the study area in a defined period, which included 17 (30.90%) males and 38 (69.09%) females. The mean age was 49 years, with age ranges from 28 years to 80 years. 13 (23.66%) patients were found to have malignant disease, whereas 42 (76.33%) patients presented with benign disease. The most common malignant cause of obstructive jaundice was carcinoma head of the pancreas (10.90%), whereas the least common causes were cholangiocarcinoma (1.82%), carcinoma gall bladder (1.82%) and malignant biliary stricture (1.82%). The most common benign cause and overall most common cause of obstructive jaundice was choledocholithiasis (32.73%), followed by referred biliary injuries (18.18%). The most common clinical feature presented in benign causes of obstructive jaundice was jaundice (100%), followed by itching (71.4%) and abdominal pain (64.28%). The most common clinical feature in malignant causes of obstructive jaundice was jaundice (100%), followed by loss of appetite (84.61%) and weight loss (84.61%). Female was the most common gender presented with obstructive jaundice. The most common benign cause of obstructive jaundice was Choledocholithiasis, while the most malignant cause was carcinoma head of the pancreas.

Obstructive jaundice: epidemiology, diagnosis and choice of optimal method of biliary decompression

Aim. To analyze epidemiological features and morbidity structure in obstructive jaundice, to estimate the results of diagnosis and treatment of this category of patients and to evaluate factors affecting the effectiveness of the treatment and diagnostic program.Materials and methods. The study was based on four-year experience of treatment of 484 patients with mechanical jaundice. Ultrasound, CT, MRI, endoscopic ultrasound, and cholangioscopy were used to diagnose the level of the obstruction and the causes of obstructive jaundice. The main method of decompression involved transpapillary interventions in 365 patients. Two patients underwent choledochoduodenostomy under endoscopic ultrasound guidance. Percutaneous transhepatic interventions and “rendezvous” method were applied in 82 cases.Results. The efficacy of transpapillary endoscopic retrograde procedures in obstructive jaundice accounted for 93.4%. Complications developed in 33 patients (8.4%) including postmanipulation pancreatitis – in 19 patients (4.9%), pancreatonecrosis – in 2 patients (0.5%), bleeding – in 9 patients (2.3%), retroduodenal perforation – in 2 patients (0.5%), Mallory–Weiss syndrome – in 1 patient (0.3%). The efficacy of antegrade percutaneous transhepatic interventions comprised 95.1%, while liver parenchyma bleeding developed in 3.7% of patients. In-hospital mortality amounted to 0.8% (malignant tumor progression).Conclusion. The increasing share of patients with mechanical jaundice in the structure of general surgical emergencies, as well as the need to use high-tech equipment for diagnosis and treatment, necessitate creating centers for treatment of patients with mechanical jaundice. Retrograde and antegrade minimally invasive surgeries for specific indications are considered to be the main methods of biliary decompression.

Lemmel Syndrome as Rare Cause of Secondary Sclerosing Cholangitis: An Unusual Presentation and Literature Review

Lemmel’s syndrome is a rare and misdiagnosed cause of obstructive jaundice, It corresponds to cholestatic disease secondary to compression of the main bile duct by a periampullary duodenal diverticulum with secondary dilation of the extra- and intra-hepatic bile ducts. Approximately 5% of duodenal diverticles can cause symptoms and 1% have complications, with colangitis being the most common. Lemmel syndrome is a type of intermittent obstructive jaundice without the presence of coledocolithiasis. Late diagnosis of this entity is common and may lead to unnecessary further investigations and therapeutic delay. There are no cases reports published associated with secondary sclerosing colangitis.

An unsuspected cause of obstructive jaundice in an infant

An unsuspected cause of obstructive jaundice in an infant

Extrahepatic bile duct fibroma in an infant: a case report

BackgroundExtrahepatic bile duct tumor in neonates and infants is extremely rare. Fibroma in neonates and infants has been reported in different body parts but not in the bile duct. We report a 2-month-old girl with common bile duct (CBD) fibroma.Case presentationA 2-month-old girl presented with jaundice and pale stool for 15 days. Her perinatal period was uneventful. On examination, we found the girl deeply icteric and having hepatomegaly but no palpable lump. Laboratory investigations revealed direct hyperbilirubinemia with elevated liver enzymes. Ultrasonography revealed hepatomegaly with focal dilatation of the extrahepatic bile duct (2.1 × 0.5 cm). Our preliminary diagnosis was a type I choledochal cyst. Considering the clinical scenario, we planned for exploration without further imaging. On exploration, we found the liver blackish and a hard mass involving the common CBD and cystic duct. The gall bladder was filled with clear mucus. After excising the mass, the gall bladder, and CBD, we reconstructed the anatomy with Roux-N-Y hepaticojejunostomy. The postoperative period was uneventful except for minor bile leakage during the first four postoperative days, which stopped spontaneously. Histopathology revealed spindle-shaped fibrous tissue admixed with collagenous tissue, compatible with a fibroma. A liver biopsy showed the features of cirrhosis. However, liver function tests were normal 2 weeks after surgery. The baby was thriving at 6 months of follow-up, hepatomegaly was resolved, and there was no clinical or radiological sign of recurrence.ConclusionsCBD fibroma is a rare cause of obstructive jaundice in neonates and infants. The earliest possible excision of the tumor and bile drainage restoration are necessary to halt the progression of liver damage.

Multidetector computed tomography evaluation of obstructive jaundice: a cross-sectional study from a tertiary hospital of Nepal

Background: The utilization of advanced multidetector computed tomography (MDCT) technology along with postprocessing reconstruction techniques has significantly enhanced the clarity of visualization of the hepato-biliary tree. Therefore, this study was conducted to evaluate the diagnostic statistics of MDCT and its associated features in the evaluation of obstructive jaundice, with respect to surgical or histopathological diagnoses. Methods and methodology: The authors conducted a cross-sectional study among 30 participants with obstructive jaundice using purposive sampling. The authors calculated the diagnostic statistics of non-neoplastic and neoplastic types, along with specific etiologies of obstructive jaundice identified through MDCT using a statistical package for social sciences (SPSS) v21 and MedCalc v12.3.0. The ethical clearance was obtained from the institutional review committee of BPKIHS, Nepal (Ref no: Acd/291/075/076-IRC). Results: The sensitivity and the negative predictive value of MDCT for non-neoplastic cause to detect obstructive jaundice were 100% (95% CI: 79.41–100.00) and 100% (95% CI: 75.29–100.00), while the specificity and the positive predictive value for neoplastic cause to detect obstructive jaundice were 100% (95% CI: 79.41–100.00) and 100% (95% CI: 75.29–100.00), respectively. Similarly, the accuracy for either non-neoplastic or neoplastic cause was 96.67% (95% CI: 82.78–99.92). The most common cause of obstructive jaundice was choledocholithiasis (33.34%) followed by cholangiocarcinoma (20%), ampullary carcinoma (13.33%), and choledochal cyst (13.33%). The diagnostic accuracy of the individual etiology of common causes of obstructive jaundice ranged from 82.78 to 100%. Biliary obstruction was most frequently observed in the periampullary region (83.33%), followed by the proximal common bile duct (6.67%), hilar region (6.67%), and intrahepatic region (3.33%). Conclusion: The MDCT could serve as the initial and time-efficient excellent imaging modality for diagnosing various causes of obstructive jaundice with greater accuracy. It can differentiate non-neoplastic from neoplastic causes of obstructive jaundice.

Successful Treatment of Refractory Inspissated Bile Syndrome Using Gastrografin

AbstractInspissated bile syndrome (IBS) is a rare cause of obstructive jaundice in infants. Despite drainage and irrigation, obstruction may persist in some cases with limited further options. This report presents a case of persistent IBS in a 5-month-old female with worsening liver failure despite cholecystostomy and saline irrigation, and a last-ditch effort was made to irrigate with Gastrografin. Patient's bilirubin decreased from 15.5 to 8.3 mg/dL after 1 day. Her bilirubin and liver functions tests normalized by 2 weeks. Gastrografin's hyperosmolarity promotes intraluminal fluid shift and stimulates smooth muscle contraction, which may explain its effectiveness in treating refractory IBS.

An unusual cause of obstructive jaundice, abdominal pain, and weight loss

A 71-year-old female patient presented to the emergency department after having developed generalized icterus for one week, associated with inappetence due to epigastric pain with a recent weight loss of 15 kg

Pattern of bile cultures and antibiotic sensitivity tests in Sudanese patients diagnosed with obstructive jaundice: A single-center prospective study.

Biliary obstruction causes bacteriobilia and significant morbidity and high mortality, which necessitates prompt and effective treatment for a good clinical outcome. Hence, the aim of this study was to determine updated knowledge of biliary microbial spectrum, antibiotic sensitivity pattern, and key clinical factors of bacteriobilia. This is a prospective study conducted during the period between November 2021 and December 2022 at Ibn Sina specialized hospital, Khartoum, Sudan, on 50 patients diagnosed with obstructive jaundice and symptomatic bacteriobilia who underwent open biliary surgeries electively. Bile samples were aspirated intra-operatively and cultured, and antibiotic sensitivity tests were performed. Fifty-four percent of patients diagnosed with obstructive jaundice who underwent elective open biliary surgeries were males with the ratio (2:1). Forty-six percent of patients were between 61 and 75 years (elderly). The most frequent cause of obstructive jaundice was migrating biliary stones (48% of cases). Thirty-two percent of patients were diabetic with bacteriobilia. The predominant isolated bacterial pathogen in this study was Escherichia coli (36% of cases). These biliary pathogens were sensitive to meropenem in 54% of cases and ciprofloxacin in 46%. Eventually, in all patients in this study, biliary bacterial pathogens were found to be resistant to a broad spectrum of antibiotics. Careful selection of empirical antibiotic therapy based on surveillance of routine bile cultures during biliary tree procedures in patients with high risk of bacteriobilia will potentially help in improving the surgical outcomes and optimizing treatment of acute cholangitis, which is associated with high mortality.

“A CLINICO-RADIOLOGICAL PRESENTATION IN PATIENTS WITH OBSTRUCTIVE JAUNDICE: A PROSPECTIVE OBSERVATIONAL STUDY”

Introduction: Obstructive jaundice is a common surgical problem. Occurs due to both benign and malignant conditions. Evaluation in patients with obstructive jaundice was a challenging problem. Presentation of obstructive jaundice patients is late and mostly present after the disease becomes advanced or uncurable. Aims And Objectives: To assess age and sex pattern, clinical presentation and radiological and biochemical pattern in obstructive jaundice patients. Material And Methods: 100 patients with obstructive jaundice were studied. Proformas form waslled and assessment of demographic and clinical patterns in benign and malignant obstructive were observed and radiological ndings in benign and malignant obstructive were carried out to assess the operability. Result: we enrolled 100 patients, 18 were male and 82 female having mean age was 52.58 yr. All patients have icterus, 60% having a lump in abdomen, 31% have pruritus. Gallbladder was palpable in 40% patients at which most were due to pancreatic and Periampullary malignancy. Our study reveals the most common overall and benign cause of biliary tract obstruction was CBD stone(33%) and most common in malignant is gall bladder carcinoma(31%) followed by Periampullary carcinoma(17%). CBD stone treated by CBD exploration(85%) and palliative treatment by ERCP(15%) and rest of benign conditions are completely treatable. Among malignant causes gallbladder carcinoma treated by radical cholecystectomy (16%), cholangiocarcinoma were mostly inoperable, Periampullary carcinoma by Whipple's procedure(25%). Conclusion: Malignant causes of obstructive jaundice outnumbered the benign causes. Most common overall and benign cause was Choleldocholithiasis and malignant was gallbladder carcinoma followed by Periampullary carcinoma. Benign causes were prevalent in younger age groups (31-40 yr) and malignant in older age groups(61-70 yr).Common presentation of obstructive jaundice was icterus and palpable gallbladder was indicative of malignant etiology. Ultrasound followed by MRCP/ERCP and CT scan was the investigation of choice. Benign conditions have better outcome and cure rate while malignant conditions were inoperable (83%).

Lemmel syndrome: a rare cause of obstructive jaundice (case report)

Obstructive jaundice through periampullary duodenal diverticulum (PAD), without gallstones or neoplasms, was first described by Lemmel in 1934 as Lemmel syndrome. PAD can be diagnosed incidentally or suspected in patients with obstructive jaundice and nonspecific abdominal pain. It is a rare and benign condition that can be misdiagnosed as malignancy. Ignoring this, can cause morbidity and mortality. Definite diagnosis is made by Endoscopic Retrograde Cholangiopancreatography (ERCP). An accurate diagnosis is very important to ensure patient management and to avoid complications of delayed care. We present a case who coincidentally was diagnosed with PAD and thus obstructive jaundice.

Limitations and Success Ratio of ERCP in the Treatment of Obstructive Jaundice

Background: Obstructive jaundice can be instigated by benign or malignant lesions. These problems are treated with biliary decompression, which can be done either endoscopically or surgically. Aim: The aim of the study is to assess the advantages and limitations as well as the therapeutic and diagnostic benefits of ERCP in the treatment of obstructive jaundice. Methods: This cross-sectional study was held in the Departments of General Surgery, Liaquat University of Medical and Health Science, Jamshoro for one-year duration from January 2022 to December 2022. A total of 70 consecutive patients were included with purposive sampling and with the diagnosis of obstructive jaundice done with ERCP. Patients with prior diagnostic or therapeutic endoscopic intervention or gastric outlet obstruction or prior gastric surgery were excluded from this study. The results are summarized in a table and conclusions are made. Results: The study included 70 patients (45 males and 25 females) with mean age of 45 years (21-70 years). The most common malignant causes of obstructive jaundice are papillary carcinoma (8.6%), cholangiocarcinoma (17.1%) and pancreatic Carcinoma (27.1%). The most common benign cause was bile duct stone (30%), followed by round warm biliary tree (5.8%) and papillary stenosis (11.4%). 30(93.8%) of patients done with stenting with 92% of success rate. Biopsies were performed to diagnose 8.6% of papillary tumors. 45.7% of patients done with stenting with 93.8% of success rate. In the remaining cases, the success rate was 100%. the most common reason for not performing ERCP; duodenal stenosis in 2.9%, abnormal papillary position in 4.3%, complete papillary obstruction in 4.3% and ampulla was not found endoscopically in 7.1% of subjects. Acute pancreatitis was the most communal complication occurred in 5.7% of cases. Bleeding occurs in 4.3% of cases; 4.3% have acute cholangitis and perforation in 2.9%. Conclusions: In spite of its complications and limitations, ERCP is a reliable stenting procedure as a palliative and tissue diagnostic tool for blockage due to malignant tumor and in the treatment of obstructive jaundice and most benign diseases. In this study, the effectiveness of ERCP in the treatment of obstructive jaundice is satisfactory. Keywords: Cholangiocarcinoma, pancreatic carcinoma, ERCP, Obstructive jaundice, and Stenting.

An uncommon cause of pancreatitis with obstructive jaundice

A 56-year-old man with a history of diabetes mellitus poorly controlled recently (HbA1c: 7.5%, <6%) presented with a 3-month history of postprandial epigastric pain, 10-kg weight loss, steatorrhea, tea-colored urine, and clay-colored stool. Physical examination revealed yellowish skin and icteric sclera but no peripheral stigmata of cirrhosis. Laboratory investigation was significant for elevated aspartate aminotransferase (289 IU/L, 8-31 IU/L), alanine aminotransferase (613 IU/L, 0-41 IU/L), alkaline phosphatase (595 U/L, 34-104 U/L), γ-glutamyl transferase (758 U/L, 0-26 U/L), total and direct bilirubin (8.0/6.1 mg/dL, 0.3-1/0.03-0.18 mg/dL), and carbohydrate antigen 19-9 (289 U/mL, <37 U/mL). Contrast-enhanced computed tomography revealed diffuse enlargement of the pancreas (arrow) and dilatation of the common bile duct (CBD) without identifiable stone or a mass lesion (arrowhead; Figure 1A). Endoscopic ultrasound (EUS) demonstrated hyperechoic foci and strands in the enlarged pancreas (arrow) and layer-by-layer whole wall thickening with a hyper-hypo-hyperechoic series (sandwich pattern) of CBD (arrowhead) as well as gallbladder (star; Figure 1B). Magnetic resonance cholangiopancreatography (MRCP) showed dilated CBD with distal tapering near the pancreatic head (arrow; Figure 1C). The diagnosis of autoimmune pancreatitis was confirmed by serologic immunoglobulin G4 (IgG 4) elevation (IgG 4: 2880 mg/dL, 3-201 mg/dL) and favored type I according to nonductal Level 1/Level 2 criteria of international consensus diagnostic criteria.1 The patient recovered from jaundice and gained weight after treatment of prednisone 40 mg/d for 4 weeks, then taper by 5 mg/wk. The 8-week follow-up EUS demonstrated normalization of pancreatic size (arrow) and CBD morphology (arrowhead; Figure 2).2 Autoimmune pancreatitis mimicking pancreatic cancer is a rare cause of obstructive jaundice with an estimated incidence of 1 per 100 000.3 In summary, we demonstrated a case of autoimmune pancreatitis diagnosed by complement image study and elevating serum IgG 4, which achieved successful medical treatment of obstructive jaundice without further invasive procedures.1 The authors declare no conflict of interest. This report was approved by the Research Ethical Committee of Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation (No. CR111-08).

Etiological Spectrum of Obstructive Jaundice: "Our Experience in Two University Hospitals in Yemen"

Background: Obstructive jaundice is a surgical emergency with preoperatively uncertain diagnosis. Its early diagnosis and treatment is important to avoid fatal complications.&#x0D; Objective: to determine the etiology of obstructive jaundice.&#x0D; Patients and methods: The data of patients, who were operated for obstructive jaundice at department of Surgery in Sana'a University Hospital and Thamar University Hospital between January 2004 and December 2008, were prospectively analyzed in relation to the causes of obstructive jaundice. The study included 134 patients (91 females and 43 males). Their ages varied from 8-90 years. &#x0D; Results: Among 9 underlying causes of obstructive jaundice, the common bile duct stones were the most common cause, 68 patients (50.7%). Malignancies of pancreas and hepatobiliary tract came in the second place, 33 patients (24.6%), followed by 13 patients with stenosis of papilla of Vater (9.7%) and benign strictures of extrahepatic biliary ducts, 10 patients (7.4%).&#x0D; Ascaris in common bile duct was found in 3 patients. Mirrizi's syndrome was found in 2 patients. Obstruction due to ruptured hydatid cyst was found in 2 patients. Two patients had amoebic liver abscesses One patient had pancreatic pseudocyst. &#x0D; Conclusion: Obstructive jaundice of Yemeni patients in our series was most often due to common bile duct stones followed by malignancy of pancreas and hepatobiliary tract. Cancer of the pancreas was the most common malignant cause. Rare causes such as ascaris in common bile duct was detected. Cooperation between surgeon and radiologist is highly recommended &#x0D;

Infantile Kaposiform hemangioendothelioma in a female patient complicated with severe obstructed jaundice: a case report

BackgroundKaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular neoplasm that occurs mainly in the pediatric population. KHE usually originates just underneath the skin and affects deeper tissues through infiltrative growth; however, visceral tissue involvement is quite rare.Case presentationAn 8-month-old girl with jaundice and acholic stool was referred to our hospital for further evaluation of a hepatoduodenal ligament tumor. A blood examination revealed high bilirubin and liver enzyme levels, but no signs of coagulopathy. The first attempt at a diagnostic surgical procedure did not provide sufficient diagnostic information. However, the histopathological diagnosis of the cystic duct excised in the second surgery indicated KHE. Therefore, in our case, KHE was considered a cause of obstructive jaundice. Sirolimus (rapamycin) was initiated, and the patient was discharged 7 months after admission.ConclusionsIn cases of atypical hypervascular lesions in the abdominal cavity, especially in the pediatric population, it is important to consider the possibility of KHE, and surgical intervention with proper strategies is required for diagnosis, followed sequentially by promising treatments.

S1674 An Unusual Cause of Septic Shock Secondary to Lemmel Syndrome

Introduction: Lemmel syndrome (LS) is a rare cause of obstructive jaundice occurring in the absence of pancreaticobiliary tumors or choledocolithiasis and commonly caused by periampullary diverticulum (PAD) with an incidence of 1-27%. They are typically asymptomatic and underreported leading to complications in ∼5% of cases. Herein, we report a case of septic shock due to acute cholangitis secondary to Lemmel Syndrome. Case Description/Methods: A 58-year-old male with a medical history significant for hypertension who presented to the ER with right upper quadrant abdominal pain, jaundice, and multiple episodes of nonbloody-nonbilious emesis. On admission, the patient was febrile, tachycardic and hypotensive. Initial laboratory tests are shown in the Table. CT of the abdomen showed cholelithiasis and diffuse gallbladder thickening with no intra or extrahepatic biliary duct dilation. He developed septic shock requiring vasopressors. Blood cultures were positive for Escherichia coli, and antibiotics were administered. Emergent ERCP was performed due to the presence of acute cholangitis. He was found to have a large diverticulum located around the major ampulla. Double wire technique was performed to access the ventral pancreatic duct (VPD) and common bile duct (CBD). Fluoroscopic images revealed biliary tapering in the distal third but no visualized stones (Figure). Plastic stents were placed into the VPD and CBD and biliary sphincterotomy was performed. He also underwent laparoscopic cholecystectomy. The patient was discharged with resolution of symptoms. Discussion: The pathophysiology of LS is due to direct mechanical irritation of the diverticulum causing papillary fibrosis. Secondly, the diverticulum may cause sphincter of Oddi dysfunction. Thirdly, the distal CBD may be directly compressed by the periampullary diverticulum. The imaging modalities of choice to confirm the diagnosis include enhanced contrast CT and MRCP. ERCP allows for direct visualization of the duodenal diverticulum. Treatment of these entities is required if the patient becomes symptomatic. The mainstay of treatment is endoscopic sphincterotomy with stent placement or papillary balloon dilation. Due to the high recurrence rate, if endoscopic treatment fails then surgical approach via diverticulectomy is performed. It is important physicians remain vigilant regarding this condition since misdiagnosis leads to delay in therapy. The incidence of septic shock in LS is unknown and to our knowledge, there has only been one other reported case in literature.Figure 1.: A: Cholangiogram of biliary tree with no stones B: Cholangiogram with diverticulum, stent in place and no obvious stones C: Periampullary diverticulum after placement of CBD and pancreatic stent D: CT abdomen showing CBD stent in place with gallbladder sludge. Table 1. - Initial Laboratory Values Blood Chemistry Reference Range Sodium 137 mmol/L 136 - 145 mmol/L Potassium 3.8 mmol/L 3.5 - 5.3 mmol/L Blood urea nitrogen (BUN) 10.0 mg/dL 6 - 24 mg/dL Creatinine 1.0 mg/dL 0.6 - 1.2 mg/dL Lactic acid 5.3 mmol/L 0 – 2.0 mmol/L C-reactive protein 21.8 mg/dL 0.0-0.8 mg/dL TSH 1.1180 µIU/mL 0.400 - 4.500 µIU/mL Total bilirubin 3.3 mg/dL 0.2–1.2 mg/dL Direct bilirubin 1.94 mg/dL 0.0-0.3 mg/dL AST 487 U/L 10-36 U/L ALT 365 U/L 9-46 U/L Alkaline phosphatase 155 U/L 40-115 U/L Complete blood count White blood cell (WBC) 4.3x10 3/ uL 4.4 - 11 × 103/µL Hemoglobin 12.2 g/dL 13.5 - 17.5 g/dL Platelet 107 x103 /uL 150 - 450 × 103/µL

Identification of signature of gene expression in biliary atresia using weighted gene co-expression network analysis.

Biliary atresia (BA) is the most common cause of obstructive jaundice during the neonatal period. This study aimed to identify gene expression signature in BA. The datasets were obtained from the Gene Expression Omnibus database. Weighted gene co-expression network analysis identified a critical module associated with BA, whereas Gene Ontology (GO) enrichment analysis and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analysis revealed the functions of the essential modules. The high-connectivity genes in the most relevant module constructed protein–protein interaction networks via the string website and Cytoscape software. Hub genes screened by lasso regression consisted of a disease classification model using the randomforest method. Receiver operating characteristic curves were used to assess models’ sensitivity and specificity and the model was verified using the internal and external validation sets. Ten gene modules were constructed by WGCNA, of which the brown module had a strong positive correlation with BA, comprising 443 genes. Functional enrichment analysis revealed that module genes were mainly involved in biological processes, such as extracellular matrix organization, cell adhesion, inflammatory response, and the Notch pathway (P < .001), whereas these genes were involved in the metabolic pathways and cell adhesion molecules (P < .001). Thirty-nine high-connectivity genes in the brown module constructed protein-protein interaction networks. keratin 7 (KRT7) and C-X-C motif chemokine ligand 8 (CXCL8) were used to construct a diagnostic model that had an accuracy of 93.6% and the area under the receiver operating curves for the model was 0.93. The study provided insight into the signature of gene expression and possible pathogenesis of BA; furthermore, it identified that the combination of KRT7 and CXCL8 could be a potential diagnostic model for BA.