National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma.

A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26, 2023, at the Pakistan Kidney and Liver Institute & Research Centre (PKLI & RC) after initial consultations with the experts. The Pakistan Society for the Study of Liver Diseases (PSSLD) and PKLI & RC jointly organised this meeting. This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma (hCCA). The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients. This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation. The diagnostic and staging workup includes high-quality computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography. Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis. However, histopathologic confirmation is not always required before resection. Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging. The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification. Selected patients with unresectable hCCA can be considered for liver transplantation. Adjuvant chemotherapy should be offered to patients with a high risk of recurrence. The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions. Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage. Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

Percutaneous Transhepatic Sphincterotome-Guided Management of Post-Living Donor Liver Transplant Biliary Anastomotic Stricture: An Innovative Approach.

Post-liver transplantation biliary complications remain a serious concern and are associated with reduced patient and graft survival. Among various biliary complications, anastomotic stricture (AS) is the most frequent and challenging one. The frequency of AS after living donor liver transplantation (LDLT) is higher as compared to deceased donor liver transplantation. The management involves endoscopic retrograde cholangiopancreatography and/or percutaneous transhepatic biliary drainage, but refractory cases necessitate surgical revision. We present a case of complex biliary AS in a 63-year-old man after LDLT. The conventional approaches including endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography, and cholangioscope-guided interventions remained unsuccessful. An innovative approach using a wire-guided sphincterotome through percutaneous transhepatic route successfully managed the complex post-LDLT AS. This is perhaps the first reported case of novel utilization of sphincterotome through transhepatic route for the management of AS in LDLT, averting major surgical interventions with related morbidity and mortality.

A Cotton Rope in the Colon: A Rare Cause of Chronic Abdominal Pain.

Retained surgical sponge is a relatively rare medical condition with potential serious medicolegal implications. The term "gossypiboma" is frequently used to describe this condition. We present a case of a 40-cm-long retained surgical sponge in a 43-year-old woman who presented with unexplained chronic abdominal pain for several years. She had a history of open cholecystectomy, hepaticojejunostomy, and enteroenterostomy. Computed tomography scan revealed a large cotton sponge anchored within the large bowel. Surgical exploration is usually required for the retrieval of gossypiboma. However, it was successfully removed endoscopically using a diathermic needle knife with no immediate complications. The patient was discharged after 48 hours with marked improvement in her abdominal pain. This case emphasizes the emerging role of novel endoscopic interventions, resulting in excellent clinical outcomes, avoiding major surgical interventions, and providing cost-effective benefits.

Calcifying Nested Stromal-Epithelial Tumor: An Extremely Rare Hepatic Tumor.

Calcifying nested stromal-epithelial tumor is a rare hepatic malignancy with approximately 50 cases reported in the literature. Its clinical presentation is nonspecific, and the diagnosis is mainly based on histology which shows nests of spindle and epithelioid cells along with a desmoplastic myofibroblastic stroma containing variable calcification and ossification. In this report, we present a case of a 24-year-old woman with a history of abdominal pain, distension, and dyspepsia. She had a palpable liver with normal liver function test results. Serum alpha-fetoprotein levels were within normal range, and serologies for hepatitis B and C virus remained negative. Radiological investigations (magnetic resonance imaging and computed tomography) showed a large, right hepatic lobe mass with tumor invasion into the right posterior portal vein, but the 2 modalities could not characterize the lesion. Finally, an ultrasound-guided biopsy of the liver lesion provided the diagnosis of calcifying nested stromal-epithelial tumor. The tumor was resected successfully.

Cholangioscope-Guided Electrohydraulic Lithotripsy as a Rescue Technique for an Impacted Dormia Basket With Large Common Bile Duct Stone.

Impaction of Dormia basket while extracting common bile duct (CBD) stones during endoscopic retrograde cholangiopancreatography is a well-known but relatively rare complication. Its management could be very challenging and may require percutaneous, endoscopic, or major surgical intervention. In this study, we present a case of a 65-year-old man with a history of obstructive jaundice secondary to a large CBD stone. For stone extraction, mechanical lithotripsy with a Dormia basket was attempted resulting in its entrapment within CBD. Subsequently, the entrapped basket and large stone were retrieved using a novel technique of cholangioscope-guided electrohydraulic lithotripsy with excellent clinical outcomes.

Gastric Linitis Plastica and role of endoscopic ultrasound: An under-recognised diagnostic modality.

Gastric linitis plastica is an aggressive malignancy with poor prognosis. Timely diagnosis is important for effective management. However, the conventional endoscopic biopsies are often inconclusive leading to delay in diagnosis and subsequent management. We present a case of a 55-year old female with high suspicion of gastric linitis plastica on gastroscopy with repeated negative endoscopic biopsies. She underwent an endoscopic ultrasound fine needle aspiration (EUS-FNA) at our center with establishment of diagnosis of gastric malignancy. There are no established guidelines about the role of EUS-FNA as a sequential diagnostic modality for this tumour. However, EUS-FNA is a highly sensitive modality to establish diagnosis in challenging cases where routine endoscopy remains inconclusive.

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study.

BACKGROUND AND OBJECTIVES: Chronic liver disease (CLD) in children present a broad spectrum of symptoms. Limited resources in Paediatric Hepatology in developing countries like Pakistan present considerable challenges in investigating and treating children with chronic liver disease in a timely fashion. This study aimed to determine the spectrum and outcomes of CLD other than chronic hep B & C virus (HBV& HCV) liver disease in children. METHODS: This retrospective descriptive study was conducted at the Paediatric Gastroenterology and Hepatology Department, Pakistan Kidney and Liver Institute and Research Centre in Lahore, Pakistan. The duration of the study was from August 2019 to January 2020. A total of 162 children of CLD were seen during this period of time. Of 162 there were 130 children with chronic HBV & HCV who were excluded from this study. 32 children aged 15 years or younger with chronic liver disease were included. The referrals were received from primary and secondary health care centres in different parts of the country. The data were collected from hospital electronic medical records database and then incorporated into a spreadsheet for analysis. The statistical analysis was performed by applying t-test with p value determined. RESULTS: Of 32 children autoimmune hepatitis (n=11; 34.3%) was the most common cause for chronic liver disease referrals, followed by progressive familial intrahepatic cholestasis type-2, (n=7; 21.8%), post Kasai for biliary atresia, (n=4; 12.5%), glycogen storage disease type-1 (n=5; 15.6%), Wilson disease (n=3; 9.3%) and primary sclerosing cholangitis (n=2; 6.2%). The diagnosis was principally established with the assistance of liver ultrasound, liver biopsy, magnetic resonance cholangiopancreatography and genetic testing. CONCLUSION: Autoimmune hepatitis was the most common chronic liver disease. Our systematic approach, in addition to an extensive workup, helped us to diagnose and then initiate an appropriate treatment, which resulted in a more optimal outcome. Prompt referrals to tertiary centres are recommended where resources and expertise are available to reduce patient morbidity and mortality.

Endoscopic papillary large balloon dilatation with sphincterotomy is safe and effective for biliary stone removal independent of timing and size of sphincterotomy.

AIM: To describe the efficacy and safety of endoscopic papillary large balloon dilatation (EPLBD) in the management of bile duct stones in a Western population. METHODS: Data was collected from the endoscopic retrograde cholangiopancreatography (ERCP) and Radiology electronic database along with a review of case notes over a period of six years from 1st August 2009 to 31st July 2015 and incorporated into Microsoft excel. Statistical analyses were performed using MedCalc for Windows, version 12.5 (MedCalc Software, Ostend, Belgium). Simple statistical applications were applied in order to determine whether significant differences exist in comparison groups. We initially used simple proportions to describe the study populations. Furthermore, we used chi-square test to compare proportions and categorical variables. Non-parametric Mann-Whitney U-test was applied in order to compare continuous variables. All comparisons were deemed to be statistically significant if P values were less than 0.05. RESULTS: EPLBD was performed in 229 patients (46 females) with mean age of 68 ± 14.3 years. 115/229 (50%) patients had failed duct clearance at previous ERCP referred from elsewhere with standard techniques. Duct clearance at the Index* ERCP (1st ERCP at our centre) was 72.5%. Final duct clearance rate was 98%. EPLBD after fresh sphincterotomy was performed in 81 (35.4%). Median balloon size was 13.5 mm (10 - 18). In addition to EPLBD, per-oral cholangioscopy (POC) and electrohydraulic lithotripsy (EHL) was performed in 35 (15%) patients at index* ERCP. 63 (27.5%) required repeat ERCP for stone clearance. 28 (44.5%) required POC and EHL and 11 (17.4%) had repeat EPLBD for complete duct clearance. Larger stone size (12.4 mm vs 17.4 mm, P < 0.000001), multiple stones (2, range (1-13) vs 3, range (1-12), P < 0.006) and dilated common bile duct (CBD) (12.4 mm vs 18.3 mm, P < 0.001) were significant predictors of failed duct clearance at index ERCP. 47 patients (20%) had ampullary or peri-ampullary diverticula. Procedure related adverse events included 2 cases of bleeding and pancreatitis (0.87%) each. CONCLUSION: EPLBD is a safe and effective technique for CBDS removal. There is no difference in outcomes whether it is performed at the time of sphincterotomy or at a later procedure or whether there is a full or limited sphincterotomy.