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.

Liver Transplantation: A Right or a Privilege? Sustainable Liver Transplant Financing With an Innovative Model for the Developing World.

Living donor liver transplant in addition to its lifesaving therapy is a cost-effective alternate to long-term disease management in patients with chronic liver disease. Financial constraint is the biggest hurdle faced by patients in developing countries in availing the liver transplantation. So, we conducted this study to report a government-funded financial support system for liver transplant services. A total of 198 patients who underwent living donor liver transplant with at least 90 days follow-up were included in the study. According to proxy means test score, 52.2% patients were from low and middle socioeconomic groups and 64.6% of patients underwent liver transplantation through government support. Out of 198 patients who underwent liver transplantation 29.6% had monthly income below 25,000 Pakistani rupees ($114). In recipients, 90-day mortality was 7.1% and morbidity was 67.1%. Donor morbidity was 23.2% without any mortality. This financial model can serve as a valuable source for middle and low income group countries to overcome the financial challenge and make liver transplant an accessible, affordable, and economically viable option.

Primary squamous cell carcinoma of liver. First case report from Pakistan and South Asia.

INTRODUCTION AND IMPORTANCE: Primary squamous cell carcinoma (PSCC) of liver is quite rare and very few cases reported in literature. It has high metastatic rate with poor prognosis. The pathogenesis is unclear, but is generally considered to be correlated with the long-term inflammation or metaplasia of biliary epithelial cells or congenital cyst of the liver. We report here a case of PSCC of liver which mimicked a complex hydatid cyst. CASE PRESENTATION: A 25 years male admitted with right hypochondrium pain associated with fever and yellowish discoloration of eyes for 20 days. He was jaundiced with epigastric tenderness and deranged liver function tests. When thoroughly investigated with ultrasound, CT abdomen and MRI liver, he was found to have a large cystic lesion in right lobe of the liver. He underwent right hepatectomy, peri-cystectomy of the cyst and T-tube placement in common bile duct. Histopathology of the resected sample showed primary squamous cell carcinoma of liver. Patient was discharged after 7 days and died after 6 months due to acute liver failure. CLINICAL DISCUSSION: Because of a very low incidence of hepatic SCC, there is not a single definite therapeutic regime and various different methods of management include surgical resection, generalized chemotherapy, radiotherapy, Hepatic Arterial ChemoEmbolization (HACE) and the combinations of these therapies. CONCLUSION: PSCC is a rare condition of the liver and is associated with other benign liver conditions such as non-parasitic and epidermoid cysts. Histopathology with radiological investigations are needed to diagnose and treat this aggressive tumor before it metastasizes.

Saphenous vein conduits for hepatic arterial reconstruction in living donor liver transplantation.

PURPOSE: Occasionally, a recipient's native hepatic arteries are not suitable for reconstruction in living donor liver transplantation (LDLT). The use of the great saphenous vein (GSV) conduits in such patients is seldom practiced since arterial conduits from deceased donors are available. Here, we share our experience with a significantly large group of LDLT recipients who underwent arterial reconstruction with GSV conduits. METHODS: We reviewed patients who underwent LDLT between 2012 and 2017. Patients who had arterial reconstruction using native hepatic arteries (group 1)(n = 452) were compared with those who had GSV interposition conduits for reconstruction (group 2)(n = 21). We compared hepatic artery thrombosis (HAT) rate, allograft dysfunction, morbidity, mortality, and actuarial 5-year survival in the two groups. RESULTS: HAT was seen in 0/452 (0%) versus 1/21(4.7%) patients (P = 0.04). Allograft dysfunction was seen in 89/423 (21%) versus 6/19(31.5%) (P = 0.2) patients. Overall mortality was 81/452 (17.9%) versus 8/21(38%) (P = 0.02). Death after a biliary complication was seen in 24/452 (5.3%) versus 4/21 (19%) patients (P = 0.02). Actuarial 1- and 5-year overall survival was 85% versus 67% and 79% versus 58% (P = 0.008). CONCLUSION: GSV conduits are a suboptimal alternative for establishing hepatic arterial inflow in LDLT, but remain valuable in ominous situations.

Living Donor Liver Transplantation for Hepatocellular Carcinoma: A Single-Center Experience from Pakistan.

BACKGROUND: Living donor liver transplantation (LDLT) is an established treatment for patients with cirrhosis and hepatocellular carcinoma (HCC) within Milan criteria. Acceptable outcomes have been demonstrated in patients fulfilling extended criteria. Here, we share our experience with LDLT for patients with HCC within and beyond Milan criteria, with emphasis on poor prognostic factors. METHODS: We retrospectively reviewed patients who underwent LDLT between 2012 and 2017 and had HCC proven on explant liver histopathology. A total of 117 patients were included. Patients who died early after transplant (in <30 days) were excluded. For outcomes, patients were divided into prognostic groups. These groups were based on (1) alpha fetoprotein >600, (2) poor differentiation, and (3) the presence of lymphovascular invasion. Recurrence-free survival (RFS) was determined using Kaplan-Meier curves. RESULTS: Median age was 53 (30-73) years. Median follow-up was 20.3 (1-63.2) months. Median model for end stage liver disease (MELD) score was 19 (9-34). Of a total of 117 patients, 74 (63.2%) patients met Milan criteria. Recurrence rate was 12/117 (10.3%). Actuarial 5-year RFS was 88% and 82% (P = 0.3) in patients within and outside Milan criteria. There was no difference in 3-year RFS in patients with 0, 1, or 2 poor prognostic factors within Milan criteria (92%, 87%, and 75%, respectively; P = 0.3). However, a significant difference in RFS was seen in patients outside Milan criteria (92%, 93%, and 53%; P = 0.03). CONCLUSIONS: Patients within Milan criteria have acceptable RFS even in the presence of poor prognostic factors. However, the presence of two or more poor prognostic variables significantly impacts RFS of patients outside Milan criteria.