Outcome of pediatric living donor liver transplant: Experience from Pakistan; a resource limited setting.

INTRODUCTION: Liver transplantation (LT) has emerged as a lifesaving modality for many liver diseases in children. Pediatric LT is an established treatment in the Western world but is relatively a new procedure in resource-limited countries like Pakistan. The study aims to highlight the outcomes and survival of pediatric recipients from the first pediatric liver transplant center in Pakistan. METHOD: A retrospective analysis of pediatric LT was done from 2012 to 2019. The study was conducted in the Hepatobiliary and liver transplant department of Shifa International Hospital (SIH), Islamabad. A detailed analysis for indications for pediatric LT, survival, and complications was done. RESULTS: Forty-five patients under 18 years of age underwent Living donor liver transplant (LDLT) in SIH. Median age was 9 years and M:F of 2:1. Cryptogenic liver disease followed by Wilson disease were the two most common indications of LT. The majority of patients had chronic liver disease 34 (75%) while 11 (24%) had acute liver failure. The right lobe graft was the most common type of graft 19 (42.2%). Thirty days, 1-year, 3-year, and 5-year survival was 77.8%, 75.6%, 73.3%, and 60.6% respectively. Mortality was highest in patients with biliary atresia 4 (33%). Causes of death included pulmonary embolism, sepsis, surgical complications, and acute kidney injury. Mean survival was 88.850 months (±7.899) (CI 73.369-104.331). CONCLUSION: Pediatric LDLT has offered disease-free survival for patients. Survival can improve further with nutritional rehabilitation and anticipation and management of post-operative complications.

Biliary atresia-An experience from the first pediatric liver transplant center in Pakistan.

BACKGROUND: Biliary atresia (BA) is the most common cause of neonatal cholestatic syndrome. The true incidence of BA in Pakistan is largely unknown. AIM: This study aimed to report the clinical features, age at diagnosis and outcomes of biliary atresia from the first pediatric liver transplant center in Pakistan. METHODS: The study was done in Shifa International hospital from 2013 to 2020. All babies who had biliary atresia confirmed by laboratory investigation were included. Demographic data, age of presentation, clinical presentation, supporting investigations like liver function tests, ultrasound abdomen, HIDA scan and liver biopsy were noted. Outcome related to Kasai portoenterostomy, liver transplant, complications and immunosuppressant agents were noted. RESULT: A total of 42 children were included, 23 (54.7%) males and 19 (45.2%) were females. Jaundice was seen in all patients (100%) followed by acholic stools (81%). Associated malformations were noted in 6 (14.2%) patients. Liver function tests confirmed obstructive cholestasis (p 0.04). Kasai was done in 19 (45%) patients only, living donor liver transplant was performed in 6 (14%) patients. Age range of transplant patients was from 3 months to 1 year. Indication for liver transplant was failed Kasai in 1(16.7%) patient and chronic liver disease in 5 (83.3%) patients. LDLT survivors were 10 months to 1 year of age at the time of transplant, mean age was 10.6 months. Maximum survival noted so far is 7 years. Acute complications seen post-transplant were sepsis (three patients), surgical site infections (two patients), biliary leaks and acute cellular rejection in one patient each. Chronic graft rejection, portal vein stricture needing stenting was done in one patient. DISCUSSION: All patients underwent LDLT from related donors wih no donor related mortality. All are deceased patients were yonger and had advanced disease. BA remains third most commo indication of transplant in our center. CONCLUSION: Liver transplant is the only lifesaving procedure after failed Kasai or as primary liver transplant due to advance liver disease. The advent of liver transplantation services offers survival and improving outlook of the disease.

Anatomical variations in living donors for liver transplantation-prevalence and relationship.

PURPOSE: Living donor liver transplantation (LDLT) is a widely accepted option to address the lack of a deceased liver program for transplantation. Understanding vascular and biliary anatomy and their variants is crucial for successful and safe graft harvesting. Anatomic variations are common, particularly in the right hepatic lobe. To provide evidence for screening potential liver transplant donors, the presence of vascular and biliary anatomic variations in Pakistan's preoperative assessment of transplantation donor candidates was explored. METHODS: This retrospective cross-sectional study evaluated the hepatic artery, portal vein, hepatic vein, and biliary variations in living liver donors. The study included 400 living liver donors; data were collected from March 2019 to March 2023. We used a CT scan and MRCP to assess the anatomical variations. RESULTS: The study examined 400 liver donors aged 18 to 53 years. Conventional arterial anatomy was the most common (65.8%), followed by replaced right hepatic artery (16%) and replaced left hepatic artery (10.8%). Conventional type 1 biliary anatomy was seen in 65.8% of cases. The dominant right hepatic vein was found in 13.3% of donors. There was a significant association between the prevalence of variant portal venous anatomy with variant biliary anatomy. CONCLUSION: Variations of the hepatic arterial, portal venous, and biliary systems are frequent and should be carefully evaluated while selecting a suitable living donor. A strong relationship between variant portal venous and biliary anatomy was found. These findings can aid in selecting suitable candidates and improving surgical planning for liver transplantation.

Exploring safety and efficacy of rivaroxaban after living donor liver transplantation: a retrospective study.

PURPOSE: Thromboembolic complications remain a significant concern in postoperative patients, particularly those who have undergone liver transplantation. Warfarin has been the standard oral anticoagulant. Direct oral anticoagulants (DOACs) have several advantages over warfarin, including rapid onset of action and standardized dose guidelines. We aimed to assess the safety of rivaroxaban in living donor liver transplantation (LDLT) recipients. METHODS: This study was a single-center, retrospective descriptive analysis of LDLT recipients who received rivaroxaban between December 2020 and April 2022. A total of 27 recipients received rivaroxaban postoperatively. Liver function tests, immunosuppression levels, serum creatinine, and INR were recorded before the initiation of rivaroxaban and then on post-therapy days 1, 7, 14, 28, 90, and 180. RESULTS: Among the 27 recipients receiving rivaroxaban postoperatively, portal venous thrombosis was the most prevalent indication for anticoagulation (44.4%), followed by Budd-Chiari syndrome (29.6%). Nine patients had a twofold increase in either ALT or AST values, two of whom were treated for biliary strictures and the others for rejection. Eighteen patients were given tacrolimus, and eight were on cyclosporine, with one patient switched from tacrolimus to cyclosporine due to insufficient therapeutic levels. There were no incidents of bleeding or re-thrombosis during the 180-day follow-up period. CONCLUSION: Rivaroxaban may be a safe and effective alternative in LDLT recipients with no significant adverse incidents. Further studies with larger sample sizes are needed to confirm these findings and determine this population's optimal dose and duration of rivaroxaban therapy.

Preoperative Endoscopic Biliary Stenting Before Pancreaticoduodenectomy: Does Timing Matter?

Background. The role of preoperative biliary stenting (PBS) before pancreaticoduodenectomy (PD) in patients with obstructive jaundice is debatable. The objective of the current study was to assess PD outcomes after upfront surgery or PBS and determine the impact of stent to surgery duration on PD outcomes. Methods. We reviewed patients who underwent PD between 2011 and 2019. Patients were grouped based on whether they underwent upfront surgery (n = 67) or PBS (n = 66). We further assessed outcomes based on stent to surgery duration. Results. There was no significant difference in 30-day mortality (3% vs. 2.9%, P = 1), 90-day mortality (7.5% vs. 4.4%, P = .4), and Grade B-C pancreatic fistula rates (7.5% vs. 4.4%, P = .4) in the PBS and upfront surgery groups, respectively. A significant increase in wound infections (22.7% vs. 7.4%, P = .01) and readmissions (10.6% vs. 0, P = .006) was seen in the PBS group. The highest rate of wound infection was seen when stent to surgery duration was 4-6 weeks (41.6%). The wound infection rates in the upfront surgery group, high-risk PBS group (4-6 weeks), and low-risk PBS group were 5/67(7.4%), 5/12(41.6%), and 7/36(19.4%), respectively (P = .008). Conclusions. PBS increases postoperative wound infections when compared with upfront surgery. Patients operated between 4 and 6 weeks after stenting have the highest rate of wound infection.

Long term outcomes after pancreaticoduodenectomy: A single center experience from Pakistan.

OBJECTIVE: To report long-term outcomes after pancreaticoduodenectomy from a single centre. METHODS: The retrospective study was conducted at Shifa International Hospital, Islamabad and comprised pancreaticoduodenectomy procedures performed by four surgeons at a single centre from January 2011 to June 2019. Outcome was assessed on the basis of morbidity, in-hospital mortality and survival. Data was analysed using SPSS 20. RESULTS: Of the 155 patients, 103(66.5%) were males. The overall mean age was 56.8±13.5 years (range: 8-85 years). Overall morbidity was 84 (54.2%). Multivisceral and venous resections were performed in 22(14.2%) and 20(12.9%) patients respectively. Grade B pancreatic fistula was seen in 5(3.2%) patients and grade C in 6(3.8%). In-hospital mortality was 5(3.2%). The difference between the estimated 5-year overall survival for pancreatic and non-pancreatic cancers was non-significant (p=0.2), while the difference in the estimated 3-year overall survival rate was significant (p<0.05). CONCLUSIONS: With standardisation of operative technique and peri-operative management, low in-hospital mortality and acceptable long-term outcomes were achieved with standard and extended pancreaticoduodenectomy.

When to call it off: defining transplant candidacy limits in liver donor liver transplantation for hepatocellular carcinoma.

BACKGROUND: Living donor liver transplantation (LDLT) is an acceptable treatment option for hepatocellular carcinoma (HCC). Traditional transplant criteria aim at best utilization of donor organs with low risk of post transplant recurrence. In LDLT, long term recurrence free survival (RFS) of 50% is considered acceptable. The objective of the current study was to determine preoperative factors associated with high recurrence rates in LDLT. METHODS: Between April 2012 and December 2019, 898 LDLTs were performed at our center. Out of these, 242 were confirmed to have HCC on explant histopathology. We looked at preoperative factors associated with ≤ 50%RFS at 4 years. For survival analysis, Kaplan Meier curves were used and Cox regression analysis was used to identify independent predictors of recurrence. RESULTS: Median AFP was 14.4(0.7-11,326.7) ng/ml. Median tumor size was 2.8(range = 0.1-11) cm and tumor number was 2(range = 1-15). On multivariate analysis, AFP > 600 ng/ml [HR:6, CI: 1.9-18.4, P = 0.002] and microvascular invasion (MVI) [HR:5.8, CI: 2.5-13.4, P <  0.001] were independent predictors of 4 year RFS ≤ 50%. When AFP was > 600 ng/ml, MVI was seen in 88.9% tumors with poor grade and 75% of tumors outside University of California San Francisco criteria. Estimated 4 year RFS was 78% for the entire cohort. When AFP was < 600 ng/ml, 4 year RFS for well-moderate and poor grade tumors was 88 and 73%. With AFP > 600 ng/ml, RFS was 53% and 0 with well-moderate and poor grade tumors respectively (P <  0.001). CONCLUSION: Patients with AFP < 600 ng/ml have acceptable outcomes after LDLT. In patients with AFP > 600 ng/ml, a preoperative biopsy to rule out poor differentiation should be considered for patient selection.

Genetic Susceptibility to Chronic Liver Disease in Individuals from Pakistan.

Chronic liver disease, with viral or non-viral etiology, is endemic in many countries and is a growing burden in Asia. Among the Asian countries, Pakistan has the highest prevalence of chronic liver disease. Despite this, the genetic susceptibility to chronic liver disease in this country has not been investigated. We performed a comprehensive analysis of the most robustly associated common genetic variants influencing chronic liver disease in a cohort of individuals from Pakistan. A total of 587 subjects with chronic liver disease and 68 healthy control individuals were genotyped for the HSD17B13 rs7261356, MBOAT7 rs641738, GCKR rs1260326, PNPLA3 rs738409, TM6SF2 rs58542926 and PPP1R3B rs4841132 variants. The variants distribution between case and control group and their association with chronic liver disease were tested by chi-square and binary logistic analysis, respectively. We report for the first time that HSD17B13 variant results in a 50% reduced risk for chronic liver disease; while MBOAT7; GCKR and PNPLA3 variants increase this risk by more than 35% in Pakistani individuals. Our genetic analysis extends the protective role of the HSD17B13 variant against chronic liver disease and disease risk conferred by the MBOAT7; GCKR and PNPLA3 variants in the Pakistani population.

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.

Spectrum of histopathological diagnosis in paediatric patients with liver disorders in Pakistan.

OBJECTIVE: To document spectrum of paediatric liver disorders confirmed on liver biopsy. METHODS: The retrospective review of patients was conducted at Shifa International Hospital, Islamabad, Pakistan, and comprised data of all children who underwent ultrasound-guided percutaneous liver biopsy between December 2008 and June 2015. Frequency of individual diagnosis was assessed and compared on gender basis. SPSS 16 was used for statistical analysis. RESULTS: There were medical records of 74 patients in the study with an overall median age of 6.9 years (interquartile range: 0.2-17.7 years). Metabolic 27(36.5%) and inflammatory 19(25.6%) disorders were the most common aetiologies. At presentation 29(39.1%) patients had cirrhosis; and 25(34%) children were <5 years of age. Metabolic disorders were the most common aetiology in children upto 10 years of age with 19(25.6%) patients. Above the age of 10 years, inflammatory disorders were more frequent and were seen in 10(13.5%). Based on gender, a trend towards significance was observed for metabolic disorders (p=0.08) and children who presented with cirrhosis (p= 0.07), but it was not statistically significant. CONCLUSIONS: Metabolic disorders were the most common cause of liver disorders. A number of children at presentation had underlying cirrhosis.

Macrovesicular steatosis in living related liver donors: correlation of biopsy findings with CT liver attenuation index and body mass index.

BACKGROUND: Hepatic steatosis threatens post-transplant graft survival; therefore, pre-operative quantification of steatosis is crucial. Gold standard for evaluation is donor liver biopsy but it is invasive. An alternative non-invasive method is a calculation of CT liver attenuation index. BMI can be an independent factor predicting grade of steatosis but it is necessary to re-define appropriate BMI cut-off points that are specific for Asians. OBJECTIVE: To retrospectively analyze CT LAI and BMI for quantitative assessment of macrovesicular steatosis in living related liver donors, using histological analysis as gold standard. MATERIALS AND METHODS: A radiologist blinded to histological grading calculated mean CT hepatic attenuation in 48 potential living related liver donors. RESULTS: CT-derived LAI correctly predicted steatosis in all except 1 patient. Parametric analysis for CT LAI and BMI showed overall weak positive correlation. No significant association was found between BMI and biopsy findings. CONCLUSION: Liver biopsy remains a gold standard for evaluation of steatosis. CT LAI of ≤0 correlates well with significant hepatic steatosis and biopsy may be avoided in such cases. Biopsy may be reserved for patients with CT LAI between 1 and 5. BMI alone is not a good predictor of hepatic steatosis in our study population.

Auxiliary partial orthotopic liver transplant for Criggler-Najjar Syndrome: Report of 2 cases from Pakistan.

Auxiliary partial orthotopic liver transplant (APOLT) is a treatment option for certain liver disorders where liver structure is preserved. It includes Criggler Najjar syndrome (CNS), urea cycle defects and familial hypercholesterolaemia. Liver transplant as a treatment modality has only recently become available in Pakistan. Here we report two paediatric cases of CNS type 1 where auxiliary liver transplant was performed to correct jaundice and prevent inevitable brain damage. Both recipients and their respective living donors had successful surgery and are doing well.

The travails of setting up a living donor liver transplant program: Experience from Pakistan and lessons learned.

Living donor liver transplantation (LDLT) is the only treatment option for patients with end-stage liver disease (ESLD) where cadaveric donors are not available. In developing countries, the inception of LDLT programs remains a challenge. The first successful liver transplantation program in Pakistan started transplantation in 2012. The objective of this study was to report outcomes of 100 LDLT recipients in a developing country and to highlight the challenges encountered by a new LDLT program in a resource-limited setting. We retrospectively reviewed recipients who underwent LDLT between April 2012 and August 2014. Demographics, etiology, graft characteristics, and operative variables were assessed. Outcome was assessed on the basis of morbidity and mortality. All complications of ≥ 3 on the Clavien-Dindo grading system were included as morbidity. Estimated 1-year survival was calculated using Kaplan-Meier curves, and a Log-rank test was used to determine the significance. Outcomes between the first 50 LDLTs (group 1) and latter 50 LDLTs (group 2) were also compared. Median age was 46.5 (0.5-72) years, whereas the median MELD score was 15.5 (7-37). The male to female ratio was 4:1. ESLD secondary to hepatitis C virus was the most common indication (73% patients). There were 52 (52%) significant (≥ grade 3) complications. The most common morbidities were bile leaks in 9 (9%) and biliary strictures in 14 (14%) patients. Overall mortality in patients who underwent LDLT for ESLD was 10.6%. Estimated 1-year survival was 87%. Patients who underwent transplantation in the latter period had a significantly lower overall complication rate (36% versus 68%; P = 0.01). Comparable outcomes can be achieved in a new LDLT program in a developing country. Outcomes improve as experience increases.

Quality of Life After Living Donor Hepatectomy for Liver Transplantation.

BACKGROUND: Living donor liver transplantation (LDLT) involves healthy individuals undergoing voluntary major hepatic resection. LDLT program only started in 2012 in Pakistan and its impact on donor's quality of life (QOL) post resection is not known. The objective of this study was to determine health-related QOL in donors who underwent hepatectomy in country's first liver transplant program. METHODS: A total of 60 donors who underwent hepatectomy between 2012 and 2014 with a minimum follow-up of 6 months were included in the study. Short form (SF-36) and Profile of mood states (POMS-65) was used to assess QOL. In addition scores were compared between patients who did and did not develop complications. RESULT: Mean time duration between hepatectomy and administration of questionnaire was 15 ± 5.1 months. Median age was 28 (19-45) years. Mean BMI was 24.4 ± 3.7. A total of 7 (11.6%) Grade 3 and above complications were observed in donors. Donors exceeded a score of 90 in 6 out of 8 evaluated categories on SF-36. The highest mean score was recorded for emotional role limitation 95.5 ± 17.1 and lowest for energy 84.8 ± 17.5. The mean score for anger was 6.6 ± 7.5. Donors also did well on the POMS vigor score with a mean of 22.7 ± 5. No significant difference in scores was observed between donors with and without complications for any of the categories except tension. Donors who developed complications post-operatively had a significantly low mean tension score of 1.5 versus 3.8 for donors without complications. CONCLUSION: Acceptable post donation QOL was achieved and surgical complications did not adversely affect SF-36 and POMS scores.

Pancreaticoduodenectomy with Para-aortic Lymph Node Dissection for Periampullary Cancer.

There is no consensus on the utility of para-aortic lymph node dissection (PALND) in patients undergoing pancreaticoduodenectomy (PD) for periampullary cancer. The objective of this study was to assess survival in patients who underwent PD with PALND for pancreatic (PAC) and non-pancreatic (non-PAC) adenocarcinoma. All patients who underwent PD and PALND between 2011 and 2019 were reviewed (n = 114). We looked at the impact of tumor type (PAC versus non-PAC) and pathologically confirmed PALN metastasis (PALNM) on overall survival (OS). Out of 114 patients, PALNM were pathologically confirmed in 17(14.9%) patients. Without PALND, pathological staging would be pN0 in1(0.8%), pN1 in 3(2.5%), and pN2 in 13(11.2%) patients. The 30-day mortality was 3(2.6%) and 65(57%) patients received adjuvant treatment. The 4-year OS for PAC and non-PAC was 9% and 39% (P = 0.001). Advanced nodal involvement (pN2) was seen in 14/17(82.4%) and 21/97(21.6%) patients with and without PALNM, respectively (P < 0.001). For PAC, 4-year OS for patients with pN0-N1, pN2, and PALNM was 12%, 8%, and not reached (P = 0.067). For non-PAC, 4-year OS was 45%, 19%, and 12% (P = 0.006). In patients with non-PAC, despite metastatic involvement of PALN, acceptable long-term survival can be achieved with curative resection. For PAC, survival benefit with curative resection remains questionable.

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.

Survival after extended resections for gallbladder cancer.

Backgrounds/Aims: Locally advanced gallbladder cancer (GBC) is associated with survival limited to a few months. Extended resections (ER) are occasionally performed in this group and outcomes remain inconclusive. This study assessed outcomes after ER for locally advanced GBC. Methods: Patients who underwent ER for GBC between 2011 and 2020 were reviewed. ER was defined as a major hepatectomy alone (n = 9), a pancreaticoduodenectomy (PD) with or without minor hepatectomy (n = 3), a major hepatectomy with PD (HPD) (n = 3) or vascular resection and reconstruction (n = 4). We assessed 30-day morbidity, mortality, and 2-year overall survival (OS). Results: Among 19 patients, negative margins were achieved in 14 (73.6%). The 30-day mortality was 1/9 (11.1%) for a major hepatectomy, 0/3 (0%) for a minor HPD, 2/3 (66.7%) for a major HPD, and 1/4 (25.0%) for vascular resection. All short term survivors (< 6 months) (n=8) had preoperative jaundice and 6/8 (75.0%) underwent a major HPD or vascular resection. There were five (26.3%) long term survivors. The median OS in patients with and without preoperative jaundice was 4.1 months (0.7-11.1 months) and 13.7 months (12-30.4 months), respectively (p = 0.009) (2-year OS = 7% vs. 75%; p = 0.008). The median OS in patients who underwent a major hepatectomy alone or a minor HPD was 11.3 months (6.8-17.3 months) versus 1.4 months (0.3-4.1 months) (p = 0.02) in patients who underwent major HPD or vascular resection (2 year OS = 33% vs. not reached) (p = 0.010) respectively. Conclusions: In selected patients with GBC, when ER is limited to a major hepatectomy alone, or a minor HPD, acceptable survival can be achieved.

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.

Donor Safety First: Postoperative Hepatectomy Outcomes in Living Liver Donors.

BACKGROUND: Living donor liver transplantation (LDLT) has become a popular treatment option because some countries lack a deceased organ program and the growing demand for liver transplants. Although postoperative outcomes are similar to deceased donor liver transplants, there is still an element of risk to the donor. The Clavien-Dindo classification system has been used to standardize reporting across different institutions and surgeons to categorize surgical outcomes. METHODS: Between January 1, 2022, and December 31, 2022, 207 living donors underwent hepatectomies at our center. All donors underwent a 3-step process of mandatory screening. Postsurgical complications were classified using the Clavien-Dindo classification. RESULTS: A total of 207 donor hepatectomies for LDLT were performed during our study period. Most donors (92.8%) were aged between 18 and 39 years. The most common type of graft used was a right lobe without the middle hepatic vein (82.6%). Most donors (91.7%) experienced an intraoperative blood loss of ≤500 mL. A total of 140 patients had an ordinary postoperative course. Grade 1 complications were observed in 16.9%, grade 2 in 12.1%, and grade 3 in 3.4% of the remaining patients. No grade 4 or grade 5 (patient death) complications were observed in this cohort. CONCLUSIONS: Living donor liver transplantation remains the most practiced liver transplant surgery in Pakistan. Our findings highlight the safety of the LDLT program with minimal risk of significant complications. The study also underscores the importance of careful screening and monitoring of living donors and the need for standardized reporting of surgical outcomes using the Clavien-Dindo classification system.