Preventing Small-for-size Syndrome in Living Donor Liver Transplantation: Guidelines From the ILTS-iLDLT-LTSI Consensus Conference.

Small-for-size syndrome (SFSS) is a well-recognized complication following liver transplantation (LT), with up to 20% developing this following living donor LT (LDLT). Preventing SFSS involves consideration of factors before the surgical procedure, including donor and recipient selection, and factors during the surgical procedure, including adequate outflow reconstruction, graft portal inflow modulation, and management of portosystemic shunts. International Liver Transplantation Society, International Living Donor Liver Transplantation Group, and Liver Transplant Society of India Consensus Conference was convened in January 2023 to develop recommendations for the prediction and management of SFSS in LDLT. The format of the conference was based on the Grading of Recommendations, Assessment, Development, and Evaluation system. International experts in this field were allocated to 4 working groups (diagnosis, prevention, anesthesia, and critical care considerations, and management of established SFSS). The working groups prepared evidence-based recommendations to answer-specific questions considering the currently available literature. The working group members, independent panel, and conference attendees served as jury to edit and confirm the final recommendations presented at the end of the conference by each working group separately. This report presents the final statements and evidence-based recommendations provided by working group 2 that can be implemented to prevent SFSS in LDLT patients.

Living Donor Liver Transplantation in a Paediatric Patient With Citrullinaemia Type 2.

Citrullinaemia is a urea cycle defect that results from a deficiency of the enzyme arginosuccinate synthetase. Type 1 disease is diagnosed in childhood, whereas type 2 disease is adult onset. But, we report the outcome in a boy (13 years) with citrullinaemia type 2 who received a live donor liver transplant (LDLT) at our centre. One advantage of LDLT over deceased donor liver transplantation is the opportunity to schedule surgery, which beneficially affects neurological consequences. In conclusion, transplantation should be considered to be the definitive treatment for citrullinaemia type 2 at this stage, although some issues remain unresolved.

Live Liver Donors: Is Right Still Right?

BACKGROUND: Conventional wisdom dictates that a larger hepatectomy is more prone to complications. Consequently, with the donor safety as paramount, the transplant community has intuitively been proponents of left lobe donation in live donor liver transplantation (LDLT), thereby satisfying the tenet of double equipoise. More recently some data suggest that this may not always be the case, especially in established centres. Our aim was to compare right and left lobe donor outcomes in LDLT from a centre with cumulative experience. METHODS: Review of a prospectively collected database of right and left lobe liver donors operated between August 2009 and July 2017 was performed. Their preoperative demographics, operative and post-operative outcomes were compared. RESULTS: Of 904 liver transplantations, 458 were right lobe donors [379 without middle hepatic vein (MHV), 79 with MHV] and 58 left lobe donors. There was a significant difference in GRWR and functional liver remnant between the right and left lobe donors (1.27 ± 0.45 vs. 1.03 ± 0.28 p = 0.004, and 63.2 ± 7.9 vs. 37.7 ± 16.3, respectively, p value). The end portal pressure (7 vs. 8 mmHg p = <0.001), peak bilirubin (1.6 ± 0.8 vs. 2.9 ± 1.5 p = <0.001) and day 5 bilirubin (0.8 ± 0.3 vs. 1.4 ± 0.9 p = <0.001) were significantly higher in right lobe donors. There was no difference in blood loss, duration of surgery or peak lactate between the groups. Complications (20.7% vs. 25.9% p = 0.48), including serious complications (Clavien-Dindo > III) (6.9% vs. 8.1% p = 0.95), duration of ICU and hospital stay, were comparable between the groups. Subgroup analysis between left lobe and right lobe with and without MHV donor was also comparable. CONCLUSION: Though biochemical differences exist between the groups, no difference in outcomes was noted. Despite larger liver mass loss in right lobe donors, a strict protocol-based approach to donor selection leads to comparable outcomes between left lobe and right lobe donations.

Feasibility and Safety of Split-Liver Transplantation in a Nascent Framework of Deceased Donation.

Split-liver transplantation (SLT) is a valuable option for optimizing the use of good-quality deceased donor grafts. It is not routinely reported outside the West because of limited deceased donor numbers, technical and organizational constraints, lack of experience, and a predominant living donor liver transplantation (LDLT) practice. At our center, 20% of the liver transplantations (LTs) are from deceased donors. We report our experience of SLT and compare outcomes with pediatric and adult LDLT recipients. A prospectively maintained database of all LT recipients between September 2009 and March 2017 was analyzed. Each pediatric SLT recipient was matched to 2 pediatric LDLT recipients for age, weight, urgency, and year of transplant. Each adult SLT recipient was similarly matched to 2 adult LDLT recipients for age, Model for End-Stage Liver Disease score, and year of transplant. Intraoperative and postoperative parameters, including recovery time, morbidity (biliary and vascular complications, Clavien grade >IIIA complications), and mortality were compared. In total, 40 SLTs were performed after splitting 20 deceased donor livers (in situ, n = 11; hybrid split, n = 3; and ex vivo, n = 6). Recipients included 22 children and 18 adults. There were 18 livers that were split conventionally (extended right lobe and left lateral segment [LLS]), and 2 were right lobe-left lobe SLTs. Also, 3 LLS grafts were used as auxiliary grafts for metabolic liver disease. Perioperative mortality in SLT recipients occurred in 3 patients (2 children and 1 adult). Incidence of vascular, biliary, and Clavien grade >IIIA complications were similar between matched adult and pediatric SLT and LDLT groups. In conclusion, SLT is an effective technique with outcomes comparable to living donor grafts for adult and pediatric recipients. Using SLT techniques at centers with limited deceased donors optimizes the use of good-quality whole grafts and reduces the gap between organ demand and availability.

Changing pattern of biliary complications in an evolving liver transplant unit.

Biliary complications (BCs) remain a significant cause of morbidity following liver transplantation (LT). This series of 640 LT recipients with a blend of living and deceased donor transplants was analyzed to determine the incidence, risk factors, management protocol, and outcomes in these patients. Review of a prospectively collected database of transplant recipients operated between August 2009 and June 2016 was performed. Patients were divided into those with and without BCs and data analyzed. The 640 LT recipients from both living (n = 481) and deceased donors (n = 159) were evaluated for BCs. The overall incidence of BCs was 13.7%. It reduced from 23% to 5% (P = 0.003) over a 6-year period. Risk factors for BCs on multivariate analysis were living donor liver transplantation, prolonged time to rearterialization, recipient age above 16 years, prolonged cold ischemia time (CIT) after deceased donor liver transplantation, and biliary reconstruction performed by anyone but the senior author. One-fifth of bile leaks progressed to strictures, and 40% of strictures followed leaks. Endoscopic therapy resolved 60% of the strictures. Surgical repair of strictures was successful in 90% of those in whom endoscopy failed, those who could not undertake the follow-up schedules endoscopic therapy entails, and those presenting with late strictures. BCs significantly prolonged hospital stay but did not alter survival after LT. BCs affect 1 in 7 recipients, although they are not associated with increased mortality. The frequency of these complications is influenced by potentially modifiable factors like evolving surgical expertise and CIT. Liver Transplantation 23 478-486 2017 AASLD.

Multidisciplinary management of hepatoblastoma in children: Experience from a developing country.

BACKGROUND: Advances in chemotherapy, liver resection techniques, and pediatric liver transplantation have vastly improved survival in children with hepatoblastoma (HB). These are best managed by a multidisciplinary team (MDT) in a setting where all treatment options are available. Until recently, this was difficult to achieve in India. METHODS: All children (<16 years) with HB treated in a pediatric liver surgery and transplantation unit between January 2011 and July 2016 were reviewed. Data regarding the clinical presentation, preoperative management, surgical treatment, postoperative course, and outcomes were extracted from a prospectively managed database. RESULTS: Thirty children were treated for HB during the study period. Nine children were PRETEXT 4, 7 were PRETEXT 3, 13 were PRETEXT 2, and 1 was PRETEXT 1 (where PRETEXT is pretreatment extension). All children received a neoadjuvant chemotherapy before surgery followed by an adjuvant chemotherapy. Nineteen children had complete resection, while six underwent primary living donor liver transplantation. There were six mortalities including five children who poorly responded to chemotherapy with progressive tumor extension. At a median follow-up of 30 months, two children who underwent resection and one child who underwent liver transplant had disease recurrence. CONCLUSION: Improved outcomes can be achieved in children with HB even in countries with limited resources when they are managed by MDTs with expertise in pediatric oncology, liver resection, and liver transplantation.

Long-term health-related quality of life in living liver donors: A south Asian experience.

AIM: The aim of this study was to evaluate long-term health-related quality of life (HRQOL), changes in lifestyle, and complications in living liver donors at a single transplant center from southern India. METHODS: A total of 64 consecutive living liver donors from 2008 to 2011 were evaluated; 46 of 64 donors completed the short form 36 (SF-36) via telephonic interviews or clinic consultations. Mean follow-up was 48 months (range: 37-84 months). RESULTS: There was no mortality in the donors evaluated. Overall morbidity was 23%, which included wound infections (4.3%), incisional hernia (2.1%), biliary leak (4.3%), and nonspecific complaints regarding the incision site (15.2%). All 46 donors who completed the SF-36 had no change in career path or predonation lifestyle. A total of 40 of 46 (87%) donors had no limitations, decrements, or disability in any domain, while six of 46 (13%) had these in some domains of which general health (GH) was most severely affected. CONCLUSIONS: Living donor hepatectomy is safe with acceptable morbidity and excellent long-term HRQOL with no change in career path or significant alteration of lifestyle for donors.

Donor Outcomes in Living Donor Liver Transplantation-Analysis of 275 Donors From a Single Centre in India.

BACKGROUND: Live donor liver transplantation is the predominant form of liver transplantation in India and in most Asian countries. Donor outcome reports are an important source of information to be shared with prospective donors at the time of informed consent. This is the first donor outcome series from India. METHODS: Analysis of donor characteristics and morbidity of 275 live donors from a single large volume center is documented. RESULTS: Two hundred seventy-five patients donated from November 2009 to October 2014, 144 were women and 131 were men, 180 donated to adults and 95 donated to children. Right lobe donors were majority at 62.2% followed by left lateral segment 28%. Two thirds of the live donors did not have any morbidity; 114 complications were encountered in 85 patients. The complications were graded as per Clavien 5 tier grading and major morbidity (grade III b, grade IV grade V) was 4.36%. Postoperative biliary complication was seen in 3 donors. CONCLUSIONS: This large single-center study is the first donor outcome report from India, and the results are comparable to other published donor series. Documentation and regular audit of donor outcomes is important to help improve the safety of donor hepatectomy and to provide a database for informed consent of prospective donors.

Liver transplantation in India.

Liver transplantation as an established form of treatment for end-stage liver disease has gained acceptance in India over the last 10 years. Liver transplantation in India has unique features that have contributed to the growth of both deceased donor and living donor transplantations of which living donor currently dominates the picture. Living donor contributes to 80% and deceased donor to 20% of the liver transplants currently performed in India. The majority of these transplants are performed within the private sector with public sector hospitals lagging behind significantly. This article gives an overview of the evolution of liver transplantation in India and the potential future challenges. Liver Transplantation 22 1019-1024 2016 AASLD.

Safety of Live Liver Donation by Individuals With G6PD Deficiency: Initial Results and Comparative Study.

BACKGROUND: G6PD deficiency (G6PDd) is the commonest genetic enzyme defect in the world. However, baring a single case report, there is no published literature regarding the safety of donor hepatectomy in G6PDd individuals. METHODS: Potential donors with World Health Organization class III or class IV G6PDd without evidence of hemolysis were evaluated for donation, if there was no other suitable donor. Postoperatively, donors were closely monitored for hemolysis and medications, which can induce hemolysis, were avoided. Outcomes of our first 14 G6PDd donors are presented. Postoperative course of these donors was also compared with a matched cohort of 30 non-G6PDd donors. RESULTS: There were 9 left lateral segment, 2 left lobe, and 3 right lobe donors. Two G6PDd donors had biochemical evidence of postoperative hemolysis not needing any specific treatment. Postoperative liver function tests, intensive care unit stay, hospital stay, and morbidity (greater than Clavien II) were similar in the G6PDd and non-G6PDd donor cohorts. Donors in the G6PDd group had lower trough hemoglobin in postoperative period (P = 0.006), greater drop in postoperative hemoglobin (P = 0.007), and a higher need for postoperative blood transfusion (4/14 vs 2/30, P = 0.071). CONCLUSIONS: This is the first case series reporting the safety of liver resection in G6PDd individuals. Hepatectomy in G6PD-deficient donors is associated with a greater drop in postoperative hemoglobin and a marginally increased need for postoperative transfusion. Use of these donors can be considered with caution, and it should not be an absolute contraindication for live liver donation.

Better innovate than compromise: a novel hepatic outflow reconstruction technique in pediatric living donor liver transplantation.

Pediatric LDLT using donors with unfavorable vascular anatomy is challenging in terms of donor safety, and complexity of reconstruction in the recipient. We describe an innovative technique of hepatic venous outflow reconstruction involving the recipient RHV, in the presence of a rudimentary RHV in the donor. The postoperative course of the donor and recipient was uneventful with satisfactory venous outflow in both. This technique avoided the use of prosthetic material, an important consideration given the recipient age and requirement for growth. This shows that donors previously considered unsuitable for donation can be utilized safely as long as principles of vascular anastomosis are adhered to. Moreover, it highlights that innovation is sometimes necessary to avoid compromise in donor safety.

Pigmented perivascular epithelioid cell tumor of the liver: report of a rare case with brief review of literature.

The perivascular epithelioid cell tumor (PEComa) family of tumors includes angiomyolipoma, lymphangioleiomyomatosis, clear cell sugar tumor of the lung, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and rare clear cell tumors of other anatomical sites (PEComas-NOS). Among the PEComas-NOS, pigmented variants are extremely rare. The case concerns a 50-year-old female who presented with pain in right hypochondrium. The resected specimen included a 24 × 18 × 9 cm mass. The tumor was histologically characterized by both spindle and epithelioid cells with round to oval nuclei and clear to eosinophilic cytoplasm containing abundant melanin pigment. The stroma demonstrated intervening, thin, fibrovascular septa. Immunohistochemically, the tumor cells were strongly positive for HMB-45, weak positive for smooth muscle actin (SMA), and negative for Hep Par 1, Glypican 3, MUM-1, and S-100 protein. The patient had no evidence of disease 24 months after surgery. Pathologists and clinicians should know about the existence of pigmented perivascular epithelioid cell tumor of the liver.