Hilar Anatomy in 3035 Living Liver Donors: A Novel Classification for Donor Surgery and Suitability, Hepatic Surgeries, and Hepatobiliary Interventions.

BACKGROUND: This study examines the vascular and biliary variations in 3035 liver donors. We propose a novel classification of hepatic arteries, portal veins, and bile ducts and clinically relevant donor classification. METHODS: Preoperative imaging and operative details of 3035 donors from 2005 to 2020 were reviewed. Hilar anatomical variations were identified and grouped on the basis of incidence and clinical relevance. RESULTS: Hilar structures are classified according to the numbers supplying or draining the graft: for the hepatic artery, right (R) and left (L), RA1/LA1 (1 artery), RA2/LA2 (2 arteries), and RA3/LA3 (3 arteries), respectively, further defined on the basis of the inflow trunk into C (for common hepatic artery), S (for superior mesenteric artery), and L (for left gastric artery); for the portal vein, RP1 (1 vein) and RP2 (2 veins) for the right lobe; and for the hepatic duct, RB1/LB1 (1 duct), RB2/LB2 (2 ducts), RB3 (3 right ducts), and RB4 (4 right ducts). Donors were classified on the basis of anatomical variations into 3 groups: class 1 and class 2 donors, who can donate liver with acceptable risks, and class 3 donors, who are high-risk donors because they are anatomically unacceptable ( Figures S1 to S4, SDC , http://links.lww.com/TP/C918 ). CONCLUSIONS: Defining hilar anatomical variations and donor grouping into anatomy-based clinical classes helps in operative planning of donors, hepatobiliary surgeries, and interventional procedures.

Incorporating Tumor Biology to Predict Hepatocellular Carcinoma Recurrence in Patients Undergoing Living Donor Liver Transplantation Using Expanded Selection Criteria.

Conventional selection criteria for liver transplantation (LT) in patients with hepatocellular carcinoma (HCC) are based on tumour size/number only, and do not consider vital surrogates of tumor biology such as alpha-fetoprotein (AFP) and tumor [18 F]fluorodeoxyglucose positron emission tomography ([18 F]FDG PET) avidity. We analyzed survival outcomes, and predictors of HCC recurrence in 405 patients with cirrhosis and HCC (HCC-cirr) who underwent living donor LT (LDLT) using our expanded selection criteria: no extrahepatic disease or major vascular invasion, irrespective of tumor size/number. Fifty-one percent patients had tumours beyond Milan, and 43% beyond the University of California San Francisco [UCSF] criteria. The 5-year overall survival (OS) and recurrence-free survival (RFS) were 64% and 70%, respectively. Three preoperatively available factors predicted recurrence: pre-LT AFP ≥100 ng/mL (P = 0.005; hazard ratio [HR], 2.190), tumor burden beyond the UCSF criteria (P = 0.001; HR, 2.640), and [18 F]FDG PET avidity (P = 0.004; HR, 2.442). A prognostic model based on the number and combination of the aforementioned preoperative risk factors was developed using a competing-risk RFS model. Three risk groups were identified: low (none or a single risk factor present, 9.3% recurrence), moderate (AFP ≥100 ng/mL and [18 F]FDG PET avidity, or beyond UCSF tumor and [18 F]FDG PET avidity, 25% recurrence), and high (AFP ≥100 ng/mL and beyond UCSF, or presence of all 3 risk factors, 46% recurrence). Acceptable long-term outcomes were achieved using our expanded selection criteria. Our prognostic model to predict recurrence based on preoperative biological and morphological factors could guide pretransplant management (downstaging versus upfront LDLT) with the aim of reducing post-LDLT recurrence.

Delayed Graft Dysfunction due to Invasive Hepatic Mucormycosis After Living Donor Liver Transplantation.

Mucormycosis is a rare but emerging fungal infection complicating solid organ transplantation. It is associated with a high mortality rate. We describe an unusual case of hepatic mucormycosis in a living donor liver transplant recipient presenting as delayed graft dysfunction, which was successfully treated with combination of liposomal amphotericin B and oral posaconazole therapy, without surgical resection. The patient had clinical improvement with normalization of liver function tests.

Experience With LDLT in Patients With Hepatocellular Carcinoma and Portal Vein Tumor Thrombosis Postdownstaging.

BACKGROUND: Median survival in patients with hepatocellular carcinoma (HCC) and portal vein tumor thrombosis (PVTT) is 2-6 months; conventionally liver transplantation is contraindicated. METHODS: We studied outcomes following living donor liver transplantation (LDLT) post-PVTT downstaging (DS) with stereotactic body radiotherapy (SBRT), and tumor ablation (with transarterial chemo- or radio-embolization). RESULTS: Of 2348 consecutive LDLTs, 451 were for HCC, including 25 with PVTT (mainly Vp1-3) after successful DS and 20 with Vp1/2 PVTT without previous treatment. DS was attempted in 43, was successful in 27 (63%), and 25 underwent LDLT. Median alpha fetoprotein (AFP) at diagnosis and pre-LDLT were 78.1 ng/mL (3-58 200) and 55 ng/mL (2-7320), respectively. Mean DS to LDLT time was 10.2 weeks (5-16). Excluding 2 postoperative deaths, 1- and 5-year overall survival (OS) and recurrence-free survival (RFS) were 82%, 57%, and 77%, 51%, respectively, comparable to survival in 382 HCC patients without PVTT undergoing upfront LDLT (5-y OS 65%, P = 0.06; RFS 66%, P = 0.33, respectively). There was a trend toward better OS in DS+LDLT versus non-DS LDLT group (5-y OS/RFS-48%/40%). OS was significantly better than in HCC-PVTT patients receiving no intervention or palliative Sorafenib alone (1-y OS of 0%) or Sorafenib with TARE/SBRT (2-y OS of 17%) at our center during the study period. Initial AFP <400 ng/mL and AFP fall (initial minus pre-LDLT) >2000 ng/mL predicted better RFS; Grade III/IV predicted worse OS in DS patients. CONCLUSIONS: HCC patients with PVTT can achieve acceptable survival with LDLT after successful DS. Low initial AFP level, a significant drop in AFP with DS and low tumor grade, favorably influence survival in these patients.

Safe Use of Right Lobe Live Donor Livers With up to 20% Macrovesicular Steatosis Without Compromising Donor Safety and Recipient Outcome.

BACKGROUND: The principle in right lobe living donor liver transplantation is to use "near-perfect" grafts to maximize recipient benefit with minimal donor risk. Whether and what degree of graft macrovesicular steatosis is safe for both recipient and donor is debatable. METHODS: We compared donor and recipient outcomes in 623 primary right lobe living donor liver transplantations, using grafts with (Group A; 10%-20% steatosis, n = 92) and without (Group B; <10%, n = 531) significant macrovesicular steatosis, on pre- or intraoperative biopsy. RESULTS: Group A donors had higher body mass index, transaminases, fasting blood sugar, triglyceride, low density lipoprotein level, and lower high density lipoprotein, and liver attenuation index on CT scan, and similar future liver remnant. Mean postoperative day (POD) 7, aspartate aminotransferase (61.13 + 24.77 vs 73.17 + 53.71 IU/L; P = 0.04), and prothrombin time-international normalized ratio (1.16 + 0.36 vs 1.28 + 0.24; P = 0.0001) were lower in Group A donors. POD3 of 7 total bilirubin and alanine aminotransferase; POD3 aspartate aminotransferase and prothrombin time-international normalized ratio; postoperative morbidity (Dindo-Clavien >3b), hospital stay were similar in both groups. Recipients in both groups had similar age, model for end-stage liver disease score. Right lobe graft weight (764.8 + 145.46 vs 703.24 + 125.53 grams; P < 0.0001) and GRWR (1.09 + 0.29 vs 1.00 + 0.21; P = 0.0004) were higher in Group A. All biochemical parameters at POD 3 of 7, as well as hospital stay, 30-day mortality were similar in recipients of both groups, even after matching both groups for age, model for end-stage liver disease, and GRWR. CONCLUSIONS: Use of well-selected right lobe grafts (adequate future liver remnant in donor, GRWR in recipient), with up to 20% macrovesicular steatosis, does not compromise graft function and outcomes and is safe for the donor.

Accuracy of preoperative CT liver volumetry in living donor hepatectomy and its clinical implications.

BACKGROUND: An accurate preoperative volumetric assessment of donor liver is essential for successful living donor liver transplant by ensuring adequate remnant and graft recipient weight ratio (GRWR). METHODS: The study cohort consisted of 744 right lobe (RL), 65 left lobe (LL) and 33 left lateral sector (LLS) grafts from July 2010 to January 2014. A semi-automated interactive commercial software called AW Volume share 6 was used for volumetry. Bland Altman plot was used for assessing the agreement between estimated graft weight (EGW) and actual graft weight (AGW). RESULTS: There was no statistically significant difference between EGW and AGW for RL graft weight (722±134 vs. 717±126 gm; P=0.06). Although Bland Altman graph showed that 95% limits of agreement was more in LL (-164 to +110) than RL (-156 to +147) and LLS grafts (-137 to +239), CT scan significantly overestimated LL graft weight (EGW =460±118 gm vs. AGW =433±102 gm; P=0.003) and underestimated LLS graft weight (EGW =203±48 gm vs. AGW =254±49 gm; P<0.001). CONCLUSIONS: CT volumetry overestimate LL graft and underestimate LLS graft weight. This should be factored in when selecting LL graft by taking higher GRWR.

Outcome of 200 Pediatric Living Donor Liver Transplantations in India.

OBJECTIVE: To describe our experience of pediatric living donor liver transplantation from India over a period of 12 years. MATERIALS AND METHODS: A retrospective analysis of 200 living donor liver transplantation in children (18 years or younger) was done for demographic features, indications, donor and graft profile and outcome. RESULTS: Between September 2004 and July 2016, 200 liver transplants were performed on 197 children. Fifty transplants were done in initial 6 years and 150 in next 6 years. All donors (51% mothers) were discharged with a mean stay of 7 days. The leading indications of liver transplants were cholestatic liver disease (46%) followed by metabolic liver disease (33%) and acute liver failure/acute on chronic liver failure (28.5%). Biliary leakage (8.5%), biliary stricture (9%), hepatic artery thrombosis (4.5%) and portal vein thrombosis (4%) were the most common surgical complications; all could be managed by surgical or interventional radiological measures, except in one child who died. Sepsis, acute rejection and CMV hepatitis in first 6 months were seen in 14.5%, 25% and 17% cases, respectively. Post-transplant lymphoproliferative disease was seen in only 1.5%. Re-transplant rate was 1.5%. The overall 1 year survival rate was 94% and 5 year actuarial survival was 87% with no statistically significant difference between children weight <10 kg vs. >10 kg. Outcome in acute liver failure did not differ significantly between those with acute on chronic liver failure vs. those with chronic liver disease. CONCLUSIONS: Advances in medical and surgical techniques associated with multidisciplinary teams including skilled pediatric liver transplant surgeons, anesthetists, dedicated pediatric hepatologists, pediatric intensivists, interventional radiologists and pathologists resulted in an excellent outcome of living related liver transplants in children. Low age and weight of the baby does not seem to be a contraindication for liver transplantation as outcome were comparable in our experience.

Yield of endoscopic ultrasound-guided fine needle aspiration for subcentimetric lymph nodes: A comparison to larger nodes.

BACKGROUND AND OBJECTIVES: Subcentimetric (defined as <1 cm at short axis) lymph nodes are considered benign and there is limited literature on the results of fine needle aspiration (FNA) of these nodes. METHODS: Endoscopic ultrasound (EUS) guided FNA was done on 189 lymph nodes in 166 patients with pyrexia of unknown origin (n = 113) or malignancy (n = 53). Subcentimetric lymph nodes (Group A) were compared to nodes with short axis diameter ≥1 cm (Group B). Data are shown as number, percentage, and median (25-75 interquartile range). RESULTS: There was no significant difference between Group A and Group B regarding site of lymph nodes (mediastinal in 73.6 and 72.5%, abdominal in 26.3 vs. 27.4%), number of slides (median 14 vs. 15), needle passes (median 2), and needle used (22 G needle in 85.5% vs. 69.9%). Group A had significantly lesser long axis diameter (1.5 [1.2-2] vs. 2.1 [1.6-2.9] cm) and short axis diameter (0.7 [0.6-0.8) vs. 1.4 [1.1-1.6] cm). A diagnosis (pathologic or reactive) could not be made in 2 (2.6%) and 11 (9.7%) lymph nodes in Group A and Group B, respectively (P = 0.078), due to inadequate material. Respective diagnoses in Group A and Group B were reactive lymphadenopathy (51.3% vs. 18.5%, P = 0.000), granulomatous lymphadenopathy (34.2% vs. 53%, P = 0.011), and malignancy (11.8% vs. 18.5%, P = 0.231). The lymph nodes with granulomatous and malignant change were significantly larger and had higher chances of having sharply demarcated borders as compared to reactive nodes. CONCLUSION: EUS-guided FNA of subcentimetric lymph nodes have comparable results to larger nodes. Almost half of the subcentimetric lymph nodes are pathologic.

Human heme oxygenase-1 deficiency presenting with hemolysis, nephritis, and asplenia.

Heme oxygenase-1 (HO-1) is a stress-induced enzyme that catalyses the oxidation of heme to biliverdin. The primary deficiency of this enzyme has been shown in HO-1 knockout mice, and is characterized by intrauterine death and chronic inflammation. The first case of human HO-1 deficiency was reported in 1999. Human HO-1 deficiency has been observed to involve the endothelial cells more severely, resulting in hemolysis and disseminated intravascular coagulation. We report another case of human HO-1 deficiency in a young girl with congenital asplenia, who presented with severe hemolysis, inflammation, nephritis, which was refractory to therapy with corticosteroids, cyclophosphamide, and rituximab.

Pediatric Hodgkin lymphoma presenting with pulmonary nodules and leukemoid reaction.

An 8-year-old female presented with fever and severe pain in the hipbones and legs for 2(1/2) months. Investigations revealed a leukemoid reaction and bilateral diffuse nodular opacities on chest X-ray. Supraclavicular lymph node biopsy was diagnostic of Hodgkin lymphoma (HL), mixed cellularity. Both pulmonary nodules and leukemoid reaction being present in the same patient with HL has not been reported.