Domino liver transplantation for maple syrup urine disease in children: A single-center case series.

BACKGROUND: Domino liver transplant (DLT) represents another type of liver donor to expand the donor pool. Recent reports of successful DLT in children with maple syrup urine disease (MSUD) show promising long-term outcomes. METHODS: It was a retrospective study. All children with MSUD were paired with either recipients with end-stage liver disease (ESLD) or non-MSUD metabolic disease. Each pair underwent simultaneous liver transplant (LT), where the MSUD recipient received the graft from a living-related donor and the liver explanted from the MSUD donor was transplanted to the respective paired domino recipient. We report our experience regarding the techniques and outcomes of DLT at our center. RESULTS: Eleven children with MSUD and 12 respective DLT recipients were enrolled, one of which was domino split-liver transplantation. DLT recipients included seven ESLD, two propionic acidemia (PA), one glycogen storage disease(GSD) type-1, one GSD type-3, and one Citrullinemia. Post-LT ICU and hospital stays were comparable (p > .05). Patient and graft survival was 100% and 66.6% in the MSUD group and DLT recipients at a mean follow-up of 13.5 and 15 months. There was no death in the MSUD group as compared to four in the DLT group. The amino acid levels rapidly normalized after the LT in the children with MSUD and they tolerated the normal unrestricted diet. No vascular, biliary, or graft-related complications were seen in the post-transplant period. No occurrence of MSUD was noted in DLT recipients. CONCLUSION: DLTs have excellent post-surgical outcomes. DLT should be strongly considered and adopted by transplant programs worldwide to circumvent organ shortage.

LTSI Consensus Guidelines: Preoperative Pulmonary Evaluation in Adult Liver Transplant Recipients.

The relationship between chronic liver disease and respiratory symptoms and hypoxia is well recognized. Over the last century, three pulmonary complications specific to chronic liver disease (CLD) have been characterized: hepatopulmonary syndrome, portopulmonary hypertension, and hepatic hydrothorax. Apart from that coexisting pulmonary disease like chronic obstructive lung disease and interstitial lung disease also complicate the outcomes after liver transplantation (LT). Assessment for evaluation of underlying pulmonary disorders is essential to improve outcomes in patients with CLD, posted for LT. This consensus guideline of the Liver Transplant Society of India (LTSI) provides a comprehensive review of pulmonary issues in CLD, related and unrelated to underlying liver disease and gives recommendations for pulmonary screening in specific clinical scenarios in adults with chronic liver disease planned for LT. This document also aims to standardize the strategies for preoperative evaluation of these pulmonary issues in this subset of patients. Proposed recommendations were based on selected single case reports, small series, registries, databases, and expert opinion. The paucity of randomized, controlled trials in either of these disorders was noted. Additionally, this review will highlight the lacunae in our current evaluation strategy, challenges faced, and will provide direction to potentially useful futuristic preoperative evaluation strategies.

Safety and feasibility of immediate tracheal extubation of small pediatric patients after living donor liver transplantation.

BACKGROUND: Immediate extubation is integral constituent of enhance recovery protocols. Purpose of this study was to examine success rates and safety of protocolized immediate extubation in pediatric living donor liver transplant recipients and to find out factors associated with non-immediate extubation in operation room. METHODS: We performed retrospective analysis for data of small (≤20 kg) pediatric patients transplanted between 2017 and 2019 (protocolized duration) and compared with data of transplants done between 2014 and 2016 (non-protocolized duration). Further, we compared data during each time duration between immediate extubation and non-immediate extubation group to find risk factors in that particular duration. RESULTS: Immediate extubation rates were significantly higher during protocolized duration compared with non-protocolized duration (85.52% vs. 48.29%, p < .001). Reintubation rates decreased during protocolized duration (10.9% vs. 4.6%). Hospital stays (20.47 ± 7.06 vs. 27.8 ± 6.2 days, p < .001) and mortality (13.2% vs. 28%, p = .04) were significantly decreased in protocolized duration. Higher age (OR: 2.85, 95% CI 1.22-6.67, p = .02), weight > 10 (OR: 4.37, 95% CI 1.16-16.46, p = .029) and high vasopressor support (OR: 32, 95% CI 6.4-160.13, p < .001) found as significant predictors of non-immediate extubation however only high vasopressor support found to be independent predictor during protocolized duration. CONCLUSIONS: Outcomes in pediatric transplants can be optimized by immediate extubation in majority of cases when protocolized as part of policy.

Living Donor Liver Transplant in patients with Hepatorenal Syndrome without the use of Intraoperative Renal Replacement Therapy, a single-center experience.

BACKGROUND: Continuous Renal Replacement Therapy (CRRT) is often used to support the intraoperative course during liver transplantation (LT) for patients with HRS. However, the use of intraoperative CRRT (IOCRRT) is not without its problems. Living donor liver transplantation (LDLT) is a planned operation and is possible without IOCRRT as the recipient can be optimized. AIM: To study the peritransplant outcomes of patients with CLD and HRS undergoing LT without IOCRRT. METHODS: Analysis of LT program database for perioperative outcomes in patients with HRS from Feb 2017 to Dec 2018. RESULTS: 87/363 (23.9%) adult LDLT patients had HRS, of whom 31 (35.6%) did not respond (NR) to standard medical therapy (SMT) prior to LT. Modified perioperative protocol enabled the NR patients (who were sicker and in persistent renal failure) to undergo LT without IOCRRT. Postoperative renal dysfunction was similar (2 in NR and 2 in R) at 1 year. Post-LT survival was also not different at one month (83.87% in NR and 87.5% in R [p = .640]) and at 1 year (77% in NR vs 80.4% in non-responders [p = .709]). CONCLUSION: IOCRRT can be avoided in HRS patients undergoing LDLT without compromising their outcomes (post-LT survival and RD), even in patients who have not responded to SMT, preoperatively.

Correlation between thromboelastography and rotational thromboelastometry values in adult liver transplant recipients.

BACKGROUND AND AIMS: Viscoelastic haemostatic assays (VHA) namely Thromboelastogram (TEG) and Rotational thromboelastometry (ROTEM) are used for global assessment of coagulopathy and guiding transfusion during living donor liver transplant (LDLT).We conducted a study to compare the interchangeability of the values obtained from these devices in patients with End stage liver disease (ESLD) undergoing LDLT. METHODS: In 76 patients undergoing LDLT, ROTEM and TEG were performed and assessed for interchangeability using Spearman Correlation. The direction and strength of correlation between equivalent parameters was calculated using Inter Class Correlation (ICC) and Bland Altman analysis. RESULTS: The correlation ρ between CT (clotting time) of ROTEM and R of TEG was 0.16 (P = 0.19).The ICC was 0.15, with 95% confidence interval (CI) of -0.38-0.48 (P = 0.25).The ρ of CFT (ROTEM) with K (TEG) was 0.425 (P=<0.001). The ICC was0.49 with 95% CI of 0.17-0.69, P = 0.003.Alpha of ROTEM correlated with Angle of TEG with ρ of 0.475 (P=<0.001). The ICC was 0.61, with 95% CI of 0.36-0.76, P=<0.001.Maximum Clot firmness (MCF) correlated with maximum amplitude (MA) with ρ=0.76 (P=<0.001).The ICC was 0.86, with 95% CI of 0.77-0.92, P=<0.001. Lysis index (L30) of ROTEM correlated clot lysis (CL30) of TEG with ρ of 0.16 (P = 0.18).However, the ICC was 0.45, with 95% CI of 0.11-0.66, P = 0.08. The correlation between CT of ROTEM and R of TEG as well as L30 of ROTEM and CL30 of TEG was not significant.The strongest correlation was found between MCF and MA (P < 0.001). However the MCF/MA showed an agreement of only 86% (ICC = 0.86). CONCLUSION: Values from ROTEM and TEG were not found to be interchangeable.

Risk Factors Assessment of the Difficult Intubation using Intubation Difficulty Scale (IDS).

BACKGROUND: The major responsibility of anaesthesiologist is to maintain adequate gas exchange in his patients in all circumstances and this require that patency of upper airway is constantly maintained. Problems with upper airway management are among the most frequent causes of anaesthetic mishaps. Using intubation difficulty scale (IDS) we made an attempt to objectively assess the predictors of difficult intubation. We assessed classical bedside tests such as modified Mallampati test, Thyromental distance test and also neck extension test. METHODS: We prospectively observed 400 patients undergoing general anaesthesia with endotracheal intubation, for each patient intubation difficulty score was recorded during intubation. Risk factors assessment of difficult intubation done using IDS. Risk factor assessed includes modified mallampati class III and IV, thyromental distance ≤ 6cm and neck extension < 5cm. Patients were categorised as Easy intubation (IDS, 0 to 2), slightly difficult intubation (IDS, 3 to 4) and difficult intubation (IDS 5). Preoperative airway assessment was done by thyromental distance measurement, neck extension measurement and modified mallampati test. Data was analysed using Receiver operating characteristic curve (ROC) and area under curve (AUC) for each test computed. p<0.05 was considered significant. RESULTS: The incidence of difficult intubation was 8% and there were no failure to intubate the trachea. The AUC were as follows: modified Mallampati test 0.473 (p<0.005), Thyromental distance test 0.753 (p<0.005) and neck extension test 0.768 (p<0.005). CONCLUSION: The results indicate that neck extension test is strongest predictor of difficult intubation (IDS≥5) in comparison to Thyromental test and modified mallampati test.

Perioperative monitoring in liver transplant patients.

Liver transplant (LT) is a major surgical undertaking involving major fluid shifts, hemodynamic instability and metabolic derangements in a patient with preexisting liver failure and multisystemic derangements. Monitoring and organ support initiated in the preoperative phase is continued intraoperatively and into the postoperative phase to ensure an optimal outcome. As cardiovascular events are the leading cause of non-graft related death among LT recipients, major emphasis is placed on cardiovascular monitoring. The other essential monitoring are the continuous assessment of coagulapathy, extent of metabolic derangements, dyselectrolytemis and intracranial pressure monitoring in patients with fulminant hepatic failure. The type and extent of monitoring differs with need according to preexisting child status of the patient and the extent of systemic derangements. It also varies among transplant centers and is mainly determined by individual or institutional practices.