Changes in branched-chain amino acids in an infant with maple syrup urine disease during perioperative pediatric liver transplant: A case report.

An 11-month-old female infant diagnosed with classic subtype IB maple syrup urine disease underwent living donor liver transplantation. Blood samples for plasma amino acid analysis were collected during the three phases of the operation. Despite the perioperative prophylactic administration of 12.5% hypertonic dextrose solution with insulin and a 20% intralipid emulsion, the blood levels of the branched-chain amino acids increased dramatically during surgery, consistent with an acute intraoperative metabolic decompensation. However, these blood levels normalized soon after liver transplantation with an excellent outcome. We suggest that the occurrence of an intraoperative metabolic crisis during liver transplantation is not necessarily a sign of graft failure.

Factors associated with immediate and early extubation in pediatric living-donor liver transplant recipients.

BACKGROUND: The practice of prompt extubation after adult liver transplantation has increasingly been applied in the pediatric population. Therefore, the factors contributing to this intervention should be identified in order to minimize failures. AIMS: We sought to determine the factors associated with immediate and early extubation in pediatric living-donor liver transplant recipients. METHODS: The medical records of pediatric liver transplant recipients at our center from January 2013 to December 2021, a 9-year period, were retrospectively collected and divided into early or delayed extubation groups. Factors associated with early extubation were determined using univariate and multivariate analyses, as the primary outcome. Comparisons of the postoperative management and outcomes between groups were evaluated as secondary outcomes. RESULTS: Seventy-nine patients were included in the analysis, of whom 19 (24%) were immediately extubated in the operating room while 12 patients (15%) were extubated early, within 24 h postoperatively. These 31 patients (39%) were assigned to the early extubation group, whereas the others were assigned to the delayed extubation group. Shorter anhepatic time was the only factor associated with early extubation (adjusted odds ratio = 0.52; 95% confidence interval: 0.30, 0.89 per 30-min increment; p-value = .018) in patients with the same characteristics, including diagnosis, total operative time, and intraoperative volume of albumin and packed red blood cells. The length of intensive care unit stay was shorter in the early extubation group than in the delayed extubation group (p = .001). The rates of total and early medical complications and total reintubation in 30 days were significantly higher in the delayed extubation group than in the early extubation group (p-value = .002, .044, and .006 respectively). There were no significant differences in the length of hospital stay between the groups. CONCLUSION: Our findings indicated that 39% of the pediatric living donor-liver transplantation patients tolerated early extubation and that the only associated factor was anhepatic time. Early extubation significantly reduces the number of days of intensive care unit stay but may not change the length of hospital stay.

Anesthetic management in an infant with tetra-amelia syndrome with congenital maxillomandibular fusion: A case report.

A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient's extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.