Sjögren-Larsson syndrome: Anesthetic considerations and practical recommendations.

BACKGROUND: Sjögren-Larsson syndrome is a rare inherited neurocutaneous disorder characterized by congenital ichthyosis, spasticity, intellectual disability, seizures, and ophthalmologic changes. Most individuals with Sjögren-Larsson syndrome live well into adulthood and often require surgical intervention to manage their symptomatology. AIMS: The aim of this work was to review the clinical aspects of Sjögren-Larsson syndrome, highlight the unique anesthetic considerations associated with this disease, and provide practical recommendations about anesthetic management. METHODS: A retrospective case review from February 2013 to October 2019 was performed based on subject participation in a Sjögren-Larsson syndrome longitudinal study at the University of Nebraska Medical Center. Anesthetic and surgical records were reviewed for the following data: age, sex, relevant comorbid conditions, anesthetic induction and maintenance agents, intravenous and oral analgesics, muscle relaxants, and anesthetic-related complications. RESULTS: Fourteen patients with Sjögren-Larsson syndrome undergoing 48 anesthetic events were identified. A variety of anesthetic techniques was utilized. No serious adverse events were encountered. The most common clinical observations were related to the ichthyosis seen in Sjögren-Larsson syndrome, which led to difficulty in adherence of electrocardiogram leads and intravenous catheter dressings. CONCLUSIONS: We found that anesthesia can be safely administered in patients with Sjögren-Larsson syndrome. Providers should be aware of anesthetic management issues in Sjögren-Larsson syndrome including challenges placing and securing lines and monitors secondary to the ichthyosis.

The Road to Transfusion-free Craniosynostosis Repair in Children Less Than 24 Months Old: A Quality Improvement Initiative.

INTRODUCTION: Pediatric craniofacial reconstruction has historically resulted in extensive blood loss necessitating transfusion. This single-center quality improvement initiative evaluates the impact of perioperative practice changes on the allogeneic transfusion rate for children 24 months and younger of age undergoing craniofacial reconstruction. METHODS: At project initiation, an appointed core group of anesthesiologists provided all intraoperative anesthetic care for patients undergoing craniofacial reconstruction. Standardized anesthetic guidelines established consistency between providers. Using the Plan-do-check-act methodology, practice changes had been implemented and studied over a 5-year period. Improvement initiatives included developing a temperature-management protocol, using a postoperative transfusion protocol, administering intraoperative tranexamic acid, and a preincisional injection of 0.25% lidocaine with epinephrine. For each year of the project, we acquired data for intraoperative and postoperative allogeneic transfusion rates. RESULTS: A cohort of 119 pediatric patients, ages 4-24 months, underwent anterior or posterior vault reconstruction for craniosynostosis at a tertiary children's hospital between March 2013 and November 2018. Intraoperative and postoperative transfusion of allogeneic blood products in this cohort decreased from 100% preintervention to 22.7% postintervention. CONCLUSIONS: Interdepartmental collaboration and practice modifications using sequential Plan-do-check-act cycles resulted in a bundle of care that leads to a sustainable decrease in the rate of intraoperative and postoperative allogeneic blood transfusions in patients less than 24 months of age undergoing craniosynostosis repair. This bundle decreases the risk of transfusion-related morbidity for these patients. Other institutions looking to achieve similar outcomes can implement this project.

Predictors of transfusion outcomes in pediatric complex cranial vault reconstruction: a multicentre observational study from the Pediatric Craniofacial Collaborative Group.

PURPOSE: Pediatric patients undergoing complex cranial vault reconstruction (CCVR) are at risk of significant perioperative blood loss requiring blood product transfusion. Minimizing allogeneic blood product transfusion is an important goal because of the associated risks and cost. The impact of patient and surgical variables on transfusion is unknown in this population. Our primary aim was to examine relationships between demographic and perioperative variables and blood product transfusion outcomes in CCVR. METHODS: The multicentre Pediatric Surgery Perioperative Registry was checked for children undergoing CCVR between June 2012 and September 2016. Univariable and multivariable analyses were performed examining patient, procedure, and blood conservation variables and their relationship to three outcomes: intraoperative red blood cell-containing product (RBC-CP) transfusion, total perioperative blood donor exposures, and transfusion-free hospitalization. RESULTS: The registry search returned data from 1,814 cases. Age and surgical duration were the only variables significantly associated with all three outcomes studied. Predictors of reduced RBC-CP transfusion included lower American Society of Anesthesiologists (ASA) physical status and antifibrinolytic administration. Total cranial vault reconstruction, intraoperative vasoactive infusion, and presence of a tracheostomy predicted increased donor exposures. Increased body weight, higher preoperative hematocrit, and utilization of intraoperative cell saver and transfusion protocols were associated with transfusion-free hospitalization. CONCLUSION: Clinical factors associated with increased allogeneic blood product transfusion in pediatric CCVR include: age ≤ 24 months, ASA status ≥ III, preoperative anemia, prolonged surgical duration, lack of intraoperative antifibrinolytic use, lack of intraoperative cell saver use, and the lack of transfusion protocols.

RéSUMé: OBJECTIF: Les patients pédiatriques subissant une reconstruction complexe de la voûte crânienne courent un risque de pertes sanguines périopératoires importantes nécessitant la transfusion de produits sanguins. La minimisation de la transfusion de produits sanguins allogènes constitue un objectif majeur étant donné les risques et les coûts associés. L'impact des variables liées au patient et à la chirurgie sur la transfusion est inconnu dans cette population. Notre objectif principal était d'examiner les liens entre les variables démographiques et périopératoires, et les résultats des transfusions sanguines suite à une reconstruction complexe de la voûte crânienne. MéTHODE: Le Registre périopératoire multicentrique de chirurgie pédiatrique (Pediatric Surgery Perioperative Registry) a été consulté afin d'en extraire les dossiers de tous les enfants ayant subi une reconstruction complexe de la voûte crânienne entre juin 2012 et septembre 2016. Des analyses univariées et multivariées ont été réalisées et ont examiné les variables concernant les patients, l'intervention et la conservation du sang ainsi que les relations entre ces données et trois critères : la transfusion peropératoire de produits contenant des érythrocytes, l'exposition durant toute la période périopératoire aux dons de sang, et l'hospitalisation sans transfusion. RéSULTATS: L'examen du Registre a permis d'extraire les données de 1814 cas. L'âge et la durée de la chirurgie étaient les deux seules variables à afficher une association significative aux trois critères à l'étude. Les prédicteurs d'une transfusion réduite d'érythrocytes étaient un statut physique ASA (American Society of Anesthesiologists) plus bas et l'administration d'agents antifibrinolytiques. La reconstruction totale de la voûte crânienne, la perfusion peropératoire d'agents vasoactifs et la présence d'une trachéostomie constituaient des prédicteurs d'exposition plus importante aux dons de sang. Un poids corporel accru, un hématocrite préopératoire plus élevé et l'utilisation de systèmes d'autotransfusion peropératoire et de protocoles de transfusion étaient associés à une hospitalisation sans transfusion. CONCLUSION: Les facteurs cliniques associés à une augmentation des transfusions de produits sanguins allogènes dans les cas de reconstruction complexe de la voûte crânienne chez l'enfant sont : un âge ≤ 24 mois, un statut ASA ≥ III, une anémie préopératoire, la durée prolongée de la chirurgie, l'absence d'utilisation peropératoire d'antifibrinolytiques, l'absence d'utilisation de systèmes d'autotransfusion peropératoire, et l'absence de protocoles de transfusion.

Cardiovascular collapse with attempted pericardial drain withdrawal.

Cardiac tamponade is a rare but serious emergency condition in the pediatric population. As treatment, a pericardial drain is often placed to evacuate the fluid. We present a case of a 4-year-old girl with cardiac tamponade secondary to renal failure. After the tamponade resolved, she suffered cardiovascular collapse upon attempted drain withdrawal. This case highlights an unusual cause for cardiovascular collapse, which occurred on blind removal of a pericardial drain.