SPA Annual Meeting Reviews
Session III: PEDx Talks
By Sean Barnes, MD, MBA
Assistant Professor
Department of Anesthesiology and Critical Care Medicine
Johns Hopkins University School of Medicine
Charlotte Bloomberg Children's Center
Williams Syndrome: New Insights into Perioperative Management
Andrew Matisoff, MD
Andrew Matisoff, MD (Childrens National Health System, Washington, DC) discussed the perioperative management of Williams Syndrome. The first ever description of Williams Syndrome can likely be traced to the 1841 Charles Dickens classic Barnaby Rudge. One hundred and twenty years later J.C.P. Williams described four patients with common atypical facial features, heart defects, and intellectual disability. In addition to the physical features described by Williams, individuals with Williams Syndrome often have strong language skills and a hyper social personality. However, beyond the elfish looks and bubbly personality may be a patient that is high risk for undergoing anesthesia.
A simple Google search of 'Anesthesia and Williams Syndrome' highlights the risk of cardiac arrest in this patient population. Common anatomical lesions are important to note and can contribute to risk stratification of Williams Syndrome patients undergoing anesthesia. These include supravalvar aortic stenosis, multi-level pulmonary stenosis, and coronary artery involvement. ‘High risk’ patients have biventricular outflow tract obstruction, coronary disease, severe supravalvar aortic stenosis (>50mm Hg), and are younger (<3 years).
Dr. Matisoff discussed his approach to the high risk Williams Syndrome patient. For these patients, he will plan for early arrival to the preoperative setting, 2-4 hours prior to procedure, allowing for IV placement and hydration. Preoperatively he will discuss plans for EMCO with CICU and surgeons. Induction is done with five-lead ECG and echo in the room used for focused exam. Typically, Dr. Matisoff will use ketamine or etomidate for induction. Any tachycardia is treated with Dexmedetomidine bolus/infusions. It is important to treat all hypotension and ST changes aggressively with vasoconstrictors.
Williams Syndrome patients can be high risk for anesthesia; however, one can estimate with some accuracy which patients represent the highest risk. When dealing with high risk Williams Syndrome patients a multidisciplinary approach to their care is recommended. Lastly, the anesthesiologist who cares for a patient with Williams Syndrome must understand:
- The physiologic goals in patients with Williams Syndrome, and
- Effects of various anesthetic drugs used.
Fast Tracking Pediatric Liver Transplantation: Pushing the Envelope
Andrew J. Costandi, MD, MMM
Andrew J. Costandi, MD, MMM (Children’s Hospital Los Angeles, Los Angeles) discussed his institution’s experience with ‘fast tracking’ pediatric liver transplantation. Dr. Costandi began his talk with a discussion about the Theory of Medicine. He postulates that medical practice is based on assumptions that dictate our decisions of what to do and what not to do. Moreover, these assumptions can limit the advancement of medicine and care we provide to patients, so we must continuously test these assumptions to achieve substantial change in how we deliver care.
‘Fast tracking’ within Enhanced Recovery After Surgery (ERAS) programs demonstrated the benefits of a multimodal approach to the patient, resulting in a substantial shift in the patient care paradigm. At the center of this paradigm change is the anesthesiologist; now becoming a ‘perioperative physician’. This served as the model for approaching fast tracking pediatric liver transplant patients. Dr. Costandi discussed two key intra- and post-operative strategies that could help fast track liver transplant patients: 1) Reduction in postoperative mechanical ventilation, and 2) Improvements in postoperative pain management.
Reduction in postoperative mechanical ventilation begins in the operating room. When the anesthesia team focuses on fluid restriction and targeted transfusion strategies they were better able to facilitate immediate extubation after liver transplantation. A small sample of studies examining immediate extubation in the operating room have found that it is:
- Safe,
- Associated with decreased ICU length of stay,
- Contributes to overall decrease in hospital length of stay, and
- Decreases the financial burden on hospitals.
Intraoperative variables favoring successful immediate extubation include older patients, non-emergent cases, shorter operative and cold ischemia times, and decreased intraoperative blood product transfusion. Improvements in postoperative pain management in the pediatric liver transplantation population are centered on applying the multimodal pain management strategies leveraged in most ERAS pathways. These strategies include an opioid PCA, ketamine infusion, Dexmedetomidine infusion or regional techniques. The regional techniques may include: Neuraxial (epidural), Transversus Abdominis Plane Block, Quadratus Lumborum Block, or Wound Catheter Local Anesthetic Infusion (Pain Busters).
When implementing these strategies it is important to recognize all stakeholders involved in fast tracking pediatric liver transplant patients. These stakeholders include the surgeon, anesthesiologist, intensivist, hepatologist, acute pain team, pharmacist, and nursing to name a few.
