Analysis of the impact of race on blood transfusion in pediatric scoliosis surgery.

INTRODUCTION: Surgical correction of pediatric scoliosis is associated with significant blood loss. Minimizing estimated blood loss and blood transfusion is beneficial as transfusions have been associated with increased morbidity, including risk of surgical site infections, longer hospitalizations, and increased cost. Although there is evidence that African-American or Black adults are more likely to require intraoperative blood transfusion compared with Caucasian or White adults, the reasons for this difference are unclear. METHODS: The electronic records for all patients <18 y/o undergoing primary corrective scoliosis surgery by a single pediatric orthopedic surgeon at a single academic medical center between 2013 and 2015 were collected and reviewed. Multivariate models were performed to assess the association between Black race and blood loss/transfusion in primary pediatric scoliosis surgery. RESULTS: In a multivariate model, Black race was independently associated with 1.61 times higher estimated blood loss than White race (P < .01; 95% CI = 1.16-2.23). Additionally, compared to a White patient, the odds a Black patient received blood transfusion was 6.25 times higher (P = .03; 95% CI = 1.56-25.06) and among the patients who received blood transfusion, Black race was independently associated with 2.61 times greater volume of blood transfusion than White race (P < .01; 95% CI = 1.54-4.41). CONCLUSION: Black race was independently associated with increased estimated blood loss, increased rate of blood transfusion, and increased amount of blood transfused during surgical correction of pediatric scoliosis. Further investigation is needed to better understand the etiology of the disparity and assess opportunities for improving outcomes.

The association between caudal anesthesia and increased risk of postoperative surgical complications in boys undergoing hypospadias repair.

INTRODUCTION: Recent reports have suggested that caudal anesthesia may be associated with an increased risk of postoperative surgical complications. We examined our experience with caudal anesthesia in hypospadias repair to evaluate for increased risk of urethrocutaneous fistula or glanular dehiscence. METHODS: All hypospadias repairs performed by a single surgeon in 2001-2014 were reviewed. Staged or revision surgeries were excluded. Patient age, weight, hypospadias severity, surgery duration, month and year of surgery, caudal anesthesia use, and postoperative complications were recorded. Bivariate and multivariate statistical analyses were performed. RESULTS: We identified 395 single-stage primary hypospadias repairs. Mean age was 15.6 months; 326 patients had distal (83%) and 69 had proximal (17%) hypospadias. Caudal anesthetics were used in 230 (58%) cases; 165 patients (42%) underwent local penile block at the discretion of the surgeon and/or anesthesiologist. Complications of urethrocutaneous fistula or glanular deshiscence occurred in 22 patients (5.6%) and were associated with caudal anesthetic use (OR 16.5, 95% CI 2.2-123.8, P = 0.007), proximal hypospadias (OR 8.2, 95% CI 3.3-20.0, P < 0.001), increased surgical duration (OR 1.01, 95% CI 1.01-1.02, P < 0.001), and earlier year of practice (OR 3.0, 95% CI 1.2-7.9, P = 0.03 for trend). After adjusting for confounding variables via multivariable logistic regression, both caudal anesthetic use (OR 13.4, 95% CI 1.8-101.8, P = 0.01) and proximal hypospadias (OR 6.8, 95% CI 2.7-16.9, P < 0.001) remained highly associated with postoperative complications. CONCLUSIONS: In our experience, caudal anesthesia was associated with an over 13-fold increase in the odds of developing postoperative surgical complications in boys undergoing hypospadias repair even after adjusting for urethral meatus location. Until further investigation occurs, clinicians should carefully consider the use of caudal anesthesia for children undergoing hypospadias repair.

Impact of hydroxyurea on perioperative management and outcomes in children with sickle cell anemia.

Hydroxyurea has enhanced the treatment for children with sickle cell anemia. The objectives of this study were to compare perioperative transfusions and outcomes for children taking hydroxyurea versus those not taking hydroxyurea. We retrospectively reviewed perioperative management and outcomes for 51 children with sickle cell anemia (HbSS genotype) who underwent surgery in our center between January 2003 and April 2008. Of the 51 patients, 30 (59%) were taking hydroxyurea and 21 (41%) were not taking hydroxyurea. Eight of 30 (27%) in the hydroxyurea group were not transfused preoperatively, 12 of 30 (40%) received a single transfusion and 10 of 30 (33%) received serial transfusions, compared with 1 of 21 (5%) children in the nonhydroxyurea group who was not transfused, 2 of 21 (10%) who received a single transfusion and 18 of 21 (85%) who received serial transfusions or pheresis (P=0.004; for comparison across groups). One patient not taking hydroxyurea developed a delayed hyperhemolytic transfusion reaction, and 2 children taking hydroxyurea developed acute chest syndrome. Overall, children taking hydroxyurea had substantially fewer transfusions compared with children not taking hydroxyurea. Both groups of children had a low complication rate. Further research should be done to optimize perioperative management of children taking hydroxyurea.

Consent issues and pediatric regional anesthesia.

The need for consent to regional anesthetic procedures varies considerably between countries. It is likely that legislation and professional guidance will tighten consent procedures, and in several countries detailed written consent is required for regional blockade. This article discusses aspects of consent to regional anesthesia in children.

The novel use of computer-generated virtual imaging to assess the difficult pediatric airway.

In a patient with a known difficult airway, history and examination may be of limited use in formulating a management plan for subsequent tracheal intubation. Further detailed and descriptive review of the airway is necessary. Virtual imaging is a recent advance in radiology that offers noninvasive airway assessment. It creates a movie clip image of the upper airway akin to the view obtained through a fiberscope. We present a patient with Goldenhar syndrome in whom virtual imaging was used to identify the cause of a previous failed tracheal intubation.

Teasing apart the heterogeneity of autism: Same behavior, different brains in toddlers with fragile X syndrome and autism.

To examine brain volumes in substructures associated with the behavioral features of children with FXS compared to children with idiopathic autism and controls. A cross-sectional study of brain substructures was conducted at the first time-point as part of an ongoing longitudinal MRI study of brain development in FXS. The study included 52 boys between 18-42 months of age with FXS and 118 comparison children (boys with autism-non FXS, developmental-delay, and typical development). Children with FXS and autistic disorder had substantially enlarged caudate volume and smaller amygdala volume; whereas those children with autistic disorder without FXS (i.e., idiopathic autism) had only modest enlargement in their caudate nucleus volumes but more robust enlargement of their amygdala volumes. Although we observed this double dissociation among selected brain volumes, no significant differences in severity of autistic behavior between these groups were observed. This study offers a unique examination of early brain development in two disorders, FXS and idiopathic autism, with overlapping behavioral features, but two distinct patterns of brain morphology. We observed that despite almost a third of our FXS sample meeting criteria for autism, the profile of brain volume differences for children with FXS and autism differed from those with idiopathic autism. These findings underscore the importance of addressing heterogeneity in studies of autistic behavior.

Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series.

BACKGROUND: Patients with infantile-onset Pompe disease suffer from marked hypertrophic cardiomyopathy and an increased risk of arrhythmia. A noncompliant left ventricle predisposes these infants to diastolic heart failure with elevated left ventricular enddiastolic pressure (LVEDP); these patients also commonly develop systolic heart failure. Given this baseline cardiac physiology, coronary perfusion pressure becomes highly sensitive to abrupt changes in diastolic blood pressure (DBP). METHODS: We retrospectively reviewed the experiences of 139 patients enrolled in clinical trials investigating the treatment of infantile-onset Pompe disease with recombinant human acid alpha-glucosidase (rhGAA). Adverse events were screened for those involving anesthesia. RESULTS: Nine patients (6%) with infantile-onset Pompe disease experienced an arrhythmia or cardiopulmonary arrest soon after the induction of general anesthesia. Of these events, propofol was involved in four arrhythmias; sevoflurane without propofol was associated with an additional two. Deaths resulting from arrhythmia appeared to correlate with left ventricular mass indices >350 g x m(-2). CONCLUSIONS: With the advent of enzyme replacement therapy (ERT) using rhGAA, and increased survivability, more infantile Pompe patients will likely present for surgical procedures. Additional care in maximizing coronary perfusion pressure and minimizing arrhythmia risk must be given. For these reasons, it is recommended that anesthesia for infantile Pompe patients specifically avoid propofol or high concentrations of sevoflurane and, instead, use an agent such as ketamine as the cornerstone for induction in order to better support coronary perfusion pressure and to avoid decreasing DBP with vasodilatory agents.

Anaesthetic management of infants with glycogen storage disease type II: a physiological approach.

Pompe or Glycogen Storage Disease type II (GSD-II) is a genetic disorder affecting both cardiac and skeletal muscle. Historically, patients with the infantile form usually die within the first year of life due to cardiac and respiratory failure. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes and a resurgence of elective anaesthesia for these patients. Understanding the unique cardiac physiology in patients with GSD-II is essential to providing safe general anaesthesia.