Evaluating the role for regional analgesia in children with spina bifida: a retrospective observational study comparing the efficacy of regional versus systemic analgesia protocols following major urological surgery.

INTRODUCTION: Regional techniques are a key component of multimodal analgesia and help decrease opioid use perioperatively, but some techniques may not be suitable for all patients, such as those with spina bifida. We hypothesized peripheral regional catheters would reduce postoperative opioid use compared with no regional analgesia without increasing pain scores in pediatric patients with spina bifida undergoing major urological surgery. METHODS: A retrospective review of a multicenter database established for the study of enhanced recovery after surgery was performed of patients from 2009 to 2021 who underwent bladder augmentation or creation of catheterizable channels. Patients without spina bifida and those receiving epidural analgesia were excluded. Opioids were converted into morphine equivalents and normalized to patient weight. RESULTS: 158 patients with pediatric spina bifida from 7 centers were included, including 87 with and 71 without regional catheters. There were no differences in baseline patient factors. Anesthesia setup increased from median 40 min (IQR 34-51) for no regional to 64 min (IQR 40-97) for regional catheters (p<0.01). The regional catheter group had lower median intraoperative opioid usage (0.24 vs 0.80 mg/kg morphine equivalents, p<0.01) as well as lower in-hospital postoperative opioid usage (0.05 vs 0.23 mg/kg/day morphine equivalents, p<0.01). Pain scores were not higher in the regional catheters group. DISCUSSION: Continuous regional analgesia following major urological surgery in children with spina bifida was associated with a 70% intraoperative and 78% postoperative reduction in opioids without higher pain scores. This approach should be considered for similar surgical interventions in this population. TRIAL REGISTRATION NUMBER: NCT03245242.

General anesthesia safety in progressive leukodystrophies: A retrospective study of patients with Krabbe disease and metachromatic leukodystrophy.

BACKGROUND: Krabbe disease and metachromatic leukodystrophy are leukodystrophies characterized by neurologic degeneration and early death. Patients often require general anesthesia for diagnostic and therapeutic interventions. METHODS: A retrospective review of medical records was conducted for patients with Krabbe disease and metachromatic leukodystrophy receiving general anesthesia at a large children's hospital between 2012 and 2017. Patient complications and American Society of Anesthesiologists Physical Status were recorded for all procedures. The Neurodevelopment in Rare Disorders classification system was created to categorize the severity of the patient's disease progression based on clinical markers. Descriptive and inferential statistics were used to compare: (a) complication rate of affected patients vs the general hospital population; (b) the accuracy of the novel Neurodevelopment in Rare Disorders classification system vs American Society of Anesthesiologists Physical Status regarding the assessment of complication risk; (c) complication rate in patients with hematopoietic stem cell transplantation vs those without transplantation; (d) complication rate in immunosuppressed patients vs nonimmunosuppressed patients; and (e) complication rate of the three most commonly performed procedures. RESULTS: A total of 96 patients underwent 287 procedures. Of these, 11 cases had complications, yielding a rate of 3.8%. This is significantly higher than the overall complication rate at our institution of 0.246%. Statistical analysis showed better correlation between the Neurodevelopment in Rare Disorders classification system and complication rate than American Society of Anesthesiologists Physical Status and complication rate. The system also showed better accuracy in differentiating low-risk and high-risk patients. No statistically significant difference in complication rate was found for patients with transplantation vs those without transplantation or for immunosuppressed vs nonimmunosuppressed patients. Of the three most common procedures, central catheter placement/removal exhibited the highest complication rate. CONCLUSIONS: Although the complication rate for patients with Krabbe disease and metachromatic leukodystrophy is higher than the general population, most complications were mild and self-limiting. These results suggest that, in experienced hands, general anesthesia is well tolerated in most children. Findings show that the Neurodevelopment in Rare Disorders classification system is a better indicator for assessing complication risk in patients with Krabbe and metachromatic leukodystrophy than American Society of Anesthesiologists Physical Status.

Personalized pediatric ophthalmology: a case report.

The availability of genetic sequencing has given physicians a new tool for diagnosis and treatment of disease, and "personalized medicine" has become an increasingly common term in general but not in pediatric ophthalmology. We present a case of a toddler who developed ataxia, opsoclonus, myoclonus, and developmental regression following anesthesia for a common otolaryngology procedure. The child was found to have a variant in the MT-ND6 gene (m.14484T>C), most commonly associated with Leber hereditary optic neuropathy, despite a phenotype more closely resembling Leigh syndrome. The incongruence of phenotype and genotype prompted whole exome sequencing, which identified an unexpected intronic missense mutation in RB1 (1960+5G>A), with a 90% penetrance for retinoblastoma. Limited evaluation of the posterior pole in clinic did not identify any lesions, and the risks and benefits of examination under anesthesia were discussed among neurology, ophthalmology, and anesthesiology. We report the outcome of these discussions. The value and risks of personalized medicine are discussed.

Cold-stored whole blood platelet function is preserved in injured children with hemorrhagic shock.

BACKGROUND: Recent data demonstrate the safety of uncrossmatched cold-stored whole blood (WB) transfusion in pediatric trauma patients. The hemostatic capabilities of platelets within the cold-stored WB unit have been demonstrated via in vitro studies and animal models. However, platelet function has not been evaluated in pediatric recipients of cold-stored WB transfusions. METHODS: Injured children, 2 years or older and 10 kg or greater with hemorrhagic shock received up to 30 mL/kg of cold-stored, low titer (<50) anti-A and -B, leukoreduced, group O- WB during their initial resuscitation. Patients were included if (1) they received WB and no conventional platelets, and (2) platelet count and thromboelastography maximum amplitude were measured both before and after transfusion. These data and relevant clinical outcomes (mortality, intensive care unit length of stay [LOS], hospital LOS and ventilator days) were compared to a historical cohort of pediatric trauma patients who received uncrossmatched red blood cells (RBC) and conventional room temperature platelets. RESULTS: Twenty-two children were included in the study; 14 in the component cohort versus 8 in the WB cohort. Neither posttransfusion platelet count (129 × 109/L vs. 135 × 109/L) nor function (thromboelastography maximum amplitude, 59.5 mm vs. 60.2 mm) differed significantly between children receiving cold-stored platelets within the WB unit versus children who received conventional warm platelets. Median (interquartile range) weight-adjusted platelet transfusion volume in the historical cohort was 4.6 (2.5-7.7) mL/kg vs. 2.4 (1.3-4.0) mL/kg in the WB cohort (p = 0.03). There was no difference between groups in age, race, mechanism of injury, Injury Severity Score, vital signs, and severe traumatic brain injury (TBI). Outcomes, including mortality, intensive care unit LOS, hospital LOS, and ventilator days, were not significantly different between groups. CONCLUSION: No difference was seen in posttransfusion platelet number or function in severely injured children receiving cold-stored WB platelets as compared to those receiving conventional room temperature-stored platelets. Larger cohorts are required to confirm these findings. LEVEL OF EVIDENCE: Therapeutic, level IV.

A Multimodal Preemptive Analgesic Protocol for Alveolar Bone Graft Surgery: Decreased Pain, Hospital Stay, and Health Care Costs.

OBJECTIVE: To evaluate postoperative pain, hospital length of stay (LOS), and associated costs of multiple perioperative analgesic strategies following alveolar bone grafting (ABG). DESIGN: Retrospective comparative cohort study. SETTING: Tertiary care pediatric hospital. PATIENTS/PARTICIPANTS: Iliac crest bone graft (ICBG) harvest techniques: "Open Harvest" (n = 22), "Trephine Only" (n = 14), or "Trephine + Pain Pump" (n = 25). INTERVENTION: The "Open Harvest" group underwent open ICBG harvest with 3-walled osteotomies. For the other 2 treatment groups, a trephine drill was used to harvest iliac crest bone with a ropivacaine infusion pump into the hip donor site ("Trephine + Pain Pump") or without ("Trephine Only"). Patients who underwent ABG with only cadaveric allograft were analyzed as a comparison group ("No Harvest"). MAIN OUTCOMES MEASURES: Outcomes were planned prior to data collection: maximum pain score, hospital LOS, and associated health care costs. RESULTS: Maximum pain scores were significantly higher in the "Open Harvest" group (7.3/10) compared to "Trephine + Pain Pump" (1.8/10; P < .0001) and "No Harvest" groups (2.8/10; P < .01). Hospital LOS decreased from 2.4 days ("Open Harvest") to 0.5 days (Trephine + Pain Pump"; P < .0001). Twelve (48%) patients from "Trephine + Pain Pump" were discharged on the day of surgery. The "Trephine + Pain Pump" saved an estimated $5336 for a unilateral ABG and $7265 for a bilateral ABG compared to "Open Harvest." CONCLUSIONS: The combined use of the trephine ICBG technique and ropivacaine infusion catheter effectively decreased pain, shortened hospital stay, and improved cost saving compared to patients who have undergone other methods of ICBG.

Safety of antifibrinolytics in cranial vault reconstructive surgery: a report from the pediatric craniofacial collaborative group.

BACKGROUND: Antifibrinolytic therapy significantly decreases blood loss and transfusion in pediatric cranial vault reconstructive surgery; however, concern regarding the side effects profile limits clinical use. AIMS: The aim was to utilize the Pediatric Craniofacial Surgery Perioperative Registry database to identify the safety profile of antifibrinolytic therapy for cranial vault reconstructive surgery by reporting the incidence of adverse events as they relate to exposure to tranexamic acid and aminocaproic acid compared to no exposure to antifibrinolytics. METHODS: The database was queried for cases of open cranial vault reconstructive surgery. Less invasive procedures such as neuro-endoscopic and spring-mediated cranioplasties were excluded. The outcomes evaluated included any perioperative neurological adverse event including seizures or seizure-like movements and thromboembolic events. RESULTS: Thirty-one institutions reported a total of 1638 cases from 2010 to 2015. Total antifibrinolytic administration accounted for 59.5% (tranexamic acid, 36.1% and aminocaproic acid, 23.4%), with 40.5% not receiving any antifibrinolytic. The overall incidence of postoperative seizures or seizure-like movements was 0.6%. No significant difference was detected in the incidence of postoperative seizures between patients receiving tranexamic acid and those receiving aminocaproic acid [the odds ratio for seizures being 0.34 (95% confidence interval: 0.07-1.85) controlling for American Society of Anesthesia (ASA) physical class] nor in patients receiving antifibrinolytics compared to those not administered antifibrinolytics (the odds ratio for seizures being 1.02 (confidence interval 0.29-3.63) controlling for ASA physical class). One complicated patient in the antifibrinolytic group with a femoral venous catheter had a postoperative deep venous thrombosis. CONCLUSIONS: This is the first report of an incidence of postoperative seizures of 0.6% in pediatric cranial vault reconstructive surgery. There was no significant difference in postoperative seizures or seizure-like events in those patients who received the tranexamic acid or aminocaproic acid vs those that did not. This report provides evidence of the safety profile of antifibrinolytic in children having noncardiac major surgery. Caution should prevail however in using antifibrinolytic in high-risk patients. Antifibrinolytic dosage regimes should be based on pharmacokinetic data avoiding high doses.

Perioperative Outcomes and Management in Pediatric Complex Cranial Vault Reconstruction: A Multicenter Study from the Pediatric Craniofacial Collaborative Group.

BACKGROUND: The Pediatric Craniofacial Collaborative Group established the Pediatric Craniofacial Surgery Perioperative Registry to elucidate practices and outcomes in children with craniosynostosis undergoing complex cranial vault reconstruction and inform quality improvement efforts. The aim of this study is to determine perioperative management, outcomes, and complications in children undergoing complex cranial vault reconstruction across North America and to delineate salient features of current practices. METHODS: Thirty-one institutions contributed data from June 2012 to September 2015. Data extracted included demographics, perioperative management, length of stay, laboratory results, and blood management techniques employed. Complications and outlier events were described. Outcomes analyzed included total blood donor exposures, intraoperative and perioperative transfusion volumes, and length of stay outcomes. RESULTS: One thousand two hundred twenty-three cases were analyzed: 935 children aged less than or equal to 24 months and 288 children aged more than 24 months. Ninety-five percent of children aged less than or equal to 24 months and 79% of children aged more than 24 months received at least one transfusion. There were no deaths. Notable complications included cardiac arrest, postoperative seizures, unplanned postoperative mechanical ventilation, large-volume transfusion, and unplanned second surgeries. Utilization of blood conservation techniques was highly variable. CONCLUSIONS: The authors present a comprehensive description of perioperative management, outcomes, and complications from a large group of North American children undergoing complex cranial vault reconstruction. Transfusion remains the rule for the vast majority of patients. The occurrence of numerous significant complications together with large variability in perioperative management and outcomes suggest targets for improvement.

Dexmedetomidine as a Rapid Bolus for Treatment and Prophylactic Prevention of Emergence Agitation in Anesthetized Children.

BACKGROUND: Administration of dexmedetomidine (DEX) in the pediatric population for its sedative, analgesic, and anxiolytic properties has been widely reported, despite there being no label indication approved by the U.S. Food and Drug Administration for pediatric patients. Infusions of DEX, rather than bolus administration, are recommended to attenuate the hemodynamic response caused by the α2-adrenoreceptor agonist. In this prospective, double-blind, randomized study, we examined the effect of rapid IV bolus injection of DEX on emergence agitation and the hemodynamic response in a large sample of children undergoing tonsillectomy with or without adenoidectomy, with or without myringotomy, and/or tympanostomy tube insertion. METHODS: Four hundred patients, aged 4 to 10 years, undergoing tonsillectomy with or without adenoidectomy, with or without myringotomy, and/or tympanostomy tube insertion, were randomized at a 1:1 ratio into 1 of the 2 treatment groups in a double-blinded fashion. After a standardized anesthetic regimen and approximately 5 minutes before the end of surgery, patients in group DEX were administered a rapid IV bolus of 4 µg·mL DEX at a dose of 0.5 µg·kg, whereas patients in group saline received a rapid IV bolus of equivalent volume saline. Baseline measurements of heart rate, systolic blood pressure, diastolic blood pressure, respiratory rate, and blood oxygen saturation were collected immediately before study drug administration and every minute thereafter for 5 minutes. In the postanesthesia care unit, vital signs were measured, emergence agitation (EA) was assessed using the Pediatric Anesthesia Emergence Delirium scale, and postoperative opioid use and complications were recorded. RESULTS: The incidence of EA in group DEX was significantly lower than that in group saline, regardless of whether EA was defined as a Pediatric Anesthesia Emergence Delirium score >10 (36% vs 66%, respectively; P < 0.0001; relative risk [95% confidence interval] = 0.527 [0.421-0.660]; number needed to treat = 3.33) or >12 (30% vs 61%, respectively; P < 0.0001; relative risk [95% confidence interval] = 0.560 [0.458-0.684]; number needed to treat = 3.23). Both groups exhibited similar baseline vital signs before study drug injection (all P ≥ 0.602). After injection, group DEX experienced a significant decrease in heart rate for all time points in comparison with group saline (all P < 0.0001). A significant, biphasic blood pressure response was observed in group DEX, specifically, a transient increase in systolic blood pressure at 1 minute after injection (P < 0.0001) and a subsequent decrease below baseline for 3, 4, and 5 minutes (all P < 0.0001). No patients required treatment for bradycardia, hypertension, or hypotension. A significantly smaller percentage of patients in group DEX received postoperative, supplemental opioid medication compared with group saline (48% vs 73%, respectively; P < 0.0001). Group DEX appeared to experience fewer adverse events than group saline as well (9% vs 17%, respectively; P = 0.025). CONCLUSIONS: Rapid IV bolus administration of DEX in children improved their recovery profile by reducing the incidence of EA. A statistically significant change in hemodynamics was observed, but no patients required any intervention for hemodynamic changes. Furthermore, DEX reduced the incidence of postoperative opioid administration, and a trend of fewer adverse events was observed in group DEX.

Development of Volunteer International Craniofacial Surgery Missions: The Komedyplast Protocol.

Volunteer surgical missions to provide cleft care to patients in developing countries has been done successfully for a number of years. Similar missions that provide craniofacial surgery introduce a dramatic step up in complexity. While articles have addressed protocols for the safe delivery of cleft care around the world, little has been written on volunteer craniofacial surgical missions. Komedyplast was established in March 2001 as a 501c(3) nonprofit organization to provide craniofacial surgical care to underserved populations and educate local surgeons in craniofacial principles. During 9 annual missions, the organization has provided surgical care to more than 150 patients with various complex, congenital, craniofacial conditions. The article addresses important safeguards that have been implemented to maximize safety and minimize risk.

Intraoperative high-dose epinephrine infiltration in cleft palate repair.

OBJECTIVE: Local infiltration of epinephrine before surgical procedures is a well-accepted technique to promote vasoconstriction. Typically, the dose of epinephrine is limited by the co-administration of local anesthetic as well as the risk for arrhythmogenesis and hemodynamic changes. In addition, some controversy exists regarding the acceptable dose of epinephrine given to children. This retrospective review examines the use and safety of "high-dose" epinephrine in palatoplasty at our cleft-craniofacial center. DESIGN: A retrospective review of epinephrine use in primary palatoplasty at a tertiary children's hospital from 2003 to 2007 was performed. Operative and anesthetic records were reviewed for hypertension (systolic blood pressure, >120 or diastolic blood pressure, >70) and tachycardia (>190 beats per min) as defined by the American Heart Association guidelines, as well as dysrhythmias, intraoperative complications, and postoperative complications. RESULTS: A total of 102 patients who underwent consecutive primary palatoplasties performed by a single surgeon were identified. After the induction of anesthesia and before incision, the patients received an initial epinephrine infiltration (without local anesthetic) up to a maximum 10 µg/kg. The average total dose of epinephrine administered during palatoplasty was 12.8 µg/kg (range, 3.2-75.0 µg/kg). Doses up to a maximum of 10 µg/kg were administered as needed at 30-minute intervals. No instances of clinically unstable tachycardia or hypertension occurred. A total of 21.6% of the patients (22/102) experienced an instance of hypertension, and only 13.7% of these (14/102) were related to epinephrine administration. One (1%) postoperative fistula was identified. CONCLUSIONS: Locally infiltrated high-dose epinephrine during palatoplasty can be safely used as a means of vasoconstriction. Doses reaching a maximum of 10 µg/kg, administered as needed at 30-minute intervals, do not seem to be a significant risk for hemodynamic instability, intraoperative complications, or postoperative complications.

Postoperative analgesia via transversus abdominis plane (TAP) catheter for small weight children-our initial experience.

Pain management in small infants and children is challenging. We report the use of unilateral transversus abdominis plane (TAP) catheters for analgesia following single-sided open lower abdominal surgery when epidural analgesia was undesirable in a series of six low-weight children. Data collection was done via a retrospective chart review. All catheters attempted were successfully placed with resultant low pain scores and the minimal use of rescue analgesic medications. No complications were reported related to these catheters. TAP catheters, although technically challenging, are feasible for the provision of analgesia for surgeries involving the lower abdominal wall in very small patients.

Perioperative management of children undergoing craniofacial reconstruction surgery: a practice survey.

OBJECTIVE/AIMS: To assess current practices in the management of children undergoing craniofacial surgery and identify areas of significant practice variability with the intent to direct future research. BACKGROUND: The perioperative management of infants and children undergoing craniofacial reconstruction surgery can be challenging because of the routine occurrence of significant blood loss with associated morbidity. A variety of techniques have been described to improve the care for these children. It is presently unknown to what extent these practices are currently employed. METHODS: A web-based survey was sent to representatives from 102 institutions. One individual per institution was surveyed to prevent larger institutions from being over-represented in the results. RESULTS: Requests to complete the survey were sent to 102 institutions; 48 surveys were completed. The survey was composed of two parts: management of infants undergoing strip craniectomies, and management of children undergoing major craniofacial reconstruction. CONCLUSIONS: Significant variability exists in the management of children undergoing these procedures; further study is required to determine the optimal management strategies. Clinical trials assessing the utility of central venous pressure and other hemodynamic monitoring modalities would enable evidence-based decision-making for monitoring in these children. The development of institutional transfusion thresholds should be encouraged, as there exists a body of evidence supporting their efficacy and safety.

Postoperative hyponatremia following calvarial vault remodeling in craniosynostosis.

BACKGROUND: The incidence, severity, and risk factors for the development of hyponatremia in patients undergoing craniosynostosis surgery are not well known. OBJECTIVE: To determine the incidence and severity of hyponatremia as a complication in surgical correction of craniosynostosis and to identify risk factors for postoperative hyponatremia. METHODS: A retrospective medical record review for 2003-2008 of the Cleft and Craniofacial Database was made. Specific data collected included sodium values, age, weight, type of surgery, duration of surgery, administration of DDAVP, composition and volume of pre-operative, intra-operative, postoperative parenteral fluids, volume of blood, colloid, and crystalloid transfused, estimated blood loss (EBL), medications, comorbidities, pre-operative signs of elevated intracranial pressure (ICP), syndromic vs nonsyndromic craniosynostosis, and the complications associated with hyponatremia. RESULTS: A total of 72 records were reviewed. The incidence of postoperative hyponatremia was 30.6%. There was no intra-operative hyponatremia. While hospital stay was not prolonged, ICU stay was significantly longer (1.9 vs 2.9 days, P = 0.001). Elevated ICP was significantly associated with hyponatremia (P < 0.002). A greater volume of blood loss (EBL) was associated with postoperative hyponatremia (P = 0.019). Patients with normal pre-operative ICP were more likely to become hyponatremic if they were female (relative risk = 2.43; P < 0.05). The average decrease in sodium was greater in patients receiving hyponatremic (hypotonic) vs normonatremic (isotonic) postoperative IVF's (5.5 vs 3.2 mEq·l(-1), P = 0.039). There were no postoperative complications related to hyponatremia. CONCLUSIONS: The incidence of postoperative hyponatremia after calvarial vault remodeling was determined to be 30.6%. Hyponatremia was significantly associated with increased pre-operative ICP, blood loss, and female gender (normal pre-operative ICP). The average decrease in sodium was greater in patients receiving hyponatremic postoperative IVF's.

Transfusion-free pediatric burn surgery: techniques and strategies.

More than 1.3 million children sustain burns each year, resulting in 40,000 admissions and more than 3000 pediatric deaths. Pediatric burn surgery has been described as excessively bloody. Strategies to reduce intraoperative blood loss include the use of topical thrombin and epinephrine, extremity tourniquets, acute normovolemic hemodilution, and hypotensive anesthesia. This study reviews the single surgeon pediatric burn experience at a children's hospital and describes a comprehensive blood conservation protocol to achieve transfusion-free pediatric burn surgery. A retrospective chart review of consecutive pediatric burn surgeries from July 2000 to April 2002 was performed. Patient demographics, burn characteristics, treatment, blood loss, laboratory values, transfusion history, and complications were reviewed. Blood loss per percent total body surface area (TBSA) treated as well as percent total blood volume (TBV) loss divided by percent TBSA treated were calculated. A total 31 burn surgeries in 23 patients were reviewed. The average age was 7 years (range, 9 months-17 years). There were 17 extremity, 6 trunk, and 2 head/neck burns. The average TBSA burned was 15% (range, 1-55%). The protocol to reduce intraoperative blood loss consisted of the debridement of full-thickness burns with electrocautery and partial-thickness burns with dermabrasion. All debrided or harvested surgical sites were treated immediately with epinephrine solution-soaked pads. All graft harvest sites were injected with an epinephrine solution before harvesting split-thickness skin grafts. The average TBSA treated per surgery was 7% (range, 1-29%). The average blood loss per percent TBSA treated was 15 mL (range, 0.7-37 mL). The average percent TBV/percent TBSA was 0.76% (range, 0.04-3.6%). All 20 patients underwent surgical debridement, 7 patients were treated with AlloDerm and ultrathin split-thickness skin grafts, 2 with full-thickness skin grafts, and 17 with split-thickness skin grafts alone. Five children required blood transfusions. These burns averaged 32% TBSA (range, 20-55%). All 5 children receiving transfusions had anemia of thermal injury and demonstrated an average preoperative drop in hematocrit of 12% (range, 10-14%). There was a 29% complication rate, with 7 patients experiencing partial graft loss, and 1 patient who developed a postgraft contracture that required revisional surgery. There was a single mortality secondary to systemic inflammatory response syndrome and acute respiratory distress syndrome. After the proposed pediatric burn treatment protocol, intraoperative blood loss requiring transfusion can be minimized or eliminated. Large TBSA burns must be surveilled for burn wound anemia that may ultimately require blood transfusion.