Free
Perioperative Medicine  |   February 2017
Perioperative Outcomes and Management in Pediatric Complex Cranial Vault Reconstruction: A Multicenter Study from the Pediatric Craniofacial Collaborative Group
Author Notes
  • From the Department of Anesthesiology and Critical Care Medicine, The Children’s Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania (P.A.S.); Department of Anesthesiology, Perioperative and Pain Medicine, Harvard Medical School, Boston Children’s Hospital, Boston, Massachusetts (S.M.G., P.M.M.); Department of Anesthesiology, The Children’s Hospital of Pittsburgh of UPMC, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania (F.P.C.); Departments of Anesthesiology and Pain Medicine and Pediatrics, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, Washington, D.C. (C.M.H.); Children’s National Health System, Division of Anesthesiology, The George Washington University School of Medicine and Health Sciences, Washington, DC (S.K.R.); Department of Anesthesiology, Monroe Carell Jr. Children’s Hospital, Vanderbilt University Medical Center, Nashville, Tennessee (T.T.N.); Department of Epidemiology and Biostatistics, Dornsife School of Public Health, Drexel University, Philadelphia, Pennsylvania (L.C., M.P.); and Department of Anesthesiology and Pain Management, University of Texas Southwestern Medical School, Children’s Medical Center Dallas, Texas and Outcome Research Consortium, Cleveland, Ohio (P.S.).
  • Submitted for publication June 26, 2016. Accepted for publication November 7, 2016.
    Submitted for publication June 26, 2016. Accepted for publication November 7, 2016.×
  • *Members of the Pediatric Craniofacial Collaborative Group are listed in appendix 2.
    Members of the Pediatric Craniofacial Collaborative Group are listed in appendix 2.×
  • Address correspondence to Dr. Stricker: Department of Anesthesiology and Critical Care Medicine, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. strickerp@email.chop.edu. Information on purchasing reprints may be found at www.anesthesiology.org or on the masthead page at the beginning of this issue. Anesthesiology’s articles are made freely accessible to all readers, for personal use only, 6 months from the cover date of the issue.
Article Information
Perioperative Medicine / Clinical Science / Coagulation and Transfusion / Pediatric Anesthesia
Perioperative Medicine   |   February 2017
Perioperative Outcomes and Management in Pediatric Complex Cranial Vault Reconstruction: A Multicenter Study from the Pediatric Craniofacial Collaborative Group
Anesthesiology 2 2017, Vol.126, 276-287. doi:10.1097/ALN.0000000000001481
Anesthesiology 2 2017, Vol.126, 276-287. doi:10.1097/ALN.0000000000001481
Abstract

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.

What We Already Know about This Topic
  • Cranial vault reconstruction for craniosynostosis is attendant with significant complications. A database of the rates of complications and modes of practice for complex cranial vault reconstruction is not available.

  • In a multicenter assessment, the Pediatric Craniofacial Surgery Perioperative Registry was queried and anesthetic management and complications were evaluated.

What This Article Tells Us That Is New
  • The majority of patients received blood transfusion and were admitted to the intensive care unit postsurgery.

  • Notable complications included cardiac arrest, hypotension, seizures, coagulopathy, and large-volume blood transfusion.

  • There were significant variations in perioperative management practices and in-hospital outcomes. These results serve as a platform for future comparisons of management practice.

SINCE the inception of the discipline of pediatric craniofacial surgery with the pioneering work of Dr. Paul Tessier in the late 1960s, morbidity and mortality have steadily declined but remain relevant with recent reports of massive blood loss and life-threatening events.1–6  Cranial vault reconstruction is performed in infants and children with craniosynostosis to improve appearance and prevent or treat neurologic impairment. Despite improvements in surgical technique and anesthetic management, many of which focused on limiting blood loss and transfusion, today, these patients may still experience massive blood loss and transfusion and are at risk of significant complications. There are currently no large-scale comprehensive analyses of both multicenter practices and outcomes for pediatric complex cranial vault reconstruction surgery (CCVR).
The Pediatric Craniofacial Collaborative Group was formed under the auspices of the Society for Pediatric Anesthesia in 2011. This group met and determined data elements to be collected and established the Pediatric Craniofacial Surgery Perioperative Registry (PCSPR) to elucidate current practices and outcomes in this population, with the ultimate goal of informing improvement efforts and optimizing care. The aim of this study is to determine perioperative management, outcomes, and complications in children undergoing CCVR and to delineate salient features of current practices. Specifically, this study aims to describe current North American trends in perioperative management of infants and children undergoing CCVR by reporting hospital outcomes including length of stay (LOS), major perioperative complications, anesthetic management techniques, fluid and transfusion practices, and the application of patient blood management strategies.
Materials and Methods
We queried the PCSPR for subjects undergoing CCVR. CCVR was defined as frontoorbital advancement/anterior cranial vault reconstruction, middle/posterior cranial vault reconstruction, or total cranial vault reconstruction. All procedures involved a craniotomy. Procedures identified as neuroendoscopic procedures, spring-mediated cranioplasties, and modified π procedures or variants thereof were excluded. Data extracted included demographic and surgical data, fluid and transfusion data, intensive care unit (ICU) and hospital LOS, perioperative management, and complications.
PCSPR
Participating institutions began entering data after local institutional review board or equivalent approval. Study data were collected and managed using research electronic data capture tools hosted at the Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania.7  Data entry began on June 25, 2012. A deidentified dataset with data through September 30, 2015, was analyzed. Individual institutions were represented using an institution code and were not identifiable.
Data Collection, Entry, and Validation
Thirty-one institutions contributed data during the study time period (appendix 1). Sites were required to provide quarterly reports quantifying case capture rates to ensure the absence of reporting bias. The aggregate capture rate across all institutions was 96%. The median (25th to 75th interquartile range) case capture rate was 100% (97 to 100%) at the 25 institutions where informed consent was not required and 99% (68 to 100%) at the six institutions where informed consent was required. All participating institutions were required to report data collection and auditing processes to ensure accuracy. At 29 institutions, the principal investigator or designee reviewed more than 50% of cases before registry entry. At two institutions, 10 to 25% of cases were reviewed before registry entry.
In addition to the above, the study principal investigator at the Data Coordinating Center (P.A.S.) audited data before analysis. This included scrutinizing cases for omissions of critical data (e.g., age and weight) and identification of outlier data. Cross-validation of data within individual records was performed to optimize data accuracy. Queries based on omissions, outliers, and discrepancies identified through this process were aggregated and sent to site investigators for rectification.
Outcomes and Analysis
Demographic data were analyzed using descriptive statistics. We divided the population into two age subgroups for the majority of the analyses: age less than or equal to 24 months (subsequently referred herein as the infant group and infants) and age more than 24 months (older group). The groups reflect the two principal demographics of children undergoing these procedures: those undergoing usually primary procedures in the first 2 yr of life (the majority of cases) and those presenting for surgery later in childhood (late presentations, redo procedures, and syndromic patients).
Outcomes analyzed included the following: total perioperative blood donor exposures, volume of erythrocyte-containing blood products (ECBPs) transfused intraoperatively, total volume of blood products transfused perioperatively (intraoperatively and in the first postoperative 48 h), hospital LOS, and ICU LOS. ECBPs include packed erythrocytes, whole blood, and reconstituted blood composed of packed erythrocytes and fresh frozen plasma (FFP).8  Perioperative blood products included packed erythrocytes, reconstituted blood, whole blood, FFP, platelets, and cryoprecipitate. Owing to profound inaccuracies/biases of blood loss estimates in these infants and children, the collaborative group decided not to include estimated blood loss in the PCSPR. Transfusion volumes were used as surrogates for blood loss since they are systematically and accurately recorded. Anesthetic and surgical management, fluid and transfusion management, complications, and the application of blood conservation strategies are also described. Data measured on a continuous scale are presented as mean ± SD or median (25th to 75th interquartile range); frequencies and percentages are presented for categorical variables. Differences in selected outcomes by age group were compared using the independent sample Student’s t test (two tailed) and the Wilcoxon–Mann–Whitney test. A P < 0.05 was considered statistically significant. Statistical analysis was done with the statistical package SAS (SAS Institute Inc., USA).
Results
Demographics
The registry query yielded a total of 1,223 subjects from 31 institutions, with 935 aged less than or equal to 24 months and 288 aged more than 24 months. Demographic data are presented in table 1. In the infant group, the most common diagnoses were metopic, sagittal, and unicoronal synostosis. In the older group, the most common diagnoses were syndromic, sagittal, and multiple suture synostosis. Seventy-one percent of subjects in the older group and 23% of subjects in the younger group underwent redo operations or had either syndromic or multiple-suture (more than or equal to three sutures) synostosis. Compared to the infant group, older subjects had higher rates of total vault reconstruction (P < 0.0001), distractor placement (P = 0.0003), previous craniofacial surgery (P < 0.0001), syndromic craniosynostosis (P < 0.0001), preoperative elevated intracranial pressure (P < 0.0001), higher preoperative hemoglobin (P < 0.0001), and higher American Society of Anesthesiologists physical status (P < 0.0001; table 1). Among patients of all ages, preoperative evidence of increased intracranial pressure was more frequent in children with syndromic craniosynostosis than in those without (34% vs. 10%, respectively; P < 0.001).
Table 1.
Patient Demographic Data
Patient Demographic Data×
Patient Demographic Data
Table 1.
Patient Demographic Data
Patient Demographic Data×
×
Perioperative Outcomes
Selected perioperative outcomes are presented in table 2. The majority of patients were transfused perioperatively in both groups; only 5% of infants were not transfused. Transfusion volumes (ml/kg) were greater in the infant group. In terms of blood donor exposures, the most striking difference was the percentage of patients having at least one blood donor exposure (95% infant group vs. 79% older group).
Table 2.
Selected Perioperative Outcomes*
Selected Perioperative Outcomes*×
Selected Perioperative Outcomes*
Table 2.
Selected Perioperative Outcomes*
Selected Perioperative Outcomes*×
×
Postoperative ICU admission was routine practice for the majority of patients at 90% (28/31) of institutions in the infant group and at 92% (22/26) of institutions in the older group. ICU LOS data were available for 872 (93%) and 279 (97%) patients in these groups, respectively; the median ICU LOS was 2 days for both groups. Only 4% (39/872) of infants and 3% (7/279) of older children were not admitted to the ICU.
Hospital LOS data were available for 887 (95%) infant group cases and 281 (98%) older group cases. The median hospital LOS was 4 days in infants: 97% stayed more than or equal to 3 days and 80% stayed more than or equal to 4 days. Findings were similar in older children, for whom median hospital LOS was 5 days: 96% stayed more than or equal to 3 days, and 79% stayed more than or equal to 4 days.
Perioperative Complications and Outlier Events
Selected perioperative complications and outlier events for all age patients are presented in table 3. There were no deaths. Notable events included cardiac arrest, hypotension, unplanned postoperative mechanical ventilation, seizures, coagulopathy (table 3), and large-volume transfusion (more than 40 ml/kg).
Table 3.
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)×
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)
Table 3.
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)×
×
Anesthetic and Intraoperative Management
Data describing the anesthetic and intraoperative management of patients in each group are presented in table 4. Nearly all patients were managed with an inhaled anesthetic-opioid technique, at least two peripheral intravenous catheters, and invasive arterial blood pressure monitoring. A central venous catheter was placed in 13% of infants and 14% of older children. Thromboelastography was utilized in a minority of patients; TEG® (Haemonetics Corporation, USA) was the modality in all but one of these cases.
Table 4.
Anesthetic/Intraoperative Management
Anesthetic/Intraoperative Management×
Anesthetic/Intraoperative Management
Table 4.
Anesthetic/Intraoperative Management
Anesthetic/Intraoperative Management×
×
Perioperative Fluids and Transfusion
There was wide variability in perioperative transfusion volumes across institutions, as shown in figure 1; intraoperative fluid and transfusion data are shown in table 5. Nearly all patients (91%) in the infant group were transfused intraoperatively.
Table 5.
Perioperative Fluids and Transfusion*
Perioperative Fluids and Transfusion*×
Perioperative Fluids and Transfusion*
Table 5.
Perioperative Fluids and Transfusion*
Perioperative Fluids and Transfusion*×
×
Fig. 1.
Box plots showing perioperative blood product transfusion (ml/kg) in the infant group (age less than or equal to 24 months) by institution. Perioperative blood products include packed red erythrocytes, whole blood, reconstituted blood, fresh frozen plasma, platelets, and cryoprecipitate administered intraoperatively and in the first postoperative 48 h. Institutions except 10, 22, 23, 24, 27, and 30 had at least six cases.
Box plots showing perioperative blood product transfusion (ml/kg) in the infant group (age less than or equal to 24 months) by institution. Perioperative blood products include packed red erythrocytes, whole blood, reconstituted blood, fresh frozen plasma, platelets, and cryoprecipitate administered intraoperatively and in the first postoperative 48 h. Institutions except 10, 22, 23, 24, 27, and 30 had at least six cases.
Fig. 1.
Box plots showing perioperative blood product transfusion (ml/kg) in the infant group (age less than or equal to 24 months) by institution. Perioperative blood products include packed red erythrocytes, whole blood, reconstituted blood, fresh frozen plasma, platelets, and cryoprecipitate administered intraoperatively and in the first postoperative 48 h. Institutions except 10, 22, 23, 24, 27, and 30 had at least six cases.
×
In the infant group, 24% of patients were transfused FFP and 3% were transfused platelets intraoperatively. In this group, the volume of intraoperative ECBPs was 46.7 ± 29.3 ml/kg in patients transfused FFP (excluding FFP in reconstituted blood), while the volume of ECBPs was 96.1 ± 51.4 ml/kg in those who received transfused platelets.
The majority of patients in both groups were not transfused postoperatively. In those who were transfused, packed erythrocytes were the predominant product: only 6% of infants and 3% of older patients were transfused FFP, platelets, or cryoprecipitate.
Blood Conservation Techniques and Transfusion-free Hospital Course
Data describing the utilization of various blood conservation strategies are presented in table 6. Specific transfusion protocols to guide transfusion were employed in a minority of cases. There were 82 infants (9%) and 69 older children (24%) who were not transfused intraoperatively. Of these, 37 (45%) and 7 (10%), respectively, received a postoperative transfusion; thus, a total of 45 infants and 62 older children experienced a transfusion-free hospital course. Among those patients with a transfusion-free hospital course, no specific conservation strategies were utilized in 9 of 45 (20%) in the infant group and 19 of 62 (31%) in the older group.
Table 6.
Blood Conservation Technique and Transfusion-free Patient Data
Blood Conservation Technique and Transfusion-free Patient Data×
Blood Conservation Technique and Transfusion-free Patient Data
Table 6.
Blood Conservation Technique and Transfusion-free Patient Data
Blood Conservation Technique and Transfusion-free Patient Data×
×
Discussion
In this study, we present a comprehensive description of perioperative management and outcomes from 1,223 children undergoing CCVR in North America during a 3-yr period. This report is from a prospective registry specifically designed for this population, with contributions from 29 institutions in the United States and two in Canada.
Patient Characteristics
Consistent with known epidemiology of craniosynostosis, males predominated in our dataset. In the infant group, metopic synostosis was the most prevalent diagnosis, followed by sagittal and unicoronal synostosis. Syndromic and multiple-suture craniosynostosis were among the most prevalent diagnoses in the older group, and more than 50% of patients in this group underwent repeat procedures. Total cranial vault reconstruction and placement of distractor hardware were more frequent in this group, reflecting the surgical complexity of these patients.
Blood Loss, Transfusion, and Blood Conservation Strategies in CCVR
We found wide variability in perioperative transfusion volumes, both between institutions and within individual institutions (fig. 1). The intraoperative transfusion volumes we report are consistent with previous studies, with 30% of patients in the infant group receiving more than 40 ml/kg intraoperative ECBPs.9–14  Despite high transfusion volumes, the median number of blood donor exposures was just one for both groups (table 2), a finding attributable to the fact that a single unit of packed erythrocytes with a volume of 320 ml represents 40 ml/kg in an 8-kg infant.
Antifibrinolytics.
Two randomized placebo-controlled prospective trials have shown that tranexamic acid decreases blood loss and transfusion in CCVR.10,15  In less rigorous observational studies, aminocaproic acid was associated with decreased blood loss and transfusion in CCVR.16,17  In our population, antifibrinolytic administration was used in 63% of children despite evidence of efficacy. Our finding that antifibrinolytics were not administered to 37% of children suggests that broadening antifibrinolytic administration is a potential improvement intervention.
Cell Saver.
Cell saver was the second most common blood conservation technique used in CCVR in our dataset. No controlled study has evaluated cell saver as a stand-alone modality in CCVR. Overall, cell saver utilization was relatively low, perhaps due to costs and availability of equipment and personnel.
Erythropoietin.
Synthetic erythropoietin has been studied as a means to increase the amount of blood loss that can be safely tolerated in infants undergoing craniofacial surgery.18–20  Despite potential benefits, only three patients in our dataset received preoperative erythropoietin. It appears that cost, inconvenience, and concerns for complications have all but eliminated the use of erythropoietin in CCVR.
Acute Preoperative Normovolemic Hemodilution.
Acute preoperative normovolemic hemodilution has been applied to pediatric craniofacial surgery patients.21,22  Mathematical modeling predicts blood savings to be modest at best with this technique.23  Moreover, it is difficult to obtain sufficient blood from patients under 30 kg.24  Acute preoperative normovolemic hemodilution was only applied in two patients in our dataset, indicating that this technique has been abandoned in CCVR.
Deliberate Hypotension.
Four percent of infants and 9% of older children had deliberate hypotension despite a lack of high-level evidence to support the efficacy or safety of this technique in CCVR. Upon closer review, the degree to which the children in our dataset were rendered hypotensive is unclear. Recent publications have argued there is no indication for deliberate hypotension in these infants.25,26 
Transfusion Thresholds/Protocols.
There is evidence in pediatric ICU patients and pediatric postsurgical patients that employing restrictive transfusion thresholds is safe and effective.27,28  There is evidence that accepting lower hemoglobin values29  and implementing postoperative transfusion protocols30  are effective in CCVR. We found formal transfusion protocols were not used in approximately two thirds of patients. The adoption of restrictive transfusion practices, and in particular the implementation of postoperative transfusion thresholds, is a potential low-cost improvement intervention to reduce transfusion in CCVR.
Bloodless Craniosynostosis Surgery.
Despite numerous publications during the past 20 yr describing methods to reduce and avoid transfusion in CCVR, a transfusion-free hospital course was achieved in only a small minority of infants, and the majority of institutions had no infants with a transfusion-free course. Interestingly, 20% of infants and 31% of older children with a transfusion-free hospital course had no specific blood conservation technique employed, including the use of transfusion protocols (infant group). Two things are suggested by this finding. First, perhaps the greatest determinant of blood loss and the need for transfusion is a variable not captured in this or other studies: surgical technique. Second, institutions with some transfusion-free patients without using these techniques might increase the number of transfusion-free patients by adopting multimodal blood management practices (e.g., antifibrinolytics and transfusion thresholds).
ICU and Hospital LOS
We observed a relatively consistent hospital LOS of 4 to 5 days. ICU admission after CCVR is a standard practice at most institutions inasmuch as only a minority of children were not admitted to the ICU postoperatively. In one study, preexisting medical conditions and higher blood loss were identified as predictors for the need of ICU admission.31  In another study, the authors proposed an algorithm for predicting an event requiring ICU admission based on risk factors.11  It remains to be determined whether these findings can be applied at different institutions and whether patients can be safely selected for ward versus ICU admission. Efforts to judiciously limit ICU LOS may be a strategy to minimize costs as many centers had ICU stays of 1 day, while at others, multiple-day stays were the rule.
Perioperative Complications and Events
Mortality rates in pediatric craniofacial surgery have steadily declined, from 1.6% in 19791  to 0.1% more recently.4  The absence of deaths in our study is consistent with a continuation of this trend; based on the rule of three, we can conclude from our dataset with 95% confidence that the mortality rate is less than 0.2%.
Although there were no deaths, there were major events indicative of severe clinical problems. Cardiovascular complications, respiratory complications, and sequelae of large-volume blood loss replacement predominated. Notable events included cardiac arrest, intravenous epinephrine bolus administration, inadvertent intraoperative extubation, unplanned postoperative intubation, postoperative seizures, coagulopathy, respiratory failure, unplanned second surgery, and massive transfusion.
Hematologic derangements were more frequent in the infant group, which likely relates to higher ECBP transfusion volumes in this group. The incidence of hyponatremia we observed is similar to that in a previous report32  in which there were no serious events although one patient had a serum sodium concentration of 121 mEq/l. Seizures from hyponatremia after CCVR have not previously been reported; the two occurrences in our population illustrate the potential severe sequelae of hyponatremia in CCVR.
Anesthetic and Intraoperative Management
Our dataset defines national perioperative management of children undergoing CCVR. Some elements of management are nearly universal: most patients were managed with general anesthesia maintained with a combined inhalation-opioid technique, at least two peripheral intravenous catheters placed, and invasive arterial blood pressure monitoring. A central venous catheter was placed in 13 to 14% of children. A practice survey in 2011 revealed that 65% of institutions place central catheters in less than 10% of patients; inadequate peripheral venous access was the most common rationale.33  A subsequent study in CCVR demonstrated no improvement in the frequency or duration of hypotension with central venous pressure monitoring.34  Central venous pressure has been consistently shown to be a poor predictor of fluid responsiveness in children.35  Central catheter insertion is associated with significant complications in children, including cardiac arrest.36  Based on the above, we suggest these catheters are not generally required for CCVR and their use be reserved for cases with inadequate peripheral venous access or other specific circumstances.
Venous air embolism occurs frequently in children undergoing open craniofacial surgical procedures, and routine use of precordial Doppler has been suggested.37,38  Doppler monitoring is sensitive but nonspecific, with the vast majority of episodes being clinically insignificant. The lack of widespread adoption of precordial Doppler in CCVR underscores its primary limitation: a subjective monitor with a signal-to-noise ratio too low to justify routine use.
Automated analysis of the invasive arterial waveform was used in approximately one third of patients. This is interesting given that parameters derived from the arterial waveform (such as systolic pressure and pulse pressure variation with respiration) remain unproven as reliable indicators of volume responsiveness in children.35,39,40 
Standard laboratory tests of coagulation have a limited ability to predict clinical bleeding.25  Thromboelastography has been purported to be a means to assess specific components of coagulation function and guide hemostatic therapy,41,42  and its use has demonstrated efficacy in CCVR.43  In our dataset, thromboelastography was utilized in only a minority of patients; this may represent an area for improvement.
Limitations
The limitations of this study include those inherent to a large multicenter clinical database analysis including inaccurate or missing data.44  For example, some complications may not have been reported, and the incidence rates presented may underestimate the true incidence for some of the variables presented. Another limitation is that, as with any observational dataset, cause and effect relationships cannot be established. Our primary intent was to assess current practices, outcomes, and complications in CCVR rather than delineate cause and effect relationships.
Future Directions
In our analysis, we identified areas of practice variability, as well as gaps between evidence-based practices and implementation of these practices. Some of these represent targets for improvement interventions within the PCCG. Specifically, we have identified the routine administration of antifibrinolytics to CCVR patients as one such intervention. As we move to expand antifibrinolytic administration, we will use the PCSPR to track administration and monitor for improved outcomes and adverse events.
Although there is robust evidence supporting transfusion thresholds in children, they were applied in just one third of our patients. We plan to pursue implementation of postoperative transfusion thresholds at PCCG institutions to improve transfusion practices, using the PCSPR to evaluate implementation and effects on transfusion outcomes.
We identified significant variability in the duration of postoperative ICU admission, a costly component of hospital care, with some centers having overnight stays and others having 3- to 4-day stays. Individual PCCG institutions may use these data to critically reevaluate whether the duration of ICU admission can be safely reduced and use the PCSPR to monitor local outcomes.
Other investigators have evaluated the relationships between craniosynostosis, corrective surgery, and neurocognitive outcomes.45,46  In the future, collaborators from the PCCG could use data from the PCSPR in conjunction with neurocognitive assessments to explore the relationships between perioperative variables, complications, and longer term outcomes in this population.
Conclusions
In this comprehensive evaluation of pediatric CCVR in North America, we identified significant variability for both perioperative management and in-hospital outcomes. In CCVR, transfusion remains the rule, especially in the youngest patients. A transfusion-free course was achieved in some cases without the use of specific blood conservation techniques, suggesting that surgical technique may be a significant determinant of blood loss. Although there were no deaths, there were numerous adverse events representing severe clinical problems. The results presented serve as a platform for future comparisons of surgical approaches and perioperative interventions. Moving forward, our goal is to leverage collaborative relationships together with the PCSPR as a tool to select, implement, and assess improvement efforts in this vulnerable pediatric population.
Acknowledgments
The authors acknowledge Ariel Vincent, B.A. (Department of Anesthesiology and Critical Care Medicine, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania; vincenta@email.chop.edu), who has managed the Pediatric Craniofacial Surgery Perioperative Registry at the Children’s Hospital of Philadelphia.
Research Support
Support was provided solely from institutional and/or departmental sources.
Competing Interests
The authors declare no competing interests.
References
Whitaker, LA, Munro, IR, Salyer, KE, Jackson, IT, Ortiz-Monasterio, F, Marchac, D Combined report of problems and complications in 793 craniofacial operations.. Plast Reconstr Surg. (1979). 64 198–203 [Article] [PubMed]
Poole, MD Complications in craniofacial surgery.. Br J Plast Surg. (1988). 41 608–13 [Article] [PubMed]
Stieg, PE, Mulliken, JB Neurosurgical complications in craniofacial surgery.. Neurosurg Clin N Am. (1991). 2 703–8 [PubMed]
Czerwinski, M, Hopper, RA, Gruss, J, Fearon, JA Major morbidity and mortality rates in craniofacial surgery: An analysis of 8101 major procedures.. Plast Reconstr Surg. (2010). 126 181–6 [PubMed]
Buntain, SG, Pabari, M Massive transfusion and hyperkalaemic cardiac arrest in craniofacial surgery in a child.. Anaesth Intensive Care. (1999). 27 530–3 [PubMed]
Ririe, DG, Lantz, PE, Glazier, SS, Argenta, LC Transfusion-related acute lung injury in an infant during craniofacial surgery.. Anesth Analg. (2005). 101 1003–6, table of contents [Article] [PubMed]
Harris, PA, Taylor, R, Thielke, R, Payne, J, Gonzalez, N, Conde, JG Research electronic data capture (REDCap)—A metadata-driven methodology and workflow process for providing translational research informatics support.. J Biomed Inform. (2009). 42 377–81 [Article] [PubMed]
Stricker, PA, Fiadjoe, JE, Davis, AR, Sussman, E, Burgess, BJ, Ciampa, B, Mendelsohn, J, Bartlett, SP, Sesok-Pizzini, DA, Jobes, DR Reconstituted blood reduces blood donor exposures in children undergoing craniofacial reconstruction surgery.. Paediatr Anaesth. (2011). 21 54–61 [Article] [PubMed]
Ali, A, Basaran, B, Yornuk, M, Altun, D, Aydoseli, A, Sencer, A, Akinci, IO Factors influencing blood loss and postoperative morbidity in children undergoing craniosynostosis surgery: A retrospective study.. Pediatr Neurosurg. (2013). 49 339–46 [Article] [PubMed]
Goobie, SM, Meier, PM, Pereira, LM, McGowan, FX, Prescilla, RP, Scharp, LA, Rogers, GF, Proctor, MR, Meara, JG, Soriano, SG, Zurakowski, D, Sethna, NF Efficacy of tranexamic acid in pediatric craniosynostosis surgery: A double-blind, placebo-controlled trial.. Anesthesiology. (2011). 114 862–71 [Article] [PubMed]
Goobie, SM, Zurakowski, D, Proctor, MR, Meara, JG, Meier, PM, Young, VJ, Rogers, GF Predictors of clinically significant postoperative events after open craniosynostosis surgery.. Anesthesiology. (2015). 122 1021–32 [Article] [PubMed]
Haas, T, Fries, D, Velik-Salchner, C, Oswald, E, Innerhofer, P Fibrinogen in craniosynostosis surgery.. Anesth Analg. (2008). 106 725–31, table of contents [Article] [PubMed]
Seruya, M, Oh, AK, Rogers, GF, Boyajian, MJ, Myseros, JS, Yaun, AL, Keating, RF Factors related to blood loss during fronto-orbital advancement.. J Craniofac Surg. (2012). 23 358–62 [Article] [PubMed]
van Uitert, A, Megens, JH, Breugem, CC, Stubenitsky, BM, Han, KS, de Graaff, JC Factors influencing blood loss and allogeneic blood transfusion practice in craniosynostosis surgery.. Paediatr Anaesth. (2011). 21 1192–7 [Article] [PubMed]
Dadure, C, Sauter, M, Bringuier, S, Bigorre, M, Raux, O, Rochette, A, Canaud, N, Capdevila, X Intraoperative tranexamic acid reduces blood transfusion in children undergoing craniosynostosis surgery: A randomized double-blind study.. Anesthesiology. (2011). 114 856–61 [Article] [PubMed]
Hsu, G, Taylor, JA, Fiadjoe, JE, Vincent, AM, Pruitt, EY, Bartlett, SP, Stricker, PA Aminocaproic acid administration is associated with reduced perioperative blood loss and transfusion in pediatric craniofacial surgery.. Acta Anaesthesiol Scand. (2016). 60 158–65 [Article] [PubMed]
Oppenheimer, AJ, Ranganathan, K, Levi, B, Strahle, JM, Kapurch, J, Muraszko, KM, Buchman, SR Minimizing transfusions in primary cranial vault remodeling: The role of aminocaproic acid.. J Craniofac Surg. (2014). 25 82–6 [Article] [PubMed]
Helfaer, MA, Carson, BS, James, CS, Gates, J, Della-Lana, D, Vander Kolk, C Increased hematocrit and decreased transfusion requirements in children given erythropoietin before undergoing craniofacial surgery.. J Neurosurg. (1998). 88 704–8 [Article] [PubMed]
Fearon, JA, Weinthal, J The use of recombinant erythropoietin in the reduction of blood transfusion rates in craniosynostosis repair in infants and children.. Plast Reconstr Surg. (2002). 109 2190–6 [Article] [PubMed]
Krajewski, K, Ashley, RK, Pung, N, Wald, S, Lazareff, J, Kawamoto, HK, Bradley, JP Successful blood conservation during craniosynostotic correction with dual therapy using procrit and cell saver.. J Craniofac Surg. (2008). 19 101–5 [Article] [PubMed]
Meneghini, L, Zadra, N, Aneloni, V, Metrangolo, S, Faggin, R, Giusti, F Erythropoietin therapy and acute preoperative normovolaemic haemodilution in infants undergoing craniosynostosis surgery.. Paediatr Anaesth. (2003). 13 392–6 [Article] [PubMed]
Hans, P, Collin, V, Bonhomme, V, Damas, F, Born, JD, Lamy, M Evaluation of acute normovolemic hemodilution for surgical repair of craniosynostosis.. J Neurosurg Anesthesiol. (2000). 12 33–6 [Article] [PubMed]
Feldman, JM, Roth, JV, Bjoraker, DG Maximum blood savings by acute normovolemic hemodilution.. Anesth Analg. (1995). 80 108–13 [PubMed]
Knuckey, MI, Wood, EM, Savoia, HF Audit of a paediatric directed donation programme.. J Paediatr Child Health. (2003). 39 364–7 [Article] [PubMed]
Goobie, SM, Haas, T Bleeding management for pediatric craniotomies and craniofacial surgery.. Paediatr Anaesth. (2014). 24 678–89 [Article] [PubMed]
Goobie, SM, Haas, T Perioperative bleeding management in pediatric patients.. Curr Opin Anaesthesiol. (2016). 29 352–8 [Article] [PubMed]
Lacroix, J, Hébert, PC, Hutchison, JS, Hume, HA, Tucci, M, Ducruet, T, Gauvin, F, Collet, JP, Toledano, BJ, Robillard, P, Joffe, A, Biarent, D, Meert, K, Peters, MJ TRIPICU Investigators; Canadian Critical Care Trials Group; Pediatric Acute Lung Injury and Sepsis Investigators Network, Transfusion strategies for patients in pediatric intensive care units.. N Engl J Med. (2007). 356 1609–19 [Article] [PubMed]
Rouette, J, Trottier, H, Ducruet, T, Beaunoyer, M, Lacroix, J, Tucci, M Canadian Critical Care Trials Group; PALISI Network, Red blood cell transfusion threshold in postsurgical pediatric intensive care patients: A randomized clinical trial.. Ann Surg. (2010). 251 421–7 [Article] [PubMed]
Steinbok, P, Heran, N, Hicdonmez, T, Cochrane, DD, Price, A Minimizing blood transfusions in the surgical correction of coronal and metopic craniosynostosis.. Childs Nerv Syst. (2004). 20 445–52 [PubMed]
Stricker, PA, Fiadjoe, JE, Kilbaugh, TJ, Pruitt, EY, Taylor, JA, Bartlett, SP, McCloskey, JJ Effect of transfusion guidelines on postoperative transfusion in children undergoing craniofacial reconstruction surgery.. Pediatr Crit Care Med. (2012). 13 e357–62 [Article] [PubMed]
Seruya, M, Sauerhammer, TM, Basci, D, Rogers, GF, Boyajian, MJ, Myseros, JS, Yaun, AL, Keating, RF, Oh, AK Analysis of routine intensive care unit admission following fronto-orbital advancement for craniosynostosis.. Plast Reconstr Surg. (2013). 131 582e–8e [Article] [PubMed]
Cladis, FP, Bykowski, M, Schmitt, E, Naran, S, Moritz, ML, Cray, J, Grunwaldt, L, Losee, J Postoperative hyponatremia following calvarial vault remodeling in craniosynostosis.. Paediatr Anaesth. (2011). 21 1020–5 [Article] [PubMed]
Stricker, PA, Cladis, FP, Fiadjoe, JE, McCloskey, JJ, Maxwell, LG Perioperative management of children undergoing craniofacial reconstruction surgery: A practice survey.. Paediatr Anaesth. (2011). 21 1026–35 [Article] [PubMed]
Stricker, PA, Lin, EE, Fiadjoe, JE, Sussman, EM, Pruitt, EY, Zhao, H, Jobes, DR Evaluation of central venous pressure monitoring in children undergoing craniofacial reconstruction surgery.. Anesth Analg. (2013). 116 411–9 [Article] [PubMed]
Gan, H, Cannesson, M, Chandler, JR, Ansermino, JM Predicting fluid responsiveness in children: A systematic review.. Anesth Analg. (2013). 117 1380–92 [Article] [PubMed]
Bhananker, SM, Ramamoorthy, C, Geiduschek, JM, Posner, KL, Domino, KB, Haberkern, CM, Campos, JS, Morray, JP Anesthesia-related cardiac arrest in children: Update from the Pediatric Perioperative Cardiac Arrest Registry.. Anesth Analg. (2007). 105 344–50 [Article] [PubMed]
Harris, MM, Yemen, TA, Davidson, A, Strafford, MA, Rowe, RW, Sanders, SP, Rockoff, MA Venous embolism during craniectomy in supine infants.. Anesthesiology. (1987). 67 816–9 [Article] [PubMed]
Faberowski, LW, Black, S, Mickle, JP Incidence of venous air embolism during craniectomy for craniosynostosis repair.. Anesthesiology. (2000). 92 20–3 [Article] [PubMed]
Pereira de Souza Neto, E, Grousson, S, Duflo, F, Ducreux, C, Joly, H, Convert, J, Mottolese, C, Dailler, F, Cannesson, M Predicting fluid responsiveness in mechanically ventilated children under general anaesthesia using dynamic parameters and transthoracic echocardiography.. Br J Anaesth. (2011). 106 856–64 [Article] [PubMed]
Durand, P, Chevret, L, Essouri, S, Haas, V, Devictor, D Respiratory variations in aortic blood flow predict fluid responsiveness in ventilated children.. Intensive Care Med. (2008). 34 888–94 [Article] [PubMed]
American Society of Anesthesiologists Task Force on Perioperative Blood Management, Practice guidelines for perioperative blood management: An updated report by the American Society of Anesthesiologists Task Force on Perioperative Blood Management.. Anesthesiology. (2015). 122 241–75 [Article] [PubMed]
Kozek-Langenecker, SA, Afshari, A, Albaladejo, P, Santullano, CA, De Robertis, E, Filipescu, DC, Fries, D, Görlinger, K, Haas, T, Imberger, G, Jacob, M, Lancé, M, Llau, J, Mallett, S, Meier, J, Rahe-Meyer, N, Samama, CM, Smith, A, Solomon, C, Van der Linden, P, Wikkelsø, AJ, Wouters, P, Wyffels, P Management of severe perioperative bleeding: Guidelines from the European Society of Anaesthesiology.. Eur J Anaesthesiol. (2013). 30 270–382 [Article] [PubMed]
Haas, T, Goobie, S, Spielmann, N, Weiss, M, Schmugge, M Improvements in patient blood management for pediatric craniosynostosis surgery using a ROTEM(®)-assisted strategy - feasibility and costs.. Paediatr Anaesth. (2014). 24 774–80 [Article] [PubMed]
Levin, PE Apples, oranges, and national databases: Commentary on an article by Daniel D. Bohl, MPH, et al.: Variations in data collection methods between national databases affect study results: A comparison of the nationwide inpatient sample and national surgical quality improvement program databases for lumbar spine fusion procedures.. J Bone Joint Surg Am. (2014). 96 e198 [Article] [PubMed]
Speltz, ML, Kapp-Simon, K, Collett, B, Keich, Y, Gaither, R, Cradock, MM, Buono, L, Cunningham, ML Neurodevelopment of infants with single-suture craniosynostosis: Presurgery comparisons with case-matched controls.. Plast Reconstr Surg. (2007). 119 1874–81 [Article] [PubMed]
Naumann, HL, Haberkern, CM, Pietila, KE, Birgfeld, CB, Starr, JR, Kapp-Simon, KA, Hopper, RA, Speltz, ML Duration of exposure to cranial vault surgery: Associations with neurodevelopment among children with single-suture craniosynostosis.. Paediatr Anaesth. (2012). 22 1053–61 [Article] [PubMed]
Appendix 1: List of Participating Institutions
  • American Family Children’s Hospital, University of Wisconsin-Madison, Madison, Wisconsin

  • Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois

  • Arkansas Children’s Hospital, Little Rock, Arkansas

  • Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts

  • Charlotte R. Bloomberg Johns Hopkins Children’s Center, Baltimore, Maryland

  • Children’s Healthcare of Atlanta at Egleston, Atlanta, Georgia

  • Children’s Hospital and Medical Center, Omaha, Nebraska

  • Children’s Hospital Colorado, Denver, Colorado

  • Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania

  • Children’s Hospital of Wisconsin, Milwaukee, Wisconsin

  • Children’s National Health System, Washington, DC

  • CHU Sainte-Justine, Montreal, Quebec, Canada

  • Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio

  • Cleveland Clinic Children’s, Cleveland, Ohio

  • Driscoll Children’s Hospital, Corpus Christi, Texas

  • Duke Children’s Hospital and Health Center, Durham, North Carolina

  • Johns Hopkins All Children’s Hospital, St. Petersburg, Florida

  • Monroe Carell Jr. Children’s Hospital at Vanderbilt, Vanderbilt University Medical Center, Nashville, Tennessee

  • Montreal Children’s Hospital, Montreal, Quebec, Canada

  • New York Presbyterian Hospital – Weill Cornell Medical College, New York, New York

  • Oregon Health & Science University, Doernbecher Children’s Hospital, Portland, Oregon

  • Seattle Children’s Hospital, Seattle, Washington, DC

  • Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas

  • The Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania

  • The University of Chicago Medicine Comer Children’s Hospital, Chicago, Illinois

  • UNC Children’s, Chapel Hill, North Carolina

  • University of California, San Diego, Rady Children’s Hospital, San Diego, California

  • University of Kansas Medical Center, Kansas City, Kansas

  • University of New Mexico, Albuquerque, New Mexico

  • University of Texas Southwestern and Children’s Medical Center, Dallas, Texas

  • UT Health-University of Texas Medical School at Houston, Houston, Texas

Appendix 2: Pediatric Craniofacial Collaborative Group (June 2012 to September 2015)
John Fiadjoe, M.D., Codruta Soneru, M.D., Ricardo Falcon, M.D., Timothy Petersen, Courtney Kowalczyk-Derderian, M.D., Nicholas Dalesio, M.D., Stefan Budac, M.D., Neels Groenewald, M.D., Daniel Rubens, M.D., Douglas Thompson, M.D., Rheana Watts, Katherine Gentry, M.D., Iskra Ivanova, M.D., Mali Hetmaniuk, M.D., Vincent Hsieh, M.D., Michael Collins, M.D., Karen Wong, M.D., Wendy Binstock, M.D., Russell Reid, M.D., Kim Poteet-Schwartz, M.D., Heike Gries, M.D., Rebecca Hall, M.D., Jeffrey Koh, M.D., Carolyn Bannister, M.D., Wai Sung, M.D., Ranu Jain, M.B.B.S., Allison Fernandez, M.D., Gerald F. Tuite, M.D., Ernesto Ruas, M.D., Oleg Drozhinin, M.D., Lisa Tetreault, R.N., B.S.N., C.C.R.P., Bridget Muldowney, M.D., Karene Ricketts, M.D., Patrick Fernandez, M.D., Lisa Sohn, M.D., John Hajduk, Brad Taicher, D.O., M.B.A., Jessica Burkhart, Allison Wright, Jane Kugler, M.D., Lea Barajas-DeLoa, Meera Gangadharan, M.D., Veronica Busso, M.D., Kayla Stallworth, Susan Staudt, M.D., Kristen L. Labovsky, M.D., Chris D. Glover, M.D., Henry Huang, M.D., Helena Karlberg-Hippard, M.D., Samantha Capehart, R.N., Cynthia Streckfus, Kim-Phuong T. Nguyen, M.D., Peter Manyang, Jose Luis Martinez, M.D., Jennifer K. Hansen, M.D., Heather Mitzel Levy, D.O., Alyssa Brzenski, M.D., Franklin Chiao, M.D., Pablo Ingelmo, M.D., Razaz Mujallid, Olutoyin A. Olutoye, M.D., Tariq Syed, M.S., Hubert Benzon, M.D., Adrian Bosenberg, M.B. Ch.B.
Fig. 1.
Box plots showing perioperative blood product transfusion (ml/kg) in the infant group (age less than or equal to 24 months) by institution. Perioperative blood products include packed red erythrocytes, whole blood, reconstituted blood, fresh frozen plasma, platelets, and cryoprecipitate administered intraoperatively and in the first postoperative 48 h. Institutions except 10, 22, 23, 24, 27, and 30 had at least six cases.
Box plots showing perioperative blood product transfusion (ml/kg) in the infant group (age less than or equal to 24 months) by institution. Perioperative blood products include packed red erythrocytes, whole blood, reconstituted blood, fresh frozen plasma, platelets, and cryoprecipitate administered intraoperatively and in the first postoperative 48 h. Institutions except 10, 22, 23, 24, 27, and 30 had at least six cases.
Fig. 1.
Box plots showing perioperative blood product transfusion (ml/kg) in the infant group (age less than or equal to 24 months) by institution. Perioperative blood products include packed red erythrocytes, whole blood, reconstituted blood, fresh frozen plasma, platelets, and cryoprecipitate administered intraoperatively and in the first postoperative 48 h. Institutions except 10, 22, 23, 24, 27, and 30 had at least six cases.
×
Table 1.
Patient Demographic Data
Patient Demographic Data×
Patient Demographic Data
Table 1.
Patient Demographic Data
Patient Demographic Data×
×
Table 2.
Selected Perioperative Outcomes*
Selected Perioperative Outcomes*×
Selected Perioperative Outcomes*
Table 2.
Selected Perioperative Outcomes*
Selected Perioperative Outcomes*×
×
Table 3.
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)×
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)
Table 3.
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)
Reported Adverse Events, Complications, and Outlier Outcomes (All Ages)×
×
Table 4.
Anesthetic/Intraoperative Management
Anesthetic/Intraoperative Management×
Anesthetic/Intraoperative Management
Table 4.
Anesthetic/Intraoperative Management
Anesthetic/Intraoperative Management×
×
Table 5.
Perioperative Fluids and Transfusion*
Perioperative Fluids and Transfusion*×
Perioperative Fluids and Transfusion*
Table 5.
Perioperative Fluids and Transfusion*
Perioperative Fluids and Transfusion*×
×
Table 6.
Blood Conservation Technique and Transfusion-free Patient Data
Blood Conservation Technique and Transfusion-free Patient Data×
Blood Conservation Technique and Transfusion-free Patient Data
Table 6.
Blood Conservation Technique and Transfusion-free Patient Data
Blood Conservation Technique and Transfusion-free Patient Data×
×