Craniosynostosis With Preoperative Iron Supplementation Exposure: A Retrospective Cohort Study Examining Preoperative Iron Supplementation and Transfusion Practice in a National Paediatric Craniofacial Centre.

BACKGROUND: Blood loss and subsequent transfusion are key concerns in the surgical management of craniosynostosis, and have been associated with increased morbidity, requirement for intensive care admission and increased length of hospital stay. Patient blood management guidelines advocate treatment of anemia before elective surgical procedures where significant blood loss is anticipated. At present there is little evidence in the literature investigating the clinical value of this practice in pediatric craniofacial surgery. AIMS: The authors examined the effect of preoperative oral iron supplementation on blood loss and transfusion rates in a national pediatric craniofacial unit. METHODS: A total of 157 patients were included in a retrospective and prospective observational cohort study conducted between July 2011 and November 2016. Eighty-five (85) patients included in the preoperative iron supplementation group were prescribed oral ferrous fumarate before total cranial vault reconstruction, frontal-orbital advancement or extended strip cranial vault remodeling procedures. This cohort was retrospectively compared to seventy-two (72) consecutive patients who did not receive iron supplementation. RESULTS: Calculated blood loss was 51.3 mL/kg in the intervention group, and 56.65 mL/kg in the control group. Transfusion rate and mean volumes for the intervention group were 85.9% and 25 mL/kg. The control group had transfusion rate of 86.1% with mean transfused volume of 24.7 mL/kg. These differences were not statistically significant. Intraoperative tranexamic acid was associated with significantly reduced transfusion volumes overall. CONCLUSIONS: This study did not show a statistically significant benefit to preoperative iron supplementation. Secondary outcomes of this study showed a statistically significant difference in estimated versus calculated intraoperative blood loss. Further research in to specific iron supplementation protocols is indicated.

Classification of Subtypes of Crouzon Syndrome Based on the Type of Vault Suture Synostosis.

BACKGROUND: Patients with Crouzon syndrome develop various types of anatomic deformities due to different forms of craniosynostosis, yet they have similar craniofacial characteristics. However, exact homology is not evident. Different pathology then may be best treated by different forms of surgical technique. Therefore, precise classification of Crouzon syndrome, based on individual patterns of cranial suture involvement is needed. METHODS: Ninety-five computed tomography (CT) scans (Crouzon, n = 33; control, n = 62) were included in this study. All the CT scans are divided into 4 types based on premature closure of sutures: class I = coronal and lambdoidal synostosis; class II = sagittal synostosis; class III = pansynostosis; and class IV = "Others." The CT scan anatomy was measured by Materialise software. RESULTS: The class III, pansynostosis, is the most prevalent (63.6%). The classes I, III, and IV of Crouzon have significantly shortened entire anteroposterior cranial base length, with the shortest base length in class III. The external cranial measurements in class I show primarily a decreased posterior facial skeleton, while the class III presented with holistic facial skeleton reduction. Class II has the least severe craniofacial malformations, while class III had the most severe. CONCLUSION: The morphology of patients with Crouzon syndrome is not identical in both cranial base and facial characteristics, especially when they associated with different subtypes of cranial suture synostosis. The classification of Crouzon syndrome proposed in this study, summarizes the differences among each subgroup of craniosynostosis suture involvement, which, theoretically, may ultimately influence both the timing and type of surgical intervention.

Post-Surgical Relapse in Metopic Synostosis and Limitations of the Interfrontal Angle as an Outcome Measure.

BACKGROUND: The interfrontal angle (IFA) has been utilized as a surrogate for morphologic assessment of the anterior cranial region in metopic synostosis with success in characterizing presenting severity. Its utility as an outcome measure has not been assessed similarly. In this study, we aimed to determine whether meaningful information relative to shape, and relapse in particular, could be assessed using the IFA. METHODS: Patients with operatively treated isolated metopic synostosis were identified and included based on the availability of "previously obtained" low-dose computerized tomography (CT) scans at (1) preoperative and 2 postoperative (2) postoperative day-3 and (3) 1 year time points. Relapse was calculated as the percentage of the operatively induced change in IFA that was retained after the first postoperative year. RESULTS: After exclusionary criteria, 19 patients were identified. The mean IFA values at the 3 time points were 119.3° (±9.8), 139.6° (±6.3), and 135.3° (±7.1), chronologically. Of the 19 patients, 14 (73.7%) experienced relapse, with this subgroup averaging 62.4% retention of their surgical change after 1 year. Increased preoperative severity and delayed age at surgical intervention were concurrently associated with an increased occurrence of relapse (P = 0.011). Another model utilizes preoperative IFA to predict the change in IFA that would accompany surgery on a case-by-case basis (P < 0.0001). CONCLUSIONS: (1) Fronto-orbital advancement offers immediate correction in the frontal bandeau. (2) Surgical impact, using fronto-orbital advancement, can be predicted using preoperative severity. (3) A component of postoperative relapse occurs within the frontal bandeau and is detectable at 1 year via the IFA. Exclusively, the IFA cannot characterize holistic postoperative relapse due to its inherent anatomical constraints; this supports the cessation of routine postoperative computerized tomography scans for determining outcomes.

[Guide to clinical practice for the diagnosis, treatment and rehabilitation of non-syndromic craniosynostosis on 3 levels of care]. / Guía de práctica clínica para el diagnóstico, tratamiento y rehabilitación de craneosinostosis no sindrómica en los 3 niveles de atención.

INTRODUCTION: Craniosynostosis is a congenital anomaly resulting from the premature fusion of the cranial sutures changing growth patterns of the skull. METHODOLOGY: Focus, scope, target population and clinical questions to be solved were defined. A systematic search for evidence in different databases (Medline, Embase, KoreaMed, Cochrane Library and the website of the World Health Organization) in stages was performed: clinical practice guidelines; systematic reviews, and clinical trials and observational studies; using MeSH, Decs and corresponding free terms, unrestricted language or temporality. Risk of bias was evaluated using appropriate tools (AMSTAR, Risk of Bias or STROBE). The quality of evidence was graduated using the GRADE system. Modified Delphi Panel technique was used to assign the recommendation's strength and direction, as well as the degree of agreement with it, taking into account the quality of evidence, balance between risks and benefits of interventions, values and preferences of patients and availability of resources. RESULTS: There were 3,712 documents obtained by search algorithms; selecting 29 documents for inclusion in the qualitative synthesis. Due to heterogeneity between studies, it was not possible to perform meta-analysis. CONCLUSIONS: We issued 7 recommendations and 8 good practice points, which will serve as support for decision-making in the comprehensive care of patients with craniosynostosis.

Minimizing transfusion requirements for children undergoing craniosynostosis repair: the CHoR protocol.

OBJECT: Children with craniosynostosis may require cranial vault remodeling to prevent or relieve elevated intracranial pressure and to correct the underlying craniofacial abnormalities. The procedure is typically associated with significant blood loss and high transfusion rates. The risks associated with transfusions are well documented and include transmission of infectious agents, bacterial contamination, acute hemolytic reactions, transfusion-related lung injury, and transfusion-related immune modulation. This study presents the Children's Hospital of Richmond (CHoR) protocol, which was developed to reduce the rate of blood transfusion in infants undergoing primary craniosynostosis repair. METHODS: A retrospective chart review of pediatric patients treated between January 2003 and Febuary 2012 was performed. The CHoR protocol was instituted in November 2008, with the following 3 components; 1) the use of preoperative erythropoietin and iron therapy, 2) the use of an intraoperative blood recycling device, and 3) acceptance of a lower level of hemoglobin as a trigger for transfusion (< 7 g/dl). Patients who underwent surgery prior to the protocol implementation served as controls. RESULTS: A total of 60 children were included in the study, 32 of whom were treated with the CHoR protocol. The control (C) and protocol (P) groups were comparable with respect to patient age (7 vs 8.4 months, p = 0.145). Recombinant erythropoietin effectively raised the mean preoperative hemoglobin level in the P group (12 vs 9.7 g/dl, p < 0.001). Although adoption of more aggressive surgical vault remodeling in 2008 resulted in a higher estimated blood loss (212 vs 114.5 ml, p = 0.004) and length of surgery (4 vs 2.8 hours, p < 0.001), transfusion was performed in significantly fewer cases in the P group (56% vs 96%, p < 0.001). The mean length of stay in the hospital was shorter for the P group (2.6 vs 3.4 days, p < 0.001). CONCLUSIONS: A protocol that includes preoperative administration of recombinant erythropoietin, intraoperative autologous blood recycling, and accepting a lower transfusion trigger significantly decreased transfusion utilization (p < 0.001). A decreased length of stay (p < 0.001) was seen, although the authors did not investigate whether composite transfusion complication reductions led to better outcomes.

Impact of supplemental vitamin K1 administration on postoperative blood component requirements after craniosynostosis repair: a prospective, placebo-controlled, randomized, blinded study.

Total cranial vault craniosynostosis repairs often require additional blood transfusions in the intensive care unit. Vitamin K1 participates in hepatic production of procoagulant proteins, and body stores of vitamin K1 are limited and dietary dependent. Surgical stress and diet interference may place infants at risk for vitamin K deficiency. Through design of a surgically stratified, randomized, placebo-controlled, blinded pilot study, we evaluated impact of vitamin K1 supplementation on coagulation parameters in infants after craniosynostosis repair. Patients received intramuscular vitamin K1 or placebo coincident with surgical incision. Serum vitamin K1 levels, protein induced in vitamin K absence-prothrombin, and factor VII were obtained at predetermined intervals after surgery. Patients received blood products in the intensive care unit in accordance with transfusion thresholds. Fifteen patients (vitamin K1 = 6, placebo = 9) completed the study procedures. Despite group assignment, patients received an average of 3 postoperative transfusions. Variations were observed with respect to intraoperative resuscitation of patients between comparably trained pediatric anesthesiologists. Thirty-three percent of patients were vitamin K1 deficient on 1 or more laboratory specimens. All breast-fed patients became deficient. Compared with placebo, elevated serum vitamin K1 levels at 6, 12, and 24 hours in the active drug group (P < 0.0001) were not associated with increased factor VII levels or reduced need for postoperative blood products. However, lack of a standardized intraoperative resuscitation plan may contribute to postoperative coagulopathy and is a major study limitation.

Does postoperative 'M' technique massage with or without mandarin oil reduce infants' distress after major craniofacial surgery?

AIM: This article is a report of a randomized controlled trial of the effects of 'M' technique massage with or without mandarin oil compared to standard postoperative care on infants' levels of pain and distress, heart rate and mean arterial pressure after major craniofacial surgery. BACKGROUND: There is a growing interest in non-pharmacological interventions such as aromatherapy massage in hospitalized children to relieve pain and distress but well performed studies are lacking. METHODS: This randomized controlled trial allocated 60 children aged 3-36 months after craniofacial surgery from January 2008 to August 2009 to one of three conditions; 'M' technique massage with carrier oil, 'M' technique massage with mandarin oil or standard postoperative care. Primary outcome measures were changes in COMFORT behaviour scores, Numeric Rating Scale pain and Numeric Rating Scale distress scores assessed from videotape by an observer blinded for the condition. RESULTS: In all three groups, the mean postintervention COMFORT behaviour scores were higher than the baseline scores, but differences were not statistically significant. Heart rate and mean arterial pressure showed a statistically significant change across the three assessment periods in all three groups. These changes were not related with the intervention. CONCLUSIONS: Results do not support a benefit of 'M' technique massage with or without mandarin oil in these young postoperative patients. Several reasons may account for this: massage given too soon after general anaesthesia, young patients' fear of strangers touching them, patients not used to massage.

Factors influencing blood loss and allogeneic blood transfusion practice in craniosynostosis surgery.

OBJECTIVE/AIMS: To identify factors influencing perioperative blood loss and transfusion practice in craniosynostotic corrections. BACKGROUND: Craniosynostotic corrections are associated with large amounts of blood loss and high transfusion rates. METHODS: A retrospective analysis was performed of all pediatric craniosynostotic corrections during the period from January 2003 to October 2009. The primary endpoint was the receipt of an allogeneic blood transfusion (ABT) during or after surgery. Pre-, intra-, and postoperative data were acquired using the electronic hospital registration systems and patients' charts. RESULTS: Forty-four patients were operated using open surgical techniques. The mean estimated blood loss during surgery was 55 ml·kg(-1). In 42 patients, red blood cells were administered during or after surgery with a mean of 38 ml·kg(-1). In 23 patients, fresh frozen plasma was administered with a mean of 28 ml·kg(-1). A median of two different donors per recipient was found. Longer duration of surgery and lower bodyweight were associated with significantly more blood loss and red blood cell transfusions. Higher perioperative blood loss and surgery at an early age were correlated with a longer duration of admission. CONCLUSIONS: In this study, craniosynostotic corrections were associated with large amounts of blood loss and high ABT rates. The amount of ABT could possibly be reduced by appointing a dedicated team of physicians, by using new less-invasive surgical techniques, and by adjusting anesthetic techniques.

Predictors of blood loss in fronto-orbital advancement and remodeling.

Fronto-orbital advancement and remodeling for craniosynostosis is extensive surgery and is associated with potential risks; the most significant of these is blood loss. We prospectively studied 116 consecutive patients undergoing fronto-orbital advancement by the same surgical team for a 5-year 6-month period to determine what factors are associated with blood loss and transfusion of blood products. The data collected on the calvarial sutures involved were whether the patient had a diagnosed syndrome, the age at operation, the length of the operation, the estimated blood volume lost during the perioperative course, the number of units of packed cells transfused (donor exposures), and the use of other blood products. The mean (SD) total blood volume lost was 116% (5.4) of the estimated preoperative volume. The median number of whole units of packed cells transfused was 2 units. Other blood products were given in 28% of the cases. There was significantly greater blood loss in those patients with recognized craniofacial syndromes, pansynostosis, an operating time longer than 5 hours, and an age of 18 months or younger at operation. The use of other blood products was associated with those patients losing a blood volume higher than the mean.

Successful blood conservation during craniosynostotic correction with dual therapy using procrit and cell saver.

BACKGROUND: Craniosynostotic correction typically performed around infant physiologic nadir of hemoglobin (approximately 3-6 months of age) is associated with high transfusion rates of packed red blood cells and other blood products. As a blood conserving strategy, we studied the use of 1) recombinant human erythropoietin or Procrit (to optimize preoperative hematocrit) and 2) Cell Saver (to recycle the slow, constant ooze of blood during the prolonged case). METHODS: UCLA Patients with craniosynostosis from 2003-2005 were divided into 1) the study group (Procrit and Cell Saver) or 2) the control group (n = 79). The study group 1) received recombinant human erythropoietin at 3 weeks, 2 weeks, and 1 week preoperatively and 2) used Cell Saver intraoperatively. Outcomes were based on morbidities and transfusion rate comparisons. RESULTS: The 2 groups were comparable with regards to age (5.66 and 5.71 months), and operative times (3.11 vs 2.59 hours). In the study group there was a marked increase in preoperative hematocrit (56.2%). The study group had significantly lower transfusions rates (5% vs 100% control group) and lower volumes transfused than in the control group (0.05 pediatric units vs 1.74 pediatric units). Additionally, of the 80% of patients in the study group who received Cell Saver blood at the end of the case, approximately 31% would have needed a transfusion if the recycled blood were unavailable. CONCLUSION: Our data showed that for elective craniosynostotic correction, successful blood conserving dual therapy with Procrit and Cell Saver might be used to decrease transfusion rates and the need for any blood products.

Reducing allogenic blood transfusions during pediatric cranial vault surgical procedures: a prospective analysis of blood recycling.

Almost all patients who undergo major craniosynostosis corrections receive allogenic blood transfusions. This study of intraoperative blood salvage was undertaken in an attempt to further reduce the need for blood transfusions and to enhance the safety of these complex procedures. This prospective nonrandomized series included 60 consecutive children undergoing major cranial vault remodeling, primarily for treatment of craniosynostosis (single-suture and syndromic). A single craniofacial surgeon performed all operations, using a cell-saver equipped with a 55-cc pediatric bowl. The average age of the patients in this series was 4 years (33 of 60 patients were less than 18 months of age), and the average length of the surgical procedure was 196 minutes. Fifty-three percent were primary procedures and 47 percent were secondary. The mean estimated blood loss was 356 cc (19 cc/kg, or 28.5 percent of the estimated total blood volume). An average of 110 cc of cell-saver blood (range, 5 to 900 cc), or 7.8 percent of the patient's estimated total blood volume (approximately 15 percent, accounting for hemoconcentration of the cell-saver blood), was recycled for transfusion. No statistically significant factors (primary versus secondary procedure, diagnosis, age, or weight) were identified as predictive indicators for the use of this technology. Although 59 of 60 patients received transfusions, only 18 (30 percent) received allogenic blood (average, approximately 140 cc). There were no complications associated with the use of the cell-saver device. Use of the cell-saver during major craniosynostosis repair seemed to be safe and was associated with an extremely low allogenic blood transfusion rate.

Comparison of perioperative blood salvage and postoperative reinfusion of drained blood during surgical correction of craniosynostosis in infants.

BACKGROUND: The surgical correction of craniosynostosis may be associated with extensive blood loss and transfusion. The aim of this study was to compare the efficacy of the perioperative use of the continuous autotransfusion system (CATS group) and of the postoperative use of the CBCII ConstaVac(R) system (CV group) to reduce homologous transfusion in infants during repair of craniosynostosis. METHODS: Two groups of consecutive infants, weighing <10 kg, and scheduled for the surgical correction of craniosynostosis, were compared retrospectively according to the blood salvaging system used: CATS group and CV group. The primary endpoint was the comparison of the total volume of homologous blood transfused. RESULTS: There was no significant difference between the CV (n = 69) and the CATS (n = 135) groups with regard to physical, preoperative and postoperative data, except for the type of craniosysnostosis with more scaphocephaly in the CV group (P = 0.03). No significant difference in blood loss and homologous transfusion was observed between the two groups during the perioperative period. Two subgroups of patients were also compared: a subgroup including patients operated on with a linear craniectomy for scaphocephaly, and a subgroup including all other patients. There was also no significant difference in blood loss and homologous transfusion between the CV and the CATS groups for these two subgroups of patients. CONCLUSION: Our results suggest that the postoperative use of the CBCII ConstaVac(R) system is as efficient as the perioperative use of the CATS(R) system for reducing homologous blood transfusion during repair of craniosynostosis in infants weighing <10 kg.

The use of recombinant erythropoietin in the reduction of blood transfusion rates in craniosynostosis repair in infants and children.

The vast majority of infants and children undergoing craniosynostosis correction receive a blood transfusion. The risks of blood transfusion include, but are not limited to, acute hemolytic reactions (approximately 1 of 250,000), human immunodeficiency virus (approximately 1 of 200,000), hepatitis B and C (approximately 1 of 30,000 each), and transfusion-related lung injuries (approximately 1 of 5000). This prospective, single-blinded, randomized study was undertaken to examine the safety and efficacy of preoperative single weekly dosing with erythropoietin (epoetin alfa) in reducing the rate of transfusion in infants and small children undergoing craniosynostosis repair. A total of 29 patients (<8 years) undergoing craniosynostosis repair were randomized into two groups: one received preoperative erythropoietin (600 U/kg) weekly for 3 weeks, and the other served as a control. All caregivers responsible for blood transfusions were blinded, and strict criteria for transfusion were established. A pediatric hematologist monitored both groups, and all patients received supplemental iron (4 mg/kg). Fourteen patients were randomized to receive erythropoietin, and eight of these 14 patients (57 percent) required transfusion (mean age, 17 months; mean weight, 10.1 kg). Of the six patients not requiring transfusion, three were younger than 12 months old (mean, 6 months). Fourteen of 15 patients (93 percent) in the control group (mean age, 13 months; mean weight, 9.3 kg) required a blood transfusion during the study. The only control patient not requiring transfusion was the eldest (5 years old). The difference between the two groups was statistically significant (Fisher's exact test = 0.03). The control group showed no change in hemoglobin levels from baseline to preoperative levels, but the erythropoietin group increased their average hemoglobin levels from 12.1 to 13.1 g/dl. There were no adverse effects noted among children receiving erythropoietin, nor were there any surgical complications. The authors conclude that the preoperative administration of erythropoietin significantly raised hemoglobin levels and reduced the need for a blood transfusion with craniosynostosis correction. More suggestions are made that may further reduce the need for blood transfusions, and a cost-benefit analysis is discussed.

The use of intraoperative autotransfusion during cranial vault remodeling for craniosynostosis.

Intraoperative autotransfusion salvages blood shed during surgery for use in immediate resuscitation of the patient. The purpose of this study was to determine whether such autotransfusion decreases the volume of homologous blood transfused in patients undergoing primary cranial vault remodeling for craniosynostosis. The Cobe-Bret 2 autologous blood recovery system (Hemo Concepts, Union, N.J.) was used in 11 cases, and an equal number of consecutive cases did not receive intraoperative autotransfusion. There were no significant differences between the groups with respect to age, sex, and weight. Mean estimated blood loss was 43.2 ml/kg (range, 20.3 to 65.0 ml/kg) in the intraoperative autotransfusion group and 40.2 ml/kg (range, 6.8 to 72.3 ml/kg) in the control group (not statistically significant; p < 0.05). There was no significant difference in volume of homologous blood transfusion between the two groups. The autotransfusion group received 34.1 ml/kg of homologous blood (range, 0 to 60.7 ml/kg), and the control group received a mean of 32.7 ml/kg (range, 14.5 to 60.2 ml/kg). The autotransfusion group received a mean of 10.4 ml/kg of recovered autologous blood (range, 0 to 21.4 ml/kg). In four of the 11 autotransfusion patients, insufficient autologous blood was recovered intraoperatively to warrant transfusion. Results of this study suggest little benefit for the use of intraoperative autotransfusion in primary cranial vault remodeling for craniosynostosis in the young patient. It was hypothesized that this finding was a result of the following: (1) intraoperative autotransfusion blood was usually available only toward the end of the procedure, after homologous blood had already been administered, and (2) the volume of recovered intraoperative autotransfusion blood is minimal, compared with the homologous transfusion volume requirements during an extensive cranial vault remodeling and fronto-orbital advancement procedure. In the context of unproven cost benefit and increasing similar evidence from other comparative studies, emphasis should be directed to other medical and surgical strategies to minimize the need for perioperative blood transfusion.

Surgical program for craniosynostosis using three-dimensional solid model, combined with autologous blood transfusion in a Japanese craniofacial unit.

Since April 1997 at St. Mary's Craniofacial Unit, simulated surgery using a three-dimensional solid model made preoperatively was carried out, which enable sufficient autologous blood banking before the surgery. This study was conducted in eight patients presenting with plagiocephaly, brachycephaly, and oxycephaly consisting of simple craniosynostosis and syndromic craniosynostosis. Four cases (numbers 1-4) conducted with simulated surgery using three-dimensional solid models and fronto-orbital advancement with reshaping, as well as autologous blood transfusion were selected for the later-term group. For the first-term group, four cases (numbers 5-8) that were performed before the introduction of the abovementioned treatment were selected. The later-term group compared with the first-term group showed a decrease in operating time by an average of 1 hour, blood loss during surgery by 90 ml, and one fifth the amount of homologous blood transfusion. Also, 2 cases in the later-term group (cases 3 and 4) were able to avoid homologous blood transfusion, and they had autologous blood transfusion of 30 to 33 ml/kg. The surgical techniques will be improved with repeated cases of simulated surgery, aiming for the minimum invasive surgery. Also, the use of autologous blood transfusion is expected to increase as a less invasive surgery.