Craniosynostosis Repair Research Articles

Impact of Supplemental Vitamin K1 Administration on Postoperative Blood Component Requirements After Craniosynostosis Repair

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.

Improvements in patient blood management for pediatric craniosynostosis surgery using a ROTEM®‐assisted strategy – feasibility and costs

BackgroundModerate to severe intraoperative bleeding and the presence of acquired coagulopathy remain serious problems in the management of major pediatric craniosynostosis surgery. After implementation of a ROTEM®-assisted patient blood management (PBM) strategy, using primarily purified coagulation factor concentrates, feasibility and costs of this new regimen were analyzed.MethodsRetrospective analysis of all consecutive children who underwent primary elective major craniofacial surgery for craniosynostosis repair was carried out at the Children's University Hospital, Zurich, between 2007 and 2013. Laboratory workup and transfusion requirements were compared.ResultsA total of 47 children (36 in the historic group and 11 after implementation of PBM) were analyzed. Although all patients in this study needed transfusion of red blood cell concentrates, there was a total avoidance of perioperative transfusion of fresh frozen plasma and a reduction in transfused platelets (one of nine children vs nine of 36 children in the historic group) after implementation of the PBM strategy. Based on a predefined ROTEM® threshold in the PBM group (FibTEM MCF <8 mm), administration of fibrinogen concentrate was necessary in all of these children. The mean total costs per patient consisting of transfused allogeneic blood products and coagulation factor concentrates were reduced by 17.1% after implementation of PBM (1071.82 EUR per patient before vs 888.93 EUR after implementation).ConclusionsThe implementation of a ROTEM®-assisted PBM is feasible and is associated with a considerable reduction in intraoperative transfusion requirements and thereby a decrease in transfusion-related direct costs.

The use of 3D stereophotogrammetry in the post-operative treatment and evaluation of endoscopic assisted repair of craniosynostosis

Background and objectives: Craniosynostosis is a congenital disorder in which one or more cranial sutures are closed prematurely. Therapy consists of an endoscopic assisted repair of craniosynostosis and postoperative helmet remoulding therapy. The goal of this study was to develop a new method to evaluate the therapy of craniosynostosis.

124 Symptomatic Recurrence Following the Open Surgical Repair of Nonsyndromic Craniosynostosis

124 Symptomatic Recurrence Following the Open Surgical Repair of Nonsyndromic Craniosynostosis

Simple and safe head positioning device for repair of craniosynostosis with the patient prone

m a roper positioning of the head is key in the operative corection of craniosynostosis. If the patient is prone it provides n overview of the skull and allows access from the supraorital margin to the external occipital protuberance. Because f the generous view provided by this position, the placeent and shaping of the bony fragments can be ideal, which eads to excellent aesthetic results. However, only a few of he available positioning devices are acceptable. In this paper we describe a simple and economical ethacrylate holder covered with a gel mat (Action Fracure Table Hip Pad; Allenspach Medical AG, Balsthal, witzerland) taped onto the operating table to place young atients prone for the surgical correction of craniosynostosis. The diagnosis, management, and treatment of craniosynstosis can be complex, and an interdisciplinary approach is ssential.1 Powiertowski et al.2 first described subtotal cranoplasty, and several more extensive procedures have been escribed subsequently.3,4 Surgical treatment can be achieved safely by keeping the atient prone.5 The use of a vacuum-stiffened beanbag was escribed for this in 1989,5 but since then, no new, more perorated device has been described. In this paper we describe he use of a methacrylate holder to allow safe and simple xation of the skull during repair of craniosynostosis. A dental technician constructed the positioning device, hich is composed of polymerised methacrylate, after taking

Venous air embolism from Tisseel use during endoscopic cranial vault remodeling for craniosynostosis repair: a case report

Venous air embolism (VAE) is a potential complication during cranial vault remodeling requiring early detection and prompt therapeutic intervention. The incidence of VAE has been reported to be as high as 82.6% during open craniectomy for craniosynostosis repair. On the other hand, two separate studies reported a much lower incidence of VAE (8% and 2%) during endoscopic strip craniectomy. As surgical advancements progress, minimally invasive neurosurgical procedures are increasing in the pediatric population with reported benefits of decreased blood loss and need for transfusion, shorter hospital stay, decreased cost, lower morbidity, and mortality. In addition, there is a heightened emphasis on achieving hemostasis, which has led to the use of products such as antifibrinolytics and fibrin sealants. We present a case where a VAE causing significant hemodynamic instability (grade III) ensued immediately following aerosolized fibrin sealant application. Exploration of the potential source of VAE pointed to the high pressure and close proximity (between spray device and tissue) during application of the sealant, likely forcing air into the vascular system.

Pancraniosynostosis following endoscope-assisted strip craniectomy and helmet orthosis for sagittal suture craniosynostosis in a nonsyndromic patient

A variety of surgical strategies are used to correct sagittal craniosynostosis. There is increasing experience with the use of endoscope-assisted techniques, although there is no consensus regarding the optimal technique. Pancraniosynostosis occurring after the surgical repair of single-suture craniosynostosis is an unusual complication. The authors describe the case of a nonsyndromic patient who underwent an endoscope-assisted strip craniectomy with the subsequent use of a helmet orthosis for correction of an isolated sagittal suture craniosynostosis. The patient's early postoperative course was uneventful. Pancraniosynostosis subsequently developed, requiring much more extensive surgical correction. Awareness of this potential complication is necessary to ensure its recognition and appropriate management.

Age at Initial Consultation for Craniosynostosis

The severity and dysmorphology that results from the premature fusion of one or more cranial sutures is not uniform. Less striking phenotypes may be more easily missed on routine screening, possibly leading to delayed diagnosis and treatment. The purpose of this study was to compare the age at initial presentation for the different forms of craniosynostosis. The authors reviewed the records of all patients who underwent open craniofacial repair of craniosynostosis at a single institution from 1996 to 2009. Relationships between type of suture fusion and age at initial consultation were compared. Two hundred eleven patients (136 males, 75 females) were identified. Indications included sagittal (n = 96), metopic (n = 39), unicoronal (n = 33), bicoronal (n = 24), multisutural (n = 15), bilambdoidal (n = 3), and unilambdoidal (n = 1) synostoses. Seventeen patients (8.1%) had a craniosynostosis syndrome and 5 (2.4%) had a syndrome or disorder not typically associated with craniosynostosis [X-linked hypophosphatemic rickets (n = 3), achondroplasia (n = 1), and Beckwith Wiedemann (n = 1)]. Median age at initial consultation was 4.1 months; there was no gender difference. Patients with X-linked hypophosphatemic rickets presented at a significantly older age than nonsyndromic patients or those with a known craniosynostosis syndrome. Those with multisutural synostosis presented at a significantly older age than patients with sagittal or bicoronal synostosis. Patients with multisutural involvement or X-linked hypophosphatemic rickets had a significant delay in presentation for craniosynostosis. The latter group of patients may especially benefit from routine surveillance for craniosynostosis given their advanced age at diagnosis.

Endoscopic-assisted treatment of trigonocephaly

Minimally invasive, endoscopic repair of metopic craniosynostosis has emerged as a potentially efficacious, safe, and aesthetically acceptable alternative to open procedures. Potential advantages of an early endoscopic approach to repair metopic craniosynostosis include a reduction in blood loss and consequent decreases in transfusion volumes, decreased hospital costs, shorter operative times, and limited duration of hospitalization. Other benefits of minimally invasive techniques would be avoidance of anaesthetic surgical scarring, decrease in postoperative swelling and discomfort, and lower rate of complications such as duramater tears, postoperative hyperthermia, or infection. However, a concern is usually raised about the achievements of the "endoscopic" techniques when compared to "standard" open approaches. The indications for endoscopic-assisted surgery in the treatment of trigonocephaly remain controversial and further series and follow-up of these patients are necessary to set up the role of these approaches.

Multicenter Study of Neurodevelopment in 3-Year-Old Children With and Without Single-Suture Craniosynostosis

To evaluate the hypothesis that 3-year-old children with single-suture craniosynostosis would receive lower neurodevelopmental scores than a comparable group of children born with patent sutures. Longitudinal comparison study. Five tertiary care craniofacial centers. Patients with craniosynostosis (cases) and a comparison group of children without craniosynostosis(controls). Patients diagnosed with single-suture craniosynostosis from 2002 to 2006 were eligible as cases.Controls were frequency-matched to cases on age, sex, race, socioeconomic status, and study site. Craniosynostosis. We administered the Bayley Scales of Infant Development, Second Edition, mental and motor development indices and the Preschool Language Scales, Third Edition, receptive and expressive communication scales. Children were evaluated at baseline (before surgery in cases and at a similar age in controls)and at 18 and 36 months of age. We compared the groups' performances at 36 months by fitting adjusted linear and logistic regression models. We also estimated adjusted associations between age at surgery and neurodevelopmental scores. Adjusted mean case deficits ranged from 3 to 6 points (P≤ .008 for all comparisons). Compared with controls, the odds of cases being delayed ranged from 1.5 to 2.0, depending on the neurodevelopmental scale (P values ranged from .03 to .09). Cases' ages at craniosynostosis repair were not strongly related to neurodevelopmental performance. In this large, carefully controlled, multicenter study, we observed consistently lower mean neurodevelopmental scores in children with single-suture craniosynostosis compared with controls. These results provide further support for neurodevelopmental screening in young children with single-suture craniosynostosis.

Controlled hypotension and blood loss during frontoorbital advancement

Controlled hypotension is routinely used during open repair of craniosynostosis to decrease blood loss, although this benefit is unproven. In this study the authors analyzed the longitudinal relationships between intraoperative mean arterial pressure (MAP) and calculated blood loss (CBL) during frontoorbital advancement (FOA) for craniosynostosis. The authors reviewed the records of infants with craniosynostosis who had undergone primary FOA between 1997 and 2009. Anesthesia records provided preoperative and serial intraoperative MAP. Interval measures of CBL had been determined during the course of the operation. The longitudinal relationships between MAP(mean), MAP(change), and CBL(change) were assessed over the same time interval and compared between adjacent time intervals to determine the directionality of associations. Ninety infants (44 males and 46 females) underwent FOA at a mean age and weight of 10.7 ± 12.9 months and 9.0 ± 7.0 kg, respectively. The average intraoperative MAP was 56.1 ± 4.8 mm Hg, 22.6 ± 12.1% lower than preoperative baseline. A negative correlation was found between CBL(change) and MAP(mean) over the same interval (r = -0.31, p < 0.05), and an inverse relationship was noted between CBL(change) of the previous interval and MAP(change) of the next interval (r = -0.07, p < 0.05). Finally, there was no significant association between MAP(change) of the previous interval and CBL(change) of the next interval. Calculated blood loss demonstrated a negative correlation with MAP during FOA. Directionality testing indicated that MAP did not affect intraoperative blood loss; instead, blood loss drove changes in MAP. Overall, these findings challenge the benefit of controlled hypotension during open craniofacial repair.

Efficacy of Minimally Invasive Sagittal Craniosynostosis Repair

Efficacy of Minimally Invasive Sagittal Craniosynostosis Repair

Unreliability of intraoperative estimated blood loss in extended sagittal synostectomies

Intraoperative blood loss represents a significant concern during open repair of craniosynostosis, and its reliable measurement remains a serious challenge. In this study of extended sagittal synostectomies, the authors analyzed the relationship between estimated blood loss (EBL) and calculated blood loss (CBL), and investigated predictors of hemodynamic outcomes. The authors reviewed outcomes in infants with sagittal synostosis who underwent primary extended synostectomies (the so-called Pi procedure) between 1997 and 2009. Patient demographic data, operating time, and mean arterial pressures (MAPs) were recorded. Serial MAPs were averaged for a MAP(mean). The EBL was based on anesthesia records, and the CBL on pre- and postoperative hemoglobin values in concert with transfusion volumes. Factors associated with EBL, CBL, red blood cell transfusion (RBCT), and hospital length of stay (LOS) were investigated. Hemodynamic outcomes were reported as percent estimated blood volume (% EBV), and relationships were analyzed using simple and multiple linear and logistic regression models. A p value < 0.05 was considered significant. Seventy-one infants with sagittal synostosis underwent primary extended synostectomies at a mean age and weight of 4.9 months and 7.3 kg, respectively. The average operating time was 1.4 hours, and intraoperative MAP was 54.6 mm Hg (21.3% lower than preoperative baseline). There was no association between mean EBL (12.7% EBV) and mean CBL (23.6% EBV) (r = 0.059, p = 0.63). The EBL inversely correlated with the patient's age (r = -0.07) and weight (r = -0.11) at surgery (p < 0.05 in both instances). With regard to intraoperative factors, EBL positively trended with operating time (r = 0.26, p = 0.09) and CBL inversely trended with MAP(mean) (r = -0.04, p = 0.10), although these relationships were only borderline significant. Intraoperative RBCT, which was required in 59.1% of patients, positively correlated with EBL (r = 1.55, p < 0.001), yet negatively trended with CBL (r = -0.40, p = 0.01). Undertransfusion was significantly more common than overtransfusion (40.8% vs 22.5%, p = 0.02, respectively). The mean hospital LOS was 2.3 days and was not significantly associated with patient demographic characteristics, intraoperative factors, blood loss, RBCT, or total fluid requirements. In extended synostectomies for sagittal synostosis, EBL and CBL demonstrated a decided lack of correlation with one another. Intraoperative blood transfusion positively correlated with EBL, but inversely correlated with CBL, with a significantly higher proportion of patients undertransfused than overtransfused. These findings highlight the need for reliable, real-time monitoring of intraoperative blood loss to provide improved guidance for blood and fluid resuscitation.

Chiari malformation associated with craniosynostosis

Chiari malformation (CM) Type I is frequently associated with craniosynostosis. Optimal management of CM in patients with craniosynostosis is not well-established. The goal of this study was to report on a series of pediatric patients with both craniosynostosis and CM and discuss their management. The authors searched the medical records of 383 consecutive patients treated for craniosynostosis at a single institution over a 15-year period to identify those with CM. They recorded demographic data as well as surgical treatment and outcomes for these patients. When MR imaging was performed, cerebellar tonsillar descent was recorded and any other associated findings, such as hydrocephalus or spinal syringes, were noted. A total of 29 patients with both CM and craniosynostosis were identified. Of these cases, 28% had associated occipital venous abnormalities, 45% were syndromic, and 52% also had hydrocephalus. Chiari malformation was more likely to be present in those patients with isolated lambdoid synostosis (55%), multisuture synostosis (35%), and pansynostosis (80%), compared with patients with coronal synostosis (6%) or sagittal synostosis (3%). All patients underwent surgical repair of craniosynostosis: 16 had craniosynostosis repair as well as CM decompression, and 13 patients did not undergo CM decompression. Of the 7 patients in whom craniosynostosis repair alone was performed, 5 had decreased tonsillar ectopia postoperatively and 5 had improved CSF flow studies postoperatively. Both patients with a spinal syrinx had imaging-documented syrinx regression after craniosynostosis repair. In 12 patients in whom CM was diagnosed after primary craniosynostosis repair, 5 had multiple cranial vault expansions and evidence of elevated intracranial pressure. In 5 cases, de novo CM development was documented following craniosynostosis repair at a mean of 3.5 years after surgery. Chiari malformation is frequently seen in patients with both multi- and single-suture lambdoid craniosynostosis. Chiari malformation, and even a spinal cord syrinx, will occasionally resolve following craniofacial repair. De novo development of CM after craniosynostosis repair is not unusual.

Endoscopically assisted versus open repair of sagittal craniosynostosis: the St. Louis Children's Hospital experience

Object This study investigated the differences in effectiveness and morbidity between endoscopically assisted wide-vertex strip craniectomy with barrel-stave osteotomies and postoperative helmet therapy versus open calvarial vault reconstruction without helmet therapy for sagittal craniosynostosis. Methods Between 2003 and 2010, the authors prospectively observed 89 children less than 12 months old who were surgically treated for a diagnosis of isolated sagittal synostosis. The endoscopic procedure was offered starting in 2006. The data associated with length of stay, blood loss, transfusion rates, operating times, and cephalic indices were reviewed. Results There were 47 endoscopically treated patients with a mean age at surgery of 3.6 months and 42 patients with open-vault reconstruction whose mean age at surgery was 6.8 months. The mean follow-up time was 13 months for endoscopic versus 25 months for open procedures. The mean operating time for the endoscopic procedure was 88 minutes, versus 179 minutes for the open surgery. The mean blood loss was 29 ml for endoscopic versus 218 ml for open procedures. Three endoscopically treated cases (6.4%) underwent transfusion, whereas all patients with open procedures underwent transfusion, with a mean of 1.6 transfusions per patient. The mean length of stay was 1.2 days for endoscopic and 3.9 days for open procedures. Of endoscopically treated patients completing helmet therapy, the mean duration for helmet therapy was 8.7 months. The mean pre- and postoperative cephalic indices for endoscopic procedures were 68% and 76% at 13 months postoperatively, versus 68% and 77% at 25 months postoperatively for open surgery. Conclusions Endoscopically assisted strip craniectomy offers a safe and effective treatment for sagittal craniosynostosis that is comparable in outcome to calvarial vault reconstruction, with no increase in morbidity and a shorter length of stay.

Long-Term Outcomes of Primary Craniofacial Reconstruction for Craniosynostosis: A 12-Year Experience

The purpose of this study was to critically assess long-term outcomes after open reconstruction of craniosynostosis within the recent decade. The authors performed a retrospective, institutional review board-approved review of open repair for craniosynostosis between 1997 and 2009. Surgical factors, complications, and long-term outcomes were assessed. Pearson chi-square, Fisher's exact, and Kaplan-Meier analyses were performed. Of 212 patients, 72 underwent primary extended synostectomy and 140 had traditional open craniofacial repair. Mean follow-up was 36.3 months (range, 0.5 to 138 months). Indications included sagittal (n = 96), metopic (n = 40), unicoronal (n = 33), bicoronal (n = 24), multisutural (n = 15), bilambdoidal (n = 3), and unilambdoidal (n = 1) synostoses; 8.5 percent of patients were syndromic. Surgical reconstruction was performed at a mean age of 11.3 months (range, 0.2 to 117.8 months), including nonsyndromic patients at an average age of 10.6 months and syndromic patients at age 19.3 months. There were no deaths. A 3.3 percent complication rate included two cerebral contusions, two hematomas, one cerebrospinal fluid leak, one infection, and one wound breakdown. Patients were categorized as 89.2 percent Whitaker class I/II and 10.8 percent Whitaker class III/IV. Major and total reoperation rates were 9.0 percent and 10.8 percent, respectively. Higher total reoperation rate and Whitaker class III/IV distribution significantly correlated with syndromic diagnosis, bicoronal synostosis, and surgical age younger than 6 months. In this experience of contemporary open craniosynostosis surgery, rates of morbidity, mortality, and reoperation were low. These results support the merits of surgical delay, targeting an age of 6 months or older, and may serve as a more accurate metric of comparison to current minimally invasive techniques for craniosynostosis repair.

Concurrent repair of orbital shallowness with craniosynostosis surgery: two late cases of simultaneous orbital decompression

Early closure of cranial sutures results in various types of cranial vault deformities, named craniosynostosis. Although mostly associated with syndromic cases, bony orbit deformities such as exorbitism can be seen with various types of craniosynostosis. This condition can be associated with papilledema and besides its effect on the patient's appearance can cause subluxation of the globe, lagophthalmos or keratitis resulting in corneal ulcers and ultimately loss of vision. Various techniques have been proposed for repair or exorbitism such as fronto-orbital advancement procedures, orbital wall decompression, periosteum scoring and tissue excision. Orbital periosteal scoring covering the globe can be extremely efficient for orbital fat decompression when combined with other orbital volume expanding procedures. We hereby present two late cases of craniosynostosis associated with bilateral exorbitism due to orbital shallowness for which cranial vault reconstruction was performed simultaneously with combinations of fronto-orbital advancement, orbital decompression and periosteal scoring. The late referral of these patients at ages older than the usual time of operation indication made the surgical procedure for craniosynostosis repair and exorbitism treatment challenging. The combined and simultaneous use of bone advancement, orbital wall decompression and specially periosteum scoring can be highly efficient in the treatment of exorbitism associated with craniosynostosis.

Penile Blood Pressure Monitoring for a Pediatric Patient with Hypomelia

To the Editor We report a case of a patient with Roberts syndrome who underwent a craniosynostosis repair for increased intracranial pressure. The patient was 3 years old, 38 cm tall, and weighed 3.5 kg. The patient had severe hypomelia (shortened limbs) as well as an unrepaired cleft lip and palate. After application of standard monitors, anesthesia was induced using sevoflurane via mask, and a 24-gauge IV catheter was inserted into a vein of the right foot. A neonatal blood pressure cuff (size 3) was placed around the penis to monitor blood pressure. The cuff was inflated intermittently (not in continuous mode) with periodic inspection of the penis. We were able to monitor the blood pressure from the penis for 2 hours intermittently (every 5 to 10 minutes) until a femoral arterial catheter was placed. There was good correlation of the penile blood pressure with the pressures recorded from the femoral arterial catheter. Penile blood pressure monitoring was discontinued before the patient was draped for the surgery. The surgery lasted approximately 3.5 hours with an estimated blood loss of 450 mL. The patient received 582 mL of packed red blood cells, 170 mL of fresh frozen plasma, 500 mL lactated Ringer solution, and 80 mL albumin 5%. Penile perfusion using Doppler technique and plethysmography has been used to assess penile blood flow for diagnosing vasculogenic impotence.1 This method of assessing blood pressure can be useful when the extremities are not available for performing noninvasive blood pressure monitoring. Harshad Gurnaney, MBBS, MPH Arjunan Ganesh, MBBS Department of Anesthesiology and Critical Care Medicine The Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine Philadelphia, Pennsylvania [email protected] Phillip B. Storm, MD Department of Neurosurgery The Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine Philadelphia, Pennsylvania

Thrombelastography-Based Monitoring for Massive Blood Loss During Elective Pediatric Surgery for Craniosynostosis Repair, a Pilot Study.

Abstract 2110Poster Board II-87The management of massive blood loss in children during trauma or major surgery is still an unsolved problem in pediatric surgery and anesthesia. Primary operative repair of craniosynostosis in infants and young children can serve as a model for excessive acute blood loss. The introduction of thromboelastography (TEG) has led to a significant decrease in transfusion of packed red blood cells (pRBC), fresh frozen plasma (FFP) and platelets in adult surgery, thereby diminishing the risks of infections and immunosuppression. Moreover a significant decrease in accompanying costs has been reported. However, no studies have evaluated the effect of TEG-guided treatment on the amount of transfused blood products in children.The primary objective of this pilot study is to obtain reference TEG-values in children during surgical repair of primary craniosynostosis. We performed a single-center pilot study on TEG-monitoring in children during craniofacial surgery. Methods:The study includes 21 children with craniosynostosis undergoing elective craniofacial repair at the Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands. Blood samples (5 ml blood taken from an arterial line) for TEG (Haemoscope®) measurement were obtained after induction of anesthesia (T1), after the application of Ringer's lactated solution (RLS) 10 ml × kg-1 body weight (T2), after the application of hetastarch 130/0.4 6% (Venofundin®, Fresenius Kabi) (T3), after transfusion of pRBC (T4), and eventually after the application of FFP (T5). Results:21 children, less than 20 months of age, with a mean body weight of 8.5 kg underwent surgical repair of craniosynostosis. They were treated according to the local protocol on massive blood loss in children during surgery. Nine children were suffering from scaphocephalie, 4 from trigonocephalie, 3 from plagiocephalie, one from brachycephalie and 4 children had a mixed or complex form of craniosynostosis such as Crouzon disease.After the induction of anaesthesia (T1) and after the administration of RLS (T2) no changes in clot strength were seen, MA remained mean 62 mm. However, between T2 and T3 all children demonstrated a significant decline in hemoglobine from mean 6.5 to 3.8 mmol/L (p<.0005). The blood loss was mean 380 ml at T3, ranging from 200 to 700 ml, requiring mean 190 ml transfusion of pRBC (range 100-390 ml). The TEG values at T3 showed a concurrent decrease of alpha (from 66° to 57°) and MA (from 62 to 48mm) with an increasing k (from 1.7 to 3.0 min) in kaolin activated TEG measurements. Together with a decrease in MA in TEG–FF at T3 (from 18 to 5.5 mm) this demonstrates a dilutional coagulopathy. All changes were highly significant with p<.0005. Transfusion of pRBC at T4 did not change TEG parameters. No signs of fibrinolysis were seen. Discussion:The administration of hetastarch 130/0.4 6% at T3 resulted in a dilutional coagulopathy. This is due to blood loss, consumption of coagulation factors and platelets, and intravascular volume replacement. During blood loss fibrinogen synthesis will be limited. Additionally, the decreasing functional fibrinogen levels (MA-FF) point to reduced strength of the clot. Administering cryoprecipitate or concentrates of fibrinogen in an early phase might maintain clot firmness and thereby decrease blood loss and reduce the number of transfused blood products. Conclusion:In an attempt to decrease the amount of transfused blood products TEG will allow tailored interventions during pediatric surgery with specific medications like antifibrinolytic agents, concentrates of fibrinogen, or activated recombinant factor VII. Finally, TEG tailored therapy may decrease blood transfusions and transfusion related complications in children. These data strongly support the evaluation of TEG-guided interventions in children during massive blood loss. Disclosures:No relevant conflicts of interest to declare.

Perioperative Management of a Child with von Willebrand Disease Undergoing Surgical Repair of Craniosynostosis: Looking at Unusual Targets

We report the successful management of a craniosynostosis repair in a child with severe Type I von Willebrand disease diagnosed during the preoperative assessment and treated by coagulation factor VIII and ristocetin cofactor. Collaboration among the anesthesiologist, the neurosurgeon, the clinical pathologist, and the pediatric hematologist is important for successful management.