Craniosynostosis In Infants Research Articles

Amount of reoperation following surgical repair of nonsyndromic craniosynostosis at a single center.

Craniosynostosis is a deformity of the skull that occurs as a result of early fusion of one or more cranial sutures and can be accompanied by neurological deficits. Craniosynostosis can be classified as syndromic or nonsyndromic according to the type of suture involved. Surgical treatment of craniosynostosis in infants basically involves loosening and opening the fused sutures to reduce intracranial pressure, allow the brain to grow, and also fix the skull shape. However, in such cases there is a risk of resynostosis after surgery. According to the literature, resynostosis rates vary between 0% and 70%. In this study, we aimed to evaluate the reoperation rate in craniosynostosis cases treated surgically in our clinic. A retrospective analysis of 70 nonsyndromic craniosynostosis cases treated surgically in the Neurosurgery Department of Bursa Uludağ University from 2005 to 2019 was performed. All patients had undergone total cranial vault remodeling surgically and had been followed up for at least a year. The study group included 70 patients, comprising 40 (57.1%) male and 30 (42.9%) female patients. The mean age of the group was 10.9 ± 7.8 months (range 3-34 months). Out of 70 patients, repeat surgery due to resynostosis had been performed once in 5 (7.1%) patients and twice in 1 (1.4%) patient. It should be kept in mind that resynostosis may occur in patients who have been operated for craniosynostosis. Patients should be examined cosmetically and if necessary, radiologically in the follow-up. Further studies based on larger sample size are recommended for more quantitative data and better results.

Developing a 3D composite training model for cranial remodeling.

Craniosynostosis correction, including cranial vault remodeling, fronto-orbital advancement (FOA), and endoscopic suturectomy, requires practical experience with complex anatomy and tools. The infrequent exposure to complex neurosurgical procedures such as these during residency limits extraoperative training. Lack of cadaveric teaching tools given the pediatric nature of synostosis compounds this challenge. The authors sought to create lifelike 3D printed models based on actual cases of craniosynostosis in infants and incorporate them into a practical course for endoscopic and open correction. The authors hypothesized that this training tool would increase extraoperative facility and familiarity with cranial vault reconstruction to better prepare surgeons for in vivo procedures. The authors utilized representative craniosynostosis patient scans to create 3D printed models of the calvaria, soft tissues, and cranial contents. Two annual courses implementing these models were held, and surveys were completed by participants (n = 18, 5 attending physicians, 4 fellows, 9 residents) on the day of the course. These participants were surveyed during the course and 1 year later to assess the impact of this training tool. A comparable cohort of trainees who did not participate in the course (n = 11) was also surveyed at the time of the 1-year follow-up to assess their preparation and confidence with performing craniosynostosis surgeries. An iterative process using multiple materials and the various printing parameters was used to create representative models. Participants performed all major surgical steps, and we quantified the fidelity and utility of the model through surveys. All attendees reported that the model was a valuable training tool for open reconstruction (n = 18/18 [100%]) and endoscopic suturectomy (n = 17/18 [94%]). In the first year, 83% of course participants (n = 14/17) agreed or strongly agreed that the skin and bone materials were realistic and appropriately detailed; the second year, 100% (n = 16/16) agreed or strongly agreed that the skin material was realistic and appropriately detailed, and 88% (n = 14/16) agreed or strongly agreed that the bone material was realistic and appropriately detailed. All participants responded that they would use the models for their own personal training and the training of residents and fellows in their programs. The authors have developed realistic 3D printed models of craniosynostosis including soft tissues that allow for surgical practice simulation. The use of these models in surgical simulation provides a level of preparedness that exceeds what currently exists through traditional resident training experience. Employing practical modules using such models as part of a standardized resident curriculum is a logical evolution in neurosurgical education and training.

S5A-05 SESSION 5A: GENERALITIES IN CRANIOSYNOSTOSIS – PART II LARGE SINGLE INSTITUTION SERIES OF ENDOSCOPICALLY TREATED INFANTS WITH CRANIOSYNOSTOSIS

Introduction: Endoscopic strip craniectomy (ESC) followed by helmeting for the treatment of craniosynostosis has gained increasing popularity since its introduction in the 1990s. Efficacy and safety remains to be determined. Methods: 500 consecutive infants who underwent ESC for craniosynostosis at a single institution from 2004 to 2018 by a single neurosurgeon were identified from a prospective database. Patient demographics, operative and postoperative variables were analyzed. Results: 500 infants (69% male) of median (IQR) age 3.0 (1.1–16.7) months and weight 5.9 (IQR 5.6–6.7) kg underwent ESC over the 15 year period. Median operative and anesthesia times were 47 and 140 minutes, respectively. Median length of hospital stay was 1 (IQR 1-1) day (3.2% admitted to ICU). The transfusion rate was 6.6% (95% CI: 5–9%) and postoperative complication rate was 1.4% (95% CI: 1–3%). Risk factors for blood transfusion were identified by multivariable logistic regression and included syndromic craniosynostosis (P=.01) and multiple fused sutures (P=.02). Complications included surgical site infections (n=4), skull defect (n=2), and CSF leak (n=1). 14 patients (2.8%; 95% CI: 2–5%) ultimately required an open procedure for correction of their craniosynostosis due to suture (re-)fusion (n=12) or inadequate cosmetic result (n=2). Infants with sagittal, coronal, and multi-suture craniosynostosis showed normalization of head circumference postoperatively at 12 months of age relative to World Health Organization normal head growth data (all P<.001, paired t-test). Head circumference remained within ±25 percentile points of baseline (WHO percentile) in 81% (95% CI: 77–84%) of infants by 12 months of age, decreasing by >25 in 7% (95%CI: 5–10%) and increasing by >25 in 12% (95% CI: 10–15%). Follow-up data demonstrated compliance with the recommended helmet therapy in 91.4% of patients (5.4% unknown); prior data from this cohort showed an average of 7 months of helmet therapy to achieve satisfactory correction. Conclusion: ESC is a safe, effective, and durable procedure for the correction of craniosynostosis in infants. ESC offers a short length of stay, low complication and transfusion rates, and excellent clinical outcomes including age-appropriate normalization of postoperative head growth in >90% of infants.

Evaluation of Long-term Outcomes of Facial Sensation following Cranial Vault Reconstruction for Craniosynostosis.

Cranial vault reshaping to correct craniosynostosis in infants may injure terminal branches of the trigeminal nerve, namely the supraorbital, supratrochlear, zygomaticofacial, and zygomaticotemporal nerves, especially if a fronto-orbital advancement is performed. Despite numerous studies demonstrating successful esthetic outcome after FOA, there are no long-term studies assessing facial sensation after possible damage to these nerves as the result of surgery. A cross-sectional case-control research design was used to evaluate facial sensory threshold in the trigeminal branches after cranial vault reconstruction in children with isolated, nonsyndromic metopic, and unicoronal craniosynostosis, compared with those with sagittal craniosynostosis and age-matched nonaffected controls. Study participants were recruited from the Hospital for Sick Children between the ages of 6 and 18 years. Sensory outcome was determined using the Weinstein Enhanced Sensory Test, the Ten Test, and self-reported facial sensibility function questionnaire. The sensory outcomes of 28 patients and 16 controls were examined at an average age of 9.6 years and 10.3 years, respectively. No subjective or objective sensory deficit was noted in supraorbital, supratrochlear, zygomaticofacial, or zygomaticotemporal nerve distributions between groups. Qualitative reports of facial sensibility function indicated no difference in subjective sensation, protective sensation, or motor behavior between groups. These results suggest that while sensory nerve injury during routine FOA may occur, it does not result in a quantifiable nor clinically significant long-term sensory deficit threshold. Patients do not develop long-term neuropathic pain following surgical intervention.

Surgical treatment of craniosynostosis in infants: open vs closed repair.

Correction of craniosynostosis may require extensive surgical interventions with related intra and postoperative complications especially hemorrhage. To reduce the intervention's impact and associated complications, less invasive surgical alternatives have evolved. The present review comprehensively summarizes surgical techniques, perioperative anesthesia management, success rates, complications, the results of outcome evaluations, and predictors of intra and postoperative complications. Recent evaluations suggest that less invasive methods represent valuable techniques with comparable cosmetic and volumetric results but reduced overall impact and complications. Furthermore, risk stratification based on predictors might optimize safety and guide decision-making concerning the required level of postoperative clinical care. Neuroendoscopic techniques, designed to minimize surgical incision, dissection, and blood loss, are becoming efficacious and valuable alternative therapeutic options reducing the need for fluid replacement and invasive hemodynamic monitoring. Since hemorrhage represents the most important complication in open craniosynostosis repair, prevention strategies such as the use of tranexamic acid should be considered. Sufficient correction of entailed coagulopathies is crucial.

Early diagnosis of craniosynostosis in infants at primary health care

Craniosynostosis or premature fusion of one or more cranial sutures in infants disturbs normal brain growth. This condition causes abnormal skull configuration, increased intracranial pressure, headache, strabismus, blurred vision, blindness, psychomotor retardation. The diagnosis of craniosynostosis is very simple. Pediatricians should routinely assess neurological status and measure head circumference and anterior fontanelle. When necessary, ultrasound of CNS, X-ray and cranial CT scan can be done. When it comes to this condition, early diagnosis and surgical intervention are of utmost importance. In this paper, we have presented a case on craniosynostosis in a female infant, discovered in the third month of life during systematic review that included measurement of head circumference, palpation of anterior fontanelle and cranial sutures. The child was referred to a neurosurgeon who performed the CT scan of endocranium and confirmed the initial diagnosis of craniosynostosis. With head circumference of 40 cm and fused anterior fontanelle, the surgery was timely performed at the sixth month of life due to early diagnosis.

Early treatment of coronal synostosis with endoscopy-assisted craniectomy and postoperative cranial orthosis therapy: 16-year experience

The objective of this study was to present the authors' 16-year experience treating coronal craniosynostosis in infants using endoscopy-assisted techniques and postoperative cranial orthoses. A total of 128 synostosed coronal sutures in 115 patients were treated between 1996 and 2012 by endoscopically resecting a strip of bone containing the stenosed suture via a 2-3 cm incision made at the ipsilateral stephanion. Data were obtained from a prospective database. Following surgery, patients were fitted with custom cranial orthoses to help correct preoperative craniofacial deformities. All patients were followed closely with cranial anthropometric measurements and photographs. The estimated mean blood loss was 20 ml (range 5-120 ml) and the estimated mean strip size was 0.6 cm × 10.7 cm. The mean surgical duration was 55 minutes (range 22-150 minutes). One patient underwent an intraoperative blood transfusion and 1 had a postoperative blood transfusion, for a total transfusion rate of 1.7%. Ninety-seven percent of patients were discharged on the first postoperative day. There were no deaths. Vertical dystopia correction of more than 80% from baseline was obtained in almost two-thirds of patients, with 51% achieving 100% correction. Nasal and sagittal craniofacial deviation (vertex-nasion-gnathion) correction greater than 80% was achieved in 80% of patients, with 77% achieving 100% correction. Supraorbital rim advancement of the ipsilateral eye was obtained in 98% of cases, with correction of frontal plagiocephaly the last deformity to achieve correction. Early treatment of coronal synostosis with endoscopy-assisted craniectomy and postoperative molding helmets leads to significant correction of craniofacial abnormalities, including vertical dystopia, nasal deviation, sagittal misalignment, and ipsilateral proptosis. This treatment method is associated with minimal trauma, blood loss, and transfusion rates, and typically only requires 1 overnight stay. This surgical approach is safe, effective, and associated with excellent results.

Immediate Spontaneous Shape Correction Using Expantile Zigzag Craniectomy in Infantile Scaphocephaly -Is There an Improvement in the Developmental Quotient Following Surgery?-

There is still debate over which method of the surgery is the most appropriate for the treatment of scaphocephalic infants. In addition, change in psychomotor development following these procedures is a very complex issue that has not yet been resolved. In this paper, the authors describe a surgical technique for immediate spontaneous shape correction of infantile scaphocephaly. There were significant differences between pre- and postoperative cephalic index. We also describe an improvement in the developmental quotient following surgery. Therefore, this expantile zigzag craniectomy should be recommended to correct for isolated sagittal craniosynostosis in infants.

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.

Application of computer-assisted design in craniofacial reconstructive surgery using a commercial image guidance system

The technology of digital image guidance systems has transformed many aspects of neurosurgery, including intracranial tumor surgery, functional neurosurgery, and spinal surgery. Despite the central role of imaging studies in diagnosis and treatment planning, intraoperative image guidance has so far had very limited application to the surgical correction of craniofacial deformities, particularly those associated with craniosynostosis. The authors report an example of the marriage of computer-assisted design methods to a commercially available neurosurgical image-guidance system in the treatment of a case of anterior plagiocephaly due to unilateral coronal synostosis. They discuss the steps that must yet be taken to make this technology applicable to the management of craniosynostosis in infants.

Venous Air Embolism During Endoscopic Strip Craniectomy for Repair of Craniosynostosis in Infants

Venous Air Embolism During Endoscopic Strip Craniectomy for Repair of Craniosynostosis in Infants

Perioperative blood salvage during surgical correction of craniosynostosis in infants

Surgical correction of craniosynostosis in infants is a very haemorrhagic procedure. The aim of this study was to determine whether the perioperative use of the continuous autotransfusion system (CATS) would reduce homologous transfusion during repair of craniosynostosis. Two groups of patients were studied according to the availability of the CATS in our hospital. The control group had surgery before the system was introduced and the study group had operations subsequently. Use of CATS was associated with a significant decrease in the median (95% confidence interval) volume of homologous blood transfused [413 (250-540) ml in the control group versus 317 (150-410) ml in the CATS group, P = 0.02] and in the median (95% confidence interval) number of packed red cell units transfused [2 (1-2) in the control group versus 1 (1-2) in the CATS group, P = 0.04] in the perioperative period. Use of CATS is associated with a reduction in homologous transfusion during the surgical correction of craniosynostosis in infants.

Non-Invasive Aortic Blood Flow Measurement in Infants During Repair of Craniosynostosis

ORLIAGUET, G. A.; MEYER, P. G.; BLANOT, S.; JARREAU, M. M.; CHARRON, B.; CUTTAREE, H.; PERIE, A. C.; CARLI, P. A.; RENIER, D. Author Information

Non-invasive aortic blood flow measurement in infants during repair of craniosynostosis

We have assessed the potential clinical benefit of a new echo-Doppler device (Dynemo 3000) which provides a continuous measure of aortic blood flow (ABF) using an aortic flowmeter and a paediatric oesophageal probe, during repair of craniosynostosis in infants under general anaesthesia. The data recorded included: ABFi (i = indexed to body surface area), stroke volume (SVi), systemic vascular resistance (TSVRi), pre-ejection period (PEP), left ventricular ejection time (LVET), mean arterial pressure (MAP), heart rate (HR) and central venous pressure (CVP). Data were collected: before (T1) and 3 min after skin incision (T2), at the time of maximal haemorrhage (T3) and at the end of the procedure (T4). Twelve infants (aged 7.0 (range 6-12) months) were included. ABFi, MAP and CVP were significantly lower at T3 compared with T1 (2.0 (0.8) vs 3.0 (0.8) litre min-1 m-2, 46.1 (5.8) vs 65.2 (8.9) mm Hg and 2.8 (1.6) vs 5.2 (2.1) mm Hg; P < 0.05). PEP/LVET ratio was significantly lower at T2 compared with T1 (0.25 (0.05) vs 0.30 (0.06)) and increased at T4 (0.36 (0.04); P < 0.05). These preliminary results suggest that this non-invasive ABF echo-Doppler device may be useful for continuous haemodynamic monitoring during a surgical procedure associated with haemorrhage in infants.