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Background: Perioperative airway management following midface advancements in children with Apert and Crouzon-Pfeiffer syndromes can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications., Methods: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc or Le Fort III procedures., Results: A total of 275 patients (monobloc surgery, n = 129; Le Fort III surgery, n = 146) were included. Sixty-two patients received immediate extubation and 162 received delayed extubation; 42 had long-term tracheostomies, and 9 had perioperative short-term tracheostomies. In most centers, short-term tracheostomies were reserved for selected cases. Patients with delayed extubation remained intubated for 3 days (interquartile range, 2 to 5 days). The rate of no or only oxygen support after extubation was comparable between immediate and delayed extubation groups (58 of 62 patients [94%] and 137 of 162 patients [85%], respectively). However, the immediate extubation group developed fewer cases of postoperative pneumonia than did the delayed group (0 of 62 [0%] versus 24 of 161 [15%]; P = 0.001). Immediate extubation also appeared safe in moderate to severe obstructive sleep apnea, as 19 of 20 patients (95%) required either no or only oxygen support after extubation. The odds of developing intubation-related complications increased by 21% with every extra day of intubation., Conclusions: Immediate extubation following midface advancements was found to be a safe option, as it was not associated with respiratory insufficiency but did lead to fewer complications. Immediate extubation should be considered routine management in patients with no or mild obstructive sleep apnea, and should be the aim in moderate to severe obstructive sleep apnea cases after careful assessment., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Plastic Surgeons.)

Objective: Guidelines for the management of pediatric severe traumatic brain injury (TBI) recommend external ventricular drainage for CSF drainage as a first-tier treatment in the intracranial pressure (ICP) pathway. However, ventriculostomy in children can sometimes be challenging because of the small size of the lateral ventricles. External lumbar drainage (ELD) may be a useful alternative; therefore, the authors analyzed the outcome of a cohort of pediatric patients who underwent ELD to manage intracranial hypertension (ICH)., Methods: This study retrospectively enrolled pediatric patients with ICH following severe TBI who underwent ELD. Radiological and clinical severity scores (Marshall classification, Rotterdam score, Injury Severity Score, and Pediatric Trauma Score) were noted. ICP and cerebral perfusion pressure (CPP) curves were analyzed 12 hours before and after the procedure. Any change in medical therapy was recorded, as well as the total volume and duration of drainage. Cerebellar tonsillar position according to the McRae line was noted before and after ELD. Glasgow Outcome Scale-Extended score at follow-up was also noted., Results: Thirty patients were included, with a mean age of 8 ± 4.4 years, and a median admission Glasgow Coma Scale score of 7 ± 4 (range 3-13). ELD was performed after a median delay of 1 day (range 0-7 days), mean drainage volume/day was 296 ± 129 ml, and median duration of drainage was 7 ± 5 (range 2-12) days. Forty-three percent of the patients underwent ELD as a part of the first-tier therapy. ICP decreased after ELD (mean difference 13.4 ± 6.2 mm Hg, p < 0.001), whereas CPP increased (mean difference 10.6 ± 6.4 mm Hg, p < 0.001). Fifty-three percent of the cohort did not need any further second-tier therapy after ELD. The study found 1 case of drain revision and 3 cases of cerebellar tonsil herniation., Conclusions: These preliminary data suggest ELD is a valuable option to treat ICH in severely head-injured children, limiting the use of second-tier treatments. This pilot study should lay the foundation for a multicenter prospective trial.

Background: Paediatric closed abdominal trauma is common, however, its severity and influence on survival are difficult to determine. No prognostic score integrating abdominal involvement exists to date in paediatrics., Objectives: To evaluate the severity and short-term and medium-term prognosis of closed abdominal trauma in children, and the performance of severity scores in predicting mortality., Design: Retrospective, cohort, observational study., Setting and Participants: Patients aged 0 to 18 years presenting at the trauma room of a French paediatric Level I Trauma Centre over the period 2015 to 2019 with an isolated closed abdominal trauma or as part of a polytrauma., Main Outcomes: Primary outcome was the six months mortality. Secondary outcomes were related complications and therapeutic interventions, and performance for predicting mortality of the scores listed. Paediatric Trauma Score (PTS), Revised Trauma Score (RTS), Shock Index Paediatric Age-adjusted (SIPA) score, Reverse shock index multiplied by Glasgow Coma Scale score (rSIG), Base Deficit, International Normalised Ratio, and Glasgow Coma Scale (BIG), Injury Severity Score (ISS) and Trauma Score and the Injury Severity (TRISS) score., Data Collection: Data collected include clinical, biological and CT scan data at admission, first 24 h management and prognosis. The PTS, RTS, SIPA, rSIG, BIG and ISS scores were calculated and mortality was predicted according to BIG score and TRISS methodology., Results: Of 1145 patients, 149 met the inclusion criteria and 12 (8.1%) died. Of the 12 deceased patients, 11 (91.7%) presented with severe head injury, 11 (91.7%) had blood products transfusion and 7 received tranexamic acid. ROC curves analysis concluded that PTS, RTS, rSIG and BIG scores accurately predict mortality in paediatric closed abdominal trauma with AUCs at least 0.92. The BIG score offered the best predictive performance for predicting mortality at a threshold of 24.8 [sensitivity 90%, specificity 92%, negative-predictive value (NPV) 99%, area under the curve (AUC) 0.93]., Conclusion: PEVALPED is the first French study to evaluate the prognosis of paediatric closed abdominal trauma. The use of PTS, rSIG and BIG scores are relevant from the acute phase and the pathophysiological interest and accuracy of the BIG score make it a powerful tool for predicting mortality of closed abdominal trauma in children., (Copyright © 2024 European Society of Anaesthesiology and Intensive Care. Unauthorized reproduction of this article is prohibited.)

Objective: Epidural hematoma (EDH) has rarely been studied specifically in infants. The objective of this study was to investigate the outcomes of patients aged < 18 months (infants) with EDH., Methods: The authors conducted a single-center retrospective study of 48 infants aged less than 18 months who underwent an operation for a supratentorial EDH in the last decade. Clinical, radiological, and biological variables were used in a statistical analysis to identify factors predictive of radiological and clinical outcome., Results: Forty-seven patients were included in the final analysis. Seventeen children (36%) had cerebral ischemia on postoperative imaging, either due to stroke (cerebral herniation) or by local compression. Factors associated with ischemia after multivariate logistic regression were the presence of an initial neurological deficit (76% vs 27%, p = 0.03), low platelet count (mean 192 vs 267 per mm3, p = 0.01), low fibrinogen level (mean 1.4 vs 2.2 g/L, p = 0.04) and long intubation time (mean 65.7 vs 10.1 hours, p = 0.03). Cerebral ischemia on MRI was predictive of a poor clinical outcome., Conclusions: Infants with EDH have a low mortality rate but a high risk of cerebral ischemia, along with long-term neurological sequelae.

Mucopolysaccharidosis type I-H (MPS I-H) is a rare lysosomal storage disorder caused by α-L-Iduronidase deficiency. Early haematopoietic stem cell transplantation (HSCT) is the sole available therapeutic option to preserve neurocognitive functions. We report long-term follow-up (median 9 years, interquartile range 8-16.5) for 51 MPS I-H patients who underwent HSCT between 1986 and 2018 in France. 4 patients died from complications of HSCT and one from disease progression. Complete chimerism and normal α-L-Iduronidase activity were obtained in 84% and 71% of patients respectively. No difference of outcomes was observed between bone marrow and cord blood stem cell sources. All patients acquired independent walking and 91% and 78% acquired intelligible language or reading and writing. Intelligence Quotient evaluation (n = 23) showed that 69% had IQ ≥ 70 at last follow-up. 58% of patients had normal or remedial schooling and 62% of the 13 adults had good socio-professional insertion. Skeletal dysplasia as well as vision and hearing impairments progressed despite HSCT, with significant disability. These results provide a long-term assessment of HSCT efficacy in MPS I-H and could be useful in the evaluation of novel promising treatments such as gene therapy., (© 2022. The Author(s).)

Importance: The COVID-19 pandemic and the containment and mitigation measures taken were feared to be associated with increased child abuse., Objective: To investigate the trend of abusive head trauma (AHT) incidence and severity in infants during the COVID-19 pandemic., Design, Setting, and Participants: In a time-series analysis of a longitudinal, population-based, cohort study, all consecutive cases of AHT in infants younger than 12 months old referred between January 2017 and December 2021 to Necker Hospital for Sick Children, the single regional pediatric neurosurgery center for the Paris metropolitan area, were included. AHT was defined as 1 or more subdural hemorrhage and a positive multidisciplinary evaluation after a social, clinical, biological, and radiological workup. Data were analyzed from January to March 2022., Main Outcomes and Measures: The primary outcome was the monthly incidence of AHT, which was analyzed using Poisson regression modeling. Secondary outcomes included mortality and severe morbidity and were studied with logistic and linear regressions. The monthly incidence of neurosurgical interventions for hydrocephalus was used as a control series., Results: Among the 99 included infants with AHT (median [IQR] age, 4 [3-6] months; 64 boys [65%]), 86 of 99 (87%) had bridging vein thrombosis, 74 of 99 (75%) had retinal hemorrhages, 23 of 72 (32%) had fractures, 26 of 99 (26%) had status epilepticus, 20 of 99 (20%) had skin injuries, 53 of 99 (54%) underwent neurosurgical interventions, and 13 of 99 (13%) died. Compared with the prepandemic period (2017-2019), AHT incidence was stable in 2020 (adjusted incidence rate ratio, 1.02; 95% CI, 0.59-1.77) and then significantly increased in 2021 (adjusted incidence rate ratio, 1.92; 95% CI, 1.23-2.99). The severity of AHT worsened in 2021 in terms of mortality (odds ratio 9.39; 95% CI, 1.88-47.00). Other secondary outcomes and the control series were not significantly modified., Conclusions and Relevance: In this cohort study, a marked increase in AHT incidence and severity occurred during the COVID-19 pandemic in the Paris metropolitan area. These results suggest the need for clinical awareness and preventive actions.

Aim: The aims of the study are to evaluate the impact of a 4% chlorhexidine (CHG4%) bathing on the occurrence of central-line-associated bloodstream infection (CLABSI) and to identify risk factors (RFs) for CLABSI in our population. This is a retrospective monocentric cohort study in the paediatric surgical intensive care unit at the Necker Enfants Malades Hospital, Paris, France., Methods: All hospitalised patients with central venous catheters (CVCs) in 2015 were included. CHG4% bathing was prescribed in CLABSI high-risk patients, defined by the presence of exposition factors (EFs): constitutive or acquired immunosuppression, presence of an invasive medical device (IMD) and the carriage of Staphylococcus aureus. The overall 2015 CLABSI incidence rate was compared with 2014 CLABSI incidence rate (before CHG4% bathing)., Results: In all, 775 patients were analysed. Some 182 had at least one EF, and 49 received CHG4%. The incidence rates of CLABSI in 2014 and 2015 were, respectively, 6.1 and 2.3/1000 days CVC (P < 0.01). The presence of at least one EF was associated with the CLABSI's occurrence: odds ratio = 15.13 (95% confidence interval: 4.26-53.71; P < 0.0001), particularly acquired immunosuppression, IMD and S. aureus colonisation. Other RFs were age <1 year and carrying duration >16 days., Conclusions: This study showed a significant reduction in incidence of CLABSI after introduction of a targeted CHG4% bathing protocol. Presence of IMD, S. aureus colonisation, immunosuppression, age <1 year and carrying duration >16 days were CLABSI RFs. Regarding the literature, the presence of IMD seems to be underestimated in CLABSI prevention., (© 2020 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Objective: To report routine practice of "perimortem" CT-scan imaging to determine the causes of death in children dying from severe accidental injuries within the first hours following hospital admission., Settings: Trauma center of a University Pediatric Hospital., Methods: A retrospective study was conducted in children (0 to 15 years old) referred for severe trauma (GCS ≤ 8) to a regional pediatric trauma center, presenting with at least spontaneous cardiac rhythm and dying within the first 12 h after admission. "Perimortem" CT-scan consisted in high-resolution, contrast-enhanced, full-body CT-scan imaging, performed whatever child's clinical status. Lethal and associated lesions found were analyzed and classified according to validated scales. The comparison between clinical and radiological examinations and CT-scan findings evaluated the accuracy of clinical examination to predict lethal lesions., Results: CT-scan performed in 73 children detected 132 potentially lethal lesions, at least 2 lesions in 63%, and 1 in 37% of the cases. More frequent lethal lesions were brain (43%), and chest injuries (33%), followed by abdominal (12%), and cervical spine injuries (12%). Clinical and minimal radiological examinations were poorly predictive for identifying abdominal/chest lesions. Clinical and imaging data provided to the medical examiner were considered sufficient to identify the cause of death, and to deliver early burial certificates in 70 children. Only three legal autopsies were commanded., Conclusions: Perimortem CT imaging could provide an insight into the causes of death in traumatized children. Performed on an emergency basis near death, it eliminates the difficulties encountered in forensic radiology. It could be a possible alternative to full-scale forensic examination, at least regarding elucidation of the potential, or highly probable causes of death.

Background: Thoracic bioreactance is a noninvasive and continuous method of cardiac output (CO) measurement that is being developed in adult patients. Very little information is available on thoracic bioreactance use in children., Objective: The aim of the study was to evaluate the ability of a bioreactance device (NICOM(®) ; Cheetah Medical, Tel Aviv, Israel) to estimate CO and to track changes in CO induced by volume expansion (VE) in children., Methods: Cardiac output values obtained using the NICOM(®) device (CONICOM ) and measured by trans-thoracic echocardiography (COTTE ) were compared in pediatric neurosurgical patients during the postoperative period., Results: Seventy-three pairs of measurements of CO obtained in 30 children were available for analysis. The bias (lower and upper limits of agreement) between CONICOM and COTTE was -0.11 (-1.4 to 1.2) l·min(-1). The percentage error (PE) was 55%. The precision of the NICOM(®) device was 45%. A significant correlation was observed between the CO values obtained using the two methods (r = 0.89, <0.001). The concordance percentage between changes in COTTE and CON icom induced by VE was 84% following exclusion of patients with changes in CO <15% (n = 5)., Conclusions: The PE observed is too large, and the limits of agreement too wide, to enable us to comment on the equivalence of the two techniques of CO measurements. However, the NICOM(®) device performs well in tracking changes in CO following VE., (© 2014 John Wiley & Sons Ltd.)

Background: Symptomatic posterior fossa hematoma in the term newborn is rare., Objective: To report on the management and outcome of posterior fossa subdural hematoma (PFSDH) in neonates., Methods: A retrospective analysis of the department database and clinical notes of neonates admitted since 1985 with a PFSDH was performed together with a literature review of similar case series., Results: Sixteen patients were included. The median gestational age was 40 weeks with a high proportion of primiparous mothers (n = 9) and forceps delivery (n = 9). Nine neonates had symptoms of brainstem dysfunction within the first 24 hours of life, whereas the other 7 had a delayed presentation (median 4 days) with signs of raised intracranial pressure due to hydrocephalus. Each patient had a cranial ultrasound followed by computed tomography scan that showed the PFSDH. Eleven neonates required surgical evacuation of the PFSDH, whereas hydrocephalus was managed by transient external ventricular drainage in 2 further patients. Eventually, 2 neonates required a permanent ventriculoperitoneal shunt. Five neonates had no operative intervention. With a mean follow-up of 7.8 years, 2 patients had mild developmental delay and 1 had severe developmental delay. The 13 other patients had a normal development., Conclusion: In neonates with a PFSDH, surgery can be safely performed in those who have clinical and radiological signs of brainstem compression or hydrocephalus. A small number of neonates require a ventriculoperitoneal shunt in the long term. Initial aggressive resuscitation should be performed even in cases of initial severe brainstem dysfunction because of the good long-term neurological outcome.

Introduction: Trigonocephaly secondary to the premature fusion of the metopic synostosis is associated to a risk of cerebral compression and several craniofacial morphological alterations. Numerous surgical techniques have been proposed. They all carry a risk of secondary temporal hollowing, Purpose: The aim of this paper is to describe the surgical technique used for trigonocephaly at the craniofacial unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis) focusing on its advantages and limitations. Resorbable osteosynthesis should be part of the current techniques.

Introduction: Nonsynostotic posterior plagiocephaly has become the most common skull deformation since pediatricians have suggested the supine position for the newborns to reduce the risk of sudden death. Prevention of such a "positional" deformation or its management once it has occurred is mainly based on physical maneuvers such as physiotherapy and active positional corrective measures., Selection Criteria: Surgical correction, however, may be suggested in rare cases where deformation of the skull is so severe or the referral of the child is so late that physical corrective treatment cannot be taken into consideration. Surgical management is based on the creation of a posterior bone flap to be repositioned after the opportune contouring and rotation., Purpose: The aim of this paper is to describe the surgical technique used for posterior vault remodeling in posterior plagiocephaly at the craniofacial unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis) focusing on its advantages and limitations.