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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.

The COVID-19 pandemic and the containment and mitigation measures taken were feared to be associated with increased child abuse.To investigate the trend of abusive head trauma (AHT) incidence and severity in infants during the COVID-19 pandemic.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.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.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.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.

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.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).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 duration16 days.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 duration16 days were CLABSI RFs. Regarding the literature, the presence of IMD seems to be underestimated in CLABSI prevention.

Resume L’activite de prelevement et de greffe d’organes pediatriques represente moins de 5 % de l’activite nationale. Chez les enfants de moins de deux ans, le prelevement est particulierement rare et exigeant, mais strictement indispensable. De nombreux obstacles tenant notamment a des contextes etiologiques traumatiques, a l’opposition parentale et a la rarete des petits receveurs compatibles, rendent ces prelevements d’organes rares et compliques. L’evolution des precedentes recommandations sur la mort encephalique (ME) pediatrique posent des problemes pratiques non encore resolus, qui peuvent impacter negativement sur le recensement des donneurs potentiels. Le Doppler transcrânien (DTC), qui permet de suivre plus facilement en pediatrie que chez l’adulte les alterations de la circulation cerebrale et l’evolution vers la ME, peut mettre en confiance les equipes de reanimation pediatrique concernees par ce diagnostic medico-legal. En preconisant une evaluation clinique et DTC repetee, les nouvelles recommandations de l’Agence de la biomedecine valorisent le diagnostic clinique de ME et permettent de simplifier la confirmation paraclinique, soit par angioscanner cerebral soit par deux electro-encephalogrammes avec un delai raccourci a 4 heures quel que soit l’âge.

Resume L’objectif, de cet article de synthese, est de decliner le concept de damage control (DC) a la pediatrie, de la phase initiale prehospitaliere a la phase hospitaliere, en insistant sur les implications des specificites physiopathologiques sur la prise en charge. Type d’article : mise au point. Source des donnees : recherche dans la base de donnees Medline des articles et des revues generales de langue francaise et anglaise, et documents personnels. Dans les grands principes, le damage control ground zero, le damage control ressuscitation et le damage control chirurgical appliques a la pediatrie, utilisent les memes concepts et techniques que chez l’adulte, malgre une litterature peu documentee. L’optimisation de la prise en charge du choc hemorragique, et singulierement la lutte contre la coagulopathie, reste au centre des preoccupations. Les particularites pediatriques se caracterisent par des objectifs, en particulier hemodynamiques, differents, et impliquent de disposer de materiel adapte a l’âge et au poids de l’enfant. L’extension du paradigme de DC concerne tous les enfants presentant un choc hemorragique avec un saignement non controle. Les specificites de cette prise en charge ne doivent pas modifier les grands principes de celle des enfants polytraumatises et plus particulierement des enfants traumatises crâniens graves. Un contexte emotionnel particulierement difficile impose egalement une organisation specifique pour l’accueil des parents et des familles.

Resume En pediatrie comme chez l’adulte, le choix du solute de remplissage vasculaire fait l’objet de nombreuses controverses : differences d’efficacite volemique entre colloides et cristalloides, effets secondaires des solutes, place des hydroxyethylamidons (HEA) et des solutions balancees, indications de plus en plus reduites de l’albumine y compris en neonatalogie. Cet article propose une mise au point concernant ces differentes controverses afin d’aider le lecteur a guider le choix du solute de remplissage vasculaire en fonction de la situation clinique.

Background Postoperative fluid management can be challenging in children after haemorrhagic surgery. The goal of this study was to assess the ability of dynamic cardiovascular variables measured using bioreactance (NICOM®, Cheetah Medical, Tel Aviv, Israel) to predict fluid responsiveness in postoperative children. Methods Children sedated and mechanically ventilated, who require volume expansion (VE) during the immediate postoperative period, were included. Indexed stroke volume (SVi), cardiac index, and stroke volume variation (SVV) were measured using the NICOM® device. Responders (Rs) to VE were patients showing an increase in SV measured using transthoracic echocardiography of at least 15% after VE. Data are median [95% confidence interval (CI)]. Results Thirty-one patients were included, but one patient was excluded because of the lack of calibration of the NICOM® device. Before VE, SVi [33 (95% CI 31–36) vs 24 (95% CI 21–28) ml m−2; P=0.006] and SVV [8 (95% CI 4–11) vs 13 (95% CI 11–15)%; P=0.004] were significantly different between non-responders and Rs. The areas under the receiver operating characteristic curves of SVi and SVV for predicting fluid responsiveness were 0.88 (95% CI 0.71–0.97) and 0.81 (95% CI 0.66–0.96), for a cut-off value of 29 ml m−2 (grey zone 27–29 ml m−2) and 10% (grey zone 9–15%), respectively. Conclusions The results of this study show that SVi and SVV non-invasively measured by bioreactance are predictive of fluid responsiveness in sedated and mechanically ventilated children after surgery.

Massive swelling of the tongue can occur after posterior fossa and craniofacial surgery. Several hypotheses have been proposed to explain the occurrence of such severe postoperative macroglossia, but this phenomenon is still poorly understood. Severe postoperative macroglossia can be a life-threatening condition due to upper airway obstruction. Three cases of severe postoperative macroglossia that occurred after cervical spine, craniofacial, and posterior fossa surgical procedures are reported here. These cases required specialized maxillofacial management and a prolonged stay in the intensive care unit. Causal factors involved in this condition are reported, in order to highlight appropriate prevention and treatment options adapted to the management of paediatric patients. An overview of the current literature on severe postoperative macroglossia in paediatric populations is also provided.

Les donneurs pediatriques d’organes sont rares et principalement la consequence de mort encephalique (ME) de causes non naturelles. Ces circonstances, plus souvent traumatiques accidentelles ou non, orientent les deces dans un processus d’obstacle medicolegal compliquant les demarches de coordination de prelevement multiorgane (PMO). Pour esperer faire participer ces jeunes defunts au pool de donneurs potentiels d’organes, les soignants doivent faire preuve d’une cooperation etroite avec les equipes judiciaires, avec en priorite la preservation des preuves utiles a la recherche de la verite. Ils doivent notamment apporter le maximum d’informations medicales sur les lesions observees cliniquement ou radiologiquement en pre- et peroperatoire. En reponse, les procureurs seront souvent plus attentifs envers ces medecins reanimateurs et les professionnels de la coordination de prelevement d’organes (CPO) qui les sollicitent pour une autorisation officielle de PMO. Ils pourront suggerer des que possible un « accord de principe » si l’enquete judiciaire le permet. Ulterieurement, si la demarche evolue favorablement avec un accord parental, une « autorisation officielle » pourra etre fournie a la CPO qui en fera la demande. Cette collaboration entre les professionnels de sante et ceux de la justice a contribue a mettre en place tous ces principes permettant un meilleur recensement avec un taux de prelevement superieur, parmi cette cohorte de 22 enfants de moins de trois ans en ME identifies par la CPO.

Le choc hemorragique est une situation a haut risque de morbimortalite. Le pronostic du patient depend de la rapidite de prise en charge du choc et de la vitesse de correction des troubles de l’hemostase. Il est donc important d’en faire rapidement le diagnostic; celui-ci est essentiellement clinique, facile en cas de saignement exteriorise. Sa reconnaissance peut cependant etre difficile chez l’enfant du fait de ses particularites physiopathologiques, particulierement en cas d’hemorragie interne. La prise en charge associe hemostase de la lesion hemorragique et reanimation hemodynamique pour maintenir une oxygenation cellulaire satisfaisante. L’efficacite du traitement s’apprecie d’abord sur les parametres hemodynamiques classiques: frequence cardiaque, pression arterielle moyenne, perfusion cutanee, conscience et diurese horaire, notamment. Neanmoins, les signes cliniques peuvent etre insuffisants pour evaluer le statut hemodynamique, et la mesure de la pression arterielle non invasive peut etre imprecise; il est souvent necessaire de monitorer la pression arterielle invasive dans les cas les plus severes. Le concept du damage control resuscitation, initialement decrit chez l’adulte, peut etre applique a l’enfant en tenant compte de ses particularites anatomophysiologiques. Le principe repose sur une reanimation ciblant la triade letale (coagulopathie–acidose–hypothermie) associee a un geste d’hemostase rapide permettant une stabilisation physiologique. Le traitement definitif de la lesion est realise une fois le patient stabilise ou quand tous les patients ont ete traites en cas d’afflux de victimes. Le principe du damage control s’applique des la prise en charge initiale, sur les lieux memes d’un accident ou a domicile et se poursuit apres l’admission a l’hopital.

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 hollowingThe 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.

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. 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. 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.

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.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.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.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 CO15% (n = 5).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.

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 Bien que la transplantation d’organes se developpe, les petits donneurs d’organes de 0 a 2 ans revele des specificites pouvant compliquer ces demarches. Les particularites ont ete etudiees a partir d’enfants en Mort Encephalique (ME) au CHU Necker. Materiel et methodes Onze dossiers d’enfants de 0 a 2 ans ou une demarche de coordination de don d’organe a ete effectuee au CHU Necker entre 2011 et 2013 ont ete etudies. Ont etes analysees les donnees epidemiologiques, les etiologies et le mode de diagnostic de la ME (utilisation d’un Doppler transcranien (DTC), electro-encephalogramme (EEG) et angiographies cerebrales), le prelevement multi-organes (organes preleves, devenir). Resultats L’âge median est de 14 mois (sex-ratio equilibre). Dans 27 % des cas, la ME est liee a un traumatisme crânien non accidentel (hematome sous-dural bilateral), dans 36 % des cas a des traumatismes par AVP ou autre, soit une cause non naturelle retrouvee dans 63 % des cas. Pour assister le diagnostic clinique de ME, le DTC est utilise chez 9/11 enfants rapportant l’effondrement circulatoire cerebral. L’EEG reste l’examen complementaire le plus utilise (8/11) pour confirmer la ME. La duree moyenne de la demarche de coordination en cas de prelevement depasse 40 h. Des organes ont ete preleves chez 4/11 enfants pour etre greffes a 8 patients, dont 2 reins monoblocs (MB) destines a des receveurs adultes et 2 greffes multiviscerales pediatriques. Trois cœurs ont ete preleves et greffes (2/3 en super U) avec succes. Un prelevement n’a pas ete realise en l’absence de receveur compatible (penurie inverse). Six refus de prelevement ont ete faits, dont 2 cas d’obstacles medicolegaux (opposition du procureur). Deux greffons sont partis a l’international. L’evolution des receveurs est bonne dans 7/8 cas. Discussion L’abondance des causes non naturelles de ME dans cette population pose des limites en termes d’obstacles medicolegaux, imposant une etroite collaboration entre coordination, equipes medicales, medicolegales et judiciaires. Concernant le diagnostic de ME, le DTC a participe dans 9/11 cas, et l’utilisation de l’EEG reste majoritaire malgre les delais imposes par les recommandations et l’acces a l’angio-TDM sur le site. Le mode diagnostic de ME, le contexte medicolegal et l’accompagnement parental delicat pour la participation des 2 parents a la decision du Don, sont autant d’arguments pouvant allonger les demarches de coordination. L’absence de receveur compatible dans cette categorie d’âge/poids le jour de la demarche peut interrompre celle-ci, douloureusement pour tout le monde. Toutefois, la mise a disposition d’organes de petits donneurs est irremplacable pour les receveurs morphologiquement contraints. Elle participe aussi a reduire la penurie nationale (rein MB pour adultes). La recherche de receveur a l’international qui est conditionnee par l’accord parental au Don, offre une alternative reciproque d’avenir, et justifie aussi une prise en charge exhaustive des petits donneurs comme les adultes, pour augmenter le pool de greffons disponibles. Les donneurs d’organes de 0 a 2 ans representent un pool restreint avec des contraintes particulieres a prendre en compte (diagnostic, contexte medicolegal, repartition) mais ils sont indispensables pour la compatibilite des petits receveurs et participent a lutter contre la penurie nationale.

Introduction Les pratiques transfusionnelles en chirurgie hemorragique evoluent vers une indication plus large du plasma frais congele (PFC). Les recommandations de l’ANSM pour la prise en charge du choc hemorragique tendent vers une administration precoce avec un ratio culot globulaire (CG)/PFC optimal entre 1/1 et 1/2 [1] . Le but de cette etude etait de definir l’interet de la transfusion de PFC en chirurgie hemorragique pediatrique, et de determiner si un ratio PFC/CG Materiel et methodes Apres accord du CPP IDF VI, une etude retrospective a ete realisee en anesthesie-reanimation neurochirurgicale pediatrique. Tous les enfants p Resultats Soixante-quatorze enfants (âge moyen 9 ± 5,2 mois) ont ete inclus ; tous ont recu une transfusion de CG, 35 enfants n’ont pas recu de PFC (PFC–), 19 ont recu du PFC (PFC+) dont 10 avec un ratio CG/PFC ≥ 1/2. La transfusion de PFC etait associee a la diminution de la transfusion postoperatoire de CG non significative (48 ± 83 mL vs 30 ± 59 mL p = 0,30). Le bilan d’hemostase postoperatoire retrouvait une thrombopenie dans le groupe PFC+, et aucune difference pour le TCA, le TP, et le fribrinogene ( Tableau 1 ). Le groupe PFC+ presentait des pertes sanguines peroperatoires plus importantes (181 mL vs 419 mL, p Discussion La transfusion precoce de PFC avec un ratio PFC/CG [2] . L’interet d’une transfusion precoce de PFC doit etre evalue prospectivement sur une plus large cohorte de patients en chirurgie hemorragique pediatrique.