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Background Nasopharyngeal tubes are useful in pediatric anesthesia for insufflating oxygen and anesthetics. During nasopharyngeal tube‐anesthesia, gas insufflation provides some positive oropharyngeal pressure that differs from the proximal airway pressure owing to the flow‐dependent pressure drop across the nasopharyngeal tube (ΔPNPT). Aims This study aimed to investigate whether ΔPNPT could be used for calculating oropharyngeal pressure during nasopharyngeal tube‐assisted anesthesia. Methods In a physical model of nasopharyngeal tube‐anesthesia, using Rohrer's equation, we calculated ΔPNPT for three nasopharyngeal tubes (3.5, 4.0, and 5.0 mm inner diameter) under oxygen and several sevoflurane in oxygen combinations in two ventilatory scenarios (continuous positive airway pressure and intermittent positive pressure ventilation). We then calculated oropharyngeal pressure as proximal airway pressure minus ΔPNPT. Calculated and measured oropharyngeal pressure couples of values were compared with the root mean square deviation to assess accuracy. We also investigated whether oropharyngeal pressure accuracy depends on the nasopharyngeal tube diameter, flow rate, gas composition, and leak size. Using ΔPNPT charts, we tested whether ΔPNPT calculation was feasible in clinical practice. Results When we tested small‐diameter nasopharyngeal tubes at high‐flow or high‐peak inspiratory pressure, proximal airway pressure measurements markedly overestimated oropharyngeal pressure. Comparing measured and calculated maximum and minimum oropharyngeal pressure couples yielded root mean square deviations less than 0.5 cmH2O regardless of ventilatory modality, nasopharyngeal tube diameter, flow rate, gas composition, and leak size. Conclusion During nasopharyngeal tube‐assisted anesthesia, proximal airway pressure readings on the anesthetic monitoring machine overestimate oropharyngeal pressure especially for smaller‐diameter nasopharyngeal tubes and higher flow, and to a lesser extent for large leaks. Given the importance of calculating oropharyngeal pressure in guiding nasopharyngeal tube ventilation in clinical practice, we propose an accurate calculation using Rohrer's equation method, or approximating oropharyngeal pressure from flow and pressure readings on the anesthetic machine using the ΔPNPT charts.

Eosinophilic esophagitis (EoE) is an immune-mediated condition characterized by symptoms of esophageal dysfunction and an eosinophilic inflammation of the esophagus.1 Swallowed topical steroids represent one of the possible strategies for inducing and maintaining remission in EoE.2 To date, a validated maintenance strategy has yet to be defined, especially in children. The available evidence suggests decreasing the dose after a successful induction therapy.3 No study has reported the efficacy of a continuous progressive dose reduction; thus, it is unknown if all patients need to use the same dosages and for how long.4,5.

The aim of the study is to assess tumor response, treatment-related toxicities, progression-free survival (PFS), and overall survival (OS) in patients with relapsed/refractory brain tumors treated with bevacizumab-containing regimen. Patients that had received I and II line treatments with or without megatherapy were included. Doses and schedule were as follows: bevacizumab (BVZ) 10 mg/kg i.v. with irinotecan (IRI) 150 mg/m2 i.v. every 2 weeks ± temozolamide (TMZ) 200 mg/m2 p.o. daily for 5 days every 4 weeks. TMZ was omitted in heavily pretreated cases. Between 2013 and 2018, 12 patients (3F/9M), median age 161 months (range 66–348), affected with medulloblastoma (n 7), or low-grade glioma (n 2), or high-grade glioma (n 3), received BVZ/IRI association (median courses 20, range 4–67); 3 of them continued single-agent BVZ (median courses 23, range 8–39). TMZ (median courses 8, range 2–26) was administered in eight patients and then stopped in three of them because of myelotoxicity or lack of compliance. Treatment was well tolerated. After 3 months, two complete responses, two partial responses, seven stable diseases, and one progressive disease were observed. Nine cases experienced an improvement in neurological symptoms. Median time to progression was 11 months (95% confidence interval, 4–18 months). Six-month and 2-year PFS were 75% and 42%, respectively. The OS is 33%; interestingly, two cases (one medulloblastoma and one high-grade glioma) are progression-free off-therapy since 30 and 48 months, respectively. BVZ/IRI association ± TMZ showed encouraging therapeutic activity and low toxicity in this series of relapsed/refractory brain tumors.

OBJECTIVES To determine whether in infants with bronchiolitis admitted to a pediatric intensive care unit (PICU) the starting rate for high-flow nasal cannula (HFNC) therapy set by the attending physicians upon clinical judgment meets patients' peak inspiratory flow (PIF) demands and how it influences respiratory mechanics and breathing effort. METHODOLOGY We simultaneously obtained respiratory flow and esophageal pressure data from 31 young infants with moderate-to-severe bronchiolitis before and after setting the HFNC rate at 1 L/kg/min (HFNC-1), 2 L/kg/min (HFNC-2) or upon clinical judgment and compared data for PIF, respiratory mechanics, and breathing effort. RESULTS Before HFNC oxygen therapy started, 16 (65%) infants had a PIF less than 1 L/kg/min (normal-PIF) and 15 (45%) had a PIF more than or equal to 1 L/kg/min (high-PIF). Normal-PIF-infants had higher airway resistance (p

After a failed mandibular osteodistraction, the wrong positioned mandible of a few patients with Pierre Robin sequence returned in the most functional position and regained a proper symmetry, without external intervention. The study aims to explain this self-adjustment and introduce the floating bone phenomenon.The inclusion criteria were severe micrognathia, Fast and Early Mandibular Distraction Osteogenesis protocol, postoperative mandibular wrong positioning, presurgery, immediate postsurgery, and long-term computed tomography scan. Five patients were included. The considered parameters were the distance between mandibular dental centerline and midsagittal facial axis, the rotation of the mandibular body, the magnitude of elongation, and the lowering of the mandibular body.Three patients went from a decentralization >4 mm in the activation phase to a normalization of the said value in the follow-up. In the same period, the interincisal point of 2 patients moved respectively from 0.5 mm on the left and 0.8 mm on the right to 1.2 mm and 1.6 mm on the right, respectively. The rotation of the mandibular body was meanly 25.6° among all patients. The mean value of the distraction was 14.1 mm. A difference of about 4.4 mm between the left and the right side was measured. The lowering of the mandible varied between 2.8 and 12.6 mm.All patients improved their symmetry. Four of them improved in all the measured parameters, while 1 patient presented a worsening in the decentralization of the interincisal point.The floating bone phenomenon could break new grounds in the management of patients with Pierre Robin sequence.

Aim To test the hypothesis that the balance of type-1/type-2 immune response differs between infants hospitalized with respiratory syncytial virus (RSV) bronchiolitis during the peak months and those during the nonpeak months. Methods We prospectively enrolled 90 unrelated full-term previously healthy infants hospitalized during the first year of life for RSV sole bronchiolitis over 2 epidemics (November 2016 to April 2017 and October 2017 to April 2018). We stratified infants as follows: hospitalized during the peak months (n: 71) and during the nonpeak months (n: 19). The frequencies of CD4+ producing interferon (IFN)-γ and interleukin (IL)-4 and of CD8+ producing IFN-γ T cells were measured by flow cytometry from infant peripheral whole blood. The T-helper cell (Th2) polarization index was calculated as the ratio between CD4+ T cells producing IL-4 and CD4+ T cells producing IFN-γ. Results Infants hospitalized during nonpeak months were significantly less frequently breast-fed, had a higher eosinophils count, a significantly higher percentage of CD4+ T cells producing IL-4 and higher Th2 polarization index than infants hospitalized during the peak months. Conclusions We elucidated the presence of different endotypes in infants with RSV sole bronchiolitis. Previously healthy full-term infants hospitalized during the nonpeak months seem to be more likely those with a possible predisposition to atopy.

Bronchiolitis is the first lower respiratory tract viral infection manifesting in infants younger than 12 months of age. Our aim was to evaluate clinical and serological differences in infants with bronchiolitis from a single or from multiple viruses. Our secondary aim was to investigate differences in recurrent wheezing episodes after 12‐24‐36 months of follow‐up. We reviewed the clinical records for 486 full‐term infants hospitalized for bronchiolitis with at least one virus detected in the nasopharyngeal aspirate. In 431 (88.7%) patients one virus was detected and in 55 (11.3%) infants more than one virus was found. No differences were observed in the length of hospitalization, clinical severity score, O2 supplementation or admission to the intensive care unit. Single virus was associated with higher serum C‐reactive protein (C‐RP) than infants with multiple viruses and higher blood neutrophil counts. Respiratory syncytial virus (RSV) was the most frequently detected virus. RSV alone was associated with higher C‐RP (P = 0.007), compared to RSV coinfection. Infants with human rhinovirus (hRV) alone had higher white blood cell counts, higher blood neutrophils, and higher serum C‐RP levels than hRV co‐infection (P = 0.029, P = 0.008, P = 0.008). RSV + hRV, the most frequent co‐infection, was associated with lower neutrophil count and lower C‐RP levels (P = 0.008, P = 0.016) and less fever (P = 0.012), when comparing RSV versus hRV versus RSV + hRV. No differences were found in the frequency of recurrent wheezing between single versus multiple viruses after bronchiolitis. Our findings suggest that in infants with bronchiolitis multiple viral co‐infections can occur, without influence in the clinical severity of the disease. Infants with co‐infection seems to mount a lower inflammatory response.

OBJECTIVES Oral viscous budesonide is a recent therapeutic option for eosinophilic oesophagitis (EoE) compared with dietary restriction and inhaled steroids. This single-centre, open-label, not blinded study aims to evaluate the efficacy and safety of a new, preprepared oral viscous budesonide suspension (PVB) in children and adolescents with EoE. METHODS We treated 36 children with PVB (29 boys; median age 12 years) with EoE diagnosed according to European Society for Paediatric Gastroenterology Hepatology and Nutrition guidelines. Patients 150 cm height received 2 and 4 mg PVB daily, respectively, for 12 weeks. Upper gastrointestinal endoscopy was performed at baseline, after 12 weeks of therapy and 24 weeks after the end of therapy. Baseline and post-treatment scores were calculated for symptoms, endoscopy, and histology. Serum cortisol was performed at baseline, 12, and 36 weeks. RESULTS At the end of PVB trial, endoscopy showed macroscopic remission in 32 patients (88.9%), whereas at histology median pre- and post-treatment peak eosinophil count/high power field (HPF) markedly decreased from 42.2 (range: 15-100) to 2.9 (range: 0-30); moreover, mean symptom and histology scores impressively improved compared with baseline (P

Post-operative pediatric cerebellar mutism syndrome (PPCMS) is a clinical syndrome arising from cerebellar injury and characterized by absence of speech and other possible symptoms and signs. Rare reports described some benefit after administration of dopamine agonist therapy, but no treatment has proven efficacy. In this paper, we report on the dramatic, sudden resolution of PPCMS induced by midazolam administration in a boy who underwent posterior fossa surgery for choroid plexus papilloma of the fourth ventricle. In addition to clinical improvement, post-midazolam single-photon emission computed tomography also demonstrated amelioration of brain perfusion.

Aim and Objectives: This study aimed to evaluate the predictive role of early post-cooling brain magnetic resonance for developmental outcome in newborns with hypoxic–ischemic encephalopathy. Materials and Methods: A retrospective cohort study was performed on 29 consecutive patients through magnetic resonance evaluation (visual analysis of the images and scoring of the detected lesions; mean diffusivity of semioval centre and lenticular nuclei; and area under the curve of basal ganglia N-acetylaspartate at proton magnetic resonance spectroscopic imaging) and Griffiths Mental Development Scales–third edition at 12 and 24 months. Results: Brain magnetic resonance was performed at a mean age of 5.7 ± 3.7 days. Newborns with no/minor magnetic resonance abnormalities had a better developmental outcome than patients with moderate or severe lesions. Structural and spectroscopic abnormalities in basal ganglia resulted in the most significant predictors for an unfavorable outcome. Conclusion: Normal magnetic resonance in early post-cooling phases is strongly associated with a favorable developmental outcome.

Background A new prepared oral viscous budesonide (PVB) has been effective in inducing clinical and histological remission in pediatric eosinophilic esophagitis (EoE). Aims To evaluate the efficacy of a 12-week maintenance therapy on clinical, endoscopic, and histological remission using half of the dose used in the induction therapy. Methods We prospectively enrolled pediatric patients with active EoE. After 12 weeks of induction therapy with PVB (

Bronchiolitis is the leading cause of hospitalization in infants under 12 months. Our aims were to analyze epidemiological characteristics of infants with bronchiolitis over 10 consecutive seasons and to evaluate whether there are any clinical differences between infants hospitalized for bronchiolitis during epidemic peak months and infants in non-peak months. We enrolled consecutive enrolled 723 previously healthy term infants hospitalized at the Paediatric Emergency Department, "Sapienza" University of Rome over the period 2004-2014. Fourteen respiratory viruses were detected from nasopharyngeal aspirates by molecular methods. Clinical and demographic data were extracted from clinical charts. Viruses were detected in 351 infants (48.5%): RSV in 234 (32.4%), RV in 44 (6.1%), hBoV in 11 (1.5%), hMPV in 12 (1.6%), co-infections in 39 (5.4%), and other viruses in 11 (1.5%). Analyzing the 10 epidemic seasons, we found higher incidence for bronchiolitis every 4 years with a peak during the months December-January. Infants hospitalized during peak months had lower family history for asthma (P = 0.003), more smoking mothers during pregnancy (P = 0.036), were slightly higher breastfed (0.056), had lower number of blood eosinophils (P = 0.015) and had a higher clinical severity score (P = 0.017). RSV was detected mostly during peak months, while RV was equally distributed during the seasons. We found some variations in bronchiolitis incidence during epidemics, and discriminative characteristics in infants hospitalized for bronchiolitis during peak months and in non-peak months, that might reflect two different populations of children. Pediatr Pulmonol. 2016;51:1330-1335. © 2016 Wiley Periodicals, Inc.

Pan-enteric capsule endoscopy (PCE) is effective for assessment of small intestinal and colonic Crohn's disease (CD) in pediatric patients. We aimed to determine whether PCE can be used to monitor mucosal healing and deep remission, in a treat to target strategy for pediatric patients with CD.We performed a prospective study of 48 children with a diagnosis of CD at a tertiary care pediatric gastroenterology unit; 46 patients were included in the final analysis. Biomarker, imaging, and PCE analyses were performed at baseline and after 24 and 52 weeks. Small bowel and colonic mucosal healing were defined by Lewis scores135 and simple endoscopic score for CD ≤1, respectively. Clinical remission was defined as defined as a pediatric CD activity index score10 and biomarker-based remission based on normal levels of biomarkers; deep remission was defined as a combination of clinical remission, biomarker-based remission, and mucosal healing. Treatments were adjusted based on findings from PCE (imaging was considered only for patients with negative findings from PCE). Therapies were introduced, optimized, switched, or combined at the discretion of treating clinicians. The primary outcome was the ability of PCE to assess mucosal healing and deep remission at 3 timepoints and to guide a treat to target strategy.PCE detected inflammation in 34 patients (71%) at baseline, 22 patients (46%) at week 24, and 18 patients (39%) at week 52 (P for comparison among timepoints.05). Findings from PCE led to a change in therapy for 34 patients (71%) at baseline and 11 patients (23%) at 24 weeks, whereas only 2 patients with negative results from PCE (4%) changed therapies based on findings from imaging. When the treat to target strategy was applied, proportions of patients with mucosal healing and deep remission increased from 21% at baseline, to 54% at week 24, to 58% at week 52 (P for comparison among timepoints.05); 2 patients (4%) did not respond to treatment.In a prospective study of 48 children with CD, we found a treat to target strategy, based on findings from PCE, to significantly increase the proportions of patients with mucosal healing and deep remission.gov no: NCT03161886.

Background Gastro-oesophageal reflux is very common in the paediatric age group. There is no single and reliable test to distinguish between physiologic and pathological gastro-oesophageal reflux, and this lack of clear distinction between disease and normal can have a negative impact on the management of children. Aims To evaluate the usefulness of 24-h oesophageal pH-impedance study in infants and children with suspected gastro-oesophageal reflux disease. Methods Patients were classified by age groups (A–C) and reflux-related symptoms (typical and atypical). All underwent pH-impedance study. If the latter suggested an abnormal reflux, patients received therapy in accordance with NASPGHAN/ESPGHAN recommendations, while those with normal study had an additional diagnostic work-up. The efficacy of therapy was evaluated with a specific standardized questionnaire for different ages. Results The study was abnormal in 203/428 patients (47%) while normal in 225/428 (53%). Of those with abnormal study, 109 exhibited typical symptoms (54%), and 94 atypical (46%). The great majority of the patients with abnormal study were responsive to medical anti-reflux therapy. Conclusions We confirm the utility of prolonged oesophageal pH-impedance study in detecting gastro-oesophageal reflux disease in children and in guiding therapy. Performing oesophageal pH-impedance monitoring in children with suspected gastro-oesophageal reflux disease is helpful to establish the diagnosis and avoid unnecessary therapy.

Measuring work of breathing (WOB) is an intricate task during high-flow nasal cannula (HFNC) therapy because the continuous unidirectional flow toward the patient makes pneumotachography technically difficult to use. We implemented a new method for measuring WOB based on a differential pneumotachography (DP) system, equipped with one pneumotachograph inserted in the HFNC circuit and another connected to a monitoring facemask, combined with a leak correction algorithm (LCA) that corrects flow measurement errors arising from leakage around the monitoring facemask. To test this system, we used a mechanical lung model that provided data to compare LCA-corrected respiratory flow, volume and time values with effective values obtained with a third pneumotachograph used instead of the LCA to measure mask flow leaks directly. Effective and corrected volume and time data showed high agreement (Bland-Altman plots) even at the highest leak. Studies on two healthy adult volunteers confirmed that corrected respiratory flow combined with esophageal pressure measurements can accurately determine WOB (relative error < 1%). We conclude that during HFNC therapy, a DP system combined with a facemask and an algorithm that corrects errors due to flow leakages allows pneumotachography to measure reliably the respiratory flow and volume data needed for calculating WOB.

Background In this study we sought to evaluate the association between viral bronchiolitis, weather conditions, and air pollution in an urban area in Italy. Methods We included infants hospitalized for acute bronchiolitis from 2004 to 2014. All infants underwent a nasal washing for virus detection. A regional agency network collected meteorological data (mean temperature, relative humidity and wind velocity) and the following air pollutants: sulfur dioxide, nitrogen oxide, carbon monoxide, ozone, benzene and suspended particulate matter measuring less than 10 µm (PM10) and less than 2.5 µm (PM2.5) in aerodynamic diameter. We obtained mean weekly concentration data for the day of admission, from the urban background monitoring sites nearest to each child's home address. Overdispersed Poisson regression model was fitted and adjusted for seasonality of the respiratory syncytial virus (RSV) infection, to evaluate the impact of individual characteristics and environmental factors on the probability of a being positive RSV. Results Of the 723 nasal washings from the infants enrolled, 266 (68%) contained RSV, 63 (16.1%) rhinovirus, 26 (6.6%) human bocavirus, 20 (5.1%) human metapneumovirus, and 16 (2.2%) other viruses. The number of RSV-positive infants correlated negatively with temperature (p < 0.001), and positively with relative humidity (p < 0.001). Air pollutant concentrations differed significantly during the peak RSV months and the other months. Benzene concentration was independently associated with RSV incidence (p = 0.0124). Conclusions Seasonal weather conditions and concentration of air pollutants seem to influence RSV-related bronchiolitis epidemics in an Italian urban area., Highlights • Peak RSV activity correlates with cold temperatures and higher relative humidity. • RSV-positive cases correlates positively with BZ, NOx, SO2, PM10 and PM2.5. • The most predictive pollutant for RSV cases (constant temperature and humidity)is BZ.

We report a case of EBV encephalitis in a seven‐yr‐old child with Ph+ ALL. Two months after an allogeneic HSCT from his HLA mismatched mother, the patient showed an altered sensorium, generalized seizures, and a left hemiparesis. Brain MRI demonstrated multiple lesions highly suggestive for viral encephalitis. Blood and CSF PCR analyses were negative for the most common viruses involved in immunocompromised patients including EBV. A cerebral biopsy was performed, which showed intense gliosis and perivascular lymphocytic cuffing. PCR analysis performed on brain tissue was positive only for the EBV genome, while extensive investigations for other viral infections were negative. The patient's neurological symptoms rapidly worsened and he died two months later. This case report suggests that in patients presenting neurological and radiological signs of encephalitis after an HSCT, an EBV involvement should be considered, even in the absence of CSF and blood PCR virus detection.

Background Kawasaki disease (KD) is an acute inflammatory vasculitis of unknown origin. Case presentation We report the case of a 5-month-old child with an atypical form of KD, characterized by undulating symptoms, who developed an aneurysm of the right coronary artery and an ectasia of the left anterior descending coronary artery. Conclusion This case report underlines the difficulties in recognizing incomplete forms of the illness in young infants, who are at higher risk of cardiac complications.

Summary. Objectives: Our study was aimed to evaluate the efficacy of 7% hypertonic saline and 0.1%hyaluronicacid (7% HS‐HA)given by inhalation, in infantshospitalized for mild-to-moderate bronchiolitis. Methods: In a double-blind controlled study, 39 infants (23 boys)

International audience; Human rhinoviruses (HRV) have been re-classified into three species (A-C), but the recently discovered HRV-C strains are not fully characterized yet. This study aimed to undertake a molecular and epidemiological characterization of HRV strains infecting children hospitalized over one year in two large research hospitals in Rome. Nasal washings from single HRV infections were retrospectively subjected to phylogenetic analysis on two genomic regions: the central part of the 5'Untranslated Region (5'UTR) and the Viral Protein (VP) 4 gene with the 5' portion of the VP2 gene (VP4/2). Forty-five different strains were identified in 73 HRV-positive children: 55 % of the cases were HRV-A, 38 % HRV-C and only 7 % HRV-B. HRV-C cases were less frequent than HRV-A during summer months and more frequent in cases presenting wheezing with respect to HRV-A. Species distribution was similar with respect to patient age, and seasonality differed during summer months with fewer HRV-C than HRV-A cases. On admission, a significantly higher number of HRV-C cases presented with wheezing with respect to HRV-A. The inter- and intra-genotype variability in VP4/2 was higher than in 5'UTR; in particular, HRV-A patient VP4/2 sequences were highly divergent (8-14 %) at the nucleotide level from those of their reference strains, but VP4 amino acid sequence was highly conserved. In HRV-C isolates, the region preceding the initiator AUG, the amino acids involved in VP4 myristoylation, the VP4-VP2 cleavage site and the cis-acting replication element were highly conserved. Differently, VP4 amino acid conservation was significantly lower in HRV-C than in HRV-A strains, especially in the transiently exposed VP4 N-terminus. This study confirmed the high number of different HRV genotypes infecting hospitalized children over one year and reveals a greater than expected variability in HRV-C VP4 protein, potentially suggestive of differences in replication.

Background: We sought to know more about how 14 common respiratory viruses manifest clinically, and to identify risk factors for specific virus-induced acute respiratory tract infections (ARTIs) in children younger than 3 years old and for wheezing at 36-month follow-up. Methods: We retrospectively studied the clinical records for 273 full-term children (median age, 2.9 months; range, 0.26–39; boys, 61.2%) hospitalized for ARTIs, whose nasopharyngeal specimen tested positive for a respiratory virus and 101 children with no history of respiratory diseases (median age, 8 months; range, 0.5–36.5; boys, 58.4%). At 12, 24 and 36 months after children’s discharge, all parents were interviewed by telephone with a structured questionnaire on wheezing episodes. Results: The most frequently detected viruses were respiratory syncytial virus in bronchiolitis, human rhinovirus in pneumonia and human bocavirus in wheezing. Multivariate analysis identified, as risk factors for virus-induced ARTIs, the presence of siblings [odds ratio (OR): 3.0 (95% confidence interval [CI]: 1.8–5.2)], smoking cohabitants (OR: 2.3 (95% CI: 2–4.2)] and breastfeeding lasting less than 3 months [OR: 0.5 (95% CI: 0.3–0.9)]. The major risk factor for respiratory syncytial virus–induced ARTIs was exposure to tobacco smoke [OR: 1.8 (95% CI: 1.1–3.2)]. Risk factors for human rhinovirus–induced ARTIs were attending day-care [OR: 5.0 (95% CI: 2.3–10.6)] and high eosinophil blood counts [OR: 2.6 (95% CI: 1.2–5.7)]. The leading risk factor for recurrent wheezing was exposure to tobacco smoke [OR: 2.5 (95% CI: 1.1–15.6)]. Conclusions: Each respiratory virus leads to a specific clinical manifestation. Avoiding exposing children to tobacco smoke might restrict viral spread from sick parents and siblings to younger children, prevent severe respiratory diseases, and possibly limit sequelae.

Although mechanical ventilation via an endotracheal tube has undoubtedly led to improvement in neonatal survival in the last 40 years, the prolonged use of this technique may predispose the infant to development of many possible complications including bronchopulmonary dysplasia. Avoiding mechanical ventilation is thought to be a critical goal, and different modes of noninvasive respiratory support beyond nasal continuous positive airway pressure, such as nasal intermittent positive pressure ventilation and synchronized nasal intermittent positive pressure ventilation, are also available and may reduce intubation rate. Several trials have demonstrated that the newer modes of noninvasive ventilation are more effective than nasal continuous positive airway pressure in reducing extubation failure and may also be more helpful as modes of primary support to treat respiratory distress syndrome after surfactant and for treatment of apnea of prematurity. With synchronized noninvasive ventilation, these benefits are more consistent, and different modes of synchronization have been reported. Although flow-triggering is the most common mode of synchronization, this technique is not reliable for noninvasive ventilation in neonates because it is affected by variable leaks at the mouth and nose. This review discusses the mechanisms of action, benefits and limitations of noninvasive ventilation, describes the different modes of synchronization and analyzes the technical characteristics, properties and clinical results of a flow-sensor expressly developed for synchronized noninvasive ventilation.

Temporomandibular joint (TMJ) ankylosis is a pathological condition characterized by articular bony or fibrous tissue fusion. TMJ ankylosis developing during childhood can lead to growth complications because of the loss of mandibular function. Hard and soft autogenous tissue grafting has been used for TMJ reconstruction in the growing patient. However, in cases where autogenous tissue grafts fail either due to unpredictable growth or ankylosis, total alloplastic temporomandibular joint replacement (TMJ TJR) can provide a viable option. The case of a 7-year old female suffering from recurrent bilateral TMJ ankylosis resulting from birth trauma, and with concomitant obstructive sleep apnea syndrome (OSAS) is presented. Due to prior surgical and autogenous graft failures, the decision was made to complete her joint reconstructions utilizing patient-fitted TMJ prostheses. Questions have been raised about the longevity of TMJ TJR devices as well as their lack of growth potential, but children with TMJ ankylosis do not have condyle-related growth potential and that replacing failed autogenous tissue graft material with more autogenous tissue will result in the same adverse outcomes. Therefore, in growing patients with recurrent TMJ ankylosis and/or failed autogenous tissue grafts, there may be a role for TMJ TJR.

High-flow nasal cannula (HFNC) is frequently used to treat respiratory distress in infants and children because of its beneficial effects on alveolar ventilation and respiratory mechanics. Setting an adequate flow rate that meets a patient's peak inspiratory flow (PIF) is thus crucially important to achieve such effects. HFNC flow rate is typically set at 1 L/min/kg +1 as suggested by the manufacturer and increased to 2 L/min/kg according to the degree of respiratory distress. However, whether this empirical flow setting actually meets a patient's PIF has not yet been investigated. In this study, we implemented our previously described respiratory mechanics monitoring system (MAES) with a new spirometry function (NSS) that allows for a simultaneous visualization of the flow tracings of HFNC and the patient's spontaneous breathing. We tested the ability of NSS-MAES to determine the adequacy of empirically set flow rates of 1 L/min/kg +1 or 2 L/min/kg on 9 infants with respiratory distress receiving HFNC. HFNC flow rate was considered adequate if its tracing was just above the patient's respiratory flow. In patients in whom 1 L/kg/min +1 was inadequate, we used NSS-MAES to identify the adequate flow by raising the HFNC flow until it reached the patient's PIF (HFNC_NSS-MAES). We also investigated which flow rate was associated with the maximal decrease of respiratory effort, namely, Pressure Time Product (PTP) and Work of Breathing (WOB). We found that 1 L/min/kg +1, but not 2 L/min/kg was often unable to meet the patient's PIF. In these cases HFNC_NSS-MAES values were around 1.6 L/min/kg. Conversely, HFNC at 2 L/min/kg always exceeded the patient's PIF. All breathing effort indexes tested improved after HFNC treatment with the maximal unloading seen at 2 L/min/kg for PTP and at HFNC_NSS-MAES.

The transfer of O2 to the tissues and the removal of CO2 are guaranteed by the simultaneous activity of the respiratory apparatus and of the cardio-circulatory system which ensures that O2 reaches the tissues and that the CO2 produced by cell metabolism is removed [1].

Although micrognathia and cleft palate have been reported in patients with Lymphedema–distichiasis syndrome (LDS), the classic Robin sequence with glossoptosis and airway obstruction has not been previously described in patients with genetically confirmed LDS. Here we report on two female siblings with LDS confirmed by a FOXC2 mutation who presented at birth with severe airway obstruction related to Robin sequence. Respiratory obstruction was successfully managed by early distraction osteogenesis. Our report highlights the unusual occurrence of Robin sequence in LDS patients and advises distraction osteogenesis to resolve breathing problems in LDS patients who present with Robin related severe airway obstruction.