Airway abnormalities in very early treated infantile-onset Pompe disease: A large-scale survey by flexible bronchoscopy.

Early enzyme replacement therapy (ERT) improve long-term outcomes in patients with infantile-onset Pompe disease (IOPD). Our cohort of patients with IOPD at Taipei Veterans General Hospital (TVGH) joined Taiwan Pompe newborn screening program from 2008, testing more than one million newborns until 2018. By 2010, we had established rapid diagnostic strategies. Now, the average age of ERT initiation starts at an average age of <10 days-old, the earliest group in the world. However, they still presented some airway problems. We present a retrospective study focused on airway abnormalities in these patients along 8 years of observation. Fifteen patients with IOPD, who received very early treatment at a mean age of 8.94 ± 3.75 days, underwent flexible bronchoscopy (FB) for dynamic assessment of the whole airway. Long-term clinical outcomes and relevant symptoms of the upper airway were assessed. All patients in the study had varying degrees of severity of upper airway abnormalities and speech disorders. The three oldest children (Age 94, 93, and 88 months, respectively) had poor movement of the vocal cords with reduced abduction and adduction and had silent aspiration of saliva through the glottis during respiration. This is the largest cohort study presented to date about airway abnormalities in very early treated patients with IOPD patients by FB. Despite very early treatment, we observed upper airway abnormalities in these IOPD patients. In IOPD, upper airway abnormalities seem inevitable over time. We suggest early and continuous monitoring for all IOPD patients, even with early and regular treatment.

Stress and uncertainty in parents of children with tracheobronchial malacia and stenosis.

BACKGROUND: An increasing number of children are undergoing flexible bronchoscopy because of tracheobronchial malacia and stenosis, but there is little research related to their parents' stress and uncertainty. AIM: To explore and identify risk factors associated with stress and uncertainty among Taiwanese parents of children with tracheobronchial malacia and tracheabronchostenosis in a paediatric intensive care unit. METHODS: A cross-sectional study design was implemented using two psychometric scales: Parenting Stress Index and Parents' Perception of Uncertainty Scale. Parents of Taiwanese children (0-18 years/o) with a diagnosis of tracheobronchial malacia or/and tracheabronchostenosis who underwent bronchoscopy in a paediatric intensive care unit were recruited. The analysis used descriptive statistics and multivariable linear regression. RESULTS: Ninety parents who were caring for a total of 51 children were recruited. Stress and uncertainty both scored high and were positively correlated with each other. Four risk factors arising from parental stress were unemployment, parental uncertainty, the child's tracheobronchial malacia and tracheabronchostenosis and use of oxygen. CONCLUSIONS: Identifying likely causes of stress and uncertainty is essential for this parental group, particularly for parents facing unemployment, feelings of uncertainty and caring for children with both tracheobronchial malacia and tracheabronchostenosis and requiring oxygen. RELEVANCE TO CLINICAL PRACTICE: Nursing practice can focus on better parental support for those parents who are unemployed, show feeling of uncertainty and care for children with combined tracheobronchial malacia and tracheabronchostenosis and other medical care, such as breathing symptom management, nasogastric feeding and oxygen therapy.

Additive effect of congenital heart disease and early developmental disorders on attention-deficit/hyperactivity disorder and autism spectrum disorder: a nationwide population-based longitudinal study.

In this retrospective nationwide population-based case-control study, we investigated the impact of congenital heart disease (CHD) on the development of attention-deficit/hyperactivity disorder (ADHD) and autism spectrum disorder (ASD), which remains unclear. Children aged <18 years that were diagnosed with CHD (n = 3552) between January 1, 1997 and December 31, 2009 were identified from the National Health Insurance Research Database in Taiwan. Non-CHD controls (n = 14,208) matched for age and sex (1:4) were selected from the same dataset. All subjects were observed until December 31, 2011 or their death. Comorbid perinatal conditions and early developmental disorders (EDD) that were diagnosed before ADHD and ASD diagnosis were also analyzed. The incidence rates of perinatal comorbidities, EDD, ADHD, and ASD were higher in the CHD group than in the control group. Multivariate Cox regression analysis revealed that the CHD group had an increased risk of developing ADHD (adjusted hazard ratio [aHR] 2.52, 95% confidence interval CI 1.96-3.25) and ASD (aHR 1.97, 95% CI 1.11-3.52) after adjusting for confounding comorbidities. EDD, but not perinatal comorbidities were also independent risk factors for ADHD and ASD after adjustment. Subgroup analysis indicated that the risk for ADHD (HR 16.59, 95% CI 12.17-22.60) and ASD (HR 80.68, 95% CI 39.96-176.12) was greatly increased in CHD subjects with EDD than in non-CHD subjects without EDD. These findings suggested that CHD at birth and EDD during early childhood were two independent risk factors for ADHD and ASD and that concurrent CHD and EDD might additively increase these risks.

Very Early Treatment for Infantile-Onset Pompe Disease Contributes to Better Outcomes.

OBJECTIVE: To evaluate whether very early treatment in our patients would result in better clinical outcomes and to compare these data with other infantile-onset Pompe disease (IOPD) cohort studies. METHODS: In this nationwide program, 669,797 newborns were screened for Pompe disease. We diagnosed IOPD in 14 of these newborns, and all were treated and followed in our hospital. RESULTS: After 2010, the mean age at first enzyme-replacement therapy (ERT) was 11.92 days. Our patients had better biological, physical, and developmental outcomes and lower anti-rh acid α-glucosidase antibodies after 2 years of treatment, even compared with one group that began ERT just 10 days later than our cohort. No patient had a hearing disorder or abnormal vision. The mean age for independent walking was 11.6 ± 1.3 months, the same age as normal children. CONCLUSIONS: ERT for patients with IOPD should be initiated as early as possible before irreversible damage occurs. Our results indicate that early identification of patients with IOPD allows for the very early initiation of ERT. Starting ERT even a few days earlier may lead to better patient outcomes.

The epidemiology and prognostic factors of mortality in critically ill children with acute kidney injury in Taiwan.

The incidence of acute kidney injury (AKI) in critically ill children varies among countries. Here we used claims data from the Taiwanese National Health Insurance program from 2006 to 2010 to investigate the epidemiological features and identify factors that predispose individuals to developing AKI and mortality in critically ill children with AKI. Of 60,338 children in this nationwide cohort, AKI was identified in 850, yielding an average incidence rate of 1.4%. Significant independent risk factors for AKI were the use of extracorporeal membrane oxygenation, mechanical ventilation or vasopressors, intrinsic renal diseases, sepsis, and age more than 1 year. Overall, of the AKI cases, 46.5% were due to sepsis, 36.1% underwent renal replacement therapy, and the mortality rate was 44.2%. Multivariate analysis showed that the use of vasopressors, mechanical ventilation, and hemato-oncological disorders were independent predictors of mortality in AKI patients. Thirty-two of the 474 patients who survived had progression to chronic kidney disease or end-stage renal disease. Thus, although not common, AKI in critically ill children still has a high mortality rate associated with a variety of factors. Long-term close follow-up to prevent progressive chronic kidney disease in survivors of critical illnesses with AKI is mandatory.

A 10-year population-based nationwide descriptive analysis of pediatric emergency care.

BACKGROUND: Pediatric emergency care medicine is an important field of health care. This study aimed to investigate the 10-year pediatric emergency care in children aged 0-17 years old in Taiwan. METHODS: Systematic random samples from the National Health Insurance Research Database of Taiwan in the period 2000-2009 were analyzed. Children recorded as undergoing emergency care were enrolled and divided into different age groups. The frequency of emergency visits, age, cost per visit, seasonality, number of hospitalizations, and diagnosis were analyzed. RESULTS: A total of 764,598 children were enrolled. These children accounted for 25% of all emergency cases and their mean age was 6.1 years. Children aged 0-5 years formed the largest group, with male predominance (57.5%). The incidence of emergency visits was 29133 ± 3104 per 100,000 children per year (mean ± SD). Acute upper airway infection, fever, and acute gastrointestinal illness were the most common diagnoses among all non-hospitalized children. Some (4.51%) required subsequent hospitalization and their most common diagnoses were fluid/electrolyte disorder, upper/lower airway infection, and acute gastrointestinal illness. The group of children aged 12-17 years had cases of traumatic injury and childbirth. CONCLUSIONS: In Taiwan, 25% of individuals seeking emergency care are children, mostly aged 0-5 years old. Costs and disease patterns vary among different age groups. Preventive measures targeting all children should focus on respiratory and gastrointestinal diseases, but should target different diseases for different age groups to improve child health.

Moderate hypothermia attenuates oxidative stress injuries in alveolar epithelial A549 cells.

Reactive oxygen species (ROS) are generally involved in lung inflammation and acute lung injury. We investigated the effects of hypothermia on ROS-induced cell damage in human alveolar type II cells. A549 cells were exposed to H2O2 and cultured at different temperatures, namely, normthermia (37°C), mild hypothermia (34°C), or moderate hypothermia (32°C). Cell damage was measured using various assays. The biochemical studies demonstrated a significant increase in apoptosis and intracellular ROS at 32°C in uninjured A549 cells. After exposure to H2O2, a marked decrease in cell viability (<50%) was demonstrated, and this was significantly ameliorated upon culture at 32°C. Significantly intracellular damage was found to affect the 24-hour H2O2-exposed cells in 37°C (P < .05), including an increase in apoptosis and necrosis, intracellular ROS, caspase-3 activity, HMGB1 protein expression, and some alterations to the cell cycle. On hypothermic treatment, the 24-hour H2O2-induced caspase-3 activation was significantly suppressed in cells cultured at both 32°C and 34°C (P < .05 versus 37°C). The cell cycle changes in 24-hour H2O2-exposed cells were significantly diminished when the cells were cultured in 32°C (P < .05 versus 37°C). However, these intracellular alterations were not seen in 6-hour H2O2-exposed cells. We concluded that moderate hypothermia (32°C) of alveolar epithelial A549 cells seems to provide protection against H2O2-induced 24-hour oxidative stress by attenuating cell death and intracellular damage. However, moderate hypothermia might cause minor damage to uninjured cells, so the use of hypothermic treatment needs to be judiciously applied.

Sustained pharyngeal inflation in infant airway-Flexible bronchoscopy measurements.

Sustained pharyngeal inflation (SPI) with pharyngeal oxygen flow and nasal closure (PhO2-NC) technique create positive inflation pressure in the airway. This study measured the peak inflation pressure (PIP) levels and image changes with SPI-assisted flexible bronchoscopy (SPI-FB) and compared the effects in the pharyngeal space and mid-tracheal lumen. This prospective study enrolled 20 participants aged 6 months to 3 years. Each participant underwent sequential SPI-FB of four different durations (0, 1s, 3s, and 5s) for three cycles. We used a 3.8 mm OD flexible bronchoscope to measure and analyze PIP levels, images, and lumen dimension scores. A total of 480 data were collected. The mean (SD) age and body weight were 12.0 (11.5) months and 7.8 (7.5) kg, respectively. The mean (IQR) PIPs were 4.2 (2.0), 18.5 (6.1), 30.6 (13.5), and 46.1 (25.0) cmH2O in the pharynx and 5.0 (1.6), 17.5 (6.5), 28.0 (12.3), 46.0 (28.5) cmH2O in the mid-trachea at SPI durations of 0, 1s, 3s, and 5s, respectively. The PIP levels had a positive correlation (p <0.001) with different SPI durations in both pharynx and trachea, and were nearly identical (p = 0.695, 0.787, and 0.725 at 1s, 3s, and 5s, respectively) at the same duration except the 0 s (p = 0.015). Lumen dimension scores also significantly increased with increasing SPI durations (p <0.05) in both locations. The identified lesions significantly increased as PIP levels increased (p <0.001). Conclusion: SPI-FB using PhO2-NC with durations up to 3s is safe and informative technique that provides controllable PIP, dilates airway lumens, and benefits lesion detection in the pharyngeal space and mid-tracheal lumen.

The specific characteristics in children with obstructive sleep apnea and cor pulmonale.

BACKGROUND: The prevalence of obstructive sleep apnea (OSA) in the pediatric population is currently estimated at 1-2% of all children. The purpose of this study was to investigate the clinical and hemodynamic characteristics in pediatric patients with cor pulmonale and OSA. METHODS: Thirty children with the diagnosis of OSA were included. These patients consisted of 26 male and 4 female children with a mean age of 7 ± 4 years old. Five of those children were found to be associated with cor pulmonale, and 25 had OSA but without cor pulmonale. RESULTS: The arousal index was much higher in children with OSA and cor pulmonale. The children with OSA and cor pulmonale had much lower mean and minimal oxygen saturation and a higher incidence of bradycardia events. All 5 patients with OSA and cor pulmonale underwent an adenotonsillectomy, and the pulmonary arterial pressure dropped significantly after the surgery. CONCLUSION: This study demonstrated that the OSA pediatric patients with cor pulmonale had the different clinical manifestations and hemodynamic characteristics from those without cor pulmonale. The adenotonsillectomy had excellent results in both the OSA pediatric patients with and without cor pulmonale.

Early detection of laryngeal cleft in infants by novel technique of flexible endoscopy with sustained pharyngeal inflation.

OBJECTIVE: We report a novel technique of flexible endoscopy with noninvasive ventilation (NIV) and sustained pharyngeal inflation (FE-NIV-SPI) in assessing aeroesophageal tracts (AET) to facilitate early detection of laryngeal clefts in infants. METHODS: Medical charts and flexible endoscopy videos of the children who were diagnosed with laryngeal cleft in a tertiary care hospital between January 2000 and December 2020 were retrospectively reviewed and analyzed. The FE-NIV-SPI technique had been applied to all these children. RESULTS: Totally, 12 infants with laryngeal cleft were identified. This equates to a prevalence of 0.28% in all the children who underwent flexible endoscopy at our institution. Their mean age was 5.0 ± 4.9 months and mean body weight was 4.7 ± 2.3 kg. Nine (75%) infants were referred in without laryngeal cleft diagnosis, which was missed by 11 prior bronchoscopy and 5 computer tomography examinations. With the FE-NIV-SPI technique, the pharyngolaryngeal space could be pneumatically dilated permitting a detailed assessment. All laryngeal cleft types and coexisting AET lesions were visualized at the first FE-NIV-SPI examination with a mean time of 4.2 ± 0.9 min; they were eight Type I, two Type II, and one Type III. Ten (83.3%) infants had coexisting airway malacia. CONCLUSION: Routine use of FE-NIV-SPI technique can help in early detection of laryngeal clefts and other associated AET lesions. Further multicenter collaborative investigations are essential to verify the early detection of this rare and occult lesion of the laryngeal cleft with this technique.

Flexible Endoscopy With Non-invasive Ventilation Enables Clinicians to Assess and Manage Infants With Severe Bronchopulmonary Dysplasia.

Objectives: The objectives of the study were to determine the efficacy of flexible endoscopy (FE) to assess the approachable aeroesophageal tract (AET) and subsequent changes in clinical management in infants with severe bronchopulmonary dysplasia (sBPD). Methods: This retrospective study investigated sBPD infants who received FE measurement from 2011 to 2020. FE was supported with non-invasive ventilation (FE-NIV) of pharyngeal oxygen with nose closure and abdominal compression without any mask or laryngeal mask airway. Data on AET lesions, changes in subsequent management, and FE therapeutic interventions were collected and analyzed. Results: Forty-two infants were enrolled in the study. Two thin scopes (1.8- and 2.6-mm outer diameter) were used. FE analysis revealed 129 AET lesions in 38 (90.5%) infants. Twenty-eight infants (66.7%) had more than one lesion. Thirty-five (83.3%) infants had 111 airway lesions where bronchial granulations (28, 25.2%), tracheomalacia (18, 16.2%), and bronchomalacia (15, 13.5%) were the main complications. Eighteen esophageal lesions were found in 15 (35.7%) infants. No significant FE-NIV complications were observed. The FE findings resulted in changes in management in all 38 infants. Thirty-six (85.7%) infants underwent altered respiratory care with pressure titrations (29, 45.3%), shortened suction depth (17, 26.6%), immediate extubation (8, 12.5%), changed insertion depth of endotracheal tube (7, 10.9%) and tracheostomy tube (3, 4.7%). Twenty-one (50%) infants had 50 pharmacotherapy changes, including added steroids, anti-reflux medicine, antibiotics, and stopped antibiotics. Eighteen (42.8%) infants received 37 therapeutic FE-NIV procedures, including 14 balloon dilatations, 13 laser-plasty, and 10 stent implantations. Seven (16.7%) infants underwent surgeries for four tracheostomies and three fundoplications. Conclusion: Flexible endoscopy with this non-invasive ventilation could be a safe and valuable technique for direct and dynamic visual measurement of AET, which is essential for subsequent medical decision making and management in infants with sBPD.

Airway abnormalities and pulmonary complications in long-term treated late-onset Pompe disease: Diagnostic and interventional by flexible bronchoscopy.

This study evaluates the whole airway abnormalities of long-term treated late-onset Pompe disease (LOPD) patients, with interventions using the flexible bronchoscope (FB). As a retrospective study, we follow up with our five LOPD patients treated with Myozyme from 2012 to 2021 regularly, but with a focus on the whole airway abnormalities of these patients visualized through FB. The long-term clinical outcomes and relevant airway symptoms were assessed. Pulmonary function test and polysomnography were performed to evaluate the degree of respiratory compromise. All patients in the study had varying degrees of airway collapsibility, pulmonary complications, sleep apnea syndrome, and facial anomalies. Pulmonary function could preserve after Myozyme treatment, but potential deterioration thereafter. This is the first study that focuses on airway abnormalities and pulmonary complications in long-term treated LOPD patients using FB. Despite years of Myozyme treatment, we still observed airway abnormalities in these patients. In our series, the pulmonary complications seem more obvious than those observed in patients with infantile-onset Pompe disease, which might be related to the late diagnosis and treatment. We might recommend that FB could provide dynamic evaluation and interventions of airway abnormalities simultaneously. Early diagnosis of respiratory dysfunction is a critical prognostic factor of the long-term outcome of treated LOPD patients.

Sustained pharyngeal inflation on upper airway effects in children-Flexible bronchoscopy measurement.

OBJECTIVE: Sustained pharyngeal inflation (SPI) with pharyngeal oxygen and nose-closure (PhO2 -NC) can create positive peak inflation pressure (PIP) inside the pharyngolaryngeal space (PLS). This study measured and compared the effects of four different SPI durations in the PLS. METHODS: A prospective study, 20 consecutive children aged between 6 months and 3 years old, scheduled for elective flexible bronchoscopy (FB) suspected positive PLS findings were enrolled. SPI was performed twice in four different durations (0, 1, 3, and 5 s) sequentially in each infant. PIP was measured for each SPI in the pharynx, while simultaneously record images at two locations of the oropharynx and supra-larynx. Patient demographic details, PIP levels, lumen expansion scores, and images of PLS were measured and analyzed. RESULTS: Twenty patients with 40 measurements were collected. The mean (SD) age and weight were 11.6 (9.1) months and 6.8 (2.4) kg, respectively. The measured mean (SD) pharyngeal PIPs were 4.1 (3.3), 21.9 (7.0), 42.2 (12.3), and 65.5 (18.5) cmH2 O at SPI duration of 0, 1, 3, and 5 s, respectively, indicating significant (p<.001) positive correlation. At assigned locations, corresponding PLS images also displayed a significant increase in lumen expansion scores and a number of detected lesions with an increase in SPI duration (p < .004). The mean (SD) procedural time was 5.7 (1.2) min. No study-related complication was noted. CONCLUSIONS: FB utilizing PhO2 -NC as SPI of 1-3 s is a simple, less invasive, and valuable ventilation modality. It provides an adequate PIP level to expand the PLS and improve FB performance in children.

Surfactant lavage decreases systemic interleukin-1 beta production in meconium aspiration syndrome.

BACKGROUND: Surfactant lavage has been used to remove meconium debris in meconium aspiration syndrome (MAS), but the influence of surfactant lavage on pro-inflammatory cytokines and cellular apoptosis is unclear. The aim of this study was to investigate the response of pro-inflammatory cytokine and the influence on alveolar cellular apoptosis using therapeutic bronchoalveolar lavage with diluted surfactant to treat MAS. METHODS: Twelve newborn piglets were anesthetized, intubated via tracheostomy, and artificially ventilated. MAS was induced by intratracheal instillation of 3-5 mL/kg of 20% human meconium. The piglets were then randomly assigned to a surfactant lavage group (n= 6) or a control group (n= 6). Piglets in the lavage group received bronchoalveolar lavage with 30 mL/kg diluted surfactant (5 mg/mL) in two aliquots. Cardiopulmonary parameters were monitored continuously. Serum was obtained hourly to measure concentrations of pro-inflammatory cytokines, including interleukin (IL)-I beta, IL-6, and tumor necrosis factor alpha. Lung tissue was histologically examined after experiments, and terminal deoxynucleotidyl transferase-mediated nick-end labeling assay for apoptotic cell death was also performed. RESULTS: The animals in the lavage group displayed significantly better gas exchange and lower serum concentrations of IL-1 beta than the animals in the control group (P < 0.05). The number of apoptotic cells in lung tissues was significantly lower in the lavage group than the control group, and also in the nondependent than the dependent site. CONCLUSION: Therapeutic surfactant lavage improves oxygenation, decreases production of systemic pro-inflammatory cytokine IL-1 beta, and alleviates the severity of lung cell apoptosis in newborn piglets with experimentally-induced MAS.

Vallecular cyst with coexisting laryngomalacia: Successful diagnosis and laser therapy by flexible endoscopy with a novel noninvasive ventilation support in infants.

OBJECTIVE: Vallecular cyst coexisting with laryngomalacia (VC-LM) can cause significant pharyngolaryngeal obstruction. Traditionally, it is diagnosed with flexible endoscopy (FE) and treated by rigid endoscopy. This study evaluates the effectiveness of solely using FE with novel noninvasive ventilation (NIV) of sustained pharyngeal inflation (SPI) support for both diagnosis and treatment in such infants. METHODS: A retrospective review of consecutive infants who were diagnosed and treated for VC-LM in the 12-year period, 2007 to 2018, was conducted. Clinical variables, techniques, and outcomes were analyzed and reported. RESULTS: Eighteen infants (10 males) were included. The mean age was 3.0 ± 0.6 months and the mean body weight was 4.6 ± 1.3 kg. Before FE, 14 infants were supported with bi-nasal prongs NIV (BN-NIV) and four infants with tracheal intubation. During diagnostic and therapeutic FE, all infants supported with a nasopharyngeal NIV (NP-NIV) only. All diagnoses were made in the first FE inspection of 3.5 ± 1.2 minutes. Thirteen lesions were immediately treated with FE laser therapy in 18.1 ± 1.7 minutes in the same FE course. Total FE time was 24.6 ± 2.8 minutes. Three infants needed revision laser therapy 4 days later. There was no desaturation (<90%), bradycardia (<100/min), or pneumothorax. After FE therapy, all infants were supported with BN-NIV only with significantly (<0.01) lower pressure and completely weaned off before being discharged 8.4 ± 1.5 days later. All infants, followed up for a 6-month period, showed many clinical improvements. CONCLUSIONS: FE, with this NP-NIV and SPI supports, could offer accurate diagnosis and successful laser therapy of the VC-LM with procedural sedation in the same session in infants.

Long-term neurodevelopmental outcomes of significant neonatal jaundice in Taiwan from 2000-2003: a nationwide, population-based cohort study.

Newborns with significant neonatal jaundice (SNJ) would admit for evaluation and/or intervention due to an earlier or more rapid increase in bilirubin level. Bilirubin-induced neurological dysfunction in this population might be underestimated. We aimed to investigate the risk of long-term neurodevelopmental sequelae of SNJ in Taiwan. An SNJ 2000-2003 follow-up cohort consisting of 66,983 neonates was extracted from the nationwide, population-based health insurance database in Taiwan to survey the accumulative incidence of long-term (7-year) neurodevelopmental sequelae in comparison to a reference general-population neonate cohort of 12,579 individuals born in 2000. The SNJ follow-up cohort was furtherly categorized into subgroups according to interventions (phototherapy, intensive phototherapy, and exchange transfusion). The SNJ follow-up cohort exhibited significantly higher cumulative rates of long-term neurodevelopmental sequelae than did the reference cohort (P < 0.05). The risks of infantile cerebral palsy, hearing loss, and developmental delay in the SNJ follow-up cohort were between twice and three times of those in the reference cohort after adjusting for gender, comorbid perinatal disorders and urbanization levels. All intervention subgroups demonstrated higher risks for long-term neurodevelopmental sequelae than the reference cohort (P < 0.05) after adjustment. Patients with SNJ are at risk of developing neurodevelopmental disorders during their growth period. A scheduled follow-up protocol of physical and neurodevelopmental assessment during early childhood for these SNJ patients would potentially be helpful for the early detection of and intervention for neurodevelopmental disorders.

Fiberoptic bronchoesophagoscopy-assisted evaluation and prognostic factor analysis in children with congenital esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are serious congenital anomalies with high morbidity and mortality. Diagnostic and therapeutic fiberoptic endoscopy has been used in children to evaluate and manage trachea-esophageal anomalies. This study aimed to evaluate the prognostic factors and the role of fiberoptic bronchoesophagoscopy (FB) in managing children with EA and TEF. METHODS: From 2000 to 2017, hospitalized children with suspected EA and TEF were enrolled in the study. All associated medical records were retrospectively reviewed. Basic characteristics, diagnoses, age of surgical reconstruction, FB findings, associated anomalies, and survival durations were reviewed. Prognostic factors associated with the patients' mortality were analyzed. RESULTS: A total of 33 children were enrolled, and 91% of them were type C. The median age at the time of hospitalization was 26 days (range, birth to 9 years), including 20 (61%) low-birth-weight infants and 26 (79 %) referred patients. FB was performed in patients preoperatively (39%) and postoperatively (96.8%). Among them, 28 patients (85%) had associated anomalies, including 17 (52%) cardiac and 23 (70%) airway anomalies. The median age of 31 patients who underwent surgical reconstruction was 3 (range, 0-39) days. Esophageal anastomotic stricture (21/31, 67.7%) was the most common postsurgical complication. Twenty-three patients (74.2%) received postoperative FB-guided interventions, including balloon dilatation, laser therapy, and stent implantation. Among the 9 mortality cases, the median age at death was 270 (range, 4-3246) days. Significant factor associated with mortality was delayed (> 48 h old) or no surgical reconstruction (p = 0.030). CONCLUSION: Delayed (>48-hour old) or no surgical reconstruction was significantly related to mortality in children with congenital EA and TEF. Preoperative and postoperative FB evaluations helped to facilitate diagnoses and nonsurgical managements and resolve the patients' tracheoesophageal problems.

Effective lavage volume of diluted surfactant improves the outcome of meconium aspiration syndrome in newborn piglets.

Meconium aspiration syndrome (MAS) is one of the top causes of severe respiratory failure in neonates. This study was designed to investigate the effective volume of therapeutic bronchoalveolar lavage (BAL) with diluted surfactant in the treatment of MAS in newborn piglets. Human meconium was instilled in 24 piglets to induce MAS, and the piglets were randomly divided into four groups: 1) control, no lavage; 2) lavage-10, BAL with diluted surfactant (5 mg/mL, Survanta) 10 mL/kg in two aliquots; 3) lavage-20, 20 mL/kg in two aliquots; 4) lavage-30, 30 mL/kg in two aliquots. Cardiopulmonary parameters were monitored, and the lung tissue was histologically examined after experiments. The changes in oxygenation and lung compliance of lavage-20 and lavage-30 groups were significantly better than control and lavage-10 groups (p < 0.05), but there was no significant difference between lavage-20 and lavage-30 groups. The lung injury scores were significantly lower in the dependent site of lavage-20 and lavage-30 groups than the other two groups. In conclusion, using 20 mL/kg diluted surfactant in two aliquots to perform therapeutic BAL was as effective as 30 mL/kg in improving the pathophysiological outcomes in MAS and may warrant consideration clinically in treating MAS.

The influence of pleurodesis on the outcome of primary spontaneous pneumothorax in children.

BACKGROUND: Primary spontaneous pneumothorax (PSP) has a high rate of recurrence, and pleurodesis has been shown to decrease the rate of recurrence in adult PSP. For pediatric PSP patients, there are only a few case series available and evidence on the benefits of pleurodesis is insufficient. This study aimed to analyze the outcome of pleurodesis among pediatric PSP patients via a nationwide population-based cohort in Taiwan. METHODS: The hospitalization data from the pediatric intensive care sampling file of the National Health Insurance Research Database from January 1 to December 31, 2010, were retrieved and analyzed. Children aged 0-18 years with a discharge diagnosis of PSP (ICD-9: 512, 512.0, and 512.8) were enrolled in the study. Demographic data, management strategies, and clinical outcomes were recorded and analyzed as well. RESULTS: A total of 1005 hospitalization cases were identified and divided into the pleurodesis (409 hospitalizations) and nonpleurodesis (596 hospitalizations) groups. In the univariate analysis, thoracoscopic surgery for PSP decreased the incidence of recurrence (hazard ratio [HR], 0.46; 95% CI, 0.32-0.67) and the need for further surgical intervention (HR, 0.29; 95% CI, 0.18-0.47); however, conventional open surgery did not. A lesser incidence of PSP recurrence (HR, 0.53; 95% CI, 0.37-0.78) and fewer subsequent surgical interventions (HR, 0.32; 95% CI, 0.20-0.52) were found in the pleurodesis group in comparison with the nonpleurodesis group. A multivariate Cox regression analysis revealed that pleurodesis was the only significant factor capable of decreasing the incidence of PSP recurrence (HR, 0.57; 95% CI, 0.38-0.86) and the need for further surgical intervention (HR, 0.40; 95% CI, 0.23-0.69). CONCLUSION: Pleurodesis reduces the rate of recurrence and the need for further surgical intervention in pediatric PSP. It may be considered as the method of choice for the management of PSP in children.

A combination of tracheoplasty and tracheal stenting is an acceptable method of treating severe congenital tracheobronchial stenosis under extracorporeal membrane oxygenation.

BACKGROUND: Congenital tracheobronchial stenosis (CTBS) is a rare congenital condition characterized by complete cartilage rings covering varying lengths of the major airway. In this study, we reviewed the outcomes of patients with CTBS receiving surgical tracheoplasty in our institute. METHODS: We retrospectively analyzed the outcomes of consecutive patients with CTBS operated between 2006 and 2017 when extracorporeal membrane oxygenation (ECMO) was used perioperatively. RESULTS: In total, 11 patients (median follow-up period, 4.2 years; interquartile range, 1.6-5.4) were included. Seven were symptomatic in the neonatal period, 10 had cardiorespiratory anomalies, 7 required preoperative bronchoscopic balloon dilatation, and 1 required preoperative stent placement. Slide tracheoplasty (STP) was performed in 9 patients, and 2 underwent pericardial patch tracheoplasty. Seven patients required postoperative balloon dilatation, and 6 required postoperative stent placement. Early stenting provided immediate ventilatory improvement in all patients and facilitated successful extubation in a median of 4 days after stenting in 80% of the patients. CONCLUSIONS: Under ECMO, severe CTBS could be successfully treated through a combination of tracheoplasty and bronchoscopic management. STP provided excellent results for solitary trachea stenosis with a minimum diameter of ≥3 mm. In selected patients, postoperative tracheobronchial stent placement was crucial in minimizing the ECMO duration and facilitating extubation. LEVEL OF EVIDENCE: IV.