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Intraventricular hemorrhage (IVH) is a serious concern for preterm infants and can predispose such infants to brain injury and poor neurodevelopmental outcomes. IVH is particularly common in preterm infants. Although advances in obstetric management and neonatal care have led to a lower mortality rate for preterm infants with IVH, the IVH-related morbidity rate in this population remains high. Therefore, the present review investigated the pathophysiology of IVH and the evidence related to interventions for prevention. The analysis of the pathophysiology of IVH was conducted with a focus on the factors associated with cerebral hemodynamics, vulnerabilities in the structure of cerebral vessels, and host or genetic predisposing factors. The findings presented in the literature indicate that fluctuations in cerebral blood flow, the presence of hemodynamic significant patent ductus arteriosus, arterial carbon dioxide tension, and impaired cerebral venous drainage; a vulnerable or fragile capillary network; and a genetic variant associated with a mechanism underlying IVH development may lead to preterm infants developing IVH. Therefore, strategies focused on antenatal management, such as routine corticosteroid administration and magnesium sulfate use; perinatal management, such as maternal transfer to a specialized center; and postnatal management, including pharmacological agent administration and circulatory management involving prevention of extreme blood pressure, hemodynamic significant patent ductus arteriosus management, and optimization of cardiac function, can lower the likelihood of IVH development in preterm infants. Incorporating neuroprotective care bundles into routine care for such infants may also reduce the likelihood of IVH development. The findings regarding the pathogenesis of IVH further indicate that cerebrovascular status and systemic hemodynamic changes must be analyzed and monitored in preterm infants and that individualized management strategies must be developed with consideration of the risk factors for and physiological status of each preterm infant.

Orthotopic liver transplantation (OLT) is an established therapeutic option for pediatric end-stage liver disease (PELD). The postoperative respiratory conditions of OLT recipients may be associated with subsequent clinical outcomes including length of stay (LOS) in the pediatric intensive care unit (PICU). This study aimed to characterize the postoperative respiratory conditions, associated factors, and outcomes after pediatric OLT.Clinical data of children receiving OLT from July 2014 to July 2020 were retrospectively collected. Postoperative respiratory conditions were defined as time to extubation, significant pleural effusion, and initial postoperative PaO2/FiO2 ratio. Logistic and multiple regressions were applied to analyze the associations among clinical factors, postoperative respiratory conditions, and clinical outcomes.Twenty-two patients with median age of 1.4-year-old (range: 25 days to 12 years old) were analyzed. Mortality within 28 days was 4.5% and median LOS in the PICU was 18 days. Of 22 patients, 11 patients (50.0%) were extubated over 24 hours after surgery, and 8 patients (36.4%) required drainage for pleural effusions. Longer LOS in the PICU were noted in patients extubated over 24 hours (p = 0.008), complicated with significant pleural effusions (p = 0.02) after surgery, and having low initial postoperative PaO2/FiO2 (300 mmHg) (p = 0.001). Among clinical factors, massive intraoperative blood transfusion (40 mL/kg) was significantly associated with prolonged intubations, significant pleural effusions, low initial postoperative PaO2/FiO2, and prolonged LOS in the PICU (14 days). The initial postoperative PaO2/FiO2 significantly depended on age, disease severity (PELD score), and whether the patient received massive intraoperative blood transfusion.Pediatric patients of OLT with poor postoperative respiratory conditions including low initial PaO2/FiO2 ratio, extubation over 24 hours or significant pleural effusions have longer LOS in the PICU, and the requirement of massive intraoperative transfusion was a risk factor for both poor postoperative respiratory conditions and prolonged LOS in the PICU.

OBJECTIVESAn extended newborn critical congenital heart disease (CCHD) screening program using oximetry has been implemented in Taipei, Taiwan since April 2014. This study was conducted to investigate the test accuracy and efficiency of this screening protocol.METHODSThis study analyzed data from 30 birthing facilities representing 87.9% of live births in Taipei. Positive screening was defined as oxygen saturation 3%. This study cohort was used to retrospectively estimate outcomes on the basis of different CCHD screening protocols.RESULTSDuring the study period, 93 058 of 94 204 (98.8%) infants who had no prenatal suspicion were screened. The referral rate was 0.17% (156/93 058), and up to 90% of test-positive infants were referred within 48 hours of life. Forty-two CCHD cases without prenatal suspicion were detected and 97.6% were diagnosed within 72 hours of life. Of the screened newborns, 4 CCHD cases passed the screening. The false positive and false negative rates were 0.12% and 0.04%, respectively. In addition, applying our database to Spanish and updated American Academy of Pediatrics screening strategies led to more CCHD case detection.CONCLUSIONSThe Taipei protocol provided an efficient and effective screening referral system in a community setting. For optimal efficiency, we advocated the updated American Academy of Pediatrics algorithm/Spanish recommendation with a modification of immediate referral if oxygen saturation ≤90% in either extremity. The updated protocol would be practicable for nationwide screening in Taiwan and could also be applied to other regions with similar medical care systems.

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

Liver transplantation (LT) for small infants6 months old is rare but becoming common as perioperative care improves. In Taiwan, living donor LT (LDLT) has expanded indications but is rarely performed for this age group because of unfavorable outcomes in the literature. We evaluated LDLT outcomes of patients6 months old.We identified infants6 months old undergoing LDLT between 2004 and 2019 at our hospital. Variables related to recipients, donors, surgeries, and outcomes were analyzed.Nine patients were identified. Indications for LT were biliary atresia (n = 2), Alagille syndrome (n = 1), protein C deficiency (n = 1), and acute liver failure (n = 5), including two patients with neonatal hemochromatosis, one with herpes simplex hepatitis, one with giant cell hepatitis with autoimmune hemolytic anemia, and one with hemophagocytic lymphohistiocytosis. Median age and weight at LT were 129 days and 4.8 kg, respectively. Graft types included left lateral segment (LLS, n = 4), hyper-reduced LLS (n = 4), and monosegment (n = 1). The median graft-to-recipient weight ratio was 4%. The median follow-up period was 14 months (range, 8 days to 127 months) with two mortalities, and two patients were totally weaned off immunosuppressants. Adjuvant therapies were required for patients with giant cell hepatitis and hemophagocytosis. Preoperative reconstructive imaging for estimating graft thickness facilitated surgical planning.Although LDLT is difficult to perform for small infants, outcomes are favorable and mainly dependent on underlying causes in addition to technical innovations.

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.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.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.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.

Background The advancements in neonatal critical care have not only improved the outcomes of extreme prematurity but also prolonged the process of death in terminally ill neonates. This study analyzed the characteristics of neonates who died at a single tertiary center in Taiwan. The utilization of neonatal hospice care before and after the legalization of life-sustaining treatment (LST) withdrawal in Taiwan in 2013 was also compared. Methods This study enrolled the neonatal mortality cases in the Taipei Veterans General Hospital during January 2008 to December 2017 through chart review. Data on birth history, primary diagnosis, complications, and death circumstances were recorded and analyzed. Results In total, 105 neonatal deaths were analyzed. The circumstances of death were as follows: 22 (21%) cases of full LST and cardiopulmonary resuscitation (CPR) performed until death; 63 (60%) cases of LST initiated but no more CPR after do-not-resuscitate (DNR) consents signed; 8 (7.6%) cases of LST withdrawn; 4 (3.8%) cases of DNR signed without LST initiation; 3 (2.9%) cases of CPR not performed, although no DNR signed; and 5 (4.8%) cases of discharge against medical advice under critical condition. The incidence of written DNR consents (57.9% in 2008-2009 vs 93.8% in 2016-2017; p = 0.02) showed an increasing trend. Regarding the incidence of comorbidities, renal failure rate was higher in the DNR group than in the non-DNR group (p = 0.002). Conclusion There was an increasing trend for written DNR consent and the utilization of neonatal hospice care. Renal failure, as a comorbidity, was significantly associated with the written DNR consent in the neonates. Further studies to evaluate the factors associated with neonatal hospice care utilization are suggested.

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

Objectives Flexible endoscopy (FE) assessed the whole approachable aeroesophageal (AE) tracks and changes of management in infants with severe bronchopulmonary dysplasia (sBPD). Methods A 10 years (2011-2020) retrospective study of sBPD infants who had FE with and without artificial airway in AE tracks. FE with noninvasive ventilation (FE-NIV) of pharyngeal oxygen with nose-close and abdomen-compression was supported. Data of found pathologies, changes of consequent management and therapeutic interventions were collected and analyzed. Results Total 42 infants enrolled. Two scopes of 1.8mm and 2.6mm were used. FE revealed 129 AE pathologies in 38 (90.5%) infants. Twenty-eight (66.7%) infants detected more than one lesion. In 35 (83.3%) infants with 111 airway lesions, bronchial granulations (28, 25.2%), tracheomalacia (18, 16.2%) and bronchial granulations (15, 13.5%) were the leadings. Fifteen (35.7%) infants had 18 esophageal lesions. No significant FE-NIV complication noted. FE findings resulted consequent changes of management in all 38 infants. Thirty-six (85.7%) infants involved respiratory care of pressure titrations (29, 45.3%), shorten suctioning depth (17, 26.6%), changed endotracheal or tracheostomy tube depth (10, 15.6%) and extubation (8, 12.5%). Twenty-one (50%) infants had 50 medication changes included add steroids, anti-reflux medicine, antibiotics and stop antibiotics. Eighteen (42.8%) infants had received 37 therapeutic FE-NIV procedures which included 14 balloon dilatation, 13 laser-plasty and 10 stent implantations. Seven (16.7%) infants had surgeries included 4 tracheostomies and 3 fundoplications. Conclusion FE-NIV can be a safe and valuable modality for direct visual assessment of AE pathologies which contributed subsequent changes of clinical management in sBPD infants.

Introduction Pre-operative management of neonates with esophageal atresia and tracheoesophageal fistula (EA/TEF) requiring positive pressure ventilation (PPV) support is clinically challenging. This study evaluates the safety, feasibility and value of flexible endoscopy with noninvasive ventilation and sustained pharyngeal inflation (FE-NIV-SPI) in diagnosis and placing a naso-tracheo-fistula-gastric (NTFG) tube before surgery. Methods A retrospective study conducted from 2017 to 2020 in neonates with Type-C EA/TEF and respiratory distress, where FE-NIV-SPI performed with NTFG tube placement before surgery. Results Five neonates were collected, one with duodenal atresia and one with transposition of great artery. At FE-NIV-SPI, median body weight was 2,399 g and mean age was 15.2 hours. Four neonates yielded severe (>80% collapsed) tracheomalacia. With this FE-NIV, all tracheal, fistulas and esophageal lumens could clearly assess and manage. All fistulas were less than 8mm proximal to carina with mean orifice width of 5 mm. All NTFG tubes placed successfully after confirmed the EA/TEF. Three neonates had co-intubated with nasal endotracheal tube and 2 neonates had received nasal prongs PPV. Mean procedural time of FE-NIV was 13.6±4.5 minutes. All neonates received gastric decompression and feeding via NTFG tubes for mean of 11.4±18.2 days and had stable pre-surgical courses. No adverse associated complication noted. Conclusion FE-NIV-SPI technique enables safe and accurate measurement of EA/TEF anatomy and placing NTFG tube. It could avert emergent gastrostomy, aid gastric decompression, feeding, and ETT intubation, improve PPV, provide pre-surgical stabilization and identify the fistula location during the surgical correction.

Objectives: Intratracheal steroid therapy for lipopolysaccharide (LPS)-induced acute lung injury (ALI) remains challenging particularly in surfactant-deficiency lungs, a common problem of preterm infants. Surfactant has been used as a vehicle for intratracheal steroid in the treatment of other types of ALI. This study investigated the efficacy of intratracheal budesonide (BUD) delivered by two concentrations of surfactant in the treatment of LPS-induced ALI in surfactant-insufficiency rat lungs. Methods: Male adult rats were anesthetized and ventilated. Our ALI model was established by repeated saline lavage to produce surfactant insufficiency, followed by intratracheal LPS instillation. Five study groups (n=5 for each) with different intratracheal treatments following ALI were used: Control (no treatment), BUD (IT-NS-BUD; BUD in saline); IT-DS-BUD (BUD in diluted surfactant); IT-FS-BUD (BUD in full-strength surfactant); IT-FS (full-strength surfactant). Cardiopulmonary variables were monitored 4 h post injury. Histological and immunohistochemical assessments of the lungs were performed. Results: The IT-FS-BUD and IT-FS groups presented better gas exchange, less metabolic acidosis, less oxygen index, and more stable hemodynamic changes than the IT-DS-BUD, IT-NS-BUD, and Control groups. The total lung injury scores assessed by histological examination were ordered as follows: IT-FS-BUD < IT-DS-BUD or IT-FS < IT-NS-BUD < Control. The immunostaining intensities of lung myeloperoxidase showed the following order: IT-NS-BUD, IT-DS-BUD, or IT-FS-BUD < Control or IT-FS. Only the IT-FS-BUD group displayed a smaller immunostaining intensity of lung TNF-α than the control group. Conclusion: Among our therapeutic strategies, intratracheal BUD delivered by full-strength surfactant confers an optimal protection against LPS-induced ALI in surfactant-insufficiency rat lungs.

OBJECTIVE: Sustained pharyngeal inflation (SPI) with pharyngeal oxygen and nose-close (PhO2-NC) can create positive peak inflation pressure (PIP) inside the pharyngolaryngeal space (PLS). This study measured and compared effects of four different SPI durations in the PLS. METHOD: In this prospective observational study, 20 consecutive infants aged less than 3 years, scheduled for elective flexible-bronchoscopy were enrolled. SPI was performed twice in four different durations (0, 1, 3 and 5 seconds) sequentially in each infant. PIP was measured for each SPI in the pharynx, and simultaneously took images at two locations of oropharynx and supra-larynx. Infants’ demographic details and PIP levels, lumen expansion scores and images of PLS were measured and analyzed. RESULTS: Twenty infants 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) cm H2O at SPI duration of 0, 1, 3 and 5 seconds, respectively; which showed significant positive association (p

Sustained pharyngeal inflation (SPI) with pharyngeal oxygen and nose-close (PhO2-NC) can create positive inflation pressure (PIP) inside the pharyngolaryngeal space (PLS). This study measured and compared effects of different SPI durations in the PLS. Method: A one-year prospective study, 20 consequent infants whose age less than 3-year-old and scheduled for elective FB were enrolled. SPI performed in four different durations (0.0, 1.0, 3.0 and 5.0 seconds) consequently. Each infant did two cycles of SPI. Measured the PIP of each SPI in the pharynx, and simultaneously took images at three locations of oropharynx, supra-larynx and larynx. Data of infant’s demographics, PIP levels, space expansion scores and images were collected and analyzed. Results: Total 20 studied infants, the mean (SD) age was 11.6 (9.1) month-old, the mean (SD) body weight was 6. 8 (2.4) kg and the mean (SD) study time was 3.8 (1.1) minutes. The measured mean (SD) pharyngeal PIPs were 4.1 (3.3), 21.9 (7.0), 42.2 (12.3) and 65.5 (18.5) cmH2O at SPI duration of 0.0, 1.0, 3.0 and 5.0 seconds, respectively. Which showed positively and significantly (p

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 P

Neonatal hyperbilirubinemia (NH) may be the initial and solitary sign of infectious condition in neonates. This retrospective cohort study aims to evaluate the risk of sepsis or urinary tract infection in well-appearing infants with NH below 7 days old.All neonates (n = 8779) born in Taipei Veterans General Hospital from 2013 to 2017 were evaluated retrospectively. A total of 2523 initially well-appearing babies were admitted because of NH. After being hospitalized, patients were categorized into two groups according to the initial transcutaneous bilirubin (TCB) level. Infectious screening results, which include C-reactive protein (CRP), differential count, blood culture, urinalysis, and urine culture, were analyzed.Regarding CRP, 2.7% (18/667) of neonates with NH had elevated CRP (≥1 mg/dL). Among 547 blood cultures, eight were positive, with 0.4% (2/547) non-coagulase-negative staphylococcus (CoNS) bacteremia and 1.1% (6/547) CoNS bacteremia. In urinalysis, 16.6% (182/1094) of NH neonates had pyuria, and 6.7% (25/372) had positive urine cultures. NH with a higher initial TCB level was related to an increased chance of elevated CRP (4.7% vs. 1.5%, odds ratio: 3.29, p = 0.024) and pyuria (20.6% vs. 12.6%, odds ratio: 1.79, p0.001). The rate of positive urine culture between the higher and lower TCB groups had no significant difference (6.6% vs. 6.9%, p0.99). Significant bacteriuria was more common in NH neonates admitted at later age (2 days) (4.9% vs. 11.5%, p = 0.035).In well-appearing neonates below 7 days old, NH with a higher initial TCB is associated with an increased rate in pyuria and abnormal CRP. No difference was found in the rate of positive urine culture between higher and lower TCB levels. Significant bacteriuria was more common in older NH neonates. Septicemia is rare among well-appearing neonates with NH.

Background Nonsteroidal anti-inflammatory drugs, such as selective phosphodiesterase type 4 (PDE4) inhibitors have potential anti-inflammatory and respiratory smooth muscle relaxation effects. This study aimed to investigate the pathophysiological effects of an intravenous PDE4 inhibitor (rolipram) and surfactant lavage (SL) in a newborn piglet model of meconium aspiration syndrome (MAS). Methods MAS was induced in 25 newborn piglets, which were randomly divided into control and four SL treatment groups administered with different doses of intravenous rolipram (0, 0.1, 0.5, and 1 mg/kg). Cardiopulmonary variables were monitored and recorded. The experimental time was 4 hours. Serial blood was drawn for blood gas and biomarker analyses. Lung tissue was examined for histological analysis. Results All SL-treated groups revealed improved oxygenation during the 4-hour experiments and had significantly lower peak inspiratory pressure levels than the control group at the end of experiments. All SL plus rolipram-treated groups exhibited significantly higher lung compliance than the control group. However, the animals receiving high-dose (0.5 and 1.0 mg/kg) rolipram demonstrated significantly elevated heart rates. Lung histology of the nondependent sites revealed significantly lower lung injury scores in all SL-treated groups compared with that in the control group, but there were no differences among the rolipram-treated groups. Conclusions In addition to SL, intravenous PDE4 inhibitors may further improve lung compliance in treating MAS; however, it is necessary to consider cardiovascular adverse effects, primarily tachycardia. Further investigations are required before the clinical application of intravenous PDE4 inhibitor as an anti-inflammatory agent to treat severe MAS.

Background: Extubation failure (EF) in acute pediatric cases causes high morbidity and prolonged hospitalization, some of which might encounter EF repeatedly. This study aims to investigate flexible bronchoscopic findings of airway problems associated with repeated EF (REF) in children. Methods: We retrospectively reviewed the medical records of intubated children from 2005 to 2013 and enrolled those with EF (reintubated within 48 h after extubation) and receiving flexible bronchoscopy (FB) examinations. We divided all subjects into two groups, the REF group (reintubated within 48 h after FB examination) and control group (no need of reintubation), and compared the related clinical conditions and outcomes. Results: We assessed 30 children (REF group, 17 cases; control group, 13 cases). Among them, no significant difference was observed in age, weight, and underlying diseases. In the REF group, the outpatient ratio, tracheostomy rate, intubation days, respiratory or oxygen supported days, and EF episodes were significantly higher than the control group (p

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.

Background: Standing height (SH) is the most reliable parameter used to predict spirometric values in children, but measurement of this parameter may be difficult in children with thoracic or spinal abnormalities. This study was designed to establish reference equations to estimate SHs of children using their arm span length (ASL) or forearm ulnar length (UL) as an index. Methods: Children aged 1–17 years were enrolled to measure their SH, body weight, ASL, and UL. Sex and age were also recorded. The relationship between SH and children's weight, age, ASL, and UL were analyzed. Regression equations using different indexes for SH of enrolled cases were used, and adults aged 18–64 years were also enrolled for comparison. Results: A total of 512 children and 144 adults were enrolled. There was a strong linear relationship between SH and both ASL and UL in children and adults. Pearson's correlation coefficients of SH for ASL and UL were 0.989 and 0.968 (p

Background Esophageal stenosis (ES) is characterized by dysphagia, failure to thrive, and long-term disability. It requires repeated management because it is refractory. Traditionally, these procedures are performed under general anesthesia with airway intubation. Purpose This study investigated the safety and efficacy of the management of interventional flexible endoscopy (IFE) performed with a novel noninvasive ventilation (NIV) support. Methods Use of a short-length flexible endoscope with NIV of pharyngeal oxygen, nose closure, and abdominal compression during IFE was investigated. Medical charts of patients aged ≤10 years with a diagnosis of ES in our hospital between 1990 and 2014 were reviewed and analyzed. The outcome measurement included the number of IFE with balloon dilatation (BD), laser therapy (LT), stent placement, procedural complications, and the success rate. Results Ten patients were enrolled. The most common etiologies were esophageal atresia with/without tracheoesophageal fistula (n = 6), followed by caustic injury (n = 2), and unknown etiology (n = 2). Nine patients who were considered successfully managed received an average of 2.8 BD sessions and 1.6 LT sessions. The complication rate of IFE in this study was 1.08% (1/93). One esophageal perforation developed after BD (1/63) and none after LT (0/30). Conclusion In this study, IFE with this NIV support is a safe, feasible and valuable modality which could rapidly examine and manage ES.

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

Objectives Flexible endoscopy (FE) is frequently used to diagnose tracheobronchial foreign bodies (TBFB). However, it is still controversial for retrieval of TBFB in pediatric field. This study aims at reporting and evaluating our experiences of using short-length FE with a non-invasive ventilation (NIV) technique and intensive care unit (ICU) support in retrieving pediatric TBFB. Methods A retrospective review of the hospital database and FE videos of pediatric patients aged less than 18 year-old who were diagnosed of TBFB and managed in our hospital over a 17-year period (1999–2015). The demographic data were collected and analyzed. A NIV technique of providing nasopharyngeal oxygen with intermittent nose closure and abdominal compression was routinely performed in procedural sedated patients throughout the whole FE procedures. Results Sixty-six consecutive patients with 76 TBFB were enrolled. Among them, 72 (94.7%) TBFB in 64 patients were successfully retrieved at the first attempt of FE immediately after the diagnosis was made. There were 13 iatrogenic TBFB in patients who already had coexisting airway problems. The median age was 16 months (range 1.5 months–17 years) and the median body weight was 10.5 kg (range 3.5–48.5 kg). Seventy (70/72, 97.2%) TBFB were retrieved by short-length FE and among them, 55 procedures (55/72, 76.4%) used FE with no working channel. No significant acute or late adverse effects were noted. The mean retrieval procedural time was 23.6 ± 15.1 min. Conclusion Using short-length FE with this NIV technique, appropriate sedation and ICU support is a safe, simple and effective modality for the retrieval of TBFB immediately after confirming the diagnosis in pediatric patients.

BACKGROUND Tracheobronchial (TB) lumen narrowing may require prolonged positive-pressure ventilation, endotracheal tube intubation or even surgical interventions. Therapeutic flexible bronchoscopy (TFB) of balloon-expandable metallic stent (BEMS) placement and subsequent forceps, laser and balloon dilatation management might be less invasive and helpful. This study aimed to analyse the placement, follow-up management with TFB and long-term outcomes in small infants with BEMS. METHODS This retrospective study reviewed the medical records and associated TFB videos of infants with a maximum body weight (BW) of 5.0 kg who had TB BEMS placement from January 2005 to December 2017 at our institution. All TFB procedures were supported with a novel noninvasive ventilation, nasopharyngeal oxygen with intermittent nose closure and abdominal compression. RESULTS Forty-one BEMSs were placed in 24 infants. The mean BW and mean age were 4.0 ± 0.7 kg and 4.9 ± 2.4 months, respectively. There were 20, 8 and 13 stents located in trachea, carina and main-stem bronchi, respectively. Seven infants with 13 stents died without obvious stent-related mortality. Seven stents in five infants were successfully retrieved by rigid endoscopy (RE). At placement, the diameters of 28 tracheal and 21 bronchial stents were 7.5 ± 1.1 (4-10) and 5.4 ± 0.9 (4-8) mm, respectively. These implanted BEMSs could be gradually and significantly (p < 0.01) expanded. At the end of the follow-up period, all the remaining 21 stents in 12 infants were functional. The diameters of the 14 remaining tracheal and 13 remaining bronchial stents were 9.6 ± 2.0 (8-14) and 7.2 ± 1.4 (4-10) mm, respectively. CONCLUSION BEMSs are practical and effective in selected small infants with benign TB narrowing and can be safely implanted and managed with TFB, and finally retrieved by RE.

Deep vein thrombosis (DVT) is a rare disease in pediatric patients. We report a pediatric patient who developed DVT in association with methicillin-resistant Staphylococcus aureus (MRSA) bacteremia complicated with septic arthritis, osteomyelitis, and myositis extensively. It is crucial to consider musculoskeletal infection associated with DVT in any child who presents with severe swollen limbs and limitations of motion. Prompt antibiotic and anticoagulant treatments should be initiated to reduce the risk of fatal complications.

Juvenile myelomonocytic leukemia (JMML) is a rare hematologic malignancy in children. Its presentations include anemia, thrombocytopenia, monocytosis, skin rash, marked hepatomegaly, and/or splenomegaly. Fever and respiratory involvement are common. Here, we report a case of a premature neonate with initial symptoms of respiratory distress. She gradually developed clinical manifestations of JMML that mimicked neonatal sepsis. Three weeks after birth, JMML was diagnosed. This is the first reported case of JMML presenting in a premature infant in Taiwan.

Supplemental Digital Content is available in the text, The comorbidities and risk factors associated with congenital airway anomalies (CAAs) in children are undecided. This study aimed to investigate the comorbidities commonly associated with CAA and to explore the prognosis and risk factors in CAA children. This nationwide, population-based cohort study was conducted between 2000 and 2011 with children aged 0 to 5 years assigned to either a CAA group (6341 patients) that diagnosed with CAA or an age- and gender-matched control group (25,159 patients) without CAA, using the Taiwan National Health Insurance Research Database (NHIRD). Descriptive, logistic regression, Kaplan–Meier, and Cox regression analyses were used for the investigation. Cleft lip/palate (adjusted odds ratio [aOR], 7.88; 95% confidence interval [CI], 6.49–9.59), chromosome (aOR, 6.85; 95% CI, 5.03–9.34), and congenital neurologic (aOR, 5.52; 95% CI, 4.45–6.87) anomalies were the comorbidities most highly associated with CAA. Of the 31,500 eligible study patients, 636 (399 in the CAA group and 237 in the control group) died during the follow-up period (6.3% vs 0.9%, P

Background Pediatricians are physicians trained to provide comprehensive nonsurgical health care for children, but parents may consult other specialists when seeking medical help for their children. This study was designed to analyze the role of pediatricians and the changes in the patterns of ambulatory visits among different specialties for children under the age of 18 years in Taiwan during the past 13 years. Methods Data on ambulatory visits of children aged 0–17 years from 1999 to 2011 were retrieved from the National Health Insurance Research Database. The physician's specialty, level of the hospital, year of visit, age of the patient, and diagnoses of each ambulatory visit were analyzed. Four of the most commonly visited specialties—pediatrics, otolaryngology, family medicine, and internal medicine—were compared. The yearly trend of ambulatory visits to different specialties, difference in various age groups, influence of hospital levels, and the top 10 diagnoses were analyzed. Results A total of 1,618,033 ambulatory visits were identified and enrolled into our study. A comparison of the proportions of ambulatory visits between 1999–2003 and 2007–2011 showed that the proportions of visits increased from 27.1 ± 1.3% to 35.4 ± 1.0% for pediatricians, decreased from 32.8 ± 1.8% to 17.0 ± 0.8% for family physicians, and did not change for otolaryngologists and internal medicine physicians. Specifically, pediatricians were visited more often if the children were younger, or if the health-care facility (level of hospital) was either a medical center or a regional hospital. Upper respiratory tract infection was the top diagnosis, followed by acute bronchitis, and acute and chronic tonsillitis. Conclusions The role of pediatricians in children's ambulatory care increased in importance from 1999 to 2011 in Taiwan. However, approximately two thirds of children sought ambulatory medical help from nonpediatric physicians. Thus, it is important to educate and encourage parents to visit pediatricians if their children require medical help.

article i nfo Purpose: The aim of this study is to examine the relationship between physician case volume and the outcomes of critically ill children with pneumonia. Materials and methods: This is a population-based cohort study analyzed data provided from by the National Health Insurance Research Database of Taiwan, 2006-2009. Children (aged 3 months to 17 years) having records of intensive care unit (ICU) admission and a diagnosis of pneumonia were included. A total of 9754 critically ill children and 1042 attending physicians were enrolled. The children were assigned to 1 of 4 groups based on the physician's pneumonia case volume. Results: The patients in the very high case volume group had a significantly lower length of hospital stay, in- hospital mortality rate, and hospitalization expenses, and a significantly higher ratio of ICU to hospital stays than the other 3 groups (P b .001). The probability of death tended to be lower when the physician's case volume was higher. The risk-adjusted odds ratio for in-hospital mortality of very-high case volume group was 0.48 (95% confidence interval, 0.35-0.65; P b .001) compared to low case volume group. Conclusions: A higher physician's pneumonia case volume is associated with a lower length of hospital stay, lower in-hospital mortality rate, and lower hospitalization expenses among critically ill children with pneumonia.

Objectives To assess the placement, surveillance management and long-term outcomes of the tracheobronchial (TB) balloon expandable metallic stent (BEMS) managed by therapeutic flexible endoscopy (TFE). Methods This is a retrospective review and analysis of all computerized medical records and related flexible endoscopy videos of pediatric patients who received TB BEMS during 20 years period, from January 1997 to December 2016. TFE techniques with forceps debridement, balloon dilatation and laser ablation were used to implant stents, perform regular surveillance, maintain their functions, and expand the diameters of BEMS. Short-length (30cm-36cm) endoscopes of OD 3.2mm to 5.0mm coupled with the noninvasive ventilation, without ventilation bag, mask or airway tube, supported the whole procedures. Results 146 BEMS were implanted in 87 consecutive children, including 84 tracheal, 15 carinal and 47 bronchial stents. At the time of placement, the mean age was 35.6 ± 54.6 month-old (range 0.3–228) and the mean body weight was 13.9 ± 10.6 kg (range 2.2–60). Surveillance period was 9.4 ± 6.7 years (range, 0.3–18.0). Satisfactory clinical improvements were noted immediately in all but two patients. Seventy-two (82.8%) patients were still alive with stable respiratory status, except two patients necessitating TFE management every two months. Fifty-one stents, including 35 tracheal and 16 bronchial ones, were successfully retrieved mainly with rigid endoscopy. Implanted stents could be significantly (< .001) further expanded for growing TB lumens. The final stent diameters were positively correlated to the implanted duration. Altogether, 33 stents expired (15 patients), 51 were retrieved (40 patients), and 62 remained and functioning well (38 patients), with their mean duration of 7.4 ± 9.5, 34.9 ± 36.3 and 82.3 ± 62.5 months, respectively. Conclusion In pediatric patients, TFE with short-length scopes coupled with this NIV support has provided a safe, feasible and effective modality in placing and subsequently managing TB BEMS with acceptable long-term outcomes.