The use of overlapping self-expandable covered stents in the management of long-segment tracheobronchomalacia: A case report.

Tracheomalacia is a condition where the tracheal wall is abnormally soft and prone to collapse during increased respiratory efforts. Airway malacia can manifest as segmental conditions like laryngomalacia, tracheomalacia and bronchomalacia, or as diffuse conditions such as tracheobronchomalacia (TBM). Unlike long-segment congenital tracheal stenosis, where surgery may be the preferred treatment, the management of long-segment TBM remains controversial.

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.

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.

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.

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.

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.

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.

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.

Very rare condition of multiple Gaucheroma: A case report and review of the literature.

BACKGROUND: This study presented a 3 years old boy with Gaucher disease (GD) who was treated with enzyme replacement therapy(ERT) for 19 months and developed multiple Gaucheroma. The literature was reviewed. METHODS: The medical chart and literature were reviewed. A boy presented at the age of 15 months with anemia, thrombocytopenia, and hepatosplenomegaly. Enzyme assay and gene mutations confirmed GD. ERT was administered. When the boy was 3 years old, multiple masses were discovered from abdominal MRI and biopsy revealed Gaucheroma. We reviewed 20 GD patients with Gaucheroma and Gaucher cell infiltrated lymphadenopathies. CONCLUSION: Gaucheroma is a rare condition in regularly treated GD patients. This patient showed poor response to doubled ERT doses. The imaging studies are necessary for Gaucher patients to detect Gaucheroma and determine their malignancy. Regular checkups are recommended in all GD patients even with ERT treatment, due to the possibility of having a deteriorating change, like Gaucheroma.

Very rare condition of multiple Gaucheroma: A case report and review of the literature.

BACKGROUND: This study presented a 3 years old boy with Gaucher disease (GD) who was treated with enzyme replacement therapy (ERT) for 19 months and then developed multiple Gaucheroma. Review of literature was performed simultaneously. METHODS: The medical chart and literature were reviewed. A boy presented at the age of 15 months with anemia, thrombocytopenia, and hepatosplenomegaly. GD was confirmed by enzyme assay and gene mutations. ERT was administered right after the diagnosis. When the boy was 3 years old, multiple masses were discovered during a regular checkup abdominal MRI and biopsy revealed Gaucheroma. We also reviewed 20 GD patients with Gaucheroma and Gaucher cell infiltrated lymphadenopathies. CONCLUSION: Gaucheroma is a rare condition of regularly treated GD patients. This patient even showed poor response to doubled ERT doses. The imaging studies are necessary for Gaucher patients to detect Gaucheroma and determine their malignancy. Regular checkups are recommended in all GD patients even with regular treatment, due to the possibility of having deteriorating change, like Gaucheroma.

Long-term management and outcomes of tracheobronchial stent by flexible bronchoscopy in infants <5 kg: A 13-year single-center experience.

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.

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.

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.

Flexible bronchoscopic findings and the relationship to repeated extubation failure in critical children.

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 < 0.05). Moreover, the FB findings in the REF group exhibited higher ratios of all airway problems and significantly in the presence of upper airway granulations (odds ratio [OR], 17.9, 95% confidence interval [CI]: 2.7-116.9) and subglottic stenosis (OR, 5.4; 95% CI: 1.1-26.0). After discharge, subjects of the REF group required higher medications than those in the control group (OR, 81.0; 95% CI: 3.9-1655.8). CONCLUSION: Upper airway granulations or stenosis significantly augment the risk of REF in children; however, these could be diagnosed early by FB, guiding the therapeutic protocol in acute cases. Thus, anatomical problems of upper airways should be considered in intubated children with EF, and FB is a useful tool for the early diagnosis and management.

The comorbidities and risk factors in children with congenital airway anomalies: A nationwide population-based study in Taiwan.

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 < .001). The mortality risk after adjusting for age, gender, and comorbidities elevated significantly among CAA patients (adjusted hazard ratio [aHR], 4.59; 95% CI, 3.85-5.48). The need for tracheostomy (aHR, 2.98; 95% CI, 2.15-4.15), comorbidity with congenital heart disease (CHD) (aHR, 2.52; 95% CI, 2.05-3.10), and chromosome anomaly (aHR, 2.34; 95% CI, 1.70-3.23) were the independent risk factors most greatly related to CAA mortality.This study demonstrated that CAA was most highly associated with the comorbidities as cleft lip/palate, chromosome, and congenital neurologic anomalies. The CAA children had a significantly elevated mortality risk; the need for tracheostomy, CHD, and chromosome anomaly were the most related risk factors of mortality for CAA. Further studies are warranted to clarify the involved mechanisms.

Outcomes of neonatal jaundice in Taiwan.

OBJECTIVE: To investigate the burden of clinically significant neonatal jaundice (SNJ) in Taiwan, 2000-2010. STUDY DESIGN: The nationwide, population-based health insurance database in Taiwan was used to investigate the incidence, kernicterus rate and mortality rates of SNJ cohort born between 2000 and 2010. RESULTS: From 2000 to 2010, up to 242 546 patients admitted with neonatal jaundice (NJ) were identified. The incidence of SNJ was 5.9% in 2000 and increased to 13.7% in 2010 (P<0.001). The mortality rate significantly decreased from 0.51% in 2000 to 0.26% in 2010 (P<0.001) and the average incidence of kernicterus was 0.86 per 100 000 live births, indicating dramatically decreased rates compared with earlier rates in Taiwan. CONCLUSIONS: In spite of the increased incidence rates, the rates of mortality and kernicterus in patients with NJ significantly declined in Taiwan. The public health prevention programme, clinicians' awareness and effective management might contribute to the reduction of these acute severe sequelae.

Reference equations for predicting standing height of children by using arm span or forearm length as an index.

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 < 0.001) in children and 0.933 and 0.845 (p < 0.001) in adults. The linear regression equations for estimating SH in children were calculated as SH = 9.363 + 0.943 ASL (r2 = 0.978, p < 0.001) and SH = 14.542 + 5.570 UL (r2 = 0.936, p < 0.001). In adults, age and sex were also added as indexes: SH = 59.849 + 0.642 ASL-0.047 Age +3.431 Sex (male = 1; female = 0) (r2 = 0.887, p < 0.001) and SH = 102.824 + 2.317 UL -0.049 age + 6.739 sex (r2 = 0.773, p < 0.001). CONCLUSION: Both ASL and UL have a significant linear relationship with SHs of children and adults. True SH can be estimated using regression equations with ASL or UL as a single index for situations where direct measurement of SH is difficult.