Exploring predisposing factors of hearing loss in prematurely born children: A nationwide case-control study.

BACKGROUND: We conducted a nationwide population-based case-control study to analyse potential predisposing factors for hearing loss (HL) that present during the fetal, perinatal, and postnatal periods in prematurely born children. METHODS: This study enrolled 21,576 children born at < 37 weeks of gestation; 3,596 with HL and 17,980 with normal hearing born between 2002 and 2015, matched for sex, age at diagnosis, and enrollment time. Data were abstracted from the concatenation of three nationwide databases for overall risk factors till the diagnosis of HL. RESULTS: Maternal HL, maternal diabetes, particularly type 1 diabetes mellitus, and at or before 32 weeks of gestation were the major obstetric risk factors for HL. Prematurely born children who were born via cesarean section and received a combination of antenatal steroids and magnesium sulfate exhibited a significantly reduced risk of developing HL. Ear malformation was a critical predictor for HL. The major postnatal risk factors included seizure and ototoxic drugs use. Premature infants diagnosed with more than 1 diagnosis of bronchopulmonary dysplasia, necrotizing enterocolitis, and intracerebral hemorrhage were at an increased risk of developing HL. Congenital CMV infection and recurrent acute otitis were also independent postnatal factors for HL in prematurely born children. CONCLUSION: To reduce the incidence of childhood HL in prematurely born children, aggressive management of premature birth-related consequences and treatable causes and longitudinal audiological follow-up with early detection and adequate intervention are crucial.

Weight growth velocity and growth outcomes in very-low-birth-weight infants developing major morbidities.

BACKGROUND: Extrauterine growth restriction (EUGR) is common in very-low-birth-weight-infants and may be associated with poor neurodevelopment. The growth velocity of preterm infants is increasing over decades, but the relationship between growth velocity, EUGR, and morbidities of preterm infants remains unknown. METHODS: A total of 263 infants born between 2012 and 2020, with birthweight <1500 g and gestational age of 24-33 weeks, were included. Birthweight and weight on day of evaluation point (corrected gestational age 36 weeks or discharged, whenever comes first) were converted to age-specific and gender-specific Z-scores and analyzed by multivariable modeling. The average growth velocity was calculated by the exponential model. RESULTS: Average growth velocity from birth to the evaluation point was 11.8 ±â€¯0.3 g/kg/day. The maximum growth velocity from birth to week 8 postnatal occurred at week 4 postnatal (16.4 ±â€¯0.9 g/kg/day). Infants with smaller birth weight, higher gestational age, and indication of intestinal surgery or those who need more days to achieve full enteral feeding were more favorable to have a weight lower than the 10th centile at the evaluation point. By contrast, most comorbidities of prematurity did not affect either lower age-specific weight Z-scores on the evaluation point or larger change in weight Z-score between birth and evaluation point. CONCLUSION: EUGR was associated with gestational age and birth weight. Infants with moderate-to-severe bronchopulmonary dysplasia, high-grade intraventricular hemorrhage, or retinopathy of prematurity tend to have slower growth velocity at 3-5 weeks postnatal, but these did not contribute to EUGR.

Increased antibiotic exposure in early life is associated with adverse outcomes in very low birth weight infants.

BACKGROUND: The use of antibiotics in the early lives of premature infants may alter the microbiota and influence their clinical outcomes. However, whether the administration of probiotics can influence these outcomes remains unknown. In our study, probiotics were routinely administered unless contraindicated. We explored whether increased antibiotic exposure with the routine use of probiotics was associated with necrotizing enterocolitis (NEC) or bronchopulmonary dysplasia (BPD). METHODS: A retrospective cohort study was conducted, enrolling very low birth weight (VLBW) infants admitted between January 1, 2016, and March 31, 2020, to a medical center. Days of antibiotic exposure in the first 14 days of life were recorded. The primary outcomes were NEC and BPD. Adjusted odds ratios (aORs) and 95% confidence intervals (CIs) were calculated using multivariable regression analyses to assess risk factors. RESULTS: Of 185 VLBW infants admitted to the medical center, 132 met the inclusion criteria. Each additional day of antibiotic treatment was associated with increased odds of NEC (aOR, 1.278; 95% CI, 1.025-1.593) and BPD (aOR, 1.630; 95% CI, 1.233-2.156). The association remained in the NEC analysis after adjustment for probiotic use. CONCLUSION: Increased antibiotic exposure in the early lives of VLBW infants was associated with increased risks of NEC and BPD. The probiotics did not influence the outcomes. Our findings suggest that clinicians should be alerted to the adverse outcomes of antibiotic use in infants with VLBWs.

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.

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.

Factors associated with postoperative respiratory conditions and critical outcomes on pediatric liver transplantation: A single-center experience.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

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.

Efficacy of intratracheal budesonide-surfactant combined therapy in surfactant-insufficient rat lungs with lipopolysaccharide insult.

BACKGROUND: Intratracheal steroid therapy for lipopolysaccharide (LPS)-induced acute lung injury (ALI) remains challenging particularly in surfactant-insufficient lungs, a common problem of neonatal or pediatric ALI. 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-insufficient 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 (NS-BUD; BUD in saline), DS-BUD (BUD in diluted surfactant), FS-BUD (BUD in full-strength surfactant), FS (full-strength surfactant). Cardiopulmonary variables were monitored 4 hours post injury. Histological and immunohistochemical assessments of the lungs were performed. RESULTS: The FS-BUD and FS groups presented better gas exchange, less metabolic acidosis, less oxygen index, and more stable hemodynamic changes than the DS-BUD, NS-BUD, and control groups. The total lung injury scores assessed by histological examination were ordered as follows: FS-BUD < DS-BUD or FS < NS-BUD < control. The immunostaining intensities of lung myeloperoxidase showed the following order: NS-BUD, DS-BUD, or FS-BUD < control or FS. Only the FS-BUD group displayed a smaller immunostaining intensity of lung tumor necrosis factor (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-insufficient rat lungs.

Living donor liver transplantation for small infants aged less than 6 months: The experience of a single institute.

PURPOSE: Liver transplantation (LT) for small infants < 6 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 patients <6 months old. METHODS: We identified infants < 6 months old undergoing LDLT between 2004 and 2019 at our hospital. Variables related to recipients, donors, surgeries, and outcomes were analyzed. RESULTS: 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. CONCLUSION: Although LDLT is difficult to perform for small infants, outcomes are favorable and mainly dependent on underlying causes in addition to technical innovations.

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.

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.

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.

Flexible endoscopy for pediatric tracheobronchial metallic stent placement, maintenance and long-term outcomes.

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.

Flexible endoscopic diagnosis and treatment of esophageal stenosis in children with noninvasive ventilation support.

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.

Retrieval of tracheobronchial foreign bodies by short flexible endoscopy in children.

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.

Juvenile Myelomonocytic Leukemia in a Premature Neonate Mimicking Neonatal Sepsis.

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.

Retrieving difficult aspirated pen caps by balloon catheter with short working-length flexible endoscopy and noninvasive ventilation support in intensive care unit.

OBJECTIVES: Aspirated pen cap (APC) is a clinical challenging issue in children because of the difficulty in both making diagnosis and performing extraction. In case of failed retrieval by rigid endoscopy (RE), more invasive surgical approaches are recommended. The objective of this study is to introduce a new retrieval technique of APC by using laser and balloon catheter (BC) guided with flexible endoscopy (FE) and supported by a novel non-invasive ventilation (NIV) in the intensive care unit (ICU) setting. METHODS: We retrospectively review the charts and FE video records of our pediatric cases with the diagnosis of APC in the past decade, 2004-2014. RESULTS: Four consecutive cases with bronchial APC which had failed extraction with RE were transferred to our hospital. All of them were under procedural sedation, topical anesthesia, NIV support and ICU monitoring. After FE confirmed the diagnosis and location, a BC parallel to the endoscope was manipulated to pass through the cap hole of the APC. Two APCs required laser pretreatment before retrieval: one debulked the entrenched granulation whereas another enlarged the size of cap hole. Guide wires also were required in three cases to assist the BC to pass through the cap hole. All four APCs were successfully retrieved on their first attempts with no significant complications. CONCLUSIONS: FE assisted by laser, guide wire and BC, with this NIV support and ICU monitoring is a feasible, safe and effective modality for retrieving those difficult bronchial APCs in pediatric cases.

The risk of cancer in patients with congenital heart disease: a nationwide population-based cohort study in Taiwan.

BACKGROUND: The relationship between congenital heart disease (CHD) and malignancies has not been determined. This study aimed to explore the association of CHD with malignancies and examine the risk factors for the development of cancer after a diagnosis of CHD. PATIENTS AND METHODS: This nationwide, population-based cohort study on cancer risk evaluated 31,961 patients with newly diagnosed CHD using the Taiwan National Health Insurance Research Database (NHIRD) between 1998 and 2006. The standardized incidence ratios (SIRs) for all and specific cancer types were analyzed, while the Cox proportional hazard model was used to evaluate risk factors of cancer occurrence. RESULTS: Among patients with newly diagnosed CHD regardless of ages, 187 (0.6%) subsequently developed cancers after a diagnosis of CHD. Patients with CHD had increased risk of cancer (SIR, 1.45; 95% CI, 1.25-1.67), as well as significantly elevated risks of hematologic (SIR, 4.04; 95% CI, 2.76-5.70), central nervous system (CNS) (SIR, 3.51; 95% CI, 1.92-5.89), and head and neck (SIR, 1.81; 95% CI, 1.03-2.94) malignancies. Age (HR, 1.06; 95% CI, 1.05-1.06) and co-morbid chronic liver disease (HR, 1.91; 95% CI, 1.27-2.87) were independent risk factors for cancer occurrence among CHD patients. CONCLUSION: Patients with CHD have significantly increased cancer risk, particularly hematologic, CNS, and head and neck malignancies. Physicians who care for patients with CHD should be aware of their predisposition to malignancy after the diagnosis of CHD. Further studies are warranted to clarify the association between CHD and malignancies.

Role of Pediatricians in the Ambulatory Care of Children in Taiwan, 1999-2011.

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.