Outcomes of infants with severe bronchopulmonary dysplasia who received tracheostomy and home ventilation.

OBJECTIVE: To describe the survival rate, timing of liberation from the ventilator, and factors favorable for decannulation among infants with severe bronchopulmonary dysplasia (sBPD) who received tracheostomy. METHODS: Demographics and clinical outcomes were obtained through retrospective chart review of 98 infants with sBPD who were born between 2004 and 2017, received tracheostomy at <1 year of age, and were followed in the Infant Tracheostomy and Home Ventilator clinic up to 4 years of age. RESULTS: The number of infants with sBPD who received tracheostomy increased significantly over the study period. The median age at tracheostomy was 4 months (IQR 3, 5) or 43 weeks corrected gestational age; the median age at NICU discharge was 7 months (IQR 6, 9). At 48 months of age, all subjects had been liberated from the ventilator, at a median age of 24 months (IQR 18, 29); 52% had been decannulated with a median age at decannulation of 32 months (IQR 26, 39). Only 1 (1%) infant died. Multivariate logistic regression showed infants who were White, liberated from the ventilator by 24 months of age and have public insurance had significantly greater odds of being decannulated by 48 months of age. Tracheobronchomalacia was associated with decreased odds of decannulation. CONCLUSION: Infants with sBPD who received tracheostomy had an excellent survival rate. Liberation from home ventilation and decannulation are likely to occur by 4 years of age.

Respiratory, growth, and survival outcomes of infants with tracheostomy and ventilator dependence.

BACKGROUND: Outcome of infants with tracheostomy have not been well described in the literature. Our objective was to describe the respiratory, growth, and survival outcomes of infants with tracheostomy. METHODS: A retrospective study was conducted on 204 infants born between 2005 and 2015 with tracheostomy at <1 year of age and follow-up in the Infant Tracheostomy and Home Ventilator Clinic up to 4 years of age. RESULTS: The mean age at tracheostomy was 4.5 months with median age of 3 months. Median age of decannulation was 32 months. The time from tracheostomy placement to complete discontinuation of mechanical ventilation was 15.4 months and from tracheostomy to decannulation was 33.8 months. Mortality rate was 21% and median age of death was 18 months. Preterm infants with acquired airway and lung disease (BPD) and born at <28 weeks' gestation had a significantly higher survival rate compared to term infants. The z-scores for weight and weight for length improved from the time of discharge (mean chronological age 6.5 months) to first year and remained consistent through 3 years. CONCLUSIONS: Premature infants had a higher rate of discontinuation of mechanical ventilation and decannulation compared to term infants. These infants showed consistent growth and comparable survival rate. IMPACT: Infants with tracheostomy and ventilator dependence followed in a multidisciplinary clinic model may have improved survival, growth, and earlier time to decannulation. Preterm infants with acquired airway and lung disease (BPD) with tracheostomy had a higher survival rate compared to term infants with various tracheostomy indications. The age at tracheostomy in infants was 4.5 months and of decannulation was 37 months. Time from tracheostomy to complete discontinuation of mechanical ventilation was 15.4 months. Addition of this data to the sparse literature will be crucial in counseling the families and education of medical staff.

Neonatal Graves' Disease with Maternal Hypothyroidism.

Neonatal Graves' disease presenting as conjugated hyperbilirubinemia is a diagnostic challenge because the differential includes a gamut of liver and systemic diseases. We present a unique case of neonatal Graves' disease in a premature infant with conjugated hyperbilirubinemia born to a mother with hypothyroidism during pregnancy and remote history of Graves' disease. Infant was treated with a combination of methimazole, propranolol, and potassium iodide for 4 weeks. Thyroid function improved after 8 weeks of treatment with full recovery of thyroid function, disappearance of thyroid-stimulating antibodies, and resolution of failure to thrive and conjugated hyperbilirubinemia. This case provides several clinical vignettes as it is a rare, severe, presentation of an uncommon neonatal disease, signs, symptoms, and clinical history presented a diagnostic challenge for neonatologists and endocrinologists, normal newborn screen was misleading, and yet timely treatment led to a full recovery.

A Novel Method of Measuring Fractional Exhaled Nitric Oxide in Tracheostomized Ventilator-Dependent Children.

BACKGROUND: The lower airway concentration of fractional exhaled nitric oxide (FENO) is unknown in children with chronic lung disease of infancy who have tracheostomy for long-term mechanical ventilation. We aimed to evaluate an online method of measuring FENO in a cohort of ventilator-dependent children with a tracheostomy and to explore the relationship between the peak FENO concentration (FENO peak) and the degree of respiratory support using the respiratory severity score. METHODS: We conducted a prospective cross-sectional study in 31 subjects who were receiving long-term respiratory support through a tracheostomy. We measured the FENO peak and FENO plateau concentration from the tip of the tracheostomy tube using a nitric oxide analyzer in subjects during a quiet state while being mechanically ventilated. We obtained 2 consecutive 2-min duration measurements from each subject. The FENO peak, exhaled NO output (equal to the FENO peak × minute ventilation), and pulmonary NO excretion (exhaled NO output/weight) were calculated and correlated with the respiratory severity score. RESULTS: The median FENO peak was 2.69 ppb, and the median FENO plateau was 1.57 ppb. The coefficients of repeatability between the 2 consecutive measurements for FENO peak and FENO plateau were 0.74 and 0.59, respectively. The intraclass coefficient between subjects within the cohort was 0.988 (95% CI 0.975-0.994, P < .001) for FENO peak and 0.991 (95% CI 0.982-0.996, P < .001) for FENO plateau. We found that the FENO peak was directly correlated with minute ventilation, but we did not find a direct relationship between the FENO peak concentration, exhaled NO output, or pulmonary NO excretion and respiratory severity score. CONCLUSIONS: FENO peak and plateau concentration can be measured online easily with a high degree of reliability and repeatability in infants and young children with a tracheostomy. FENO peak concentration from the lower airway is low and influenced by minute ventilation in children receiving mechanical ventilation.

Outcomes of Tracheostomy in Children Requiring Surgery for Congenital Heart Disease.

Outcomes after discharge in children requiring tracheostomy after cardiac surgery have not been fully described. A retrospective, single-center study was performed on all children <18 years of age requiring both tracheostomy and surgery for congenital heart disease from January 2002 to May 2015. Forty-six tracheostomies were placed after surgery and four before. Single-ventricle anatomy was present in 12 (33%) patients. Incidence of tracheostomy after heart surgery increased from 0.8% the first half of the study period to 2% the second half. Median time between cardiac surgery and tracheostomy was 58 days. The most common indication for tracheostomy was multifactorial (30%) followed by airway malacia (22%). Median length to follow-up for survivors was 3.9 years (range 0.4-11.8 years). Survival to hospital discharge was 72%, and intermediate survival was 48%. Survival in those with systemic to pulmonary artery shunts at the time of tracheostomy was 22% compared to 59% for those with biventricular anatomy. Heart failure and multiple indications for tracheostomy were associated with worse outcome. There was no difference in survival for those discharged with a ventilator compared to those that were not. The most common cause of death after discharge was tracheostomy tube dislodgement/obstruction, accounting for 5 of 11 that died. Survival with a tracheostomy after cardiac surgery is poor, and children with systemic to pulmonary artery shunts are at especially high risk of death.

Tracheobronchomalacia diagnosed by tracheobronchography in ventilator-dependent infants.

BACKGROUND: Tracheobronchomalacia prevalence in premature infants on prolonged mechanical ventilation is high. OBJECTIVE: To examine the prevalence of tracheobronchomalacia diagnosed by tracheobronchography in ventilator-dependent infants, and describe the demographic, clinical and dynamic airway characteristics of those infants with tracheobronchomalacia. MATERIALS AND METHODS: This retrospective review studies 198 tracheobronchograms performed from 1998 to 2011 in a cohort of 158 ventilator-dependent infants <2 years of age. Dynamic airway assessment during tracheobronchography determined the optimal positive end-expiratory pressure to maintain airway patency at expiration in those infants with tracheobronchomalacia. RESULTS: Tracheobronchograms were performed at a median age of 52 weeks post menstrual age. The primary diagnoses in these infants were bronchopulmonary dysplasia (53%), other causes of chronic lung disease of infancy (28%) and upper airway anomaly (13%). Of those with bronchopulmonary dysplasia, 48% had tracheobronchomalacia. Prematurity (P=0.01) and higher baseline - pre-tracheobronchogram positive end-expiratory pressure (P=0.04) were significantly associated with tracheobronchomalacia. Dynamic airway collapse during tracheobronchography showed statistically significant airway opening at optimal positive end-expiratory pressure (P < 0.001). There were no significant complications noted during and immediately following tracheobronchography. CONCLUSION: The overall prevalence of tracheobronchomalacia in this cohort of ventilator-dependent infants is 40% and in those with bronchopulmonary dysplasia is 48%. Infants born prematurely and requiring high pre-tracheobronchogram positive end-expiratory pressure were likely to have tracheobronchomalacia. Tracheobronchography can be used to safely assess the dynamic function of the airway and can provide the clinician the optimal positive end-expiratory pressure to maintain airway patency.