Type one chiari malformation as a cause of central sleep apnea and hypoventilation in children.

OBJECTIVES: Chiari type 1 malformation (CM1) may occasionally lead to central sleep apnea (CSA). We studied, in a large clinical cohort of pediatric CM1 patients, the effect of CM1 on breathing during sleep. METHODS: This is a retrospective single pediatric pulmonology center study with a systematic evaluation of pediatric CM1 patients under age 18 with polysomnography (PSG) during 2008-2020. Children with syndromes were excluded. All patients had undergone head and spine magnetic resonance imaging. RESULTS: We included 104 children with CM1 with a median age of 7 (interquartile range (IQR) 5-13) years. The median extent of tonsillar descent (TD) was 13 (IQR 10-18) mm. Syringomyelia was present in 19 children (18%). Of all children, 53 (51%) had normal PSG, 35 (34%) showed periodic breathing or central apnea and hypopnea index ≥5 h-1, and 16 (15%) displayed features of compensated central hypoventilation and end-tidal or transcutaneous carbon dioxide 99th percentile level above 50 mmHg. TD had the best predictive value for central breathing disorders. In a linear model, both age (61%) and TD (39%) predicted median breathing frequency (R = 0.33, p < 0.001). CONCLUSIONS: Although severe CSA is a rare complication of brainstem compression in pediatric patients with CM1, short arousal-triggered episodes of periodic breathing and mild compensated central hypoventilation are common. TD shows the best but still poor prediction of the presence of a central breathing disorder. This highlights the use of PSG in patient evaluation. Posterior fossa decompression surgery effectively treats central breathing disorders.

Children's interstitial lung disease: Multidetector computed tomography patterns and correlations between imaging and histopathology.

PURPOSE: Childhood interstitial lung disease (chILD) is an umbrella concept covering a wide range of rare lung diseases, many of which are unique to childhood. The diagnosis is based on clinical presentation, multidetector computed tomography (MDCT), genetic testing, lung-function testing, and lung biopsy. Because knowledge of the usefulness of MDCT pattern recognition in ChILD is at present limited, we examined the occurrence of MDCT patterns in children with histologically confirmed interstitial lung disease. METHOD: We searched the biopsy, MDCT, and clinical information database of a single national paediatric referral hospital for 2004-2020. Data were from affected children under age 18. MDCT images we reanalysed while blinded to the identity and referral information. RESULTS: We included 90 patients, of whom 63 (70 %) were male. The median age at biopsy was 1.3 years (interquartile range 0.1-16.8). Biopsy findings fell into 26 histological classes covering all nine chILD classification categories. We recognized six distinct MDCT patterns: neuroendocrine cell hyperplasia of infancy (23), organizing pneumonia (5), non-specific interstitial pneumonia (4), bronchiolitis obliterans (3), pulmonary alveolar proteinosis (2), and bronchopulmonary dysplasia (n = 2). Of the total 90, in 51 (57 %) children, none of these six MDCT patterns appeared. Of those 39 children with a recognizable MDCT pattern, in 34 (87 %), that pattern predicted their final diagnosis. CONCLUSIONS: Among cases of chILD, we identified a specific predefined MDCT pattern in only 43 %. However, when such a recognizable pattern occurred, it was predictive of the final chILD diagnosis.

Sleep-related breathing disorder in non-infectious pulmonary complications after pediatric allogeneic stem cell transplantation.

BACKGROUND: Chronic lung problems are a rare but serious complication of allogeneic hematopoietic stem cell transplantation (HSCT). We studied clinical phenotypes and polysomnography appearance of breathing abnormality in late onset non-infectious pulmonary complications (NIPS). METHODS: We reviewed Finnish national reference database between the years 1999 and 2016. We identified 12 children with most severely decreased pulmonary function and performed polysomnography and 24 aged-matched controls out of 325 performed pediatric allogeneic HSCTs. RESULTS: All patients with NIPS had severely decreased pulmonary function already at 6 months post HSCT with median FEV1 value 42% (interquartile range (IQR) 30-52%) of predicted normal values. Seven children had obstructive and five children more restrictive lung function. Children with obstructive lung function showed laborious breathing (7/7), decreased oxygenation and ventilation-to-perfusion mismatch (6/7), or REM-sleep-related hypoventilation (4/7) on polysomnography. Children with restrictive lung function (5/12) did not show sleep-related breathing disorder. CONCLUSIONS: Children going through allogeneic HSCT who develop severe chronic obstructive lung function are more likely to present with sleep-related hypoxia and hypoventilation than children with restrictive lung function. IMPACT: Children with severe obstructive lung function and chronic lung graft-versus-host disease following hematopoietic stem cell transplantation are more likely to present with sleep-related mild hypoxia and hypoventilation than children with restrictive lung disease. To our knowledge there are no reports on sleep-related breathing disorders and ventilatory function measured by polysomnography in children with pulmonary complications after allogeneic HSCT. Polysomnography may add to the differential diagnostics between patients with BOS and other non-infectious pulmonary complications.

Ventilator-derived dynamic respiratory system compliance: Comparison with static compliance in children.

Measurement of dynamic lung compliance during breathing requires measurement of esophageal pressure, whereas static respiratory system compliance (Crs) method requires several airway occlusions. Despite their precision these compliance methods are cumbersome and not suitable for evaluation of pulmonary system in intensive care. The current ventilators display dynamic Crs, which, however, is seldom utilized in clinical practice. We studied the feasibility of ventilator-derived dynamic Crs measurement in pulmonary evaluation after congenital cardiac surgery in children. In 50 children static Crs was measured by double-occlusion technique, and compared with simultaneous ventilator-derived dynamic Crs values. The early postoperative dynamic and static Crs showed a correlation (r = 0.57, p < 0.0001), but static Crs was 48% higher than dynamic (p < 0.0001). Dynamic Crs measurement showed no correlation with radiographic lung edema findings, whereas the static Crs showed a negative correlation with radiographic lung edema scoring (r = -0.50, p = 0.0002). Thus ventilator-derived dynamic Crs seems less reliable in postoperative pulmonary evaluation than static Crs.

Assessment of extravascular lung water by ultrasound after congenital cardiac surgery.

BACKGROUND: Lung ultrasounds show vertical artifacts known as B-lines in the presence of increased extravascular lung water (EVLW). We aimed to investigate whether lung ultrasound could estimate EVLW after congenital cardiac surgery. METHODS: This prospective observational study comprised 61 children (age range 3 days to 7.4 years) undergoing congenital cardiac surgery. We compared postoperative B-line scores from lung ultrasounds, early postoperative ultrasound as our primary interest, with corresponding postoperative chest radiography (CXR) lung edema scores, with static lung compliance, and with short-term clinical outcome interpreted as time on mechanical ventilation and length of pediatric intensive care unit (PICU) stay. RESULTS: Our findings showed lung ultrasound B-line scores and CXR lung edema scores as correlating 1-6 hr postoperatively (r2 = 0.41, P < 0.0001), on the first postoperative day (r2 = 0.15, P = 0.004) and on the fourth postoperative day (r2 = 0.28, P = 0.008). The B-line score or CXR lung edema score showed no correlation with lung compliance. We found that in multivariable analyses, with length of perfusion and presence of postoperative complications as covariates, both lung ultrasound (P ≤ 0.02) and CXR (P ≤ 0.002) 1-6 hr postoperatively predicted the length of mechanical ventilation and PICU stay. The interobserver variability was less for lung ultrasound B-line score than for CXR lung edema score (P = 0.001). CONCLUSIONS: Our results show that lung ultrasound in assessment of postoperative EVLW predicted length of mechanical ventilation and stay in the PICU, and it had less interobserver variability than CXR. Accordingly, lung ultrasound may complement CXR in assessment of lung edema after surgery for congenital heart defect. Pediatr Pulmonol. 2017;52:345-352. © 2016 Wiley Periodicals, Inc.

Chronic Hypoxemia in Children With Congenital Heart Defect Impairs Airway Epithelial Sodium Transport.

OBJECTIVE: Ambient hypoxia impairs the airway epithelial Na transport, which is crucial in lung edema reabsorption. Whether chronic systemic hypoxemia affects airway Na transport has remained largely unknown. We have therefore investigated whether chronic systemic hypoxemia in children with congenital heart defect affects airway epithelial Na transport, Na transporter-gene expression, and short-term lung edema accumulation. DESIGN: Prospective, observational study. SETTING: Tertiary care medical center responsible for nationwide pediatric cardiac surgery. PATIENTS: Ninety-nine children with congenital heart defect or acquired heart disease (age range, 6 d to 14.8 yr) were divided into three groups based on their level of preoperative systemic hypoxemia: 1) normoxemic patients (SpO2% ≥ 95%; n = 44), 2) patients with cyanotic congenital heart defect and moderate hypoxemia (SpO2 86-94%; n = 16), and 3) patients with cyanotic congenital heart defect and profound systemic hypoxemia (SpO2 ≤ 85%; n = 39). MEASUREMENTS AND MAIN RESULTS: Nasal transepithelial potential difference served as a surrogate measure for epithelial Na transport of the respiratory tract. Profoundly hypoxemic patients had 29% lower basal nasal transepithelial potential difference (p = 0.02) and 55% lower amiloride-sensitive nasal transepithelial potential difference (p = 0.0003) than normoxemic patients. In profoundly hypoxemic patients, nasal epithelial messenger RNA expressions of two airway Na transporters (amiloride-sensitive epithelial Na channel and ß1- Na-K-ATPase) were not attenuated, but instead α1-Na-K-ATPase messenger RNA levels were higher (p = 0.03) than in the normoxemic patients, indicating that posttranscriptional factors may impair airway Na transport. The chest radiograph lung edema score increased after congenital cardiac surgery in profoundly hypoxemic patients (p = 0.0004) but not in patients with normoxemia or moderate hypoxemia. CONCLUSIONS: The impaired airway epithelial amiloride-sensitive Na transport activity in profoundly hypoxemic children with cyanotic congenital heart defect may hinder defense against lung edema after cardiac surgery.

All snoring is not adenoids in young children.

OBJECTIVE: To determine the prevalence of snoring in young children and to assess age, growth, previous surgery therapy, respiratory problems and sleep-related symptoms in relation to child's snoring, and to evaluate the relationship between child's snoring and parents' snoring and smoking. CHILDREN AND METHODS: A cross-sectional study evaluated 2100 children 1-6 years of age in Helsinki, Finland. Child's frequency of snoring on a five-point scale (never to every night) and age, height, weight and body mass index, previous adenotonsillectomies, tympanostomies, allergic rhinitis and respiratory infections were determined as was frequency of parental snoring and smoking. Sleep problems were determined based on Finnish or Swedish modified version of the sleep disturbance scale for Children. RESULTS: Of the 2100 eligible children, 1471 (71%) returned questionnaires. Children always or often snoring numbered 92 (6.3%), sometimes snoring, 183 (12.4%), and never or occasionally snoring, 1196 (81.3%). No difference in age (p=0.06) or gender (p=0.39) existed between snorers and non-snorers. History of previous adenotonsillectomies (p<0.001), allergic rhinitis (p<0.001), recurrent respiratory infections (p<0.001), and otitis media (p<0.001) were more common among snorers than among occasional or never-snorers. Nocturnal symptoms such as breathing problems, sleep hyperhydrosis, sleep-wake transition disorders, and daytime somnolence were associated with children's snoring. Frequency of children's snoring was also associated with parental snoring (p<0.001) and smoking (p<0.001). CONCLUSIONS: Snoring is common among young children and is associated with previous adenotonsillectomy, allergic rhinitis, respiratory infections, nocturnal symptoms, and parents' snoring and smoking. The high prevalence of snoring among children with adenotonsillectomy raises the question whether adenotonsillectomy alone is adequate treatment for snoring in young children.

Altered cardiovascular control in preterm infants with bronchopulmonary dysplasia.

Vestibulo-mediated cardiovascular control in hazardous situations is important. Our hypothesis is that the prerequisite for sudden infant death syndrome (SIDS) is impaired vestibulo-mediated cardiovascular control. Prematurity is a risk factor for SIDS, and postnatal intermittent hypoxia may contribute to this risk. We studied heart rate (HR) and blood pressure (BP) responses in 10 infants with bronchopulmonary dysplasia (BPD) who were born at 27 +/- 2.4 (23-30) wk of gestation. Twenty healthy term infants served as controls. Cardiovascular tests were performed under polysomnographic control during slow-wave sleep (SWS) at a corrected age of 12 +/- 3.5 (7-19) wk. Control infants showed biphasic HR and BP responses to side motion with an immediate increase followed by a modest decrease and return to baseline. Compared with the controls, half of the BPD infants had altered BP responses (p < 0.005) without an early increase, followed by a more prominent decrease in BP. BPD infants also presented with a greater variability in BP responses to head-up tilts than did the controls (p < 0.001). In conclusion, these findings suggest that some BPD infants have impaired vestibular sympathoreflex-mediated cardiovascular control. This dysfunction may become critical in life-threatening situations.

Sodium transport in airway epithelium correlates with lung compliance in healthy newborn infants.

To study the relation between sodium transport in airway epithelium and postnatal pulmonary adaptation, we measured nasal potential difference at 1 to 4 hours and lung compliance at 21 to 48 hours after birth in 20 healthy infants. Sodium transport correlated with lung compliance ( r 2 = 0.40, P < .003). Airway sodium transport plays a role in postnatal pulmonary adaptation.