Pediatric bronchoscopy: recent advances and clinical challenges.

Introduction: During the last 40 years equipment has been improved with smaller instruments and sufficient size working channels. This has ensured that bronchoscopy offers therapeutic and interventional options.Areas covered: We provide a review of recent advances and clinical challenges in pediatric bronchoscopy. This includes single-use bronchoscopes, endobronchial ultrasound, and cryoprobe. Bronchoscopy in persistent preschool wheezing and asthma is included. The indications for interventional bronchoscopy have amplified and included balloon dilatation, endoscopic intubation, the use of airway stents, whole lung lavage, closing of fistulas and air leak, as well as an update on removal of foreign bodies. Others include the use of laser and microdebrider in airway surgery. Experience with bronchoscope during the COVID-19 pandemic has been included in this review. PubMed was searched for articles on pediatric bronchoscopy, including rigid bronchoscopy as well as interventional bronchoscopy with a focus on reviewing literature in the past 5 years.Expert opinion: As the proficiency of pediatric interventional pulmonologists continues to grow more interventions are being performed. There is a scarcity of published evidence in this field. Courses for pediatric interventional bronchoscopy need to be developed. The COVID-19 experience resulted in safer bronchoscopy practice for all involved.

Short-term effects of physiotherapy on ventilation inhomogeneity in cystic fibrosis patients with a wide range of lung disease severity.

BACKGROUND: Lung clearance index (LCI) is increasingly used as a study endpoint for therapeutic interventions in cystic fibrosis (CF) patients. We set out to assess the effect of chest physiotherapy on ventilation inhomogeneity in clinically stable patients with CF lung disease of varying severity. METHODS: In 29 CF patients (7.3-43.7 years) N2MBW (nitrogen multiple breath washout), plethysmography, and spirometry measurements were conducted, followed by 30 min of supervised PEP mask chest physiotherapy and repeated measurements 30 min after therapy. RESULTS: We observed a mean change in LCI after physiotherapy from 15.00 to 14.80 (range, -4.84 to 3.37; p=0.578). In seven patients, LCI decreased, and in ten patients, LCI increased by ≥1. For the whole group, statistically significant improvements were seen in Reff, FEV1, FVC, and MEF50. CONCLUSIONS: By opening up previously poorly ventilated lung regions, physiotherapy may either increase or decrease ventilation inhomogeneity; the short-term effect of physiotherapy on LCI appears to be unpredictable.

Antenatal and postnatal management of congenital cystic adenomatoid malformation.

Congenital thoracic malformations (CTMs) are a heterogeneous group of rare disorders that may involve the airways or lung parenchyma. The authors have focused on the condition that causes the most controversy, namely, congenital cystic adenomatoid malformation (CCAM). The reported incidence is 3.5 and 0.94 per 10,000 live births for CTMs and CCAMs respectively. Ultrasound is the antenatal imaging modality of choice for screening for CCAMs whilst magnetic resonance imaging is complimentary for morphological and volumetric evaluation of the foetal lung. Most CCAMs are detected antenatally with only a small proportion presenting postnatally. Only a few CCAMs cause foetal problems, with foetal hydrops being the best predictor of death. Although many CCAMs regress during pregnancy, most remain detectable postnatally by CT scans. Surgical excision of symptomatic lesions is relatively straightforward, but management of asymptomatic lesions is controversial. Some surgeons adopt a "wait and see" approach operating only on those patients who develop symptoms, but others operate on asymptomatic patients usually within the first year of life. Due to the potential of malignant transformation, children should have long term follow up. There is an urgent need to delineate the natural history of antenatally detected CCAMs to guide future management.

Prospective evaluation of clinical scoring systems in infants with bronchiolitis admitted to the intensive care unit.

The objective of this investigation was to compare different scoring systems to assess the severity of illness in infants with bronchiolitis admitted to a tertiary paediatric intensive care unit (PICU). Over an 18-year period (1990-2007), infants with bronchiolitis aged up to 12 months and admitted to the PICU were prospectively scored using the Pediatric Risk of Mortality III (PRISM III) score, the Organ System Failure (OSF) score and the Acute Physiologic Score for Children (APSC) within 24 h. Infants were compared as to whether or not bronchiolitis was associated with respiratory syncytial virus (RSV). There was no difference between 113 RSV-positive and 80 RSV-negative infants regarding gestational age, birth weight, rate of premature delivery or bronchopulmonary dysplasia (BPD). The PRISM III score differed significantly between RSV-positive and RSV-negative cases (3.27 ± 0.39 vs. 1.96 ± 0.44, p = 0.006), as did the OSF score (0.56 ± 0.05 vs. 0.35 ± 0.06, p = 0.049) and the APSC (5.16 ± 0.46 vs. 4.1 ± 0.53, p = 0.048). All scores were significantly higher in the subgroup with mechanical ventilation (p < 0.0001). The mean time of ventilation was significantly higher in the RSV-positive group compared to the RSV-negative group (6.39 ± 1.74 days vs. 2.4 ± 0.47 days, p < 0.001). Infants suffering from RSV-positive bronchiolitis had higher clinical scores corresponding with the severity of bronchiolitis.

Congenital diaphragmatic hernia.

Infants with congenital diaphragmatic hernia (CDH) have significant mortality and long-term morbidity. Only 60-70% survive and usually those in high-volume centres. The current Task Force, therefore, has convened experts to evaluate the current literature and make recommendations on both the antenatal and post-natal management of CDH. The incidence of CDH varies from 1.7 to 5.7 per 10,000 live-born infants depending on the study population. Antenatal ultrasound scanning is routine and increasingly complemented by the use of magnetic resonance imaging. For isolated CDH, antenatal interventions should be considered, but the techniques need vigorous evaluation. After birth, management protocols are often used and have improved outcome in nonrandomised studies, but immediate intubation at birth and gentle ventilation are important. Pulmonary hypertension is common and its optimal management is crucial as its severity predicts the outcome. Usually, surgery is delayed to allow optimal medical stabilisation. The role of minimal invasive post-natal surgery remains to be further defined. There are differences in opinion about whether extracorporeal membrane oxygenation improves outcome. Survivors of CDH can have a high incidence of comorbidities; thus, multidisciplinary follow-up is recommended. Multicentre international trials are necessary to optimise the antenatal and post-natal management of CDH patients.

Paediatrics in Barcelona: highlights from the 2010 ERS Annual Congress.

The aim of this update is to describe the paediatric highlights from the 2010 European Respiratory Society Annual Congress in Barcelona, Spain. Abstracts from the seven groups of the Paediatric Assembly (Respiratory physiology, Asthma and allergy, Cystic fibrosis, Respiratory infection and immunology, Neonatology and paediatric intensive care, Respiratory epidemiology and Bronchology) are presented in the context of the current literature.

Medicines used in respiratory diseases only seen in children.

Detailed literature searches were carried out in seven respiratory disease areas. Therapeutic evidence for efficacy of medicinal products was assessed using the Grades of Recommendation, Assessment and Evaluation (GRADE) methodology, as well as an assessment of safety and side-effects. Systemic corticosteroids may reduce the development of bronchopulmonary dysplasia but have serious side-effects. Antioxidants need further study to demonstrate whether they have long-term benefits. Treatments for acute bronchiolitis have shown little benefit but new antiviral and monoclonal antibodies need further assessment. Well-constructed studies are needed to confirm the value of inhaled corticosteroids and/or montelukast in the management of viral-induced wheeze. Corticosteroids are the treatment of choice in croup. Minimal or no information is available for the treatment of congenital lung abnormalities, bronchiolitis obliterans and interstitial lung disease.

Definition, assessment and treatment of wheezing disorders in preschool children: an evidence-based approach.

There is poor agreement on definitions of different phenotypes of preschool wheezing disorders. The present Task Force proposes to use the terms episodic (viral) wheeze to describe children who wheeze intermittently and are well between episodes, and multiple-trigger wheeze for children who wheeze both during and outside discrete episodes. Investigations are only needed when in doubt about the diagnosis. Based on the limited evidence available, inhaled short-acting beta(2)-agonists by metered-dose inhaler/spacer combination are recommended for symptomatic relief. Educating parents regarding causative factors and treatment is useful. Exposure to tobacco smoke should be avoided; allergen avoidance may be considered when sensitisation has been established. Maintenance treatment with inhaled corticosteroids is recommended for multiple-trigger wheeze; benefits are often small. Montelukast is recommended for the treatment of episodic (viral) wheeze and can be started when symptoms of a viral cold develop. Given the large overlap in phenotypes, and the fact that patients can move from one phenotype to another, inhaled corticosteroids and montelukast may be considered on a trial basis in almost any preschool child with recurrent wheeze, but should be discontinued if there is no clear clinical benefit. Large well-designed randomised controlled trials with clear descriptions of patients are needed to improve the present recommendations on the treatment of these common syndromes.

Arthralgia and digital clubbing in a child: hypertrophic osteoarthropathy with inflammatory pseudotumour of the lung.

Arthralgia in childhood is an unspecific symptom. One rare cause of arthralgia is hypertrophic osteoarthropathy associated with digital clubbing. We present a child where hypertrophic osteoarthropathy led to the rare diagnosis of an inflammatory pseudotumour of the lung. In a 12-year-old girl with arthralgia and digital clubbing, a chest radiograph disclosed a large round mass in the right upper lobe, and the following chest computed tomography scan showed a large solid homogenous, round, well marginated lesion with little contrast enhancement. A lobectomy of the right upper lobe was performed, and histological examination showed an inflammatory pseudotumour. The postoperative course was without problems: arthralgia and digital clubbing disappeared.

Flexible endoscopy of paediatric airways.

Paediatric fibreoptic bronchoscopy is used for ever wider indications, and increasingly used in many contexts, including paediatric and neonatal intensive care. The report of this Task Force contains an overview on the current applications of paediatric bronchoscopy. The report discusses the facilities and equipment needed for the procedure, including the newly developed bronchoscopes which are allowing intervention even in very small children. The indications of both flexible and rigid bronchoscopes in the context of newer and smaller flexible endoscopic equipment are also considered. The care of the instruments, including disinfection and sterilisation, is fully documented. Patient management is described, including the relative merits of conscious sedation and general anaesthesia, as well as special settings for the procedure, including the needs in intensive care. Special procedures, increasingly performed bronchoscopically are described. These include bronchoalveolar lavage, endobronchial and transbronchial biopsy, laser therapy, bronchography, and endoscopic intubation and drug therapy. Finally, neonatal bronchoscopy is discussed, and the ethics of bronchoscopic procedures, including bronchoscopic research in children. Advances in instrumentation, and also improved anaesthetic techniques, allow fibreoptic bronchoscopy to be safely performed in even very small, sick infants, provided proper precautions are taken.

Partial splenectomy in cystic fibrosis patients with hypersplenism.

We report three cystic fibrosis (CF) patients with hypersplenism who underwent partial splenectomy. The postoperative course was uneventful in two patients; one patient developed a complication necessitating resection of the rest of the spleen. Haematological parameters improved and oesophageal varices regressed in all patients. On follow up, one patient showed a normal spleen, the other a normally functioning accessory spleen; the third patient again developed splenomegaly with hypersplenism. Partial splenectomy is a promising therapeutic option for CF patients with hypersplenism.

Laser acupuncture in children and adolescents with exercise induced asthma.

BACKGROUND: Laser acupuncture, a painless technique, is a widely used alternative treatment method for childhood asthma, although its efficacy has not been proved in controlled clinical studies. METHODS: A double blind, placebo controlled, crossover study was performed to investigate the possible protective effect of a single laser acupuncture treatment on cold dry air hyperventilation induced bronchoconstriction in 44 children and adolescents of mean age 11.9 years (range 7.5-16.7) with exercise induced asthma. Laser acupuncture was performed on real and placebo points in random order on two consecutive days. Lung function was measured before laser acupuncture, immediately after laser acupuncture (just before cold dry air challenge (CACh)), and 3 and 15 minutes after CACh. CACh consisted of a 4 minute isocapnic hyperventilation of -10 degrees C absolute dry air. RESULTS: Comparison of real acupuncture with placebo acupuncture showed no significant differences in the mean maximum CACh induced decrease in forced expiratory volume in 1 second (27.2 (18.2)% v 23.8 (16.2)%) and maximal expiratory flow at 25% remaining vital capacity (51.6 (20.8)% v 44.4 (22.3)%). CONCLUSIONS: A single laser acupuncture treatment offers no protection against exercise induced bronchoconstriction in paediatric and adolescent patients.

Clinical and histopathological findings in two Turkish children with follicular bronchiolitis.

UNLABELLED: We report on two Turkish children who presented with progressive airway obstruction. Open lung biopsy revealed follicular bronchiolitis. The children were treated with systemic steroids and various topical medications. Whereas the respiratory situation of patient 1 required immunosuppressive therapy, the condition of patient 2 stabilised without systemic medication. CONCLUSION: Diagnosis of follicular bronchiolitis should be considered when children present with recurrent respiratory tract infections, progressive dyspnoea, and chronic bronchial obstruction. Children in whom follicular bronchiolitis is suspected should undergo open lung biopsy for confirmation of diagnosis.