Management of obstructive sleep apnea syndrome type 1 in children and adolescents - A French consensus.

This document is the outcome of a group of experts brought together at the request of the French Society of Sleep Research and Medicine to provide recommendations for the management of obstructive sleep apnea syndrome type 1 (OSA1) in children. The recommendations are based on shared experience and published literature. OSA1 is suspected when several nighttime respiratory symptoms related to upper airway obstruction are identified on clinical history taking. A specialist otolaryngologist examination, including nasofibroscopy, is essential during diagnosis. A sleep study for OSA1 is not mandatory when at least two nighttime symptoms (including snoring) are noted. Therapeutic management must be individualized according to the location of the obstruction. Ear, nose, and throat (ENT) surgery is often required, as hypertrophy of the lymphoid tissues is the main cause of OSA1 in children. According to clinical findings, orthodontic treatment generally associated with specialized orofacial-myofunctional therapy might also be indicated. Whatever treatment is chosen, follow-up must be continuous and multidisciplinary, in a network of trained specialists.

Sniff nasal inspiratory pressure in children with muscular, chest wall or lung disease.

Sniff nasal inspiratory pressure is proposed as a noninvasive test of inspiratory muscle strength. During this manoeuvre, the nasal pressure is supposed to reflect oesophageal pressure. The aim of the present study was to compare the nasal pressure with the oesophageal pressure during a maximal sniff in children with neuromuscular disease (NM, n = 78), thoracic scoliosis (n = 12) and cystic fibrosis (CF, n = 23). A significant correlation was observed between the sniff nasal and oesophageal pressure. The ratio of the sniff nasal/oesophageal pressure was lower in patients with CF (0.72+/-0.13) than in NM patients (0.83+/-0.17) or patients with thoracic scoliosis (0.86+/-0.10). In patients with CF and NM disease, this ratio was not correlated to age or spirometric data. The difference between the sniff oesophageal and nasal pressure exceeded 15 cm H(2)O in 17, 33 and 87% of the NM, thoracic scoliosis and CF patients, respectively. Sniff nasal pressure often underestimates the strength of inspiratory muscles in cystic fibrosis. Such an underestimation occurs more rarely in neuromuscular disease disorders and thoracic scoliosis. A normal value excludes inspiratory muscle weakness but a low value requires the measurement of the oesophageal pressure.

[Obesity in childhood: What are the respiratory risks?] / Prise de poids chez l'enfant : quels risques respiratoires ?

In childhood and adolescence overweight is defined as a body mass index (BMI) above the 97th percentile for age and sex, according to the curves established by the International Obesity Task Force (IOTF). In France, it is estimated that 25 % of children under 18 years old are overweight. Overweight and obesity in this population are multifactorial, with an important influence of genetic factors, modulated by pre and post-natal (maternal smoking), societal and psychological determinants. The impact of obesity on respiratory function in children is mostly characterized by a decreased FEV1/FCV. Moreover, several studies have shown an association between asthma and overweight/obesity, with a pejorative impact of BMI on asthma control. However, asthma is still poorly characterized in this population, and the determinants of bronchial obstruction seem to differ from non-obese children, with less eosinophilic inflammation. Obstructive sleep apnea syndrome (OSAS) is a frequent complication of obesity, affecting up to 80% of obese children and adolescents. It has a specific polysomnographic definition in children. Symptoms are similar to adult OSAS, but with cognitive and neurobehavioral alterations often more important in adolescents. The treatment consists in ENT surgery when indicated (with systematic post-operative polysomnography), and nocturnal continuous positive airway pressure (CPAP). The obesity-hypoventilation syndrome (OHS) has the same definition in children as in adults and affects up to 20% of obese patients. Treatment consists in nocturnal ventilation using bilevel positive airway pressure (BiPAP). Finally, in some extreme cases, bariatric surgery can be performed. The indication should be discussed in a specialised paediatric reference centre.

[Pneumocystis jiroveci pneumonia during prolonged corticosteroid therapy in an immunocompetent infant]. / Pneumonie àPneumocystis jiroveci au cours d'une corticothérapie prolongée chez un nourrisson immunocompétent.

INTRODUCTION: Pneumocystis jiroveci (PJ) infection is rare in infants and is suggestive of primary or secondary immunodeficiency. We report on a case of severe PJ pneumonia in an immunocompetent infant after prolonged corticosteroid treatment. CASE REPORT: A 5 1/2 month-old girl presented with hypoxemic respiratory distress. Her medical record was remarkable only for a bulky parotid haemangioma, which was treated with prolonged oral corticosteroid therapy. The chest X-ray showed a mixed alveolar-interstitial pattern, and bronchoalveolar lavage revealed the presence of PJ. A favourable outcome was obtained after three weeks of intravenous trimethoprim-sulfamethoxazole treatment. CONCLUSION: PJ infection should be suspected in infants presenting with progressive respiratory distress associated with a mixed alveolar-interstitial pattern. Its potential seriousness justifies prophylactic therapy during prolonged immunosuppressive treatment (chemotherapy, corticosteroid treatment).

[Obstructive sleep apnea-hypopnea syndrome in children: Clinical diagnosis]. / Diagnostic clinique du syndrome d'apnées obstructives du sommeil de l'enfant.

The French Society of Research and Sleep Medicine (SFRMS) organized a meeting on obstructive sleep apnea syndrome (OSAS) in children. A multidisciplinary group of specialists (pulmonologist, ENT surgeons, pediatricians, orofacial myofunctional therapists, neurophysiologists, and sleep specialists) reached a consensus on the value of isolated or clustered clinical symptoms and of questionnaires completed by parents in the clinical diagnosis and in assessing the severity of OSAS. Are clinical history with validated questionnaires and a rigorous physical examination sufficient to suspect OSAS, to appreciate its severity, and finally to confirm the diagnosis? Usually, a sleep recording of respiratory parameters remains mandatory for the diagnosis of OSAS to be made. However, clinical symptoms are very useful for estimating the probability of the diagnosis and the severity of the disease, and therefore for classifying which children will benefit form polysomnography and for proposing an adapted follow-up after OSAS therapy. Even if they are not able to ascertain the diagnosis of OSAS in children, clinical history, questionnaires, and physical examination are very important. Finally, we propose a classification of the indications for polysomnography in children suspected of having OSAS.

[Chronic otitis and hearing loss revealing a disseminated tuberculosis in a child]. / Otorrhée chronique et hypoacousie révélant une tuberculose disséminée chez l'enfant: à propos d'un cas.

UNLABELLED: Ear localization is sometimes the first symptom of tuberculosis. CASE REPORT: We report a case of a teen with a chronic otitis revealing a disseminated tuberculosis. The investigations showed ear, bones and pulmonary localisations. The outcome with treatment showed a persistent hearing loss. CONCLUSION: Middle ear tuberculosis should be suspected in patients with chronic otitis and risk factors of tuberculosis. A disseminated tuberculosis should be investigated and an early treatment is necessary to prevent hearing loss.

[Diagnostic criteria for obstructive sleep apnea syndrome]. / Critères diagnostiques du syndrome d'apnées obstructives du sommeil.

The prevalence of obstructive sleep apnea syndrome (OSAS) is 1-4 % in school-aged children. Adenotonsillar hypertrophy is the most common etiology of OSAS in children. Other causes are obesity; facial or skeletal malformations; and neuromuscular, respiratory, or metabolic diseases. OSAS has been associated with sleep quality disturbance (frequent arousals) and nocturnal gas exchange abnormalities (hypoxemia and sometimes hypercapnia), which can both result in negative health outcomes. The analysis of clinical symptoms and physical examination cannot always distinguish between children with primary snoring and children with OSAS. However, the association of at least one sign of nocturnal upper airway obstruction with other diurnal or nocturnal symptoms can be sufficient to establish OSAS diagnosis in a child more than 3 years of age with clear enlarged tonsils but who is otherwise healthy. In all other cases, polysomnography (the gold standard for the diagnosis of sleep-disordered breathing) must be performed either to declare the diagnosis when clinical assessment is not conclusive or when risk factors are present, or to follow up children with an associated health condition or initial severe OSAS. The equipment used to record sleep and the interpretation criteria are all pediatric-specific. Other methods, such as respiratory polygraphy, are simpler to implement, but further studies are warranted to validate the interpretation criteria of these methods in children. However, in centers with experienced personnel, polygraphy can be used in place of polysomnography. In all cases, the analysis of sleep traces must be manual and performed by personnel under the supervision of medical staff trained to interpret pediatric sleep studies.

[Obstructive sleep apnea syndrome in children]. / Le syndrome d'apnées obstructives du sommeil chez l'enfant.

Obstructive sleep apnea (OSA) is highly prevalent in school-aged children. Tonsillar and/or adenoids hypertrophy is the most common etiology of OSA in children. OSA has been associated with sleep quality disturbance (frequent arousals) and nocturnal gas-exchange abnormalities (hypoxemia and sometimes hypercapnia), complicated with a large array of negative health outcomes. The clinical symptoms are not able to distinguish primary snoring from OSA. Polysomnography remains the gold standard for the diagnosis of sleep disordered breathing, but the demand is increasing for this highly technical sleep test. So, some other simpler diagnostic methods are available, as respiratory polygraphy, but need to be validated in children. Treatment of OSA in children must be based on a mutlidisciplinary approach with pediatricians, ENT surgeons and orthodontists.

[Screening: from prescription to interpretation in children's obstructive sleep apnea syndrome]. / Explorer : de la prescription à l'interprétation : exploration du syndrome d'apnée du sommeil de l'enfant.

The supply and the demand for sleep recording in children are increasing. With no lower age limit, obstructive sleep apnea syndrome can be diagnosed in a lab run by a staff trained in conducting and interpreting children's sleep traces recorded with an adapted material. Whichever the criteria used to quote respiratory events, a relevant diagnosis is performed using both clinical presentation and results of sleep traces.

Management of laryngomalacia.

Laryngomalacia is the most common laryngeal disease of infancy. It is poorly tolerated in 10% of cases, requiring assessment and management, generally surgical. Surgery often consists of supraglottoplasty, for which a large number of technical variants have been described. This surgery, performed in an appropriate setting, relieves the symptoms in the great majority of cases with low morbidity. However, few data are available concerning the objective results: preoperative and postoperative objective assessment of these infants is therefore necessary whenever possible. Noninvasive ventilation (NIV) may be indicated in some infants with comorbid conditions or failing to respond to surgical management.

[Recommendations for pediatric oxygen therapy in acute and chronic settings: Needs assessment, implementation criteria, prescription practices and follow-up]. / Recommandations pour l'oxygénothérapie chez l'enfant en situations aiguës et chroniques : évaluation du besoin, critères de mise en route, modalités de prescriptions et de surveillance.

Recommendations for acute and long-term oxygen therapy (needs assessment, implementation criteria, prescription practices, and follow-up) in children were produced by the Groupe de Recherche sur les Avancées en Pneumo-Pédiatrie (GRAPP) under the auspices of the French Paediatric Pulmonology and Allergology Society (SP2A). The Haute Autorité de Santé (HAS) methodology, based on the Formalized Consensus, was used. A first panel of experts analyzed the English and French literature to provide a second panel of experts with recommendations to validate. Only the recommendations are presented here, but the full text (arguments+recommendations) is available at the website of the French Paediatric Society: www.sfpediatrie.com.

Pathophysiology and diagnostic approach to laryngomalacia in infants.

Laryngomalacia is defined as collapse of supraglottic structures during inspiration. It is the most common laryngeal disease of infancy. Laryngomalacia presents in the form of stridor, a high-pitched, musical, vibrating, multiphase inspiratory noise appearing within the first 10 days of life. Signs of severity are present in 10% of cases: poor weight gain (probably the most contributive element), dyspnoea with permanent and severe intercostal or xyphoid retraction, episodes of respiratory distress, obstructive sleep apnoea, and/or episodes of suffocation while feeding or feeding difficulties. The diagnosis is based on systematic office flexible laryngoscopy to confirm laryngomalacia and exclude other causes of supraglottic obstruction. Rigid endoscopy under general anaesthesia is only performed in the following cases: absence of laryngomalacia on flexible laryngoscopy, presence of laryngomalacia with signs of severity, search for any associated lesions prior to surgery, discrepancy between the severity of symptoms and the appearance on flexible laryngoscopy, and/or atypical symptoms (mostly aspirations). The work-up must be adapted to each child; however, guidelines recommend objective respiratory investigations in infants presenting signs of severity.

[Recommendations for pediatric oxygen therapy in acute and chronic settings: needs assessment, implementation criteria, prescription practices and follow-up]. / Recommandations pour l'oxygénothérapie chez l'enfant en situations aiguës et chroniques : évaluation du besoin, critères de mise en route, modalités de prescription et de surveillance.

Recommendations for acute and long-term oxygen therapy (needs assessment, implementation criteria, prescription practices, and follow-up) in children were produced by the Groupe de Recherche sur les Avancées en Pneumo-Pédiatrie (GRAPP) under the auspices of the French Paediatric Pulmonology and Allergology Society (SP2A). The Haute Autorité de Santé (HAS) methodology, based on the Formalized Consensus, was used. A first panel of experts analyzed the English and French literature to provide a second panel of experts with recommendations to validate. Only the recommendations are presented here, but the full text (arguments+recommendations) is available at the website of the French Paediatric Society: www.sfpediatrie.com.

Conservative use of chest-tube insertion in children with pleural effusion.

The aim of this work was to evaluate the effect of a more conservative use of chest-tube insertion on the short-term and long-term outcome of pleural infection. Sixty-five patients with pleural infection, aged 1 month to 16 years were each treated according to one of the two protocols: classical management with chest-tube insertion (classical group, n = 33), or conservative use of chest-tube insertion (conservative group, n = 32), with drainage indicated only in the case of voluminous pleural effusion defined by a mediastinal shift and respiratory distress and/or an uncontrolled septic situation. The two groups were comparable with regard to age, baseline C-reactive protein (CRP) value and white blood cell counts, pleural thickness, identified bacteria, and antibiotic treatment. Chest-tube insertion was performed in 17 patients (52%) of the classical group compared to eight patients (25%) of the conservative group (P = 0.03). Duration of temperature above 39 degrees C was shorter in the conservative group (10 +/- 1 vs. 14 +/- 1 days, P = 0.01), as was the normalization of CRP (13 +/- 1 vs. 17 +/- 1 days, P = 0.03). Duration of hospitalization and intravenous (IV) antibiotherapy as well as the delay of chest-radiograph normalization was not significantly different between the two groups. A more conservative use of chest-tube insertion did not change short- and long-term outcome of the pleural infection in children. Drainage could be restricted to the most severely affected patients with pleural empyema causing a mediastinal shift and respiratory distress and/or presenting with an uncontrolled septic situation.

Evaluating age from arbitrary forms of injection functions of tracer.

The age of the air in a room is normally determined either from a pulse response or from a step change response (up or down). There are a certain number of problems involved in applying these two theoretical models, especially those associated with the duration of the injection, which must either be infinitely short or infinitely long. A hybrid method that consists of injecting a known quantity of tracer for a given time offers the advantages of both methods. The equation for calculating age is exact, regardless of the type of flow considered, and is derived from the expressions already established for a pulse response to which a correction is included to account for the tracer generation function. If a rectangular pulse is used for the injection, the solution is particularly simple.

Prenatal diagnosis of apparently isolated unilateral multicystic kidney: implications for counselling and management.

Cases where initial prenatal diagnosis was made of isolated unilateral multicystic kidney (UMCK) were reviewed to determine appropriate counselling and management strategies. For the 73 cases, chromosome abnormalities, pregnancy complications and family histories were reviewed. In addition, subsequently diagnosed birth defects, and pediatric medical and surgical outcomes were available for 54 cases. Of those with outcome information available renal/genital-urinary tract abnormalities were diagnosed subsequently in 33% and non-renal abnormalities in 16% of cases. Of the non-renal abnormalities, congenital heart defects were most frequent (7%). One chromosome abnormality, a trisomy 21, was present among 32 cases where karyotypes were known (3%). Amniotic fluid volume abnormalities were present in 11 cases but not predictive of associated anomalies, with the exception of one case where polyhydramnios accompanied multiple malformations consistent with VATER association. A family history of structural renal anomalies was reported in 11 cases (20%). There were 14 cases of partial or complete involution (25%), including two cases of complete prenatal involution of the cystic kidneys. No long-term associated morbidity such as hypertension or malignancy was present in our cohort. Based on our study and corroborating literature, amniocentesis should be offered to women when a seemingly isolated UMCK is detected on routine prenatal ultrasound. Furthermore, a detailed ultrasound with careful assessment of the fetal heart and contralateral kidney is indicated at diagnosis and during the third trimester to assess for further evidence of structural abnormalities, as well as amniotic fluid volume abnormalities. Careful assessment of the newborn is indicated with appropriate speciality referral as required.