Evaluation of the efficacy of tongue-lip adhesion in Pierre Robin sequence.

OBJECTIVE: Tongue-lip adhesion may be used to relieve obstructive sleep apnoea in infants with Pierre Robin sequence (PRS), but only a few studies have objectively evaluated its efficacy. The purpose of this study was to evaluate the results of tongue-lip adhesion by polysomnography. MATERIEL AND METHODS: A single-centre retrospective study was conducted in infants with PRS treated by tongue-lip adhesion from 2004 to 2015, in whom at least laryngotracheal endoscopy and polysomnography were performed. The variables collected were the syndromic diagnosis, demographic data, respiratory management before tongue-lip adhesion, symptoms, and additional airway interventions. Obstructive sleep apnoea was classified into 3 groups according to severity. Polysomnography was performed one month after tongue-lip adhesion. Statistical analysis was performed with Wilcoxon signed-rank test with a limit of statistical significance of P<0.005. RESULTS: Thirty-seven subjects in whom tongue-lip adhesion was performed at a mean age of 45 days (8 to 210 days) were included. Thirty-one patients had isolated PRS and 6 patients had associated anomalies. All patients had confirmed severe obstructive sleep apnoea. All patients required respiratory support prior to surgery: 8 intubated patients, 15 patients with noninvasive ventilation and 14 patients with nasopharyngeal airways. Eight patients had bradycardia before tongue-lip adhesion. All parameters were improved on postoperative polysomnography: oxygen saturation, hypercapnia, apnoea-hypopnoea index, bradycardia (P<0.005). Only 8 patients had persistent severe obstructive sleep apnoea and required tracheostomy (n=5) or noninvasive ventilation (n=3). No significant correlation was observed between treatment success and any predictive variables. CONCLUSION: Tongue-lip adhesion improved airway obstruction in all infants with PRS and resolved obstructive sleep apnoea in 29 patients. However, 8 patients required additional airway interventions.

[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.

French recommendations on electroencephalography.

Electroencephalography allows the functional analysis of electrical brain cortical activity and is the gold standard for analyzing electrophysiological processes involved in epilepsy but also in several other dysfunctions of the central nervous system. Morphological imaging yields complementary data, yet it cannot replace the essential functional analysis tool that is EEG. Furthermore, EEG has the great advantage of being non-invasive, easy to perform and allows repeat testing when follow-up is necessary, even at the patient's bedside. Faced with advances in knowledge, techniques and indications, the Société de neurophysiologie clinique de langue française (SNCLF) and the Ligue française contre l'épilepsie (LFCE) found it necessary to provide an update on EEG recommendations. This article will review the methodology applied to this work, refine the various topics detailed in the following chapters. It will go over the summary of recommendations for each of these chapters and highlight proposals for writing an EEG report. Some questions could not be answered by review of the literature; in such cases, in addition to the guidelines the working and reading groups provided their expert opinion.

EEG in children, in the laboratory or at the patient's bedside.

In pediatrics, EEG recordings are performed on patients from the neonatal period up to young adults. This means adapting techniques to many different conditions, concerning not only the patient's age, the need for asepsis and the patient's behavior, but also the environment (e.g. in the laboratory, at the patient's bedside, or in the neonatal intensive care unit [NICU]). Technical requirements depend on age, indication and the type of examination; in infancy, there should be a minimum of 12 EEG electrodes, ECG and respiration recording. In epileptology, surface EMG is also necessary to characterize the type of seizures and refine the diagnosis of epilepsy syndrome, on which physicians will base their treatment choice. The role of the EEG technician is essential because the quality of the recording, its analysis and conclusion will depend on the quality of the technical set-up and the interaction with the child. Sleep is a systematic part of the study up to the age of 5 years for several reasons: sleep EEG yields information on brain maturation; the EEG tracing during wakefulness can contain too many artefacts; and some grapho-elements, key to the diagnosis, only appear during sleep. The time of the examination must be chosen according to the child's usual nap times, possibly after sleep deprivation. Grapho-elements and spatio-temporal organization of the EEG vary with age, and normal variants and unusual aspects are quite wide for any given age; this is why a physician experienced in pediatric EEG should perform the interpretation. This chapter concerns EEG performed in infants, children and adolescents, its technical aspects according to age and indications (general pediatrics, emergency, epilepsy).

EEG guidelines in the diagnosis of brain death.

In France, for the determination and diagnostic validation of brain death the law requires either two EEG recordings separated by a 4-hour observation period, both showing electrocerebral inactivity; or cerebral angiography examination. Since EEG is available in most hospitals and clinics, it is often used in this indication, at the patient's bedside, especially in the context of organ donation. However, very precise methodology must be followed. The last French guidelines date back to 1989, before the development of digital EEG recording. We present the new guidelines from the Société de Neurophysiologie Clinique de Langue Française. Electrocerebral inactivity may be confirmed when a 30-minute good quality EEG recording shows complete electrocerebral silence, defined as no cerebral activity greater than 2 uV, having first ruled out the possible influence of sedative drugs, metabolic disorders or hypothermia. In the presence of sedative drugs, CT brain angiography will be the gold standard test for this diagnosis. In the newborn, the utmost caution is indicated since electrocerebral inactivity can be observed in the absence of cerebral death. In the infant, the criterion for the observation period to be respected between both EEG recordings needs to be more clearly refined.

EEG in the neonatal unit.

The execution and interpretation of neonatal EEG adheres to strict and specific criteria related to this very early age. In preterm newborns, the dedicated healthcare staff needs to respect EEG indications and chronology of EEG recordings in order to diagnose and manage various pathologies, and use EEG in addition to cerebral imaging. EEG analysis focuses on a global vision of the recording according to the neonate's state of alertness and various age-related patterns. Monitoring of continuous conventional EEG and simplified EEG signal processing can help screen for seizures and monitor the effect of antiepileptic treatment, as well as appreciating changes in EEG background activity, for diagnostic and prognostic purposes. EEG reports should be highly explanatory to meet the expectations of the physician's clinical request.

[French guidelines on electroencephalogram]. / Recommandations françaises sur l'électroencéphalogramme.

Electroencephalography allows the functional analysis of electrical brain cortical activity and is the gold standard for analyzing electrophysiological processes involved in epilepsy but also in several other dysfunctions of the central nervous system. Morphological imaging yields complementary data, yet it cannot replace the essential functional analysis tool that is EEG. Furthermore, EEG has the great advantage of being non-invasive, easy to perform and allows control tests when follow-up is necessary, even at the patient's bedside. Faced with the advances in knowledge, techniques and indications, the Société de Neurophysiologie Clinique de Langue Française (SNCLF) and the Ligue Française Contre l'Épilepsie (LFCE) found it necessary to provide an update on EEG recommendations. This article will review the methodology applied to this work, refine the various topics detailed in the following chapters. It will go over the summary of recommendations for each of these chapters and underline proposals for writing an EEG report. Some questions could not be answered by the review of the literature; in those cases, an expert advice was given by the working and reading groups in addition to the guidelines.

The electroencephalogram of the full-term newborn: review of normal features and hypoxic-ischemic encephalopathy patterns.

OBJECTIVE: The objective of this study is to specify, by reference to the normal newborn, the current contribution of the electroencephalogram (EEG) to the diagnosis and prognosis of hypoxic-ischemic encephalopathy (HIE) in the full-term newborn. Both digitized traditional EEG and cerebral function monitoring will be considered. DISCUSSION: A good knowledge of the EEG features of the sleep-wake cycle (SWC) is a prerequisite. We first describe the main features of normal and pathological EEGs. Very early recordings (before 6hours of life) are needed to indicate any required neuroprotective (hypothermia) and other (sedation, anticonvulsivants) treatments. CONCLUSIONS: Between the normal or near-normal tracings, which are associated with a good prognosis, and the very pathological ones (inactive, paroxysmal), which are associated with a poor vital or neurological prognosis, the interpretation of "intermediate" tracings--mainly represented by other types of discontinuous tracings--must take into account characteristics such as bursts and discontinuities, postnatal age, evolution of successive tracings, and treatments.

[Esophageal atresia]. / Devenir à moyen et long terme des enfants atteints d'atrésie de l'œsophage.

Most of the children operated for esophageal atresia will survive the neonatal period. However, medium-term and late complications are frequent in this population. Gastroesophageal reflux disease is observed in 26 to 75% of the cases and can be responsible for peptic esophagitis, anastomotic stenosis, and Barrett esophagus, which is a risk factor for adenocarcinoma of the esophagus. Dysphagia is frequently observed, sometimes several years after the surgery, affecting up to 45% of children at the age of 5 years. Growth retardation is present in nearly one-third of children at the age of 5 years. Ear, nose, and throat and respiratory complications are also very frequent but tend to improve with time. Tracheomalacia is found in 75% of these children at birth, sometimes responsible for severe complications (malaise, bradycardia). Respiratory symptoms are dominated by chronic cough, wheezing, and infections reported in 29% of the children by the age of 5 years. Restrictive, obstructive syndromes and bronchial hyperactivity can be observed, but usually remain moderate. All these complications can influence the patient's quality of life, which is moderately impaired compared to healthy controls. The high frequency of late sequelae in esophageal atresia justifies regular and multidisciplinary follow-up through adulthood.

[Electroencephalogram of the full-term newborn. Normal features and hypoxic-ischemic encephalopathy]. / Électroencéphalogramme du nouveau-né à terme. Aspects normaux et encéphalopathie hypoxo-ischémique.

The objective of this work is to specify, by reference to the normal newborn, the current contribution of the electroencephalogram in the hypoxic-ischemic encephalopathy of the full-term newborn. Both digitized traditional EEG and cerebral function monitoring (CFM) will be considered. We first describe the main features of normal and pathological EEGs. A good knowledge of the organization of the sleep-wakefulness cycles, in relationship with the EEG, is essential. Very early recordings (before 6 hours of life) are needed to put the indications of neuroprotective treatments (hypothermia). Between the normal or near-normal tracings, which are associated with a good prognosis, and the very pathological tracings (inactive, paroxysmal), which are associated with a poor vital or functional prognosis, the interpretation of "intermediate" tracings - mainly represented by other types of discontinuous tracings - must take into account characteristics of bursts and discontinuities, postnatal age, the evolution of successive tracings, and pharmacological treatments. A flowchart is used to illustrate our strategy of EEG watching over a full-term newborn after an acute fetal distress.

Electroencephalography in premature and full-term infants. Developmental features and glossary.

Following the pioneering work of C. Dreyfus-Brisac and N. Monod, research into neonatal electroencephalography (EEG) has developed tremendously in France. French neurophysiologists who had been trained in Paris (France) collaborated on a joint project on the introduction, development, and currently available neonatal EEG recording techniques. They assessed the analytical criteria for the different maturational stages and standardized neonatal EEG terminology on the basis of the large amount of data available in the French and the English literature. The results of their work were presented in 1999. Since the first edition, technology has moved towards the widespread use of digitized recordings. Although the data obtained with analog recordings can be applied to digitized EEG tracings, the present edition, including new published data, is illustrated with digitized recordings. Herein, the reader can find a comprehensive description of EEG features and neonatal behavioural states at different gestational ages, and also a definition of the main aspects and patterns of both pathological and normal EEGs, presented in glossary form. In both sections, numerous illustrations have been provided. This precise neonatal EEG terminology should improve homogeneity in the analysis of neonatal EEG recordings, and facilitate the setting up of multicentric studies on certain aspects of normal EEG recordings and various pathological patterns.

Childhood-onset narcolepsy, obesity and puberty in four consecutive children: a close temporal link.

Narcolepsy is a rare but disabling condition that causes excessive daytime sleepiness. Interestingly, weight gain is frequent in patients with narcolepsy and it has sometimes been described very early in the course of the disease. Here, we report four consecutive obese children who were referred to our sleep laboratory for excessive daytime sleepiness and suspected sleep apnoea syndrome. They underwent nocturnal polysomnography associated with multiple sleep latency tests. Narcolepsy was diagnosed in all children with a close temporal link between the onset of narcolepsy, obesity and puberty. Scientifically, the relationship between sleep, weight, growth rate and puberty onset is striking and merits further investigation. From the clinical point of view, narcolepsy must be investigated in obese sleepy children along with obstructive sleep apnoea. Indeed, it can be controlled with appropriate treatment but the proper diagnosis relies not only upon nocturnal polysomnography but involves the systematic use of multiple sleep latency tests.

Treatments for pediatric achalasia: Heller myotomy or pneumatic dilatation?

AIM: The treatment of achalasia consists of reducing distal esophageal obstruction by either Heller myotomy surgery or endoscopic pneumatic dilatation. The aim of the present study was to evaluate the short- and middle-term results of these procedures in children. METHODOLOGY: For technical reasons, children under six years old (n=8) were treated by surgery only, whereas patients over six years old (n=14) were treated by either Heller myotomy or pneumatic dilatation. RESULTS: Of the children aged under six years, 75% were symptom-free at six months and 83% at 24 months of follow-up. Of the patients aged over six years, complete remission was achieved by Heller myotomy in 44.5% vs. 55.5% by pneumatic dilatation after six months, and in 40% vs. 65%, respectively, after 24 months. Both pneumatic dilatation and Heller myotomy showed significant rates of failure. CONCLUSION: These results suggest that pneumatic dilatation may be considered a primary treatment in children over six years old. Also, where necessary, Heller myotomy and pneumatic dilatation may be used as complementary treatments.

[Use of midazolam for refractory status epilepticus in children]. / Utilisation du midazolam dans l'état de mal épileptique réfractaire de l'enfant.

UNLABELLED: Morbidity and mortality are high in children with refractory status epilepticus (RSE). Here, we assess the efficacy of midazolam for RSE in children. METHODS: This was a retrospective analysis of 29 children admitted to the Lille University Hospital pediatric intensive care unit (PICU) for RSE between May 2006 and July 2008. The onset of the study corresponded with a new therapeutic protocol applied in the PICU for RSE where midazolam was proposed as the first-line treatment (bolus ten continuous infusion until control) to be replaced by thiopenthal in case of failure. RESULTS: We recorded 29 patients with RSE during the study period: 26 were treated with midazolam, including two where midazolam replaced thiopenthal because of hypotension. Midazolam successfully controlled RSE in 58% of patients. Mean delay to cessation of RSE was 48+/-65 minutes. Hypotension was observed in 8% of midazolam-treated patients and 71% of thiopenthal-treated patients. Overall mortality was 15% (4/26). Two deaths occurred long after the cessation of RSE. None of the deaths occurred in midazolam-treated patients. CONCLUSION: Midazolam is an efficient treatment for RSE in children. Morbidity and mortality appear to be lower with midazolam compared with other antiepileptic drugs used for the treatment of RSE.

[Syncope and pre-syncope in children and adolescents: a prospective study in a pediatric emergency care unit]. / Syncopes et lipothymies chez l'enfant et l'adolescent : étude prospective dans une unité d'urgence pédiatrique.

OBJECTIVES: To assess in a pediatric emergency care unit (PECU): 1. The frequency of syncope and pre-syncope, 2. The incidence of diagnoses, 3. The value of investigations and cardiology and neurology consultations. METHODS: The data of PECU patients aged 2 years to 15 years and 3 months were prospectively collected over 1 year. Standard electrocardiogram and serum glucose were compulsory investigations. Schellong's orthostatic test was performed whenever possible. RESULTS: One hundred and fity-nine children (mean age, 11+/-4 years) were included, accounting for 0.8% of the PECU's visits: 48% had syncope, 52% had pre-syncope. The most common cause was neurally mediated syncope - 98 patients (62%), with vasovagal syncope for 80 patients - followed by neurological causes: 29 patients (18%). Neither cardiac arrhythmia nor obstructive cardiomyopathy was diagnosed. There were discrepancies between cardiologists' and pediatricians' ECG interpretations in 9% of cases. Diagnoses differed between cardiologists and pediatricians in 54% of 41 consultations. Diagnoses differed between neurologists and pediatricians in 54% of 42 consultations. No investigation except Schellong's orthostatic test led to modification of a previous diagnosis. CONCLUSION: This study emphasizes that the routine workup of pediatric syncope should focus on the patient's history and physical examination. Diagnostic testing should be minimal: ECG and Schellong's orthostatic test. The leading cause was neurocardiogenic syncope.

Consensus on the use of neurophysiological tests in the intensive care unit (ICU): electroencephalogram (EEG), evoked potentials (EP), and electroneuromyography (ENMG).

STUDY AIM: To provide a consensus of European leading authorities about the optimal use of clinical neurophysiological (CN) tests (electroencephalogram [EEG]; evoked potentials [EP]; electroneuromyography [ENMG]) in the intensive care unit (ICU) and, particularly, about the way to make these tests clinically useful for the management of individual patients. METHODS: This study gathered together several European clinical neurophysiologists and neurointensivists whose leading contributions in the adult or paediatric ICU and in continuous neuromonitoring had been peer-acknowledged. It was based on both a literature review and each participant's own experience. Given the methodological impossibility to gather studies fulfilling criteria of evidence-based medicine, this article essentially relies on expert opinions that were gained after several rounds, in which each expert was invited to communicate his own contribution to all other experts. A complete consensus has been reached when submitting the manuscript. RESULTS: What the group considered as the best classification systems for EEG and EP abnormalities in the ICU is first presented. CN tests are useful for diagnosis (epilepsy, brain death, and neuromuscular disorders), prognosis (anoxic ischemic encephalopathy, head trauma, and neurologic disturbances of metabolic and toxic origin), and follow-up, in the adult, paediatric, and neonatal ICU. Regarding prognosis, a clear distinction is made between these tests whose abnormalities are indicative of an ominous prognosis and those whose relative normalcy is indicative of a good prognosis. The prognostic significance of any test may vary as a function of coma etiology. CONCLUSION: CN provides quantitative functional assessment of the nervous system. It can be used in sedated or curarized patients. Therefore, it should play a major role in the individual assessment of ICU patients.

[Encopresis revealing myotonic dystrophy in 2 children]. / Encoprésie révélatrice d'une dystrophie myotonique de Steinert : à propos de 2 observations.

Gastrointestinal symptoms are very frequent in myotonic dystrophy but largely unrecognized. They can be the revealing factors of the disease. We report 2 cases of 10 and 17-year-old children with persistent encopresis starting at the age of 3 and 5 years in spite of laxative treatment. Neurological examination and anorectal manometry provided the diagnosis of myotonic dystrophy. Procainamide treatment was introduced and the digestive symptoms improved. Any child with encopresis should have complete evaluation to rule out the diagnosis of myotonic dystrophy and physicians should look for upper and/or lower gastrointestinal symptoms in every patient with myotonic dystrophy.

[Myoclonic astatic seizures in a child with Sturge-Weber syndrome]. / Crises myoclonoastatiques chez un patient présentant un syndrome de Sturge-Weber.

INTRODUCTION: Sturge-Weber syndrome is a neurocutaneous disease associating facial and pial angioma. Focal epilepsy is a common sign. In a few cases, generalized seizures have been reported. CASE REPORT: We report on a four-year-old girl with Sturge-Weber syndrome. The first focal seizures occurred at three years of age. She developed refractory status epilepticus. At discharge from the PICU, she was on a ketogenic diet and received three antiepileptic drugs. No seizures were observed for four months. The patient then developed several types of seizures: myoclonic seizures, focal clonic seizures, and sudden falls. We were unable to determine the etiology of the falls. Typical myoclonic astatic seizures were identified on video-electroencephalographic recordings. CONCLUSION: Seizures in Sturge-Weber syndrome are usually focal. Four patients with Sturge-Weber syndrome and myoclonoastatic seizures are reported in the literature. We discuss the pathophysiological mechanisms leading from a focal lesion to generalized myoclonoastatic seizures.

Pathological features of neonatal EEG in preterm babies born before 30 weeks of gestationnal age.

Pathological features on very premature EEG concern background abnormalities and abnormal ictal and nonictal patterns. Positive rolandic sharp waves keep an important place regarding diagnosis and prognosis of white-matter lesions. Background abnormalities, that may be classified as acute-stage or chronic-stage abnormalities, give essential complementary information. These abnormal patterns remain precocious markers of cerebral lesions and are complementary to cerebral imaging. Analysis of these abnormalities has always to take into account medication received by the baby during the recording and that could modify the EEG.