[Secondary surgeries in craniosynostosis and faciocraniosynostosis]. / Chirurgie secondaire des craniosténoses et faciocraniosténoses.

Secondary surgeries for single craniosynostosis surgeries are mainly esthetic refinements rather than functional indications. However, cranioplasties for bone defects correction or insufficient corrections may be undertaken. Management of syndromic craniosynostoses usually requires multiple surgical interventions, the sequence of which might vary per the genetic mutation. It is commonplace to start with posterior vault expansion before age 6 months, then treat cerebellar tonsillar herniation by the age of twelve months, and delay fronto-facial monobloc advancement until at least 18-24 months of age. Ventricular shunting is preferably avoided or delayed. Failure to respect these guidelines can significantly complicate the subsequent management. Primary fronto-orbital advancement or early facial osteotomy type Le Fort3, may compromise the subsequent fronto-facial monobloc advancement. However, this salvage secondary monobloc may be undertaken in some instances despite previous anterior osteotomies with a higher morbidity.

[Craniofacial strategy for syndromic craniosynostosis]. / Stratégie craniofaciale pour les faciocraniosténoses.

The complexity of treatment of faciocraniosynostosis justifies the treatment in a reference center for rare diseases. The growth disturbances in the skull and face being variable according to the type of mutation in the FGFr (Crouzon, Pfeiffer, Apert), the strategy is adapted to the phenotype according to the following principles: posterior expansion with or without distraction around 6 months to limit the descent of the cerebellum tonsils and to prevent the turricephalic development; fronto-facial monobloc advancement with internal distraction around the age of 18 months in case of severe exorbitism or breathing impairment. The dissociated strategy (fronto-orbital advancement first, followed by facial osteotomy of Le Fort 3 type). The growing evolution dictates the sequence of subsequent surgeries according to the monitoring of intracranial pressure by fundus examination and of the respiration by polysomnography. Le Fort 3 and transversal maxillary distraction may be repeated if necessary. Orthognathic surgery is almost always compulsory after the age of 14, before the aesthetic refinements which can be undertaken ultimately (rhinoplasty, genioplasty, canthopexies, fat grafting…).

Prevalence of residual excessive sleepiness in CPAP-treated sleep apnoea patients: the French multicentre study.

The percentage of compliant continuous positive airway pressure (CPAP)-treated apnoeic patients that continue to experience residual excessive sleepiness (RES) is unknown. RES was defined by an Epworth Sleepiness Scale (ESS) score of >or=11. In total, 502 patients from 37 French sleep centres using CPAP >3 h night(-1) attending their 1-yr follow-up visit were eligible. ESS and polysomnographic data as well as symptoms, quality of life, depression scores and objective CPAP compliance at 1 yr were collected. Overall, 60 patients remained sleepy on CPAP (ESS 14.3+/-2.5) leading to a prevalence rate of RES of 12.0% (95% confidence interval (CI) 9.1-14.8). After having excluded associated restless leg syndrome, major depressive disorder and narcolepsy as confounding causes, the final prevalence rate of RES was 6.0% (95% CI 3.9-8.01). Patients with RES were younger and more sleepy at diagnosis. The relative risk of having RES was 5.3 (95% CI 1.6-22.1), when ESS before treatment was >or=11. Scores of emotional and energy Nottingham Health Profile domains were two times worse in patients with RES. As 230,000 obstructive sleep apnoea patients are currently treated in France by continuous positive airway pressure, more than 13,800 of them might suffer from residual excessive sleepiness.

Psychological health in central hypersomnias: the French Harmony study.

BACKGROUND: A large observational French study of central hypersomnia, including narcolepsy with cataplexy (C+), without cataplexy (C-) and idiopathic hypersomnia (IH), was conducted to clarify the relationships between the severity of the condition, psychological health and treatment response. METHODS: 601 consecutive patients over 15 years of age suffering from central hypersomnia were recruited on excessive daytime sleepiness, polysomnography and Multiple Sleep Latency Test (MSLT) results. 517 (47.6% men, 52.4% women) were finally included: 82.0% C+, 13.2% C- and 4.8% IH. Face to face standardised clinical interviews plus questionnaires (Epworth Sleepiness Scale (ESS), short version Beck Depression Inventory (S-BDI), Pittsburgh Sleep Quality Index (PSQI) and 36-item Short Form Health Survey (SF-36)) were performed. Patients affected with a different diagnosis and with and without depressive symptoms were compared. RESULTS: Mean ESS and body mass index were higher in C+ compared with C-/IH patients. Half of the patients (44.9%) had no depressive symptoms while 26.3% had mild, 23.2% moderate and 5.6% severe depressive symptoms. C+ patients had higher S-BDI and PSQI and lower SF-36 scores than C-/IH patients. Depressed patients had higher ESS scores than non-depressed patients, with no difference in age, gender, duration of disease or MSLT parameters. Finally, C+ patients treated with anticataplectic drugs (38.7%) had higher S-BDI and lower SF-36 scores than C+ patients treated with stimulants alone. CONCLUSION: Our data confirmed the high frequency of depressive symptoms and the major impact of central hypersomnias on health related quality of life, especially in patients with cataplexy. We recommend a more thorough assessment of mood impairment in central hypersomnias, especially in narcolepsy-cataplexy.

[Sleep duration and metabolism]. / Durée de sommeil et métabolisme.

Sleep duration has gradually diminished during the last decade while obesity and type 2 diabetes have become epidemics. Experimental sleep curtailment leads to increased appetite, hormonal disturbances and, especially, insulin resistance. Numerous epidemiological studies have therefore examined whether habitual short sleep is associated with obesity and type 2 diabetes. A large majority of cross-sectional studies have confirmed an association between short, and also long sleep duration and obesity in adults more than in the elderly. Short sleep is strongly associated to obesity in children and adolescents. Prospective studies, including studies in children, are not conclusive with regard to the effect of short sleep on the incidence of obesity. Both short and long sleep durations are associated with diabetes, but only short sleep duration seems predictive of future diabetes. Insomnia seems to be a strong contributor to short sleep duration but the association of insomnia with obesity is not clear. Insomnia is associated with type 2 diabetes and also predictive of a higher incidence. Other studies have shown that short sleep duration and insomnia are associated with, and sometime predictive of, other components of the metabolic syndrome, especially hypertension and the risk of coronary disease. The treatment of short sleep duration and insomnia with regard to their effects on the metabolic syndrome merits further study.

[Biological models of depression: effect of antidepressants on sleep]. / Modeles biologiques de la depression : l'effet des antidepresseurs sur le sommeil.

The relationship between insomnia and depression cannot be summarized as a symptom/disease relationship. It is well admitted now that sleep deprivation has an antidepressant effect on depressed patients. The effect is immediate, global but transient : the relapse occurs after subsequent sleep, diurnal nap or recovery night. On an other hand, sleep architecture is impaired in depressed patients, some of these alterations, especially in REM sleep, might have been considered specific of depressive disease. Antidepressant drugs exert an effect on sleep architecture which is different. This effect varies over time and generally tend to correct sleep impairment. This has led some authors to propose the hypothesis that sleep himself might be involved in the causal process of depression. Three main hypotheses will be considered, excluding those involving circadian rhythm impairment, according to their strong and weak points. For G. Vogel, an excess of REM sleep is the causal process in depression. As a matter of fact, he did show that selective REM sleep deprivation exerts an antidepressant effect following the same temporal profile as antidepressant drugs. An other argument is that the shortening of REM latency and the increased amount of REM sleep in the first half of the night are evidences of the excess of REM sleep and at last, most antidepressant drugs are REM suppressors and may act through REM sleep suppression. The second hypothesis is the process S deficiency proposed by Alexander Borbely, according to his two process model of sleep homeostasis. The impairment of process S, which is reflected by slow wave activity, is responsible for depression. This explain disruptions and shortening of sleep in depressed patients, REM sleep abnormalities being only secondary to the slow wave sleep reduction. More recently, D. Beersma and R. van den Hoofdakker proposed that non REM sleep might be depressogenic after an experiment of selective REM sleep deprivation in normals which showed that non REM sleep deprivation was also largely reduced. REM suppression effects might therefore also be attributed to non REM suppression. All these hypothesis must explain the effect of antidepressant drugs on sleep. There is a large heterogeneity of effects on slow wave, non REM and even REM sleep, hardly compatible with a causal role of sleep, REM or non REM.

[Two cases of pneumocephalus following noninvasive continuous positive airway ventilation after transsphenoidal neurosurgery]. / Pneumencéphalie après ventilation non invasive en postopératoire de neurochirurgie par voie trans-sphénoïdale: à propos de deux cas.

The early use of continuous positive airway pressure ventilation has been shown to be effective and is recommended for patients with obstructive sleep apnea. The complications of continuous positive airway pressure ventilation are not well described. We report two cases of pneumocephalus following the use of continuous positive airway pressure ventilation after transsphenoidal surgery. One patient had an obstructive sleep apnea and the other suffered acute respiratory failure. In both cases, pneumocephalus caused major morbidity and required specific treatment and prolonged considerably hospital stay. Based on these observations we believe new precautions in the use of noninvasive continuous positive airway pressure ventilation should be recommended.