RESUMEN

BACKGROUND: This study aims to assess the improvement of sleep apnea after secondary Le Fort III facial advancement with distraction (LF3) in faciocraniosynostosis (FCS) patients with sleep apnea who have previously undergone fronto-facial monobloc advancement (FFMBA) with distraction. METHODS: Patients having undergone secondary LF3 were selected from a cohort of FCS patients with documented sleep apnea who had previously undergone fronto-facial monobloc advancement. Patient charts and polysomnographic records were reviewed. Apnea-hypopnea index (AHI) was recorded before and at least 6 months after secondary LF3. The primary outcome was normalization of AHI (less than 5/h was considered normal). Hierarchical multilevel analysis was performed to predict postoperative AHI evolution. RESULTS: Seventeen patients underwent a secondary LF3, 7.0 ± 3.9 years after the primary FFMBA. The mean age was 9.6 ± 3.9 years. A total of 15 patients (88%) normalized their AHI. Two of four patients were decannulated (50%). There was a statistically significant decrease in AHI (preoperative AHI 21.5/h vs. 3.9/h postoperatively, p=0.003). Hierarchic multilevel modeling showed progressive AHI decrease postoperatively. CONCLUSION: Secondary LF3 improves residual or relapsing sleep apnea in FCS patients who have previously had FFMBA.

Asunto(s)

RESUMEN

Obstructive Sleep Apnea (OSA) in children, which has a multifactorial origin, can lead, if not treated, to severe medical complications, growth disturbances, behavioural changes and reduced quality of life. Nowadays, it is underdiagnosed whereas early screening, diagnosis and interdisciplinary treatment are essential. Furthermore, many families and health professionals do not often know where to go when there is suspicion of OSA for a child. Orthodontists are uniquely positioned to screen, to refer to the appropriate specialist and to treat, if needed, patients who may be at high risk for OSA. The authors describe the synergistic means to screen, diagnose and treat paediatric OSA in a collaborative and interactive approach between ENT, orthodontists, pneumo-allergologists, sleep physicians, endocrinologists, orofacial myo-functional therapists and speech therapists. These means which are clinically illustrated in this paper fit the guidelines which have been recently published as white papers by official professional specialists organisations involved in paediatric OSA treatment (AAPD, AAO, FFO, SFORL, SFRMS…). The development of multidisciplinary teams gathering specialists who are conscious about the mutual benefits of the specialties involved in paediatric OSA should contribute to optimize the child treatment care pathway and the short, mid and long term outcomes.

Asunto(s)

RESUMEN

Obstructive sleep apnea syndrome is prevalent in children with syndromic craniosynostoses. Here we assessed the effects of fronto-facial monobloc advancement with internal distraction on obstructive sleep apnea in syndromic craniosynostoses. All patients managed for syndromic craniosynostosis over a period of 14 years were assessed based on apnea-hyponea index (AHI) before and after fronto-facial surgery. AHI values were analyzed using multivariate models with focuses on (1) absolute decrease in AHI values after fronto-facial surgery and (2) AHI normalization (AHI < 5) after fronto-facial surgery. One hundred and nine patients were included with 407 polysomnographic studies. Higher pre-operative AHI (p < 0.001) and pre-operative vault expansion (p = 0.008) were associated with more AHI decrease. Early airways surgery (p = 0.002) and fronto-facial surgery at older ages (p < 0.001) were associated with more AHI normalization. Our results indicate that fronto-facial surgery is specifically efficient in reducing severe (AHI > 20) obstructive sleep apnea in syndromic craniosynostoses. Early airways surgery, early vault expansion and fronto-facial surgery at older ages are recommended for better respiratory results. We provide support for a protocol involving (1) early posterior vault expansion and airways surgery and (2) fronto-facial advancement performed as late as possible (>2.5 years).

Asunto(s)

Asunto(s)

RESUMEN

Upper airway stenosis in patients with faciocraniosynostosis is very common and often severe. Mid-face advancement, either with a Le Fort III or concomitantly to a monobloc frontofacial advancement, may prevent a tracheotomy or result in its ablation. The amelioration of respiratory function appears to be much better if the mid-face advancement is combined with distraction osteogenesis, although large studies with long-term follow-up are rare. In this study we reviewed the respiratory outcome between Le Fort III with distraction and monobloc advancement with distraction in 55 faciocraniosynostotic patients. Early respiratory results of both procedures were very good and stable at long-term follow-up. The choice between a Le Fort III and a monobloc procedure is made based on presenting morphology, previous surgery, and age. Both can be expected to give a long-lasting improvement of upper airway obstruction.

Asunto(s)

RESUMEN

Upper airway stenosis in patients with faciocraniosynostosis is very common and often severe. Midface advancement, either with a Le Fort III or concomitantly to a monobloc frontofacial advancement, may prevent a tracheotomy or result in its ablation. The amelioration of respiratory function appears to be much better if the midface advancement is combined with distraction osteogenesis, although large studies with long-term follow-up are rare. In this study, we reviewed the respiratory outcome between Le Fort III with distraction and monobloc advancement with distraction in 54 faciocraniosynostotic patients. Early respiratory results of both procedures were very good and stable at long-term follow-up. The choice between a Le Fort III and a monobloc procedure is made on the basis of presenting morphology, previous surgery, and age. Both can be expected to give a long-lasting improvement of upper airway obstruction.

Asunto(s)

RESUMEN

UNLABELLED: Pierre Robin sequence (posterior U-shape cleft palate, glossoptosis, retrognathia) (PRS) is a frequent and heterogeneous neonatal condition of obscure origin. We show here that orodigestive and cardiorespiratory functional disorders are very frequent in PRS and that these functional disorders, as well as anatomical and embryological data, argue for the involvement of brainstem dysfunction in the pathogenesis of some cases of isolated PRS. A total of 66 infants consecutively admitted for isolated PRS were followed-up with observations and investigations focused on their orodigestive and cardiorespiratory disorders. Neonatal clinical examination and neonatal anatomical aspects of the three orofacial features of the sequence were evaluated. Feeding difficulties and respiratory disorders were recorded and infants were classified according to three grades of severity. The relation between functional severity grade and neonatal orofacial features was evaluated, as well as the relation between functional severity grade and specific criteria characterising oesophageal and laryngeal motility and cardiac orthoparasympathetic imbalance. In the first weeks of life, sucking and swallowing disorders (100%), excessive regurgitation (94%), upper airways obstruction (50%), and cardiac vagal overactivity (59%) were noted. Correlation of anatomical features with functional severity grades was poor except for extreme forms of glossoptosis and retrognathia. Specific anomalies of oesophageal motility, pharyngolaryngeal tone and parasympathetic cardiac regulation were described. These anomalies were more frequent in children with the two higher grades of functional severity. CONCLUSION: infants with Pierre Robin sequence have early and severe anomalies of orodigestive and cardiorespiratory function which do not appear to be related solely to anatomical features and which require proper medical management. We suggest a prenatal and neonatal brainstem dysfunction as a neuroembryological hypothesis to explain the onset of some cases of Pierre Robin sequence.

Asunto(s)