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Query: UMLS:C0037315 (sleep apnea)
 8,000 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Most patients with Apert syndrome experience some degree of upper airway obstruction during the first few years of life. The pathophysiology of this form of obstruction and the clinical features are covered in detail in this article. Sleep apnea syndrome is seen commonly in association with Apert syndrome. The investigation of sleep apnea and various therapeutic modalities used to relieve the airway obstruction are presented. In addition, the otologic aspects of Apert syndrome are discussed.
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PMID:Otolaryngologic aspects of Apert syndrome. 206 91

A new technique of using implantable springs as an adjunct after corrective surgery for craniofacial malformations is presented. A 6-month-old boy with multiple premature craniosynostoses and extreme turricephaly underwent surgery of limited extensiveness but supplemented with a set of indwelling springs for gradual postoperative skull reshaping. At spring removal three months later the skull was normalised both clinically and on cephalogram. A 5-year-old boy with Apert syndrome, severe midface retrusion, exorbitism, and sleep apnoea underwent a monobloc full face disjunction without repositioning, but was fitted with two springs for postoperative facial advancement. Three months postoperatively cephalometric analysis revealed 14 mm advancement at incisor level and at least 16 mm in the frontal region. There was no more exorbitism or clinically noticeable midface retrusion. Sleep studies revealed that the sleep apnoea was significantly improved, meaning complete cure except when sleeping flat on the back. It was concluded from these first clinical applications of spring assisted craniofacial distraction that springs hold significant promise for the future in many respects.
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PMID:Spring mediated dynamic craniofacial reshaping. Case report. 978 39

Treatment of the craniofacial dysostoses (e.g., Crouzon, Apert, Pfeiffer, Saethre-Chotzen syndromes) is critically dependent on the successful advancement of the midface with a Le Fort III procedure. The purpose of this retrospective clinical outcome study was to evaluate a new technique for distracting the Le Fort III procedure and to compare its results in growing children with those of the standard Le Fort III osteotomy. The records of 22 children were reviewed; 10 patients (mean age, 6.5 years) underwent a standard Le Fort III procedure, and 12 patients (mean age, 7.5 years) underwent a Le Fort III distraction procedure. The distraction group included two separate techniques, bilateral buried distraction (n = 2) and halo distraction (n = 10). Preoperative and 2- to 3-month postoperative cephalograms were analyzed. The average horizontal advancement achieved in the standard Le Fort III group was 6 mm, compared with 19 mm of advancement in the distraction group (p </= 0.005). Complications were evenly distributed between the two groups (one infection and one tracheostomy in each group), and the lengths of hospitalization were similar. No documented improvement in sleep apnea was identified in the standard Le Fort III group. However, in the distraction group two patients experienced normalization of sleep studies postoperatively as measured by respiratory disturbance index, and two patients underwent successful decannulation of tracheotomies. For aesthetic reasons, halo distraction was preferred over bilateral buried distraction. With halo distraction the vector of traction is focused in the facial midline, which helps to reposition the concave midface and to provide a more convex facial profile. In growing children, the ideal vector for distraction is determined by the malar position and not by dental occlusion. The amount of overcorrection can be calculated from tables of normal anthropologic data. On this preliminary review, it was concluded that the use of halo distraction, in combination with a modified Le Fort III osteotomy, provided a significantly further forward-positioned midface and seemed to offer a better correction of sleep apnea than did the standard Le Fort III osteotomy.
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PMID:The Le Fort III osteotomy: to distract or not to distract? 1137 47

Syndromic craniosynostosis is a congenital disorder characterised by premature fusion of calvarial sutures combined with other anomalies. The facial appearance is different and patients may show physical impairment, mental or developmental disabilities, elevated intracranial pressure and obstructive sleep apnoea. The impact of this condition on daily functioning has not been studied before. The aim of this study is to assess the health-related quality of life in children and adolescents with syndromic or complex craniosynostosis and to determine the impact of these syndromes on parents. A prospective study was performed in 111 children. Health-related quality of life was measured by international standardised quality-of-life questionnaires, the Infant Toddler Quality of Life Questionnaire (ITQoL), Child Health Questionnaire Parental Form 50 (CHQ-PF50), Child Health Questionnaire Child Form 87 (CHQ-CF87) and Short-Form Health Survey (SF-36). For comparison, we used Dutch population norms of health-related quality-of-life-scores. Parents' scores for patients with syndromic or complex craniosynostosis were significantly lower than those for the norm population. Apert syndrome had the largest impact on the different domains. Scores on the CHQ-PF50 scales for 'physical functioning', 'parental impact emotional' and 'family activities' for these patients were significantly lower than scores for patients with other syndromes, possibly due to the complexity of the syndrome, which includes complex syndactyly, cognitive impairment and behaviour problems. Parents reported a reduced health-related quality of life for themselves, mostly psychosocial with clearly significantly lower general health perceptions. In conclusion, syndromic craniosynostosis has a large impact on the health-related quality of life of these children and their parents, both physical and psychosocial.
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PMID:Health-related quality of life in children and adolescents with syndromic craniosynostosis. 2022 16

Syndromic craniosynostosis is known to be associated with various obstructive respiratory disorders, including sleep apnoea. We performed early midfacial distraction in 11 syndromic craniosynostotic patients with obstructive respiratory apnoea and obtained good results. There were four patients with Crouzon syndrome, three patients with Pfeiffer syndrome and four patients with Apert syndrome. Their ages ranged from 7 months to 3 years, 9 months (mean: 2 years, 5 months). Midfacial distraction was performed using an internal or external device to improve obstructive respiratory disorders, such as sleep apnoea, and to avoid tracheostomy. Evaluation was performed by comparison of the pre- and postoperative polysomnograms (PSGs) and cephalograms. All patients had markedly improved respiratory disorders during sleep and avoided tracheostomy. Preoperative PSGs and cephalograms were compared with those from 1 year to 1 year, 6 months postoperatively. The postoperative PSGs and cephalograms showed marked improvements compared with preoperative PSGs and cephalograms. Syndromic craniosynostotic patients have facial bone hypoplasia, particularly of the maxilla. The tongue and parapharyngeal soft tissue become enlarged, and the enlarged tissues can cause airway obstruction. In this study, marked improvements were observed in physical evaluation using PSGs and morphological evaluation using cephalograms. Early midfacial distraction is thought to be a very useful method to reconstruct a large pharyngeal cavity, enlarge the airway, improve obstructive respiratory disorders and to avoid tracheostomy. It is important to select a device suitable for each case when surgery and distraction are performed.
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PMID:Early midfacial distraction for syndromic craniosynostotic patients with obstructive sleep apnoea. 2370 95