The Effect of Midface Advancement Surgery on Obstructive Sleep Apnoea in Syndromic Craniosynostosis.

BACKGROUND: Children with syndromic craniosynostosis frequently suffer from obstructive sleep apnoea (OSA). The aim of the authors' study was to investigate if midface advancement surgery for patients with SC improved the severity of OSA by examining the results of sleep studies before and after surgery. METHODS: A retrospective comparison of the pre and postoperative sleep study data of children undergoing midface advancement surgery at Great Ormond Street Hospital between 2007 and 2016. RESULTS: A total of 65 children underwent midface advancement surgery between 2007 and 2016 at Great Ormond Street Hospital and had recorded pre- and postoperative sleep studies. Thirteen patients were excluded from the analysis as their sleep study techniques before and after surgery were not comparable (e.g., different conditions with prong/continuous positive airway pressure use). Fifty-six percent of the patients were treated by monobloc surgery and the remainder with bipartition surgery. A greater proportion of patients had a normal OSA grading following midface advancement (42.3% postoperatively vs. 23.1% preoperatively, P = 0.059) although no statistically significant categorical changes in OSA grade were observed. Seventy-one percent of the patients had a decrease in Apnoea-Hypopnoea Index after surgery (21 patients 2011 onward). Similarly, there was no significant change in median oxygen desaturation index or in oxygen saturation nadir following surgery. CONCLUSION: The authors report one of the largest reviews of the effects of midface advancement surgery on sleep study parameters. Most patients showed improvements in Apnoea-Hypopnoea Index and OSA grading, although measures of oxygenation showed no consistent change.

Impact of long-term nasopharyngeal airway on health-related quality of life of children with obstructive sleep apnea caused by syndromic craniosynostosis.

Obstructive sleep apnea in children with syndromic craniosynostosis is a well-documented entity, and airway management in these group of children is difficult, with well-documented cardiorespiratory and neurodevelopment consequences. Numerous well-documented techniques are available for airway management in this group of children. In our center, the nasopharyngeal airway (NPA) is the first-line treatment. This study aimed to evaluate the improvement in health-related quality of life in this group of children using the Glasgow Children's Benefit Inventory. We conducted a retrospective postal study of 24 patients with craniofacial disorders who had an NPA inserted as part of their airway management. A 79.2% (n = 19) response rate was obtained. Mean (SD) age was 5.8 (4.1) years. Patients were classified into 3 main groups: Crouzon (n = 11), Apert (n = 6), and Pfeiffer (n = 2) syndromes. There was improvement in 3 of the 4 domains, namely, learning (P = 0.006), vitality (P = 0.003), physical (range, -3 to 8; mean, 2; P = 0.005). There was significant improvement in the sleep study parameters; however, no correlation was found between this and the Glasgow Children's Benefit Inventory findings. Parents also preferred an NPA as opposed to a tracheostomy when given a choice. Our study shows that NPA confers significant improvement not only in health-related quality of life but also in severity of obstructive sleep apnea as judged by sleep study parameters. The NPA is also well tolerated by patients and is much preferred by parents for airway management for their children as opposed to having a tracheostomy. We think that this should be considered in other centers as first-line management in children with syndromic craniosynostosis.

International Pediatric Otolaryngology group (IPOG) consensus on the diagnosis and management of pediatric obstructive sleep apnea (OSA).

OBJECTIVE: To develop an expert-based consensus of recommendations for the diagnosis and management of pediatric obstructive sleep apnea. METHODS: A two-iterative Delphi method questionnaire was used to formulate expert recommendations by the members of the International Pediatric Otolaryngology Group (IPOG). RESULTS: Twenty-six members completed the survey. Consensus recommendations (>90% agreement) are formulated for 15 different items related to the clinical evaluation, diagnosis, treatment, postoperative management and follow-up of children with OSA. CONCLUSION: The recommendations formulated in this IPOG consensus statement may be used along with existing clinical practice guidelines to improve the quality of care and to reduce variation in care for children with OSA.

Adenotonsillectomy for sleep-disordered breathing in children with syndromic craniosynostosis.

Syndromic craniosynostosis (SCS) is known to be associated with sleep-disordered breathing (SDB) in childhood. Problems can occur at various levels, but midface hypoplasia is a major factor in affected infants.Adenotonsillectomy (AT) has been proven to be efficacious as a first-line treatment of SDB in healthy children. However, its role in the treatment of this issue in those with SCS has not been evaluated. Owing to the multiple possible levels of obstruction in such individuals, AT may have no benefit. This study therefore aimed to evaluate the effectiveness of AT in this group of patients.Twenty-six children with SCS with moderate to severe obstructive sleep apnea (OSA) were treated with AT. The mean age at surgery was 4.5 years (range, 1.6-13.9 y). Seven had severe OSA, 11 had moderate OSA, and 7 had mild OSA preoperatively. Of 25 children, 15 (60%) demonstrated an improvement in sleep severity scores postoperatively.Overall, there was a significant improvement in the mean number of saturation dips greater than 4%/h and in the mean nadir of dips in saturation after the operation. There was no significant difference in mean oxygen saturation, number of pulse rate rises per hour, or percentage time spent less than 90% SaO2.Clinically, there is thought to be some benefit in performing AT in these groups of patients. In children with SCS, in whom the cause of obstruction might be at multiple levels, AT should still to be considered as first-line treatment in the management of OSA/SDB.We acknowledge that some children may still go on to require further airway intervention. Further multicenter trials would be useful to examine more closely the significance of the effects of AT in children with craniofacial disorders experiencing SDB.

Glottic stenosis.

Glottic stenosis is a fixed, focal narrowing at the level of the laryngeal inlet, the true vocal cords. It may be either congenital or acquired and be related to a wide range of etiologies. The stenosis may be either anterior, posterior, or in rare cases, complete. Isolated glottic stenosis is rare; lesions often involve adjacent regions, namely the subglottis. A diagnosis is made from careful history and examination, including evaluation by microlaryngoscopy and bronchoscopy. The management of glottic stenosis is challenging and should be tailored to each individual case. A secure and adequate airway is the treatment priority alongside optimization of voice and laryngeal competence. Endoscopic and open techniques in either single or multiple stages have been described.

Partial adenoidectomy by suction diathermy in children with cleft palate, to avoid velopharyngeal insufficiency.

OBJECTIVES: Adenoidectomy is indicated for the relief of paediatric nasal obstruction, sleep-disordered breathing and otitis media with effusion (OME). Velopharyngeal insufficiency (VPI) is a rare complication. The main risk factor is the presence of pre-existing velopharyngeal closure-impaired mechanisms, including submucosal or overt cleft palate. Despite possible benefits, adenoidectomy is frequently withheld in such children to avoid VPI. This study aims to demonstrate the efficacy and safety of partial adenoidectomy using suction diathermy in children who previously underwent overt cleft palate repair during infancy, to allow selective resection of tissue and symptom resolution without producing VPI. METHODS: Since 1994, 18 patients with previously corrected overt cleft palate have undergone partial adenoidectomy at this centre, for the treatment of nasal obstruction or sleep-disordered breathing, with or without OME. Three had existing VPI following their cleft correction surgery. Selective resection of the adenoid was performed transorally under indirect vision, using a malleable suction coagulator. This allowed exposure of the posterior choanae, leaving the remaining adenoid bulk intact. RESULTS: Patients were followed up at 4 weeks, and subsequently at regular intervals (total follow up 30-180 months, median 92 months), including perceptual speech assessment in all cases. All demonstrated symptomatic improvement with respect to the original indications for surgery. None developed worsening hypernasal speech or other features of VPI, and there were no cases of symptomatic adenoidal re-growth. CONCLUSIONS: Partial adenoidectomy, employing a variety of methods, has been used successfully in children with submucosal cleft palate. This study demonstrates the safe and effective use of suction diathermy to enable partial adenoidectomy in children who have previously undergone surgical correction of overt cleft palate, allowing symptom resolution without producing VPI.

The role of the nasopharyngeal airway for obstructive sleep apnea in syndromic craniosynostosis.

Syndromic craniosynostosis is known to be associated with obstructive sleep apnea (OSA), which can often present in infancy. Although multifactorial, a predominant contributing factor is midface hypoplasia. Nasal continuous positive airway pressure has proven to be an effective treatment modality but may be poorly tolerated in certain cases. This study looks at the effectiveness of bypassing midface obstruction with a nasopharyngeal airway (NPA). Twenty-seven children with syndromic craniosynostosis with confirmed moderate to severe OSA were initially treated with an NPA. The mean age of NPA insertion was 12.3 months (range, 0.5-48 mo). Seventeen had severe OSA, and 10 had moderate OSA preinsertion. Post-NPA insertion, 26 of 27 children (96%) demonstrated an improvement in sleep severity scores, resulting in 3 with moderate OSA and 24 with mild OSA. There was a significant improvement in mean oxygen saturation, mean number of saturation dips greater than 4% per hour, percentage time spent less than 90% SpO2, and number of pulse rate rises per hour. There were no significant differences in mean pulse rate. The NPA was well tolerated by this patient group, with 24 of 26 children retaining it for at least 6 weeks. We believe that an NPA is therefore an effective first-line treatment modality in the management of OSA in children with syndromic craniosynostosis. It is well tolerated by the patient and may obviate the need for continuous positive airway pressure or tracheostomy.