Management of sleep-disordered breathing in patients with syndromic hemifacial macrosomia.

IF 2.1 4区医学 Q3 CLINICAL NEUROLOGY Sleep and Breathing Pub Date : 2024-10-01 Epub Date: 2024-06-06 DOI:10.1007/s11325-024-03032-1

Barbara Madini, Sonia Khirani, Meryl Vedrenne-Cloquet, Eva Galliani, Catherine Tomat, Charlotte Célérier, Maria Francesca Patria, Lucie Griffon, Natacha Kadlub, Vincent Couloigner, Arnaud Picard, Françoise Denoyelle, Brigitte Fauroux

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Abstract

Purpose: Patients with syndromic hemifacial microsomia (SHFM) are at risk of obstructive sleep apnea (OSA). The aim of the study was to describe the prevalence of OSA and its management, especially in patients with Goldenhar syndrome (GS).

Methods: The respiratory polygraphies and clinical management of 15 patients, aged 2 to 23 years, evaluated at a national reference center, were analyzed.

Results: Four (27%) patients had no OSA, 4 (27%) had mild OSA, and 7 (46%), of whom 5 were ≤ 2 years old, had severe OSA. None of the patients had central apneas. Only one patient had alveolar hypoventilation, and another one had nocturnal hypoxemia. Two patients had severe OSA despite prior adenoidectomy or mandibular distraction osteogenesis. Median duration of follow-up was 3.5 years (range 0.5-9 years). None of the patients without OSA or with mild OSA at baseline respiratory polygraphy developed OSA during the follow up. Among the 7 patients with severe OSA, 3 required continuous positive airway pressure or noninvasive ventilation, and one patient required a tracheostomy.

Conclusion: In conclusion, patients with SHFM are at high risk of severe OSA at any age, underlining the importance of systematic sleep studies to diagnose and evaluate the severity of OSA. Individualized treatment should be privileged, based on a careful examination of the entire upper airway, taking in account potential associated risk factors. All patients with SHFM should be managed by a pediatric expert multidisciplinary medical/surgical team until the end of post pubertal growth.

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综合症半面大畸形患者的睡眠呼吸障碍管理。

目的：综合征性半颜面小畸形（SHFM）患者有患阻塞性睡眠呼吸暂停（OSA）的风险。本研究旨在描述 OSA 的患病率及其管理，尤其是在戈登哈尔综合征（GS）患者中的情况：方法：分析了在一家国家参考中心接受评估的 15 名 2 至 23 岁患者的呼吸系统测谎和临床治疗情况：结果：4 名患者（27%）无 OSA，4 名患者（27%）有轻度 OSA，7 名患者（46%）有重度 OSA，其中 5 名患者年龄小于 2 岁。所有患者均无中心性呼吸暂停。只有一名患者存在肺泡通气不足，另一名患者存在夜间低氧血症。两名患者曾接受过腺样体切除术或下颌骨牵引成骨术，但仍有严重的 OSA。中位随访时间为 3.5 年（0.5-9 年不等）。在随访期间，没有 OSA 或在基线呼吸测谎时有轻度 OSA 的患者均未发展为 OSA。在7名重度OSA患者中，3人需要持续气道正压或无创通气，1人需要气管造口术：总之，SHFM 患者在任何年龄段都是重度 OSA 的高危人群，这凸显了系统性睡眠研究对诊断和评估 OSA 严重程度的重要性。在对整个上气道进行仔细检查的基础上，考虑到潜在的相关风险因素，应优先考虑个性化治疗。所有 SHFM 患者均应由儿科专家组成的多学科医疗/外科团队进行管理，直至青春期发育结束。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Sleep and Breathing 医学-呼吸系统

CiteScore

5.20

自引率

4.00%

发文量

222

审稿时长

3-8 weeks

期刊介绍： The journal Sleep and Breathing aims to reflect the state of the art in the international science and practice of sleep medicine. The journal is based on the recognition that management of sleep disorders requires a multi-disciplinary approach and diverse perspectives. The initial focus of Sleep and Breathing is on timely and original studies that collect, intervene, or otherwise inform all clinicians and scientists in medicine, dentistry and oral surgery, otolaryngology, and epidemiology on the management of the upper airway during sleep. Furthermore, Sleep and Breathing endeavors to bring readers cutting edge information about all evolving aspects of common sleep disorders or disruptions, such as insomnia and shift work. The journal includes not only patient studies, but also studies that emphasize the principles of physiology and pathophysiology or illustrate potentially novel approaches to diagnosis and treatment. In addition, the journal features articles that describe patient-oriented and cost-benefit health outcomes research. Thus, with peer review by an international Editorial Board and prompt English-language publication, Sleep and Breathing provides rapid dissemination of clinical and clinically related scientific information. But it also does more: it is dedicated to making the most important developments in sleep disordered breathing easily accessible to clinicians who are treating sleep apnea by presenting well-chosen, well-written, and highly organized information that is useful for patient care.