Harrison Rebecca, Schaefer Simone, Warner Laura, Mercer Jean, Jones Simon, Bruce Iain
Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
Int J Pediatr Otorhinolaryngol. 2018 Aug;111:149-152. doi: 10.1016/j.ijporl.2018.04.028. Epub 2018 May 1.
Mucopolysaccharide (MPS) diseases are a heterogeneous group of inherited, metabolic disorders characterized by accumulation of partially degraded glycosaminoglycans (GAG) in multiple organ systems. Due to accumulation in the airway, patients often present with multilevel airway obstruction and obstructive sleep apnoea (OSA). Adenotonsillar surgery leads to a significant improvement in the severity of OSA in MPS patients. However, access to secure the airway and for conventional surgery can be challenging, due to limited neck extension, macroglossia and reduced mouth opening. This study was undertaken to evaluate the role of transnasal microdebridement and radiofrequent plasma ablation (Coblation) in adenoidectomy to treat OSA in patients with MPS and restricted airway access.
A retrospective case review was performed including patients with MPS undergoing adenoidectomy for OSA in the period between June 2015 and March 2017. In all cases, either a microdebrider (Gyrus Diablo) or a Coblation wand (EVAC70, Smith&Nephew) was used via a transnasal approach guided by nasendoscopy. The primary outcome was effect upon OSA, measured by sleep oximetry and parental report of benefit. The secondary outcomes were surgical complications and risk factors for persistent OSA after surgery.
A total of nine patients were identified with a mean age of 9 years (range 3-14 years) at surgery. Post-operative sleep study data was available for eight patients (8/9). Six patients (6/8) had improvement in 4% oxygen desaturation index (ODI-4) with a mean of 8.11 pre-operatively (range 2.69-14.0) and 4.99 postoperatively (range 0.68-8.48). ODI-4 did not improve in two (2/8) patients. Irrespective of sleep oximetry results, improvement in OSA-related symptoms was noted by all parents postoperatively. No risk factors for persistent OSA were identified. Furthermore, no complications were noted in this cohort.
Transnasal Coblation and Microdebrider adenoidectomy is a safe and effective surgical treatment for OSA in patients with Mucopolysaccharidosis and adenoidal hypertrophy. As lifespan increases for patients with the Mucopolysaccharidoses, greater emphasis is being given to optimising airway management over the longer-term. This technical note describes the novel application of endoscopic techniques for the management of primary adenoidal hypertrophy when transoral access is restricted, or to debulk recurrent disease that would be challenging to remove via the standard transoral route.
黏多糖（MPS）病是一组遗传性代谢紊乱疾病，其特征是部分降解的糖胺聚糖（GAG）在多个器官系统中蓄积。由于在气道内蓄积，患者常出现多级气道阻塞和阻塞性睡眠呼吸暂停（OSA）。腺样体扁桃体手术可显著改善MPS患者的OSA严重程度。然而，由于颈部伸展受限、巨舌症和张口受限，确保气道安全和进行传统手术可能具有挑战性。本研究旨在评估经鼻微创清创术和射频等离子体消融术（Coblation）在腺样体切除术中治疗气道受限的MPS患者OSA的作用。
进行一项回顾性病例分析，纳入2015年6月至2017年3月期间因OSA接受腺样体切除术的MPS患者。在所有病例中，通过鼻内镜引导经鼻途径使用微创清创器（Gyrus Diablo）或Coblation棒（EVAC70，施乐辉）。主要结局是对OSA的影响，通过睡眠血氧饱和度测定和家长报告的获益情况来衡量。次要结局是手术并发症和术后持续性OSA的危险因素。
共确定9例患者，手术时平均年龄为9岁（范围3 - 14岁）。8例患者（8/9）有术后睡眠研究数据。6例患者（6/8）的4%氧饱和度下降指数（ODI - 4）有所改善，术前平均为8.11（范围2.69 - 14.0），术后为4.99（范围0.68 - 8.48）。2例（2/8）患者的ODI - 4未改善。无论睡眠血氧饱和度测定结果如何，所有家长均指出术后OSA相关症状有所改善。未发现持续性OSA的危险因素。此外，该队列中未观察到并发症。
经鼻Coblation和微创清创器腺样体切除术是治疗黏多糖贮积症和腺样体肥大患者OSA的一种安全有效的手术方法。随着黏多糖贮积症患者寿命的延长，人们越来越重视长期优化气道管理。本技术说明描述了在内镜技术在经口入路受限的情况下用于治疗原发性腺样体肥大，或用于清除通过标准经口途径难以切除的复发性疾病的新应用。
