科尼莉亚-德-朗格综合征儿科患者鼻息肉病的治疗:病例系列和文献综述。

Ian F Caplan, Michael Ye, Aaron N Pearlman
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引用次数: 0

摘要

科尼莉亚-德-朗格综合征(CdLS)是一种罕见的遗传性多发性畸形疾病,有许多耳鼻喉科合并症。CdLS 患者中,慢性鼻炎(CRS)和慢性鼻炎伴鼻息肉病(CRSwNP)的发病率似乎较高,但描述 CdLS 患者中 CRSwNP 的表现、评估和管理的文献却很有限。在此,我们对 Embase、PubMed、Cochrane 图书馆和谷歌学术进行了文献综述,并报告了本院发现的一例 CRSwNP 并发 CdLS 的病例。我们描述了一名 8 岁男性患者的病史、体格检查、鼻内窥镜检查和计算机断层扫描证实其患有 CdLS 和 CRSwNP。鼻塞症状经药物治疗无效,需要再次手术治疗,但鼻塞症状有所改善。文献综述发现,还有 2 例鼻息肉病例采取了类似的治疗策略。此外,一个病例系列显示 33% 的 CdLS 患者有复发性鼻窦炎,而第二个病例系列发现 39% 的 CdLS 患者有 CRS,12% 有 CRSwNP。这些数据表明,与普通人相比,CdLS 患者中 CRSwNP 的发病率更高,可以通过药物和手术治疗相结合的方法安全有效地控制 CRSwNP。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Management of Nasal Polyposis in Pediatric Patients With Cornelia de Lange Syndrome: A Case Series and Literature Review.

Cornelia de Lange syndrome (CdLS) is a rare genetic multiple malformation disorder with many otolaryngologic comorbidities. Patients with CdLS appear to have an increased prevalence of chronic rhinosinusitis (CRS) and chronic rhinosinusitis with nasal polyposis (CRSwNP), however, there is limited literature describing the presentation, evaluation, and management of CRSwNP within the CdLS population. Here we performed a literature review from Embase, PubMed, Cochrane Library, and Google Scholar and reported a case of CRSwNP with concomitant CdLS that was identified at our institution. We describe an 8-year-old male with CdLS and CRSwNP confirmed by history, physical exam, nasal endoscopy, and computed tomography. Symptoms of nasal obstruction were refractory to medical management and required repeat surgical management with improvement in nasal obstruction. Literature review identified 2 additional cases of nasal polyposis with similar management strategies. Additionally, one case series showed 33% of CdLS patients had recurrent sinusitis while a second series identified 39% of CdLS patients with CRS and 12% with CRSwNP. These data suggest that CRSwNP is more prevalent in patients with CdLS compared to the general public and can be both safely and effectively managed with a combination of medical and surgical therapy.

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