Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, MA, USA.
Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, MA, USA.
Int J Pediatr Otorhinolaryngol. 2024 Nov;186:112112. doi: 10.1016/j.ijporl.2024.112112. Epub 2024 Sep 14.
Pediatric dysphagia is a commonly encountered clinical problem, with primary cricopharyngeal achalasia being a rare etiology. Management options for this condition include observation, medical and surgical management. The goal of this review paper was to summarize the current literature on the topic with respect to patient presentation, diagnosis and surgical management options.
The inclusion criteria were as follows: pediatric patients less than age 18 years, case series featuring equal to or greater than two patients, and any article that described surgical interventions pertaining to primary cricopharyngeal achalasia. Exclusion criteria included patients greater than 18 years of age, those with lower esophageal sphincter pathology (achalasia), non-English articles and case reports consisting of only one patient. A search was run on the PubMed/Medline, OVID, EMBASE, Cochrane, and Web of Science databases on November 1, 2023. The validated bias tool from the Institute of Health Economics was used for bias screening. The results were synthesized using pooled analysis and descriptive statistics.
Ten case series were identified that met inclusion criteria. The most common symptoms at presentation were choking/regurgitation, aspiration and failure to thrive. The most utilized diagnostic test that identified cricopharyngeal achalasia abnormality was the video fluoroscopic swallow study. Management options are dictated by the patient's symptoms and include observation, treating concomitant co-morbidities, balloon dilation, botulinum injection, and endoscopic or open cricopharyngeal myotomies. While botulinum toxin injections are reported to be effective, they often need to be repeated and thus offer a temporizing strategy to allow the natural history of the condition to declare itself. Surgical myotomy represents a more definitive management strategy compared to balloon dilation but has associated risks.
There are limited studies available regarding the diagnosis and management of CPA in children. The studies that are available are mainly case series, with low sample sizes and heterogeneous data. This systematic review highlights the importance of keeping this diagnosis within the differential for infants and pediatric patients with dysphagia to minimize delays in diagnosis and provides updated data on the presentation and surgical management of this condition. There was a limitation of evidence in this systematic review, including the small number of articles that were identified, and limited sample size of patients within the articles themselves. Consequently, the analysis was not amenable to a meta-analysis.
小儿吞咽困难是一种常见的临床问题，原发性环咽肌失弛缓症是一种罕见的病因。这种情况的治疗选择包括观察、药物和手术治疗。本文的目的是总结目前关于该疾病的文献，包括患者的临床表现、诊断和手术治疗选择。
纳入标准为：年龄小于 18 岁的儿科患者，病例系列研究中包含等于或多于 2 例患者，以及任何描述与原发性环咽肌失弛缓症相关的手术干预的文章。排除标准包括年龄大于 18 岁的患者、食管下括约肌病理（失弛缓症）患者、非英文文章以及仅包含 1 例患者的病例报告。2023 年 11 月 1 日，在 PubMed/Medline、OVID、EMBASE、Cochrane 和 Web of Science 数据库中进行了搜索。使用来自卫生经济学研究所的验证偏倚工具进行偏倚筛选。结果使用合并分析和描述性统计进行综合。
确定了 10 篇符合纳入标准的病例系列研究。最常见的表现症状为呛咳/反流、吸入和生长发育不良。确定环咽肌失弛缓症异常的最常用诊断测试是视频荧光透视吞咽研究。治疗方案取决于患者的症状，包括观察、治疗并存的合并症、球囊扩张、肉毒杆菌毒素注射以及内镜或开放环咽肌切开术。虽然肉毒杆菌毒素注射被报道有效，但它们通常需要重复注射，因此是一种暂时的治疗策略，可以让疾病的自然病程自行显现。与球囊扩张相比，手术切开术是一种更有效的治疗策略，但有相关风险。
关于儿童 CPA 的诊断和治疗，可用的研究有限。现有的研究主要是病例系列研究，样本量小且数据异质性大。本系统综述强调了在婴儿和小儿吞咽困难患者中保持这种诊断的重要性，以尽量减少诊断延误，并提供了关于这种疾病的表现和手术治疗的最新数据。本系统综述存在证据局限性，包括确定的文章数量较少，以及文章中患者的样本量有限。因此，分析不适合进行荟萃分析。
