局限性喉淀粉样变:儿科患者的罕见诊断。病例报告。

Javiera Pardo Jadue, Camila Ramírez Pelayo, Andrés Lanas Volz
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引用次数: 0

摘要

目的:本研究的主要目的是报道一例罕见的小儿局限性喉淀粉样变病例,这种情况在全球范围内只有18例18岁以下的病例。此外,它比较了诊断方法和管理的情况下,概述了那些在有限的现有文献。方法:一名10岁男性患者表现为逐渐恶化的语音障碍。临床评估包括鼻纤维镜、CT增强成像和切除组织的组织病理学检查。采用刚果红染色证实组织学诊断。其他检查包括血清生化和超声心动图,以排除全身性淀粉样变性。术后随访3年。结果:经组织病理学分析,确诊为喉部局限性淀粉样变。手术治疗包括冷解剖部分切除,然后用CO2激光完全宏观切除。术后恢复显示轻微的发音障碍,无呼吸困难。随访评估,包括鼻纤维镜检查,显示三年后没有明显的疾病复发,只有一个小的右后部白斑,解释为撞击相关病变。结论:局限性喉淀粉样变性是一种罕见的儿科疾病。早期诊断至关重要,经口内镜切除手术治疗对改善症状有效。由于复发的可能性很高,特别是在头五年内,定期随访是必要的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Localized laryngeal amyloidosis: an unusual diagnosis in pediatric patients. Case report.

Purpose: The primary objective of this study was to present a rare case of localized laryngeal amyloidosis in a pediatric patient, a condition with only 18 documented cases worldwide in individuals under eighteen years of age. Furthermore, it compares the diagnostic approach and management of this case with those outlined in the limited existing literature.

Methods: A 10-year-old male patient presented with progressively worsening dysphonia. Clinical evaluation included nasofibroscopy, contrast-enhanced CT imaging, and histopathological examination of resected tissue. The histological diagnosis was confirmed using Congo red staining. Additional tests were conducted to rule out systemic amyloidosis, including serum biochemistry and echocardiogram. The patient was followed for three years postoperatively.

Results: The patient was diagnosed with localized laryngeal amyloidosis, confirmed through histopathological analysis. Surgical management included a partial excision using cold dissection, followed by complete macroscopic resection with CO2 laser. Postoperative recovery showed minimal dysphonia with no dyspnea. Follow-up evaluations, including nasofibroscopy, revealed no significant recurrence of disease after three years, with only a small right posterior leukoplakia, interpreted as an impact-related lesion.

Conclusion: Localized laryngeal amyloidosis is an rare condition in pediatric patients. Early diagnosis is critical, and surgical management via transoral endoscopic resection is effective in improving symptoms. Regular follow-up is necessary due to the high likelihood of recurrence, especially within the first five years.

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