一名5岁女孩的口腔肿块

文献信息

中文摘要

背景： 淋巴管畸形是罕见的先天性血管异常，最常累及头颈部区域。延伸至口腔或上气道的颈面部病变可导致吞咽困难、出血、营养不良和气道梗阻。急诊评估尤其具有挑战性，因为即使生理参数保持稳定，也可能存在显著的解剖变形，因此需要进行全面的气道评估并及早预判潜在的临床恶化。
病例报告： 一名近期从秘鲁移民的5岁女孩因进行性增大的口腔肿块就诊，导致言语受损、无法闭口、吞咽困难、间歇性黏膜出血和体重增长不良，需行胃造瘘术。她既往有需行气管切开术的气道梗阻病史。就诊时，她心动过速，但氧合维持充分，无喘鸣或呼吸窘迫。体格检查显示巨舌症伴舌部紫癜性水疱、下颌骨增大和黏膜出血。急诊处理重点为气道评估、持续监测、气道准备和早期多学科会诊。实验室检查显示重度小细胞性贫血，可能继发于慢性出血。患者入院在麻醉支持下接受控制性磁共振成像，发现一个巨大的跨间隙颈面部肿块，累及下面部、口底、下颌下区和舌根。活检证实为微囊型淋巴管畸形。治疗包括输血、营养优化、分期硬化治疗、淋巴管栓塞、部分舌切除术和黏膜重建。为什么急诊医师应了解此病？：患有广泛颈面部淋巴管畸形的儿童尽管存在高危气道解剖结构，临床上仍可能表现稳定。急诊医师应认识到病情可能迅速恶化，避免不必要的 airway 操作，评估出血和贫血等并发症，尽早寻求多学科会诊，并考虑在受控条件下收治入院以进行影像学检查或操作。

英文摘要

BACKGROUND: Lymphatic malformations are rare congenital vascular anomalies that most commonly affect the head and neck regions. Cervicofacial lesions extending into the oral cavity or upper airway can result in dysphagia, bleeding, malnutrition, and airway obstruction. Emergency assessment is particularly challenging because significant anatomic distortion can exist even when physiologic parameters remain stable, necessitating thorough airway evaluation and early anticipation of potential clinical deterioration.
CASE REPORT: A 5-year-old girl who had recently emigrated from Peru presented with a progressively enlarging oral mass, resulting in impaired speech, inability to close her mouth, dysphagia, intermittent mucosal bleeding, and poor weight gain necessitating gastrostomy. She had a history of prior airway obstruction requiring tracheostomy. On presentation, she was tachycardic but maintained adequate oxygenation without stridor or respiratory distress. Physical examination revealed macroglossia with purpuric lingual blebs, mandibulomegaly, and mucosal bleeding. Emergency management focused on airway assessment, continuous monitoring, airway preparedness, and early multidisciplinary consultation. Laboratory evaluation demonstrated severe microcytic anemia, likely secondary to chronic bleeding. The patient was admitted for controlled magnetic resonance imaging with anesthesia support, which identified a large transspatial cervicofacial mass involving the lower face, floor of mouth, submandibular regions, and tongue base. Biopsy confirmed a microcystic lymphatic malformation. Management included transfusion, nutritional optimization, staged sclerotherapy, lymphatic embolization, partial glossectomy, and mucosal resurfacing. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Children with extensive cervicofacial lymphatic malformations may appear clinically stable despite having high-risk airway anatomy. Emergency physicians should recognize the potential for rapid deterioration, avoid unnecessary airway manipulation, assess for complications such as bleeding and anemia, seek early multidisciplinary consultation, and consider admission for imaging or procedures under controlled conditions.