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儿童淋巴管畸形所致先天性巨舌症的管理及文献综述

Management of Congenital Macroglossia Due to Lymphatic Malformation in a Child with Review of Literature.

作者信息

Achugatla Sushma, Singh Surendra, Thakur Sonia, Redkar Rajeev

机构信息

Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India.

出版信息

J Indian Assoc Pediatr Surg. 2025 May-Jun;30(3):403-406. doi: 10.4103/jiaps.jiaps_281_24. Epub 2025 Apr 10.

Abstract

Lymphangiomas are uncommon congenital hamartomas of the lymphatic system with a marked predilection for the head-and-neck region accounting for 75% of all cases. Lymphangiomas rarely affect the oral cavity. Affected sites in the oral cavity may include the tongue, palate, gingiva, lips, and alveolar ridge of the mandible. The anterior two-thirds on the dorsal surface of the tongue is the most common site for intraoral lymphangiomas leading to macroglossia, similar to the presentation in our case. Congenital macroglossia is a rare clinical condition characterized by an enlarged tongue. This enlargement can lead to a variety of oral and facial problems, with structural deformities such as diastema, disproportionate mandibular growth, and functional disorders such as difficulty in eating and swallowing, speech impairment, or even difficulty in breathing if it is severe. Macroglossia may be attributed to multiple underlying causes. These include conditions such as idiopathic muscular hypertrophy (notably in Beckwith-Wiedemann syndrome), vascular malformations (such as angiomas and lymphangiomas), tumors, and endocrine disorders. Among these causes, lymphangiomatous macroglossia is particularly rare and is characterized by diffuse involvement. This type of macroglossia typically progresses chronically, reaching a developmental plateau at puberty without any spontaneous regression. We report a case of congenital macroglossia in an 8-year-old male due to lymphatic malformation and managed successfully.

摘要

淋巴管瘤是一种罕见的先天性淋巴系统错构瘤,显著好发于头颈部,占所有病例的75%。淋巴管瘤很少累及口腔。口腔内的受累部位可能包括舌、腭、牙龈、唇以及下颌牙槽嵴。舌背前三分之二是口腔内淋巴管瘤最常见的部位,可导致巨舌症,与我们病例中的表现相似。先天性巨舌症是一种罕见的临床病症,其特征为舌体增大。这种增大可导致多种口腔和面部问题,包括结构畸形,如牙间隙、下颌生长不均衡,以及功能障碍,如进食和吞咽困难、言语障碍,严重时甚至呼吸困难。巨舌症可能由多种潜在原因引起。这些原因包括特发性肌肉肥大(尤其是在贝克威思-维德曼综合征中)、血管畸形(如血管瘤和淋巴管瘤)、肿瘤以及内分泌紊乱。在这些原因中,淋巴管瘤性巨舌症尤为罕见,其特点是弥漫性受累。这种类型的巨舌症通常呈慢性进展,在青春期达到发育稳定状态,且不会自发消退。我们报告一例8岁男性因淋巴管畸形导致先天性巨舌症并成功治疗的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/606f/12094591/0c71b82d8f5c/JIAPS-30-403-g001.jpg

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