Kajal Pradeep, Dhingra Himanshi, Bhutani Namita
Department of Paediatric Surgery, PGIMS Rohtak, Haryana, India.
Department of Paediatric Surgery, PGIMS Rohtak, Haryana, India.
Int J Surg Case Rep. 2023 Nov;112:108940. doi: 10.1016/j.ijscr.2023.108940. Epub 2023 Oct 11.
Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. Its aetiology is still unknown, and its clinical spectrum is quite wide.
A 2-year-old-male child presented to us with a solitary swelling in left mandibular region which was painless and increasing in size with time. It was diagnosed to be unifocal LCH of mandible on the basis of X-ray, ultrasonography of the involved mandible and fine needle aspiration cytology of the swelling and managed conservatively with oral steroids.
LCH is often classified as single system, when the disease affects only one part of the body; or multisystem, when it affects more than one part of the body (Jezierska et al., 2018 [1]). In children, histiocytosis usually involves the bones and may consist of single or multiple sites. The skull is frequently affected. Children over five years of age usually have the single system disease, with just bone involvement but our patient was 2-year-old and had unifocal disease involving mandible. Young children, especially infants, are more likely to have the multisystem disease (Jezierska et al., 2018 [1]).
Mandibular involvement associated with LCH is quite uncommon in paediatric population. Early diagnosis and prompt initiation of treatment are key to a good eventual outcome.
朗格汉斯细胞组织细胞增多症（LCH）是一种树突状单核细胞增殖性疾病，可局部或弥漫性浸润器官。其病因尚不清楚，临床谱较广。
一名2岁男童因左下颌区出现孤立性肿胀前来就诊，该肿胀无痛，且随时间推移逐渐增大。根据X线、受累下颌骨的超声检查以及肿胀部位的细针穿刺细胞学检查，诊断为下颌骨单灶性LCH，并采用口服类固醇进行保守治疗。
当疾病仅累及身体的一个部位时，LCH通常被分类为单系统疾病；当累及身体多个部位时，则被分类为多系统疾病（Jezierska等人，2018 [1]）。在儿童中，组织细胞增多症通常累及骨骼，可能为单发病灶或多发病灶。颅骨常受累。五岁以上儿童通常患有单系统疾病，仅累及骨骼，但我们的患者为2岁，患有累及下颌骨的单灶性疾病。幼儿，尤其是婴儿，更易患多系统疾病（Jezierska等人，2018 [1]）。
在儿科人群中，与LCH相关的下颌骨受累情况相当罕见。早期诊断和及时开始治疗是获得良好最终结局的关键。
