Xu Joyce, Gilbert Julie R, Sutton Kathryn S, Goudy Steven L, Abramowicz Shelly
US/CA OMS resident; Resident in Training, Oral and Maxillofacial Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, Georgia.
Fellow in Hematology and Oncology, Department of Pediatrics, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia.
J Oral Maxillofac Surg. 2022 Mar;80(3):545-552. doi: 10.1016/j.joms.2021.10.014. Epub 2021 Nov 5.
Controversy exists among head and neck surgical specialties regarding management of Langerhan's Cell Histiocytosis (LCH). The purpose of this study was to evaluate diagnosis, management, and treatment outcomes in children with LCH of the head and neck.
This is a retrospective cohort study of children with LCH of the head and neck who presented to Children's Healthcare of Atlanta hospital from 2009 to 2021. The independent variables were demographic information, lesion locations, clinical presentation, radiographic findings, diagnostic workup, treatment, and length of follow-up. The patients were grouped based on these variables. The outcome variable was disease reactivation. Descriptive statistics were calculated.
There were 3 presentations of LCH of the head and neck. Group 1 presented as a lesion in 1 system without CNS risk (SS-). There were 24 patients with an average age of 10 years. Lesions were located in calvaria and/or mandible. Majority of the patients were treated with only debridement. Two of the patients experienced reactivation. Group 2 presented as a lesion in 1 system with CNS risk (SS+). There were 30 patients with an average age of 6 years. Common locations were temporal bone and/or orbit. These patients present with recurrent ear infections and ptosis. Majority of the patients were treated with chemotherapy (n = 28). One patient had disease reactivation. Group 3 presented with multisystem involvement. There were 13 patients with an average age of 2 years. LCH was found in skin and the lymphatic system. Imaging demonstrated extracranial organ involvement. All of them were treated with chemotherapy. There was 40% reactivation of LCH.
Treatment of LCH depends on presentation. SS- subgroup can be adequately treated via surgical debridement. SS+ and multisystem groups benefit from an early disease diagnosis and require chemotherapy.
头颈部外科各专业在朗格汉斯细胞组织细胞增多症（LCH）的管理方面存在争议。本研究的目的是评估头颈部LCH患儿的诊断、管理及治疗结果。
这是一项对2009年至2021年在亚特兰大儿童医疗保健医院就诊的头颈部LCH患儿的回顾性队列研究。自变量为人口统计学信息、病变部位、临床表现、影像学检查结果、诊断检查、治疗及随访时间。患者根据这些变量进行分组。结果变量为疾病复发。计算描述性统计数据。
头颈部LCH有3种表现形式。第1组表现为1个系统中的病变，无中枢神经系统风险（SS-）。有24例患者，平均年龄10岁。病变位于颅骨和/或下颌骨。大多数患者仅接受清创治疗。2例患者出现疾病复发。第2组表现为1个系统中的病变，有中枢神经系统风险（SS+）。有30例患者，平均年龄6岁。常见部位为颞骨和/或眼眶。这些患者表现为反复耳部感染和上睑下垂。大多数患者接受化疗（n = 28）。1例患者疾病复发。第3组表现为多系统受累。有13例患者，平均年龄2岁。LCH见于皮肤和淋巴系统。影像学显示颅外器官受累。所有患者均接受化疗。LCH复发率为40%。
LCH的治疗取决于表现形式。SS-亚组可通过手术清创得到充分治疗。SS+组和多系统受累组受益于早期疾病诊断，需要化疗。
