朗格汉斯细胞组织细胞增生症 (Langerhans cell histiocytosis,LCH) 是一种由骨髓细胞肿瘤性增殖引起的罕见疾病,多见于儿童。LCH临床表现多样,以骨骼受累最常见。该文报道了一例儿童颅眶沟通LCH,影像学检查结果提示患儿右侧眉弓处类圆形穿凿样骨质破坏,通过手术切除病灶,送组织病理学检查明确诊断,同时选择通过3D打印聚醚醚酮(polyetheretherketone,PEEK)材料修补颅眶缺损部位,PEEK材料匹配度高、安全性好,改善患儿预后,提升患儿生存质量。
Langerhans cell histiocyte (LCH) is a rare disease caused by the tumor-like proliferation of bone marrow cells, which is mostly seen in children. Its clinical manifestations can be diverse, in which the skeletal system is most involved. This paper reports a case of LCH in cranio-orbital communication of a child. The imaging results suggest that there is a round chisel damage at the patient’s right brow ridge. In terms of definitive diagnosis and treatment, this patient underwent surgical resection and histopathological examination. 3D-printed polyether-ether-ketone (PEEK) material was selected to repair the cranio-orbital defect. The material can achieve better biocompatibility, while 3D-printing technique allows higher matching degree, both help to improve the prognosis and quality of life of the patient.
患者,男性,1岁9个月。以“发现右眼上、下眼睑肿物25 d”首诊于眼科,要求切除,但经影像学及病理学检查,诊断为多发性朗格汉斯细胞组织细胞增生症,且全身骨骼多处出现溶骨性改变,不符合切除指征。给予多次全身化学治疗后眼部肿物明显变小。该例诊治提醒眼科医生,眼部肿物可由全身系统性疾病引起,不可盲目切除,必要时做进一步检查。术中切除物均建议行病理活组织检查,以免延误治疗。
patient, male, 1year and 9months old, was first diagnosed as “eye tumor” in the ophthalmology department and requested for excision. But it was diagnosed as multiple Langerhans cell histiocytosis (LCH) through imaging and pathological examination ultimately.Bone lytic changes appeared in many parts of the whole body, which did not meet the indication of excision.The tumor was smaller after systemic chemotherapy. The diagnosis and treatment of this case suggests ophthalmologists that eye tumors can be caused by caused by systemic diseases, systemic diseases. During operation, it is recommended to perform pathological biopsy to avoid treatment delay.