Grouteau E, Chaix Y, Armbruster V, Lesueur L, Sevely A, Rubie H, Carrière J P
Service de médecine infantile A, médecine infantile G, CHU Purpan, Toulouse, France.
Arch Pediatr. 1998 Feb;5(2):153-8. doi: 10.1016/s0929-693x(97)86829-7.
Orbital pseudo-tumors account for approximately 8% of the orbital tumors. They are scarcely described in children and raise diagnostic dilemas, especially when they present as an isolated extra-ocular muscle swelling called idiopathic orbital myositis (IOM).
A diagnosis of inflammatory orbital pseudotumors (IOPT) was made in three children aged 7, 13 and 14 years from clinical and CT scan and/or MRI findings associated with histological data in two of them. Two of these IOPT presented as IOM. Analysis was made in a effort to eliminate intra-orbital tumors, especially rhabdomyosarcoma.
The abrupt onset of the orbital signs, often related in the literature, was not a specific diagnostic criterion regarding to embryonic rhabdomyosarcoma. The inconstant presence of inflammatory signs and the absence of local osseous defect could be more discriminating. Although diagnostic procedure, including histological documentation, was difficult, even dangerous, surgical biopsy should be recommended. Eventually, considering the mandatory multidisciplinary care, steroid therapy response should be evaluated as a diagnostic test.
眼眶假瘤约占眼眶肿瘤的8%。儿童眼眶假瘤鲜有报道,且会引发诊断难题,尤其是当它表现为孤立的眼外肌肿胀,即特发性眼眶肌炎(IOM)时。
根据临床、CT扫描和/或MRI检查结果,并结合其中两例的组织学数据,对3名年龄分别为7岁、13岁和14岁的儿童诊断为炎性眼眶假瘤(IOPT)。其中两例IOPT表现为IOM。进行分析以排除眶内肿瘤,尤其是横纹肌肉瘤。
眼眶症状的突然发作在文献中常被提及,但并非胚胎性横纹肌肉瘤的特异性诊断标准。炎症体征的不恒定出现以及局部骨质缺损的缺失可能更具鉴别意义。尽管包括组织学记录在内的诊断过程困难甚至危险,但仍应建议进行手术活检。最终,考虑到必须进行多学科治疗,应将类固醇治疗反应作为一种诊断试验进行评估。