儿童颅骨嗜酸性肉芽肿51例

Eosinophilic glanuloma of skull in children： reports of 51 cases

目的 探讨儿童颅骨嗜酸性肉芽肿的外科诊断、治疗及预后.方法 回顾性分析2003年1月至2011年12月南京儿童医院神经外科收治的51例颅骨嗜酸性肉芽肿病例的诊断和治疗经过,并进行分析.患儿入院均行头颅CT检查,48例单发颅骨病灶行扩大病灶切除术,2例多发病例行骨髓穿刺活检,1例眶部病灶仅行活检.术后行病理检查,并与血液科合作进一步治疗.结果 男女比例2.6∶1.0,年龄为（3.99±2.96）岁,主诉多为偶然发现逐渐增大的头部肿块.其中顶部最多,其次为额部、枕部、颞部及眼眶.有明显压痛的有40例（78.4％）.术后48例转血液科进一步检查,3例发现其他部位骨病灶.颅骨病变（以外板为准）为直径1.1 ～ 6.7 cm,其中病灶直径＞2 cm的有39例.所有患儿经1～5年的临床随访,平均随访时间2.1年,45例单病灶性朗格汉斯细胞增生症患儿术后转血液科进一步诊治的均末复发.1例术后自行去外院就诊未经血液科进一步检查及治疗,于2年后发现颅盖骨新病灶再次手术后转血液科化疗,随访至今未见复发.结论 单发颅骨病灶手术切除后大多痊愈无复发,预后良好,术后联合血液科医师进行全身综合筛查并坚持长期随访很重要.

Objective To investigate the clinical manifestation,diagnosis,treatment and prognosis of skull eosinophilic granuloma.Methods A retrospective analysis was performed on patients with eosinophilic granuloma of the skull who were admitted into Department of Neurosurgery Nanjing Children＇s Hospital from Jan.2003 to Dec.2011.All clinical data of 51 cases,especially in diagnosis and surgical treatment were analyzed in present study.All patients underwent head CT examination when admitted.Forty-eight patients with solitary skull lesion underwent expanded lesion resection operation.The bone marrow biopsy was performed on 2 patients with multiple skull lesions.The other one case in which lesion invaded the orbital bone got just the lesion biopsy.Every lesion removed from patient＇s skull underwent the pathological examination after the operatiou.All patients were transferred to the department of hematology for further treatment.Results In this study,male to female ratio was 2.6 ∶ 1,with an average age of （3.99 ± 2.96） years old.The most common complaint was that they accidentally discovered the head mass which was enlarged every year.The lesions were mainly located in the parietal bones,followed by frontal bones,occipital bones,temporal bones and orbital bones.There was tenderness in 40 patients （78.4%）.Forty-eight cases were transferred to hematology department for further examination.Three cases of them were found to have other parts of the bone lesions.Skull lesion diameters were between 1.1 cm and 6.7 cm.The lesion diameter over 2 cm were 39 cases.All patients had clinical follow-up of 1 to 5 years,with an average of 2.1 years.Forty-five cases with single lesion were transferred to hematology department to receive further treatment,and no relapse was found.Only one patient without further examination and treatment in hematology was found to have new bone lesions in calvaria.Conclusions Surgical resection is an effective treatment to children with skull unifocal eosinophilic granuloma.It is important to do the systemic inspection and follow up the patients with hematologists post-operation.