21例儿童髂骨骨质破坏性病变的临床分析

Analysis of 21 children with iliac bone destruction

目的总结儿童髂骨骨质破坏性病变的临床特点。方法回顾性分析2013年1月至2015年12月,西安市儿童医院收治的髂骨骨质破坏患儿的临床资料。对21例患儿术后通过切除的病灶标本确定病变性质,分析其临床特点。结果患儿年龄为24天~11.5岁,中位年龄3.5岁;男︰女=2.5:1。朗格汉斯细胞增生症11例(9例多发,2例单发),骨髓炎3例,尤文肉瘤2例,多发性纤维结构不良2例,骨软骨瘤、臀部横纹肌肉瘤和急性淋巴细胞白血病各1例;有12例患儿在髂骨病灶之外还有多处病灶。所有病例影像学均表现为髂骨不同形态的骨质破坏,发生在髂骨翼19例,髋臼2例。8例伴有大小不等的软组织肿块,5例见骨膜反应。以髋部疼痛为首发症状9例,以下肢跛行为首发症状6例。经99锝全身骨扫描阳性率仅占57.1%,多病灶者准确率为66.7%。结论儿童髂骨骨质破坏以朗格汉斯细胞增生症最多见,常合并多处骨病灶。临床表现特异性不高,确诊仍需病理检查。

Objective To summarize the clinical characteristics of iliac bone destruction in children.Methods The clinical features of 21 cases with iliac bone destruction admitted in Xi ＇an Children ＇s Hospital from January 2013 to December 2015 were analyzed retrospectively.The nature of lesions incised after surgery in 21 cases was confirmed to analyze their clinical features.Results The children aged from 24 days to 11.5 years old with median age of 3.5 years.The proportion of male and female was 2.5：1.Eleven cases were found with Langerhans cell histiocytosis（9 cases of multiple histiocytosis and 2 cases of single histiocytosis）,3 cases with osteomyelitis,2 cases with Ewing＇s sarcoma,2 cases with multiple fibrous dysplasia,1 case with osteochondroma,1 case with hip rhabdomyosarcoma and 1 case with acute lymphocytic leukemia.In 12 patients there were multiple lesions besides iliac lesions.Imaging features of all cases showed iliac bone destruction in different shapes,with 19 cases in iliac wing and 2 cases in acetabulum.Eight cases had soft tissue masses of various sizes,and 5 cases had periosteal reaction.Hip pain as the first symptom was in 9 cases,and limping as the first symptom was in 6 cases.Technetium-99 bone scan revealed positive rate of 57.1%,and the accuracy for patients with more lesions was 66.7%.Conclusion Langerhans cell histiocytosis is the most common disease in children with iliac bone destruction,which often is associated with multiple bone lesions.The specificity of clinical manifestations is not high.Diagnosis still requires pathological examination.