Pelc Hugh J S, Walker Jenny, Davies Geraint
Sheffield Children's Hospital, Western Bank, Sheffield, UK.
J Pediatr Orthop B. 2013 Sep;22(5):505-8. doi: 10.1097/BPB.0b013e32835875d2.
This report outlines the management of a large intrathoracic lesion found in a 2-year-old girl with hereditary multiple exostosis. The lesion arose from the right eighth rib and comprised two separate osteochondromata that had coalesced into a single lesion and caused significant deformity to the chest wall. Aside from the deformity, the lesions were asymptomatic. Further growth of the lesions could cause respiratory complications, worsening of the visible deformity and, being lesions of the axial skeleton, bear an increased risk of malignant change. The lesions and the attached eighth rib were removed operatively and the patient recovered without complications. We demonstrate a place for the operative management of asymptomatic lesions in anticipation of future difficulties or malignant changes.
本报告概述了一名患有遗传性多发性骨软骨瘤的2岁女童体内发现的巨大胸腔内病变的处理情况。该病变起源于右第八肋骨，由两个独立的骨软骨瘤融合形成单个病变，导致胸壁严重畸形。除畸形外，病变无症状。病变进一步生长可能导致呼吸并发症、可见畸形加重，并且作为中轴骨病变，发生恶性变的风险增加。通过手术切除病变及附着的第八肋骨，患者康复且无并发症。我们证明了对于无症状病变进行手术处理以预防未来出现困难或恶性变是有必要的。
