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一名5岁男孩,患有一个长入胸腔的巨大遗传性多发性骨软骨瘤肿块。

A 5-year-old boy with a large hereditary multiple exostoses lump grown into thoracic cavity.

作者信息

Huang Lijun, Lu Qiang, Yan Xiaolong, Han Yong

机构信息

Department of Thoracic Surgery, Tangdu Hospital, Fourth Military Medical University, Xi'an, China.

出版信息

Ann Thorac Cardiovasc Surg. 2014;20 Suppl:528-30. doi: 10.5761/atcs.cr.12.01989. Epub 2013 Jan 31.

Abstract

Multiple exostosis is associated with the autosomal dominant disorder and hereditary multiple exostoses (HMEs), are rarely growing around the ribs and the formation of a large lump into the thoracic cavity. It's generally agreed that when there are no symptoms present, a HME patient could either avoid any treatment or postpone the operation until adolescence. We present a 5-year-old boy with 4 cm × 3.5 cm HMEs lump invasion into the thoracic cavity, with symptoms of chest distress and right chest pain. This patient received video-assisted thoracoscopic surgery (VATS) and a the lump and 1 cm rib involved was resected. During a 2 years follow-up, the patient remained in good condition without recurrence and complications.

摘要

多发性外生骨疣与常染色体显性疾病及遗传性多发性外生骨疣(HMEs)相关,很少在肋骨周围生长并在胸腔内形成大肿块。人们普遍认为,当HME患者没有症状时,可以避免任何治疗或推迟手术至青春期。我们报告一名5岁男孩,其有一个4 cm×3.5 cm的HMEs肿块侵入胸腔,伴有胸闷和右胸痛症状。该患者接受了电视辅助胸腔镜手术(VATS),切除了肿块及受累的1 cm肋骨。在2年的随访中,患者情况良好,无复发及并发症。

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