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一名患有软骨发育不全的儿童的胸椎朗格汉斯细胞组织细胞增多症。

Thoracic spine Langerhans cell histiocytosis in a child with achondroplasia.

作者信息

Chan Zoe, Simpson Lesley, Gallo Pasquale

机构信息

Neurosurgery, NHS Lothian, Edinburgh, UK.

Oncology, Royal Hospital for Sick Children, Edinburgh, UK.

出版信息

BMJ Case Rep. 2019 Jul 24;12(7):e228801. doi: 10.1136/bcr-2018-228801.

DOI:10.1136/bcr-2018-228801
PMID:31345829
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6663273/
Abstract

Multifocal bone Langerhans cell histiocytosis (LCH) is usually treated with prednisolone and vinblastine. We present a case conservatively treated with indomethacin with good clinical and radiological response. A 7-year-old achondroplastic boy presented with worsening thoracic back pain and leg weakness. An admission MRI spine showed a pathological T1 vertebrae fracture with posterior soft tissue extension compressing and distorting the spinal cord. A CT guided biopsy revealed an LCH. Steroids were avoided to reduce osteopenia risk and further vertebral fragility. Considering the risk of a thoracic surgical approach in a child with this background, he was managed conservatively with indomethacin and a Sternal Occipital Mandibular Immobilizer (SOMI) Brace. Pain resolved completely within 6 months and the brace was discontinued. Serial follow-up scans showed progressive resolution of the pathological T1 fracture and complete resolution of the spinal cord compression.

摘要

多灶性骨朗格汉斯细胞组织细胞增多症(LCH)通常采用泼尼松龙和长春碱进行治疗。我们报告了1例采用吲哚美辛保守治疗且临床和影像学反应良好的病例。一名7岁的软骨发育不全男孩出现胸背部疼痛加重和腿部无力症状。入院时的脊柱MRI显示T1椎体病理性骨折,伴有后方软组织延伸,压迫并扭曲脊髓。CT引导下活检显示为LCH。为降低骨质减少风险和进一步的椎体脆性,未使用类固醇。考虑到在此背景下对儿童进行开胸手术的风险,对他采用吲哚美辛和胸骨枕下颌固定器(SOMI)支具进行保守治疗。疼痛在6个月内完全缓解,支具停用。系列随访扫描显示病理性T1骨折逐渐愈合,脊髓压迫完全解除。

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Pediatr Blood Cancer. 2013 Feb;60(2):175-84. doi: 10.1002/pbc.24367. Epub 2012 Oct 25.
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