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首页> 外文期刊>Skeletal radiology >Progressive melorheostosis in the peripheral and axial skeleton with associated vascular malformations: imaging findings over three decades.
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Progressive melorheostosis in the peripheral and axial skeleton with associated vascular malformations: imaging findings over three decades.

机译：周围和轴向骨骼进行性进展性血管造血术伴有相关的血管畸形：三十年来的影像学表现。

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摘要

A 28-year old woman presented with Leri's disease (melorheostosis) and the rare combination of complex vascular malformations and lymphatic anomalies. Multifocal melorheostosis was segmental and unilateral, located in the left axial and peripheral skeleton, fifth thoracic vertebral body, fifth rib. left upper limb and lumbosacral spine (third lumbar body to first sacral segment). Sacral involvement was associated with spinal canal stenosis. Additionally the patient had multiple nevi and had suffered from left hemiplegia since birth. Lymphangiectasia of the mesentery and thorax led to chylothorax resistant to therapy for which the patient underwent a pleuropericardiectomy. Death ensued due to respiratory failure.
机译：一名28岁的女性表现为Leri病（melheheostosis）以及复杂的血管畸形和淋巴异常的罕见结合体。多灶性股骨造血术呈节段性和单侧性，位于左轴和外周骨架，第五胸椎椎体，第五肋骨。左上肢和腰ac脊柱（第三腰椎至第一to骨节）。 involvement骨受累与椎管狭窄有关。另外，该患者患有多发性痣并且自出生以来就患有左偏瘫。肠系膜和胸腔的淋巴管扩张症导致乳糜胸对患者进行了胸膜腹膜切除术的治疗耐药。因呼吸衰竭而导致死亡。

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