Kalbermatten N T, Vock P, Rüfenacht D, Anderson S E
Department of Diagnostic Radiology, University Hospital, Bern, Switzerland.
Skeletal Radiol. 2001 Jan;30(1):48-52. doi: 10.1007/s002560000283.
A 28-year old woman presented with Léri'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.
一名28岁女性患有勒里氏病（肢骨纹状肥大症），并伴有复杂血管畸形和淋巴管异常这种罕见的组合情况。多灶性肢骨纹状肥大症呈节段性且为单侧，位于左轴性骨骼和周围骨骼、第五胸椎椎体、第五肋骨、左上肢以及腰骶椎（第三腰椎体至第一骶椎节段）。骶骨受累伴有椎管狭窄。此外，该患者有多处痣，自出生以来一直患有左侧偏瘫。肠系膜和胸部的淋巴管扩张导致乳糜胸，对治疗耐药，为此患者接受了胸膜心包切除术。最终因呼吸衰竭死亡。
