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一例伴有严重椎管狭窄、脊柱侧凸和胸壁畸形的脑-内脏脂肪瘤病,合并脊髓栓系。

A case of Proteus syndrome with severe spinal canal stenosis, scoliosis, and thoracic deformity associated with tethered cord.

机构信息

Department of Radiology, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8606, Japan.

出版信息

Jpn J Radiol. 2012 May;30(4):336-9. doi: 10.1007/s11604-011-0044-5. Epub 2012 Jan 7.

Abstract

Proteus syndrome is a rare, sporadic, hamartomatous disorder manifesting with multifocal overgrowth of tissue. The features seem to develop most often during childhood. Vertebral overgrowth with severe spinal canal stenosis is unusual, although scoliosis with abnormal vertebral bodies is one of the typical features of Proteus syndrome. We report a case of Proteus syndrome with severe spinal canal stenosis, scoliosis, cervical kyphosis, and thoracic deformity with airway obstruction because of asymmetrical overgrowth of vertebrae and ribs associated with a tethered cord, lipomas, strawberry hemangioma, flat nasal bridge, and bilateral hypoplasty of the first metatarsal bones with hyperplasty of soft tissue.

摘要

脑颜面血管瘤病合并脊髓拴系

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