Thoracolumbar scoliosis in a patient with Proteus syndrome: a case report and literature review.
Thoracolumbar scoliosis in a patient with Proteus syndrome: a case report and literature review.
复制标题
变形杆菌综合征胸腰段脊柱侧凸一例报告及文献复习
DOI：
10.1097/md.0000000000000360
复制
发表时间：
2015-02
期刊：
影响因子：
1.6
通讯作者：
Liang J
中科院分区：
文献类型：
--
作者：
Li Z;Shen J;Liang J
Proteus 综合征 (PS) 是一种复杂且罕见的先天性错构瘤疾病，具有多种畸形。关于与这种综合征相关的脊柱畸形的报道很少。本研究介绍了 PS 背景下发生的脊柱侧凸病例，并探讨了两种疾病之间的可能机制。患者为一名17岁的中国女性，患有脊柱侧凸、右上、下肢偏肥大，以及右小腿关节（包括髋、膝、踝、趾）外生骨疣。这些表现都暗示着PS。她使用 Moss-SI 脊柱系统在胸椎 2-腰椎 4 (T5-L4) 水平接受了后路矫正。在 3 个月的随访中，患者临床上无疼痛且平衡良好。平片显示脊柱融合牢固，没有丧失畸形矫正能力。 PS 中脊柱侧凸的严重程度逐渐加重，广泛的脊柱畸形的矫正通常很困难。因此，需要早期诊断以进行充分的跨学科治疗。
The Proteus syndrome (PS) is a complex and rare congenital hamartomatous condition with a wide range of malformations. Little is reported about spinal deformity associated with this syndrome. This study presents a case of scoliosis occurring in the setting of PS and explores the possible mechanisms between the 2 diseases. The patient is a 17-year-old Chinese female with scoliosis and hemihypertrophy of the right upper and lower extremity as well as exostosis of the right lower leg joint including the hip, knee, ankle, and toes. These manifestations were suggestive of PS. She underwent a posterior correction at thoracic 2-lumbar 4 (T5–L4) levels, using the Moss-SI spinal system. At 3-month follow-ups, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. The severity of scoliosis in PS is progressively aggravated and the correction of the extensive spinal deformities is generally difficult. Therefore, early diagnosis is required for adequate interdisciplinary treatment.