马凡综合征伴脊柱畸形患者脊柱-骨盆矢状面的形态特征

Spino-pelvic alignment in patients with spinal deformity secondary to Marfan syndrome

中文关键词:  马凡综合征  脊柱畸形  矢状面  骨盆

英文关键词:Marfan syndrome  Spinal deformity  Sagittal  Pelvis

中文摘要:

  【摘要】 目的：探索马凡综合征伴脊柱畸形患者脊柱-骨盆矢状面的形态特征。方法：收集以脊柱畸形来我院就诊的马凡综合征患者35例，男18例，女17例，年龄10~20岁，平均14.4±2.3岁。在站立位全脊柱侧位X线片上测量脊柱及骨盆矢状面参数，包括：（1）胸椎后凸角（thoracic kyphosis，TK），（2）胸腰段后凸角（thoracolumbar kyphosis，TL），（3）腰椎前凸角（lumbar lodorsis，LL），（4）骨盆入射角（pelvic incidence，PI），（5）骨盆倾斜角（pelvic tilt，PT），（6）骶骨倾斜角（sacral slope，SS），（7）矢状面平衡（sagittal vertical axis，SVA）。定义顶椎在T12、L1或者T12/L1椎间盘，后凸角度>10°的后凸为胸腰段后凸；顶椎在L1/2椎间盘或以下椎体、椎间盘，后凸角度>10°的后凸为腰椎后凸。采用Sponseller分型方法对患者脊柱矢状面形态进行分型，比较不同分型患者脊柱-骨盆矢状面形态。结果：本组患者在冠状面上以胸腰双弯（40.0%）、单胸弯（22.8%）以及三弯（20.0%）最常见，最大Cobb角43°～165°，平均75.2°±26.0°。在脊柱矢状面上，TK为-25°～73°（19.0°±24.1°），其中胸椎后凸正常者（20°≤TK≤50°）10例（28.6%）；胸椎后凸增大患者（TK>50°）5例（14.3%）；胸椎后凸减小者（0°≤TK<20°）13例（37.1%）；另有7例（20.0%）患者表现为胸椎前凸。TL为-25°～73°（14.0°±19.0°）；LL为-17°～70°（37.1°±23.3°）；SVA为-9.0~7.2cm（-2.0±4.3cm）。15例（42.9%）患者表现为胸腰段后凸或腰椎后凸（9例ⅡA型，6例ⅡB型），5例患者表现为后凸区明显的椎体楔形变。骨盆矢状面上，PI为25°～74°（40.1°±12.7°）；PT为-12°～34°（6.9°±9.6°）；SS为14°～68°（33.3°±12.6°）。Sponseller分型Ⅰ型患者TK、LL、PI、SS明显大于Ⅱ型患者，而Ⅱ型患者TL明显大于Ⅰ型患者。未见腰椎滑脱现象。结论：马凡综合征伴脊柱畸形患者脊柱-骨盆矢状面形态差异较大，手术医生应该根据不同分型制定不同的手术策略。

英文摘要:

  【Abstract】 Objectives: To investigate the spino-pelvic alignment in Marfan syndrome patients. Methods: A retrospective study was performed on 35 patients with spinal deformity secondary to Marfan syndrome(18 males and 17 females), the average age was 14.4±2.3 years(10-20). The following spinal and pelvic parameters were measured on the standing lateral radiographs of the whole spine: (1)thoracic kyphosis(TK), (2)thoracolumbar kyphosis(TL), (3)lumbar lodorsis(LL), (4)pelvic incidence(PI), (5)pelvic tilt(PT), (6)sacral slope(SS), (7)sagittal vertical axis(SVA). The thoracolumbar kyphosis was defined as the kyphosis Cobb angle larger than 10° and the apex sitting at T12, L1 or T12/L1 disc; the lumbar kyphosis was defined as the kyphosis Cobb angle larger than 10° and the apex below L1/2 disc. The patients were divided into two groups according to Sponseller′s classification, and the spino-pelvic parameters were compared between two groups. Results: In the frontal plane, the most common curve types were double major(40.0%), thoracic(22.8%) and triple(20.0%), and the mean maximum Cobb angle was 75.2°±26.0°(43°-165°). In the sagittal plane of the spine, the TK was 19.0°±24.1°(-25°-73°), 28.6%(10/35) for normal thoracic kyphosis(20°≤TK≤50°), 14.3%(5/35) for hyperkyphosis(TK>50°), 37.1%(13/35) for hypokyphosis(0°≤TK<20°), and 20.0%(7/35) for thoracic lordosis. TL was 14.0°±19.0°(-25°-73°); LL was 37.1°±23.3°(-17°-70°); SVA was -9.0-7.2cm(-2.0±4.3cm). According to our definition, 42.9%(15/35, 9 type ⅡA, 6 type ⅡB) of cases had thoracolumbar kyphosis or lumbar kyphosis, and 5 presented with vertebral wedging. In the sagittal plane of the pelvis, PI was 40.1°±12.7°(25°-74°)； PT was 6.9°±9.6°(-12°-34°); SS was 33.3°±12.6°(14°-68°). Type Ⅰ had larger TK, LL, PI, SS than type Ⅱ, while type Ⅱ had larger TL and PT than type Ⅰ. Furthermore, there was no spondylolisthesis occurred in this series. Conclusions: The patients with Marfan syndrome differ greatly in the spino-pelvic alignments, which indicate different surgical strategies according to different spino-pelvic alignments.