Remes V, Poussa M, Peltonen J
Hospital for Children's and Adolescents, Helsinki, Finland.
Spine (Phila Pa 1976). 2001 Aug 1;26(15):1689-97. doi: 10.1097/00007632-200108010-00011.
Scoliosis in patients with diastrophic dysplasia was analyzed.
To study the natural history of scoliosis and to classify the patients with different types of scoliosis.
Typical findings in diastrophic dysplasia are short-limbed short stature, multiple joint contractures, early degeneration of joints, and spinal deformities. The largest studies have reported scoliosis in 37% to 88% of the patients with this rare skeletal dysplasia. The natural history of the deformity is unknown.
Of the 130 unselected patients, 98 (75%) who were older than 16 years and/or had undergone surgery at the time of the last radiograph were included in the final analysis. These 98 patients included 37 males and 61 females. Their ages at the first radiograph ranged from newborn to 78 years (average, 21 years). The mean follow-up period was 20 years (range, 2-41 years) for 80 patients. Standard standing anteroposterior and lateral radiographs were taken. The degrees of scoliosis, kyphosis, and lordosis were measured according to the Cobb method. Classification of the scoliosis was based on the patient's age at onset of scoliosis, the rate of progression, the magnitude of the scoliosis at the end of growth, and the curve pattern.
Of the 98 patients in this study, 86 (88%) had scoliosis. This difference was highly significant statistically (P < 0.001), as compared with the normal population. The frequency of scoliosis was 90% among females and 84% among males. Scoliosis can be divided further into three subtypes: early progressive (11 patients), idiopathic-like (41 patients), and mild nonprogressive (33 patients). One patient had a congenital scoliosis.
Scoliosis is very common in patients with diastrophic dysplasia. The natural history of scoliosis varies from severe deformity with rapid progression to mild deformity without any progression. The authors suggest that the classification described in this report offers a tool for the predicting natural history of scoliosis in diastrophic dysplasia, and for adjusting the timing of surgery in individual patients.
分析了粘多糖病性发育不良患者的脊柱侧凸情况。
研究脊柱侧凸的自然病史，并对不同类型脊柱侧凸患者进行分类。
粘多糖病性发育不良的典型表现为短肢矮小、多关节挛缩、关节早期退变和脊柱畸形。规模最大的研究报告称，在患有这种罕见骨骼发育不良的患者中，37%至88%存在脊柱侧凸。畸形的自然病史尚不清楚。
在130例未经挑选的患者中，98例（75%）年龄大于16岁和/或在最后一次X光检查时已接受手术的患者被纳入最终分析。这98例患者包括37例男性和61例女性。他们首次X光检查时的年龄从新生儿到78岁不等（平均21岁）。80例患者的平均随访期为20年（范围2至41年）。拍摄标准站立位前后位和侧位X光片。根据Cobb法测量脊柱侧凸、后凸和前凸的度数。脊柱侧凸的分类基于患者脊柱侧凸发病时的年龄、进展速度、生长末期脊柱侧凸的严重程度以及曲线类型。
本研究的98例患者中，86例（88%）患有脊柱侧凸。与正常人群相比，这一差异具有高度统计学意义（P<0.001）。女性脊柱侧凸的发生率为90%，男性为84%。脊柱侧凸可进一步分为三种亚型：早期进展型（11例）、特发性样型（41例）和轻度非进展型（33例）。1例患者患有先天性脊柱侧凸。
脊柱侧凸在粘多糖病性发育不良患者中非常常见。脊柱侧凸的自然病史各不相同，从进展迅速的严重畸形到无任何进展的轻度畸形。作者认为，本报告中描述的分类为预测粘多糖病性发育不良患者脊柱侧凸的自然病史以及调整个体患者的手术时机提供了一种工具。
