Gottschalk Hilton P, Kanauchi Yumiko, Bednar Michael S, Light Terry R
Department of Orthopaedic Surgery and Rehabilitation, Loyola University Medical Center, Maywood, Illinois 60153, USA.
J Hand Surg Am. 2012 Nov;37(11):2286-93. doi: 10.1016/j.jhsa.2012.07.033. Epub 2012 Oct 4.
Multiple hereditary osteochondromatosis (MHO) is an autosomal-dominant skeletal dysplasia that may result in forearm deformity. The purpose of this study was 2-fold: to describe the natural history of forearm deformity in patients with MHO, with particular attention to those who develop radial head dislocation, and to determine predictors of deformity.
We retrospectively reviewed charts of all patients with MHO evaluated at our institution. Patients with the presence of a radiographically visible osteochondroma in the forearm were divided into 5 groups or types based on location of the osteochondroma(s). Radiographic measurements included radial articular angle, percent ulnar variance, radial bow, radial length, ulnar length, and ulnar bow. The predictive values of each measure were statistically evaluated for each type with relation to radial head dislocation.
Of 146 patients with MHO, 102 patients (70%) had forearm involvement. Appropriate anteroposterior and lateral radiographs were available on 48 patients (76 forearms). Average age at initial radiographic evaluation was 12 years (range, 2-18 y). Average follow-up period was 7 years (range 1-19 y). Thirteen forearms demonstrated radial head dislocation, with all but 1 reported in the type 1 limbs (solitary distal ulna osteochondroma). Radial head dislocation was noted in 34% (12/35 forearms) of type 1 limbs.
Forearms with isolated osteochondromas of the distal ulna are the ones most likely to develop radial head dislocation. Because the ulna growth is disproportionately less than radial growth, the soft tissues may act as a tether, linking the distal radius and ulna, and lead to radial head dislocation. Changes in radiographic measurements may predict limbs at risk for radial head dislocation.
多发性遗传性骨软骨瘤病（MHO）是一种常染色体显性遗传性骨骼发育不良疾病，可能导致前臂畸形。本研究的目的有两个：描述MHO患者前臂畸形的自然病程，尤其关注那些发生桡骨头脱位的患者，并确定畸形的预测因素。
我们回顾性分析了在本机构接受评估的所有MHO患者的病历。根据前臂骨软骨瘤的位置，将在前臂X线片上可见骨软骨瘤的患者分为5组或5种类型。X线测量包括桡骨关节角、尺骨变异百分比、桡骨弓形、桡骨长度、尺骨长度和尺骨弓形。针对每种类型，统计评估各项测量指标与桡骨头脱位的预测价值。
在146例MHO患者中，102例（70%）有前臂受累。48例患者（76侧前臂）有合适的前后位和侧位X线片。首次X线评估时的平均年龄为12岁（范围2 - 18岁）。平均随访期为7年（范围1 - 19年）。13侧前臂出现桡骨头脱位，除1例外均发生在1型肢体（孤立性尺骨远端骨软骨瘤）。1型肢体中34%（12/35侧前臂）出现桡骨头脱位。
尺骨远端孤立性骨软骨瘤的前臂最易发生桡骨头脱位。由于尺骨生长明显慢于桡骨生长，软组织可能起到束缚作用，连接桡骨远端和尺骨，导致桡骨头脱位。X线测量的变化可能预测有桡骨头脱位风险的肢体。
