Treatment of Deformity of the Lower Limb in Adults Who Have Osteogenesis Imperfecta*
Treatment of Deformity of the Lower Limb in Adults Who Have Osteogenesis Imperfecta*
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成骨不全成人下肢畸形的治疗*
DOI：
10.2106/00004623-199602000-00008
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发表时间：
1996
期刊：
影响因子：
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通讯作者：
Donald M. Spencer
中科院分区：
文献类型：
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作者：
D. Ring;J. Jupiter;Panos K. Labropoulos;John J. Guggenheim;Deborah F. Stanitsky;Donald M. Spencer
采用Ilizarov延长法矫正了6名患有Sillence等人分类的I型成骨不全的患者的下肢畸形。平均年龄为31岁（范围：14 - 51岁）。畸形包括4个胫骨和3个股骨缩短，以及所有4个胫骨和1个股骨的角度对线不良（平均28度;范围20 - 40度）。1例患者还出现右侧股骨骨不连。平均角度矫正为23度（范围，20至30度）。七个肢体节段的长度平均增加了6.6厘米（范围，2到11厘米）。所有肢体长度的差异都被纠正到对侧肢体长度的两厘米以内。在平均3年零4个月（范围：1年零7个月至6年）时，完全成熟骨的X线表现与原始骨相当。没有骨折或新骨段成角增加。两名患者行走时疼痛：一名患者与慢性针道感染有关，另一名患者与踝关节骨关节病有关。4例患者的功能状态得到改善，另2例患者的功能状态无变化。所有6名患者都对手术结果感到满意。有18个并发症：2例患者出现膝关节僵硬; 2例患者出现腓神经麻痹; 3例患者出现浅表针道感染; 1例患者出现深部针道感染、术中失血多于正常、2个针松动、膝关节不稳定性恶化和膝关节感染。在另一名患者中，在畸形矫正前放置的Rush棒向近端移位，在完成矫正后不得不取出。有五处骨折。
The Ilizarov method of lengthening was used to correct deformities of the lower extremity in six patients who had type-I osteogenesis imperfecta, as categorized by Sillence et al. The average age was thirty-one years (range, fourteen to fifty-one years). The deformities included shortening of four tibiae and three femora as well as an angular malalignment (average, 28 degrees; range, 20 to 40 degrees) of all four tibiae and one femur. One patient also had a non-union of the right femur. The average angular correction was 23 degrees (range, 20 to 30 degrees). The seven limb segments gained an average of 6.6 centimeters (range, two to eleven centimeters) in length. All limb-length discrepancies were corrected to within two centimeters of the length of the contralateral limb. At an average of three years and four months (range, one year and seven months to six years), the roentgenographic appearance of the fully matured bone was comparable with that of the original bone. There were no fractures or increases in the angulation of the segment of new bone. Two patients had pain when walking: it was related to a chronic pin-track infection in one and to osteoarthrosis of the ankle in the other. The functional status of four patients was improved and that of the other two patients was unchanged. All six patients were pleased with the outcome of the procedure. There were eighteen complications: stiffness of the knee in two patients; a peroneal nerve palsy in two; a superficial pin-track infection in three; and a deep pin-track infection, greater-than-normal loss of blood intraoperatively, loosening of two pins, worsening of the instability of the knee, and an infection in the knee in one patient each. In another patient, a Rush rod that had been placed before correction of the deformity migrated proximally and had to be removed after completion of the correction. There were five fractures.