Shadi Milud, Koczewski Paweł
Katedra i Klinika Ortopedii Dzieciecej i Traumatologii UM im K. Marcinkowskiego w Poznaniu.
Pediatr Endocrinol Diabetes Metab. 2007;13(3):121-4.
In achondroplasia patients the shortening of upper limb (mainly the arms) is an important part of pathology in addition to low stature. Not all patients who are treated for increasing their height decided to have humeral lengthening and the indication for treatment is not only for cosmetic, psychological aspects but also limitation of upper limb function like self-services and personal hygiene.
5 patients were evaluated (4 girls and 1 boy) at age of 14 to 18 years (mean 15.7) in whom 10 humeral lengthening were do-ne using monolateral external fixator "Pumed". All patients have lower limb lengthening with the Ilizarov method 4-5 years before humeral lengthening. Observation time was 6 to 34 months (mean 20). In all cases the Pumed external fixator was fixed to humerus by 4 Schanz screws, open humeral distraction osteotomy was done below the insertion of deltoid muscle. In one case 15 degrees anteflexion correction was done intraoperatively. Distraction began at 5-6th day postoperatively with rate of 1 mm/day. Because of hypertrophic bone regenerate the distraction rate was often increased up to 1.5 mm/day.
8 to 9 cm lengthening was achieved (mean 8.5) which represents more than 50% of the primary segmental length. The average time of fixator application was 7 months and the lengthening index ranged from 0.8 to 1.1 months/cm (average 0.85). No shoulder and elbow joint range of motion deterioration was observed. At the follow-up transient radial nerve palsy was observed in one case after acute limb axis correction.
Humerus lengthening, in achondroplasia patients with the use of monolateral external fixator is an effective and reliable method of treatment, with relatively low lengthening index. Monolateral External Fixator are well tolerated by patients.
在软骨发育不全患者中，上肢（主要是手臂）缩短是除身材矮小之外病理的重要组成部分。并非所有接受增高治疗的患者都决定进行肱骨延长术，其治疗指征不仅关乎美观、心理方面，还涉及上肢功能受限，如自理和个人卫生。
对5例患者（4名女孩和1名男孩）进行了评估，年龄在14至18岁（平均15.7岁），其中使用单侧“Pumed”外固定器进行了10次肱骨延长术。所有患者在肱骨延长术前4至5年采用伊里扎洛夫方法进行了下肢延长。观察时间为6至34个月（平均20个月）。在所有病例中，通过4枚斯氏针将“Pumed”外固定器固定于肱骨，在三角肌止点下方进行开放性肱骨延长截骨术。1例术中进行了15度的前屈矫正。术后第5至6天开始牵引，牵引速度为1毫米/天。由于骨再生过度，牵引速度常增至1.5毫米/天。
实现了8至9厘米的延长（平均8.5厘米），超过了原节段长度的50%。外固定器平均应用时间为7个月，延长指数为0.8至1.1个月/厘米（平均0.85）。未观察到肩、肘关节活动范围恶化。随访时，1例在急性肢体轴线矫正后出现了短暂性桡神经麻痹。
对于软骨发育不全患者，使用单侧外固定器进行肱骨延长是一种有效且可靠的治疗方法，延长指数相对较低。患者对单侧外固定器耐受性良好。
