A dominantly inherited malformation syndrome with short stature,upper limb anomaly,minor craniofacial anomalies,and absence of TBX5 mutations: Report of a Thai family

We report on a Thai family with dominantly inherited malformation syndrome with upper limb anomalies, short stature, quadricuspid aortic valve, and minor craniofacial anomalies. The affected individuals comprised a mildly affected mother, a moderately affected daughter, and a most severely affected son. The daughter and son had short stature. The craniofacial abnormalities comprised frontal bossing, hypoplastic nasal bones, depressed nasal bridge, and broad nasal alae. The upper limb defects varies among the patients, ranging from radial ray defects in the mother through radial and ulnar ray defects with unilateral humeral hypoplasia in the daughter to radial ray defects with severe oligodactyly and bilateral humeral hypoplasia in the son. All patients in this family had hypoplasia of the shoulder girdle and resembled what is observed in many families with Holt‐Oram syndrome. Moreover, the son showed quadricuspid aortic valve with mild aortic regurgitation. However, the present family did not show any mutation of the TBX5 gene, a disease‐causing gene of Holt‐Oram syndrome. The present family deserves further investigation on other genes that play a role in the development of the upper limbs, particularly of radial rays. © 2002 Wiley‐Liss, Inc.     

肘关节后脱位并尺骨冠状突骨折9例临床疗效分析

目的 探讨肘关节后脱位并尺骨冠状突骨折的治疗方法。方法 总结2000年3月～2003年5月9例肘关节后脱位合并尺骨冠状突骨折患者的治疗经验，根据骨折类型分别采用内外侧入路对8例患者进行手术治疗，术后配合早期功能锻炼。结果 所有患者随访8～24个月，根据HSS评分标准，优5例（55.6%)，良2例（22.2%)，一般2例（22.2%)，优良率77.8%，无严重肘关节并发症发生。结论 选择恰当的手术入路和早期功能锻炼是提高该类骨折效果的关键。     

Effect of wrist and ulna head position on gliding resistance of the extensor digitorum minimi and extensor digitorum communis III tendons: a cadaver study.

While attrition from sharp bony surfaces is the most common cause of extensor digiti minimi (EDM) tendon rupture, the etiology of other cases of spontaneous EDM tendon rupture is still unknown. Friction within the compartment may play a role, especially with ulna dislocation. The purpose of this study was to compare gliding resistance of the EDM tendon with that of a tendon which rarely ruptures spontaneously, the extensor digitorum communis of the middle finger (EDC III) tendon, under various wrist and ulna head positions. Eight fresh frozen cadavers were used. Gliding resistance between the tendon and its sheath in each compartment was measured in five different wrist positions and three different ulna head positions. Gliding resistance of the EDM tendon (0.13 +/- 0.03 N) was significantly greater than the EDC III tendon (0.09 +/- 0.03 N) (p < 0.05). For the EDM tendon, the gliding resistance in ulnar deviation or pronation was higher than the gliding resistance in neutral, radial deviation, or supination (p < 0.05), and the gliding resistance with ulnar lengthening (over 6 mm) or dorsal ulnar dislocation (over 9 mm) was higher than in neutral ulnar head positioning. For the EDC III tendon, the gliding resistance in ulnar deviation was significantly higher than the gliding resistance in neutral, radial deviation, or supination, or dorsal dislocation with ulnar lengthening (p < 0.05). Wrist ulnar deviation, ulnar dorsal dislocation (over 9 mm), and ulnar lengthening (over 6 mm) increased the gliding resistance of the EDM tendon. In patients at risk for EDM rupture, such as those with rheumatoid arthritis or distal radioulnar joint osteoarthritis, avoiding such positions may be advantageous.     

尺骨头进针固定尺骨的解剖及临床应用研究

目的 :报道尺骨头进针固定尺骨骨折的解剖基础及其临床应用效果。方法 :观察 7具 1 4侧成人上肢标本 ,测量尺骨茎突的长度。以尺侧腕伸肌腱沟内缘 2mm尺骨茎突远端以上 1 5mm处为进针点 ,测量该点到尺神经手背支的距离、该点在前后两侧到尺骨头环状关节面的距离。以该点进针模拟手术并试用于临床。结果 :①尺骨茎突长为 (6 .0± 1 .2 )mm ;②进针点与尺神经手背支距离为 (1 4 .1± 3 .8)mm ;③进针点在前后两侧到尺骨头环状关节面的距离分别为 (1 6 .7± 0 .7)mm、(1 6 .6± 1 .1 )mm ,配对t检验比较两者差异 (P >0 .0 5) ;④术后随访未见严重并发症。结论 :自尺骨头背侧进针能避免传统的从尺骨鹰嘴进针的并发症 ,且钢针尾部不影响前臂的旋转运动和腕关节活动     

Fractures of the proximal radius and ulna

Acute injuries of the proximal radius and ulna can be challenging to manage. This review article summarizes the classification, injury patterns, treatment and outcome of fractures of the olecranon, coronoid process, adult Monteggia fracture dislocations and radial head fractures. Undisplaced fractures and stable injuries can be treated conservatively and displaced and complex injuries should be treated surgically to permit early range of motion with a functional rehabilitation programme.     

Ulnar shortening osteotomy for ulnar ideopathic impaction syndrome

Twenty-eight patients were treated by ulnar shortening osteotomy for static or dynamic ulnar impaction syndrome. Ulnar variance was measured on a true anteroposterior radiograph. There were 25 wrists that were too long, two neutral, and one that was short. Bones were shortened by a mean of 3.5 mm. Mean follow-up time was 29 months (range 7–60), all with confirmed consolidation. At final follow-up mean grip strength had improved from 67% to 75%, mean Disabilities of the Arm, Shoulder, and Hand (DASH) score from 40 (range 12–83) to 26 (range 0–61) and mean range of movement from 80% (range 40%–100%) to 88% (range 50%–100%). Smoking, age at operation, type of osteotomy (transverse or oblique), dominance of hand, and sex did not influence consolidation or functionality. Special attention was paid to the anatomy of the distal radioulnar joint and the inclination of the sigmoid notch of the radius. There was no correlation between the anatomy and the functional outcome scores. Mean consolidation time (10 months) (range 2–32) and return to work were longer than in similar studies. Our findings confirm the usefulness of ulnar shortening osteotomy in the relief of ulnocarpal impingement symptoms.     

尺骨冠状突骨折合并肘关节后脱位有限元模型的建立与分析

目的：建立尺骨冠状突骨折合并肘关节后脱位的有限元模型,并对肘关节后脱位在关节稳定性方面的影响进行数字化研究。方法：建立正常肘关节-前臂（IEJF）有限元模型与尺骨冠状突骨折肘关节-前臂（FUCF）有限元模型。采用压缩、屈曲两组生理荷载（每组3种幅值）,进行计算、验证和应力分析。结果：建立了结构完整的IEJF与FUCF有限元模型,并对模型进行验证,情况与临床实际符合。计算得出FUCF有限元模型较IEJF有限元模型桡骨头出现了严重的应力集中现象,不同压缩荷载作用下的肘关节纵向压缩位移与不同屈曲力矩导致的屈曲角都有所增加。结论：尺骨冠状突骨折及肘关节后脱位对肘关节-前臂纵向不稳有一定程度的影响。本研究所构建的肘关节-前臂有限元模型对尺骨冠状突骨折合并肘关节后脱位的临床稳定性评估研究及手术治疗方案的制定具有一定的参考价值。