Diagnosis of Atlantoaxial Subluxation in Morquio's Syndrome and Spondyloepiphyseal Dysplasia Congenita

Compression of the spinal cord due to atlantoaxial subluxation was diagnosed in a patient with Morquio's syndrome and in another with spondyloepiphyseal dysplasia (SED) congenita by cervical radiography and magnetic resonance imaging (MRI). The patient with Morquio's syndrome, a 15 year old boy, had no neurologic symptoms and his somatosensory evoked potential (SSEP) was normal. However, MRI demonstrated spinal cord compression at C1-C2. In contrast, the patient with SED congenita, an 11 year old girl, had neck pain, hyperreflexia and loss of vibration sense in both legs. These findings were explained by the absence of P3 and later waves in SSEP and by compression of the spinal cord observed on MRI. Both SSEP and MRI should be used for evaluating disorders in which atlantoaxial subluxation might be present.     

小切口晶状体手术在晶状体脱位继发青光眼中的应用

目的观察小切口手术治疗晶状体脱位继发青光眼的临床疗效。方法回顾性研究自1999年4月至2005年2月在我院因晶状体脱位继发青光眼而行小切口白内障手术治疗的一组患者共21例22只眼。记录患者眼压、视力变化及术中术后并发症等。观察时间为3-72个月。结果22只眼术后最后一次复查时平均眼压为(12.47±3.77)mmHg,明显低于术前平均眼压(42.29±18.99)mmHg,差异有非常显著意义(t=7.185,p=0.000)。22只眼术后最后一次复查最佳矫正视力在NLP～1.2之间,较术前有明显改善,差异有非常显著意义(t=-2.929,p=0.008)。术中术后均未出现严重并发症。结论小切口晶状体手术是治疗晶状体脱位继发青光眼的有效手术方式,且技术较成熟,临床上如有条件应广泛开展。     

Marfan综合征晶状体半脱位的手术治疗探讨

目的　探讨Marfan氏综合征晶状体半脱位手术治疗的安全有效方法。方法　采用小切口 ,晶状体干性吸除 ,保留晶状体后囊膜悬韧带 ,必要时干性玻璃体切除 ,折叠人工晶状体一襻睫状沟悬吊 ,另一襻及人工晶状体的光学部则置于保留的晶状体囊膜上的方法治疗 8例 16眼Marfan氏综合征晶状体严重半脱位 ,晶状体脱位范围均大于 180度 ,脱位一侧的晶状体边缘在自然状态下已达瞳孔区 ,最佳矫正视力 <0 3者 14眼 ,≥ 0 3者 2眼。结果　术后视力均较术前视力有明显的提高 ,术后视力 0 1～ 0 3者 6眼 ,0 4～ 0 9者 10眼。术后最佳矫正视力 0 1～ 0 3者 4眼 ,0 4～ 0 9者 12眼。正常瞳孔下人工晶状体正位 ,无一例发生偏斜。术后无角膜水肿 ,眼压高 ,脉络膜上腔出血 ,睫状体脉络膜脱离 ,玻璃体浑浊、增殖 ,视网膜脱离 ,黄斑囊样水肿等严重并发症的发生。术后瞳孔完全居中 14眼 ,轻度上移但不超过人工晶状体光学面边缘 2眼。结论　采用小切口 ,晶状体皮质干性吸除 ,保留晶状体后囊膜悬韧带 ,必要时干性玻璃体切除 ,折叠人工晶状体一襻睫状沟悬吊 ,另一襻及人工晶状体的光学部则置于保留的晶状体囊膜上的方法治疗Marfan氏综合征晶状体严重半脱位是一种安全、有效的方法。     

手法治疗寰枢关节半脱位及其伴随病症疗效观察

为观察手法治疗寰枢关节半脱位及其伴随病症的疗效。采用规范的手法治疗与枕颌牵引组对照 ,观察其疗效。结果手法治疗组对伴随病症的总有效率为 96 .7% ,对照组总有效率为 6 3.3% ,手法治疗组对寰枢轴线分离的整复改善有效率为 73.3% ,对照组为 5 3.33% ,经统计学处理 ,有非常显著意义。表明手法治疗寰枢关节半脱位及其伴随病症有较好的疗效。     

VERTICAL SUBLUXATION OF THE PATELLA: AN UNUSUAL FEATURE OF MULTIPLE EPIPHYSEAL DYSPLASIA

Book reviews in this article: Abnormally shaped, usually double-layered, patellae are recognized as occurring in multiple epiphyseal dysplasia. The case described is unusual in that an associated defect appeared in the femur and resulted in mechanical symptoms.     

Signs of acute carpal instability associated with distal radial fracture

The posteroanterior and lateral wrist radiographs of 52 adults who sustained acute distal radial fractures were reviewed to determine the incidence of radiographic signs of concomitant acute scapholunate ligamentous injury. Three radiographic criteria, either separately or in concert, were used to identify scapholunate ligamentous injury. They are (a) scapholunate angle >60°; (b) widening of the scapholunate space >2 mm; and (c) the cortical ring sign (also called the scaphoid or signet ring sign). Thirty-six of 52 cases (69%; 95% confidence interval =52.1–82.2%) showed evidence of scapholunate ligamentous injury; 16 of 52 cases (31%; 95% confidence interval =16.8–46.6%) showed no evidence of scapholunate ligamentous injury. Statistical analysis suggests that a minimum of 52.1% of all adult distal radial fractures are associated with signs of concurrent acute scapholunate ligamentous injury. The analysis also suggests that independent variables such as age, gender, severity of radius fracture, or mechanism of injury do not significantly alter the incidence of associated carpal ligamentous instability. This study has established a greater than 52.1% incidence of radiographic signs of scapholunate instability in adults with acute distal radial fractures. It is incumbent upon radiologists to search for the signs of scapholunate dissociation in all adult cases of distal radius fracture, regardless of age, gender, severity of radius fracture, or mechanism of injury.     

Grisel's syndrome in head and neck practice

Grisel’s syndrome is a rare condition of uncertain etiology characterized by a non-traumatic atlanto-axial subluxation, secondary to an infection in the head and neck region. The authors discuss the history, pathogenesis, terminology, classification, clinical characteristics, diagnosis, treatment, prognosis and prevention of this syndrome.     

Longitudinal Tears of Both Peroneal Tendons Associated With Tophaceous Gouty Infiltration. A Case Report

The authors report a case of longitudinal tendon tears of the peroneus longus and brevis in the presence of tophaceous gouty infiltration. There are a limited number of reports discussing similar processes affecting various tendons throughout the body. There has been 1 prior case of peroneal tendon involvement affecting only the peroneus brevis. A 35-year-old man presented with a 4-year history of left-sided lateral ankle pain, redness, and swelling. The patient described the "attacks" as occurring off and on, with a recent increase in frequency. The symptoms were relieved with indomethacin, colchicine, and narcotic analgesics. Upon clinical evaluation, there was a cavus foot type with moderate calcaneal varus. Peroneal subluxation was elicited with resisted eversion. An MRI evaluation revealed longitudinal tears of both peroneal tendons. During surgical repair, a chalky-white substance resembling gouty tophi was present within both tendon tears. The tendons were remodeled and repaired. The patient had an uneventful postoperative course with nearly completed resolution of his symptoms at 1-year follow-up. The patient was referred to his primary care physician for further evaluation and long-term control of elevated uric acid levels.     

Humero-spinal dysostosis

A 2 year old boy withhumero-spinal dysostosis is described. This is the third case of this disease reported in the literature.Humero-spinal dysostosis is characterised radiologically by distal humeral bifurcation, elbow subluxation and coronal cleft vertebrae. Congenital, progressive heart disease, possibly with fatal outcome, is probably part of the syndrome.