Secondary correction of the unilateral cleft nose deformity with an endonasal technique

Summary Malposition of the nasal bone, septum and the alar cartilage are striking features of the unilateral cleft nose deformity. An endonasal technique (the extramucosal) was used in 26 patients, aged 13–38 (median 19), to correct aesthetic and functional problems. Twenty-four patients were secondary and two were tertiary. The patients were followed from 1–9 years. The pathological anatomy of the septum varied considerably, so different types of septoplasties had to be done. In 20 patients, satisfactory aesthetic and functional results were obtained in one operation. Secondary corrections were indicated in six patients. In cases with no gross scarring in or around the alar cartilage, the form and position of this cartilage will be more normal once the alar cartilages have been undermined and the deviation of the nasal bone and the septum is corrected.     

Nasal fractures in children

Summary A group of thirty children with nasal fractures was evaluated retrospectively by means of a questionnaire and hospital records. Age at the time of injury ranged from age 3 to 12 (mean = 8.6) years and mean follow-up period was 9 years. Eight patients reported some degree of nasal obstruction post reduction, but only one patient required submucous resection and two patients underwent septorhinoplasty for appearance. No patients reported class III malocclusion, or required orthodontic treatment or maxillofacial corrective surgery for maxillary hypoplasia. We concluded that a childhood nasal fracture treated by closed reduction does not have deleterious effects on facial or nasal growth.This work was supported in part by the Brigham Surgical Group Foundation, Inc., Boston, Massachusetts, USA     

Mutational analysis of HOXD13 and HOXA13 genes in the triphalangeal thumb-brachyectrodactyly syndrome.

The triphalangeal thumb-brachyectrodactyly syndrome is a very rare autosomal dominant disorder of unknown etiology characterized by an unusual pattern of limb malformations: triphalangeal thumbs and brachyectrodactyly in the hands, and ectrodactyly in the feet. In a previous report, we described the clinical and radiographical features of three related subjects with the disease and suggest that due to the unusual combination of limb defects and to its phenotypic similarity with the limb malformative pattern induced by disrupting the Hoxd13 gene in mouse, the triphalangeal thumb-brachyectrodactyly syndrome might be caused by mutations in a HOX gene. After sequencing the entire coding region of HOXD13 and the highly conserved homeodomain encoding region of HOXA13, we do not detect any deleterious mutation in any of the patients excluding that alterations at these sequences are responsible for the disease. Mutations in regulatory regions of these genes or in other genes involved in limb development might be responsible for the disease.     

粒细胞集落刺激因子治疗下肢缺血性疾病

目的探讨粒细胞集落刺激因子(rhG-CSF)对下肢缺血模型血管新生的影响。方法制作兔左下肢缺血模型,术后随机分为rhG-CSF治疗实验组(n=24)和对照组(n=24);应用流式细胞学技术、动脉造影、免疫组织化学染色检查,比较两组外周血CD34 细胞的含量、缺血下肢侧枝血管计数及肌肉毛细血管密度。结果治疗后3 d实验组CD34 含量(%)为(0.7150±0.0873)明显高于对照组(0.3983±0.0853),差异有统计学意义(P<0.01);实验组在第15、30天时侧枝血管计数(6.33±0.82、9.17±0.75)均高于对照组(3.33±0.52、4.17±0.75)(P<0.01);第40天实验组内收肌毛细血管密度平均为8.5/HP,明显高于对照组4.2/HP(P<0.01)。结论rhG-CSF可以增加兔缺血下肢的毛细血管数量,有促进血管新生的作用。     

The relationship between jaw deformity and scoliosis

Objectives To analyze the relationship between lateral displacement of the mandible and scoliosis. Methods From April 2002 through July 2003, we examined posteroanterior cephalometric radiographs and chest X-rays from 85 patients with jaw deformities and a control group of 20 patients with no jaw deformities. To measure the lateral shift of the mandible, we drew a horizontal baseline (X axis) on the cephalogram connecting the intersection of the external margins of the orbits and the most lateral points of the greater wings of the sphenoid. A vertical baseline (Y axis) was then marked perpendicular to the X axis, intersecting the ethmoid crista galli. Then, we measured the lateral displacement of the mandibular mentum from the Y axis. Displacement to the right was designated positive; that to the left was designated negative. Cobb's method was used to measure scoliosis curves on chest X-rays; the direction of the curve was designated similarly. Results Of the 85 patients with jaw deformity, 23 (27.1%) had a Cobb angle exceeding 10°. None of the control group had scoliosis exceeding 10°. No correlation was found between the direction of mandibular displacement and the direction of scoliosis. Conclusion This study suggests a relationship between jaw deformities and scoliosis, as scoliosis was found in 27.1% of the patients with a main complaint of jaw deformity.     

真皮-脂肪颗粒混合液注射移植修复面部凹陷性组织缺损

目的探索一种新的面部凹陷性组织缺损的修复方法。方法将真皮颗粒和脂肪颗粒混合制成真皮-脂肪颗粒混合液,用2mm脂肪抽吸针注射移植修复面部凹陷性组织缺损。结果本组18例,17例1次填充成功,仅1例因缺损面积过大而补充填充1次,全部病例伤口均Ⅰ期愈合,无感染,外形明显改善。结论真皮-脂肪颗粒混合移植修复面部凹陷性组织缺损,手术简单易行,术后吸收少,效果较好,值得推广。     

Selective dorsal rhizotomy: long-term experience from Cape Town

Introduction Given the large number of cerebral palsy patients who have undergone selective dorsal rhizotomy in the past two decades, it is clearly imperative that the clinical community be provided with objective and compelling evidence of the long-term sequelae of the procedure. Materials and methods In the early 1980s, Peacock in Cape Town shifted the site of the rhizotomy from the conus medullaris to the cauda equina, and in the past 25 years, more than 200 children have been operated on. We have studied the incidence of spinal deformities after multiple-level laminectomy and recorded a 20% incidence of isthmic spondylolysis or grade-I spondylolisthesis. We have also conducted a long-term prospective gait analysis study on a cohort of 14 ambulatory patients who were operated on in 1985. Results Ten years after surgery, our patients had increased ranges of motion that were within normal limits. Step length was significantly improved, although cadence was unchanged postoperatively and was significantly less than normal age-matched control subjects. Discussion We have recently tracked down all 14 patients from the original cohort and are currently completing a 20-year prospective follow-up analysis of their neuromuscular function and gait. Our preliminary data suggest that selective dorsal rhizotomy is not only an effective method for alleviating spasticity but it also leads to long-term functional benefits.     

大鼠肢体缺血再灌注后内皮素-1含量的变化及BQ-123的干预作用

①目的　探讨肢体缺血再灌注 (LIR)后内皮素 - 1 (ET - 1 )含量的变化及BQ - 1 2 3的干预作用。②方法　采用大鼠肢体缺血再灌注损伤模型 ,将Wistar大鼠随机分 8组 ,即 :对照组、单纯缺血组、再灌注 0 .5、2、4、6、1 0小时组与BQ - 1 2 3组 ,分别测定血浆LDH、CK活性、ET - 1水平及骨骼肌中MDA含量、ET - 1放免活性和组织湿 /干重比值。③结果　IR各组与对照组比较 ,血浆和骨骼肌的各项生化指标显著增高 (P <0 .0 1orP <0 .0 5) ;BQ - 1 2 3组与再灌注 4小时组比较 ,各项指标明显降低 (P <0 .0 1orP <0 .0 5)。④结论　ET - 1可能参与了肢体缺血再灌注损伤。BQ - 1 2 3可减轻肢体缺血再灌注损伤 ,对骨骼肌有保护作用。     

GIEBEL系统在胫骨高位截骨术中的应用

[目的]探讨GIEBEL内固定系统固定的胫骨高位截骨术治疗膝内翻畸形的效果。[方法]对19例31膝,平均53.5岁的膝内翻患者进行评价。术前内翻畸形平均12.29°;膝关节症状以内侧间室疼痛为主。不伴有其他关节间室病变。对患膝进行精确的胫骨高位截骨术后应用GIEBEL系统内固定截骨端。手术前后拍摄双下肢负重位全长X线片,测量内翻角、机械轴线和解剖轴线。记录内外侧关节间隙距离的差值以及关节活动度。同时进行Lysholm评分。术后对患者进行主观满意度调查。[结果]对19例患者平均随访22个月。术后1例腓总神经一过性麻痹,2例切口脂肪液化延迟愈合。截骨处至术后12~16周均骨性愈合。测量矫正角度平均12.32°。3例患者残留膝前疼痛和关节绞锁症状。在随访期间膝内外侧间隙无明显退变加重。手术效果优良率为89.5%。84.2%患者对手术效果表示满意。除膝关节活动度外,Lysholm评分、内外关节间隙差以及内翻角度在手术前后差异均有统计学显著性差异。[结论]GIEBEL系统可有效地固定胫骨高位截骨术截骨端,对轻中度膝关节内翻畸形患者固定强度大,骨愈合率高。