Treatment of extensive bone and soft tissue defects of the lower limb by traction and free-flap transfer

Twenty patients with extensive bone and soft tissue defects and posttraumatic osteomyelitis were treated between 1983 and 1995. In all cases an external fixator was used for bone fixation. Bone defects were managed with the Ilizarov intercalary bone transport. Two types of traction were used: the Ilizarov type and a 'new' Ljubljana type. The results of treatment were compared between the two types of traction. In all cases delayed bony union was observed. Osteomyelitis never reactivated. All patients were satisfied with treatment. They were all independent except for one amputee. The Ljubljana traction method was found to have the following advantages: no discrepancy in leg length, no orthopaedic support was needed, the aesthetic outcome was better, the traction time was reduced and there was less soft tissue damage during bone traction.     

A new variant of type II von Willebrand disease with aberrant multimeric structure of plasma but not platelet von Willebrand factor (type IIF)

A patient with a lifelong bleeding disorder was diagnosed as having Type II von Willebrand disease. The larger multimers of von Willebrand factor were absent from her plasma but present in platelets. A high- resolution electrophoretic technique was used to study the complex structure of individual von Willebrand factor multimers. In normal plasma, each multimer could be resolved into five bands: a more intense central one and four less intense, two moving faster and two slower than the central band. In normal platelets, each multimer could also be resolved into five bands. The central one had a mobility similar to that in plasma, whereas the four satellite bands had a mobility that differed from that of the corresponding plasma bands. In the patient, platelet von Willebrand factor antigen content and ristocetin cofactor activity were normal, and von Willebrand factor showed the same structure of individual multimers as seen in normal platelets. On the other hand, plasma von Willebrand factor antigen and ristocetin cofactor activity were decreased, and the structure of individual von Willebrand factor multimers was different from that of normal plasma and similar to that seen in normal and patient's platelets. After infusion of 1-deamino-8-D-arginine vasopressin, the largest von Willebrand factor multimers, as well as new satellite bands with a mobility similar to those in normal plasma, appeared in the patient plasma, and the levels of von Willebrand factor antigen and ristocetin cofactor activity became normal. Yet no relevant change in the prolonged bleeding time was observed. This new variant of von Willebrand disease, therefore, is characterized by the presence of a dysfunctional von Willebrand factor molecule that exhibits unique structural abnormalities in plasma but appears to be normal in platelets. The designation of Type IIF is proposed for this type of von Willebrand disease in accordance with the terminology that has been previously used.     

Prevention of congenital toxoplasmosis in Slovenia by serological screening of pregnant women

A programme for the prevention of congenital toxoplasmosis in Slovenia involving the screening of pregnant women for Toxoplasma infection is presented. Of 21,270 pregnant women screened for toxoplasmosis between, 1996 and the end of 1999, 13,987 (66%) were seronegative, 7,151 (34%) seropositive and 132 had primary infection; approximately 9/1,000 women were at risk of acquiring the primary infection. One hundred live-born infants of primary infected women were available for follow-up. Nine infected but asymptomatic children were born to mothers who were screened and treated in time and two congenitally infected babies were born to mothers in whom infection was detected too late in pregnancy and who therefore received no adequate treatment. It is suggested that the results obtained in this study outweigh the cost of screening for toxoplasmosis in pregnancy. Pregnant women should always be tested at the beginning of pregnancy and, in cases of seronegativity, should be re-tested in the second and third trimesters of the pregnancy. Toxoplasma primary infected pregnant women and neonates should be treated as soon as possible. However, long-term follow-up of children born to primary infected women would be necessary for an accurate evaluation of the effectiveness of the screening because of the possibility of late onset of symptoms.     

Immediate reconstruction of the lower extremity--an update

The objective of emergency free-tissue transfer of complex lower extremity injuries is to provide primary coverage of the wound at the time of the first surgical procedure during the first 24 hours after the accident. This is achieved by meticulous debridement of soft tissues and bone in the zone of injury, by fracture stabilization by either external or internal fixation, by assuring good circulation with a direct artery repair or by the use of venous or arterial grafts, and by closure of the soft-tissue defect with a suitable free flap with microvascular anastomoses. Such a repair on an emergency basis requires cooperation between orthopedic and plastic surgeons and organization of a continuous microsurgical service. With these prerequisites fulfilled, emergency treatment of complex lower leg injuries gives predictably better results than delayed primary treatment in terms of lower free flap failure rate, lower infection rate, lower number of operations required to obtain the final result, shorter time of hospitalization, shorter time to bone healing and weightbearing, and lower cost of treatment. Emergency free-tissue transfer is not indicated in life-threatening situations.     

Free lateral arm osteocutaneous neurosensory flap for thumb reconstruction.

Three cases are described to illustrate the use of the lateral arm osteocutaneous neurosensory flap in thumb reconstruction. The merits of such a method and its place in the repertoire of techniques for this problem are discussed.     

Demographic features, clinical characteristics and laboratory findings in children with multiple erythema migrans in Slovenia

OBJECTIVE: To identify demographic, clinical and laboratory characteristics of children with multiple erythema migrans (EM) in Slovenia. METHODS: We prospectively studied patients aged 15 years and less, examined at our department for multiple EM in 1996 and 1997. Demographic and clinical data were collected by means of a questionnaire. In addition, basic haematological and biochemical investigations, serologic testing, and Borrelia cerebrospinal fluid and blood cultures were performed. RESULTS: Ninety-five children (44 girls, 55 boys) aged 1 to 13.5 (median, 4.5) years fulfilled the inclusion criteria. A tick bite was recalled by 23%. The incubation period was 10.5 (range, 1 to 150) days, the duration of skin lesions before the initial examination 4 (range, 1 to 54) days, and the median number of skin lesions, 4.5 (range, 2 to 35). The initial disease was mild in 81% of patients. Local and systemic symptoms were reported by 11% and 30% of children, respectively. Clinical signs accompanying EM lesions were found in 42%. Cerebrospinal pleocytosis (predominantly lymphocytic) was seen in 18% of patients; none of them had frank clinical evidence of central nervous system involvement. Serum IgM and IgG antibodies were detected in 28% and 22% of children, respectively. In 3/79 (4%) patients, Borreliae were isolated from the blood and in 2/83 (2%) from the cerebrospinal fluid. In 2/81 (2%) children, borrelial IgG intrathecal antibody production was demonstrated. CONCLUSIONS: Multiple EM in Slovene children is a mild disease. However, some patients had an associated, usually asymptomatic, infection of the central nervous system.     

自体髂骨植骨带锁钢板内固定材料置入并一期前后路减压治疗多节段脊髓型颈椎病12例:临床资料回顾分析

目的:严重的多节段脊髓型颈椎病单纯前路或后路手术都有其局限性。观察一期前后路联合手术并自体髂骨植骨及带锁钢板内固定材料置入在治疗多节段脊髓型颈椎病中的应用价值。方法:选择2004-11/2006-12本院12例多节段脊髓型颈椎病患者,均采用一期前后路减压、自体髂骨植骨融合、带锁钢板内固定联合手术。其中男9例,女性3例,年龄49～75岁;3节段受累9例,4节段受累3例(突出节段分布:C3～66例,C4～73例,C3～73例)。全部病例进行临床随访,患者均对本试验知情同意。采用mJOA评分标准对患者神经功能改善情况进行评定;术前颈椎侧位片测量,以D值(C4椎体后下缘到齿突后缘与C7椎体后下缘连线的垂直距离)评价颈椎(C2～7)弧度;根据颈椎伸屈动态侧位片C2和C7椎体后缘切线相交所成的夹角之和评价颈椎(C2～7)活动范围。主要以电话随访和问卷填写的方式,分别从神经功能改善情况、颈椎弧度、活动范围及术后并发症等进行随访观察。结果:①12例患者全部得到随访,术后随访时间6～28个月,平均(16±6)个月。②所有植骨均获得骨性愈合;疗效结果中优4例(33.3%);良6例(50%);无效2例(16.7%);颈椎D值术前(3.9±1.4)mm,术后即刻(8.5±1.7)mm,随访时(8.1±2.5)mm。术前与术后差异有显著性(P<0.01),术后与随访时差异无显著性(P=0.251);颈椎活动范围术前(36.3±4.0)°,随访时(10.6±2.7)°,与术前相比差异具有显著性(P<0.01)。③术后C5神经根麻痹1例,为感觉及运动混合型,8个月随访时,感觉功能恢复,肩关节外展肌力从术后Ⅱ级恢复至Ⅳ级;1例术后6个月出现"S"畸形而再次压迫脊髓,神经功能改善停滞,目前处于随访中。结论:一期前后路手术并自体髂骨植骨及带锁钢板内固定材料置入减压充分、彻底,而且前路手术能重建颈椎稳定性,恢复颈椎生理前凸和椎间高度,并且后路减压术又能预防相邻颈椎退变引起的脊髓继发的压迫。     

DFNB74, a novel autosomal recessive nonsyndromic hearing impairment locus on chromosome 12q14.2-q15

Autosomal recessive nonsyndromic hearing impairment (ARNSHI) segregating in three unrelated, large consanguineous Pakistani families (PKDF528, PKDF859 and PKDF326) is linked to markers on chromosome 12q14.2-q15. This novel locus is designated DFNB74 . Maximum two-point limit of detection (LOD) scores of 5.6, 5.7 and 2.6 were estimated for markers D 12 S 313, D 12 S 83 and D 12 S 75 at θ = 0 for recessive deafness segregating in these three families. Haplotype analyses identified a critical linkage interval of 5.35 cM (5.36 Mb) defined by D 12 S 329 at 74.58 cM and D 12 S 313 at 79.93 cM. DFNB74 is the second ARNSHI locus mapped to chromosome 12, but the physical intervals do not overlap with one another. A locus contributing to the early onset, rapidly progressing hearing loss of A/J mice ( ahl4 , age-related hearing loss 4) was reported to map to chromosome 10 in a region of conserved synteny to DFNB74 , suggesting that ahl4 and DFNB74 may be due to mutations of the same gene in these two species.