Management of sagittal craniosynostosis: morphological comparison of eight surgical techniques

The aim of this study was to carry out a retrospective multicentre study comparing the morphological outcome of 8 techniques used for the management of sagittal synostosis versus a large cohort of control patients. Computed tomographic (CT) images were obtained from children CT-scanned for non-craniosynostosis related events (n = 241) and SS patients at preoperative and postoperative follow-up stages (n = 101). No significant difference in morphological outcomes was observed between the techniques considered in this study. However, the majority of techniques showed a tendency for relapse. Further, the more invasive procedures at older ages seem to lead to larger intracranial volume compared to less invasive techniques at younger ages. This study can be a first step towards future multicentre studies, comparing surgical results and offering a possibility for objective benchmarking of outcomes between methods and centres.     

胸廓矢状面倾斜度的测定及其对放疗的意义

目的了解人体前胸矢状面倾斜度的大小及对放疗剂量分布的影响。方法选择126例患者行胸部CT扫描并予正中矢状面重建。分别测定上中下胸廓层面前后体表中线至脊髓距离及各层上下间距,计算出前胸倾斜度。查TMR表得出中平面深度及脊髓深度处上中胸及中下胸层面剂量误差。结果前野各层面至脊髓距离变化较大,后野变化较小。人体前胸倾斜度范围为25~56°。上中胸段组的上下缘剂量误差较大,躯体中平面平均相差8%,脊髓17%。中下胸段组剂量误差较小,躯体中平面4%,脊髓7%。误差随倾斜角度增加而增加。根据倾斜度得出合适的楔形板修正角度大约为10~35°。结论人体前胸矢状面存在自然倾斜度,这种倾斜影响前野剂量分布,应予以个体化修正。建议根据角度的大小选择合适的头足向楔形板优化。     

Two-level posterior lumbar interbody fusion for degenerative disc disease: improved clinical outcome with restoration of lumbar lordosis

BACKGROUND CONTEXT: Although posterior lumbar interbody fusion (PLIF) for degenerative lumbar diseases is routine, there are few reports on double-level PLIF. PURPOSE: To evaluate the clinical outcomes of double-level PLIF. STUDY DESIGN/SETTING: A retrospective study of operated cases in Gifu, Japan. PATIENT SAMPLE: Nineteen patients (8 men and 11 women, 59.5+/-10.2 years) who underwent double-level PLIF between 1996 and 2001. OUTCOME MEASURES: Operation time, blood loss, complications, the Japanese Orthopaedic Association (JOA) score for back pain and lumbar sagittal alignment were evaluated. METHODS: Patients were examined retrospectively at follow-ups of 3.6+/-1.7 years. Primary diseases were spondylolisthesis, spinal canal stenosis, degenerative scoliosis and herniated intervertebral disc. Fusion areas were L3 to L5 in 15 cases and L4 to S1 in 4 cases. RESULTS: The mean JOA score increased from an initial score of 12.9+/-3.5 to 21.3+/-4.9 at the final follow-up. There was a positive correlation (R=0.718, p<.001) between the increase in lordotic angle and the increase in the JOA score. Several parameters suggested that the surgical invasiveness was not minimal. CONCLUSION: Double-level PLIF provided satisfactory results and preserved lumbar spine lordosis.     

Significance of beaten copper appearance on skull radiographs in children with isolated sagittal synostosis

Aims and Objectives The significance of beaten copper appearance (BCA) on skull radiographs in children following surgery for isolated sagittal craniosynostosis has not been studied. This study was designed to look for any correlation between BCA and symptoms suggestive of intracranial hypertension in this group of patients. Materials and Methods Forty-eight consecutive children, who were operated for isolated sagittal synostosis from1987 to 2000 and had postoperative skull radiographs, were included. Patients were divided into: (a) BCA group (n = 20), consisting of children who had beaten copper appearance on skull radiographs at last follow up, and (b) Non-BCA group (n = 28), consisting of children who did not have this finding. Records were reviewed to look for symptoms suggestive of intracranial hypertension, such as headache, head banging, and irritability. Results Median age at surgery was 4.8 months for BCA group and 4 months for the non-BCA group. Follow up ranged from 4 to 156 months with a mean of 36.2 months. Total of 28.6% (n = 6) of the children with follow up radiographs done at ≤18 months of age had BCA. The incidence of BCA increased to 83.3% in children with skull radiographs performed after 48 months of age. In 18 (90%) children, the BCA was ‘diffuse’ with 5 (25%) children having the maximum possible score of 8. In the BCA group, 45% (n = 9) had symptoms compared to 10.7% (n = 3) in the control group (p = 0.0068). Conclusions This study suggests a significant number of children with BCA on radiographs develop symptoms suggestive of raised ICP following surgical treatment in infancy and prolonged follow up may be warranted in this group of patients.     

Segmental Liver Transplantation from Non-Heart Beating Donors—An Early Experience with Implications for the Future

We report our experience of pediatric liver transplantation with partial grafts from non-heart beating donors (NHBD). Controlled donors less than 40 years of age with a warm ischemia time (WI) of less than 30 min were considered for pediatric recipients. Death was declared 5 min after asystole. A super-rapid recovery technique with aortic and portal perfusion was utilized. Mean donor age was 29 years and WI 14.6 min (range 11–18). Seven children, mean age 4.9 years (0.7–11), median weight 20 kg (8.4–53) received NHBD segmental liver grafts. Diagnoses included seronegative hepatitis, neonatal sclerosing cholangitis, familial intrahepatic cholestasis, hepatoblastoma, primary hyperoxaluria and factor VII deficiency (n = 2).The grafts included four reduced and one split left lateral segments, one left lobe and one right auxiliary graft. Mean cold ischemia was 7.3 h (6.2–8.8). Complications included one pleural effusion and one biliary collection drained percutaneously. At 20 months (10–36) follow-up all children are alive and well with functioning grafts.
Donation after cardiac death is a significant source of liver grafts for adults and children with careful donor selection and short cold ischemic times.     

矢状缝早闭(舟状头)全颅成形术

目的探讨治疗矢状缝早闭(舟状头)畸形的手术方法。方法采用David“‖”形颅缝重建法(6例)、旋转骨瓣截骨法(2例)、梅花颅骨瓣头颅盖成型法(3例)等术式对舟状头进行矫治。结果11例患儿均治愈,外形满意。结论1~3个月舟状头宜采用简单的David“‖”形颅缝重建法进行治疗,3个月以上的患儿可选用浮动颅骨瓣头颅成型术、梅花瓣法颅骨瓣头颅盖成型术等方法进行治疗。     

To market! To market! A new dawn for community care?

The public sector in Britain has been subjected to over a decade of major reform aimed at breaking up public service monopolies, at containing costs while at the same time opening services up to greater consumer choice. Health and social services have not been exempt from this revolution in the organization and management of public sector services. The long-standing policy of care in the community is being subjected to market principles and the introduction of a ‘contract culture’ very similar to the NHS reforms introduced in 1991. This paper reviews the origins of these developments in the doctrines of ‘new public management’, a movement which has proved attractive to policy-makers in many countries. Local authority social services departments have been identified as the lead agency for the development of a mixed economy of care following a review of community care policy by the government's health adviser, Sir Roy Griffiths, and a subsequent white paper. This paper examines the limited empirical evidence available on how managers and providers are meeting the challenge bestowed upon them, and concludes that most authorities are moving ahead cautiously if at all. Only a handful of authorities studied have embraced the reforms with any degree of enthusiasm. The paper concludes with an assessment of the reforms from two perspectives: a pessimistic one and an optimistic one. There are many worrying features of the reforms, not least among these being a lack of clarity over their intended purpose. Tensions and contradictions are plentiful, which places in jeopardy the certainty of the reforms in becoming user led rather than provider driven. A more optimistic scenario is that the changes are leading to a loosening up of services and practices which have often suffered from sclerotic tendencies, paternalism and sometimes complacency. If the reform process is skilfully handled and not rushed and if the ends are clearly established and communicated then users and carers could prove to be the principal beneficiaries.     

Effect of prolonged physical exercise on the fibrinolytic system

Summary The effect of a test marathon race on plasma fibrinolytic activity (FA) was studied in 16 endurance athletes before, immediately after, 3 h, and 31 h after the run. Tissue plasminogen activator (t-PA) activity increased about 31-fold immediately after the run. Similar increases were found in t-PA antigen concentration. Plasminogen activator inhibitor (PAI) was not detectable immediately after the race and was significantly decreased 3 h (P < 0.05) and 31 h (P < 0.01) later. B_15–42 peptide increased by 0.63 pmol · ml^–1 (P<0.001),d-dimer by 68.3 ng · ml^–1 (P< 0.05). Euglobulin lysis time (ELT) was reduced from 109 to 18 min (P<0.001). The increased t-PA activity and t-PA antigen concentration disappeared in the course of the first 3 h after exertion. ELT also reached its pre-exercise levels at this time. Thirty-one hours after the race ELT and t-PA antigen levels were slightly but significantly reduced (P<0.05), whereas B_15–42 peptide remained increased (P<0.05). t-PA activity was unchanged compared with pre-exercise values. It seems that the exercise-induced FA is mainly caused by the marked increase of t-PA antigen and t-PA activity.     

WNT10B variants in split hand/foot malformation: Report of three novel families and review of the literature

Split‐hand/foot malformation (SHFM) is a genetically heterogeneous congenital limb malformation typically limited to a defect of the central rays of the autopod, presenting as a median cleft of hands and feet. It can be associated with long bone deficiency or included in more complex syndromes. Among the numerous genetic causes, WNT10B homozygous variants have been recently identified in consanguineous families, but remain still rarely described (SHFM6; MIM225300). We report on three novel SHFM families harboring WNT10B variants and review the literature, allowing us to highlight some clinical findings. The feet are more severely affected than the hands and there is a frequent asymmetry without obvious side‐bias. Syndactyly of third–fourth fingers was a frequent finding (62%). Polydactyly, which was classically described in SHFM6, was only present in 27% of patients. No genotype–phenotype correlation is delineated but heterozygous individuals might have mild features of SHFM, suggesting a dose‐effect of the WNT10B loss‐of‐function.     

Autosomal recessive acrorenal syndrome

We describe two sibs with tetraectrodactyly and oligomeganephronic renal hypoplasia. The parents were unaffected. This syndrome of apparently autosomal recessive origin appears to be the first Mendelian form of the acrorenal developmental field defect identified so far. © 1992 Wiley-Liss, Inc.