Cognitive, behavioral and psychological functioning of children and adults with conservatively managed metopic synostosis.

Children diagnosed with metopic synostosis (MS) commonly experience poor neuropsychological outcomes, with research suggesting that children whose MS is managed conservatively (without surgery) potentially having worse outcomes than their operated peers. However, studies of children whose MS was managed conservatively are scarce. This study therefore examined the cognitive, behavioral, and psychological functioning of children/adults with conservatively managed MS (N = 38) and compares their outcomes to individually matched healthy controls (N = 38) of the same age and sex (matched-pairs design) from the general community. Age-appropriate, validated assessments measuring general cognition, verbal and visuospatial ability, attention and working memory, executive functioning, behavior, depression, anxiety, and satisfaction with appearance were utilized. Group differences were estimated using linear regression for (a) the overall sample and (b) by broad developmental stages: 2&3 yrs; ≥6-≤17. Moderate to large negative effects (g = -0.38 to -1.30) were evident before controlling for socio-economic status (SES), with the MS group performing significantly worse on 8 out of the 10 cognitive domains (general cognition, visuospatial ability, working memory, information processing, executive functioning: semantic & initial letter verbal fluency, switching, inhibition+switching). However, only initial letter verbal fluency (g = -0.99) and switching (g = -1.19) remained significant after adjusting for SES. The MS group displayed more behavioral problems, although this was not significant. Depression, anxiety, and satisfaction with appearance did not differ between the groups. Regular monitoring of cognitive functioning, particularly executive functioning, should be undertaken for those with conservatively managed MS.

Cognitive, behavioral and psychological functioning in children with metopic synostosis: a meta-analysis examining the impact of surgical status.

Neurodevelopmental delays are known to occur in children with metopic synostosis, but it is presently unclear whether the cognitive, behavioral and psychological outcomes of children with metopic synostosis differ to those of their healthy peers. This meta-analysis consolidated data from 17 studies (published prior to August 2017) that examined the cognitive, behavioral and psychological outcomes of children (n = 666; aged ≤19 yrs) with metopic synostosis. Hedges'g (gw) effect sizes compared the outcomes of samples with metopic synostosis (unoperated, operated) to healthy controls or normative data and, where available, the prevalence of problems/disorders was calculated. Children with unoperated metopic synostosis performed significantly worse than their healthy peers on measures of: general cognition (gw = -.38), motor functioning, (gw = -.81), and verbal (gw = -.82) and visuospatial (gw = -.92) abilities. Children with operated metopic synostosis performed significantly worse on measures of motor functioning (gw = -.45), visuospatial skills (gw = -.32), attention (gw = -.50), executive functioning (gw = -.36), arithmetic ability (gw = -.37), and behavior (gw = -.34). Cognitive, behavioral, and psychological problems were prevalent, but variable. Overall, the cognitive, behavioral, and psychological outcomes of children with metopic synostosis are generally worse than their healthy peers, regardless of surgical status. However, research is sparse, samples small, controls are rarely recruited, and the severity of metopic synostosis often not stated. Nevertheless, the findings suggest that children with metopic synostosis are likely to experience a variety of negative outcomes and should therefore receive ongoing monitoring and support.

Cranial distraction osteogenesis in sheep using a totally implantable hydraulic mechanism: early results.

Conventional distraction osteogenesis has been performed by using either percutaneous pins attached to a thread and screw mechanism, or a thread and screw mechanism that is implanted but still requires turning by a percutaneous instrument or device. In these situations there is always the risk of infection passing to the bone via the percutaneous connection. We have developed a totally implantable hydraulic mechanism, which is activated in much the same way as injection into the port of a tissue expander. Preliminary in vitro testing has suggested that our mechanism can produce sufficient force at appropriate rates of distraction to have a promising role in distraction osteogenesis. We examined the effect of osseous distraction on the cranial vault. Expansion of the cranial vault by distraction osteogenesis is not widespread in clinical practice, in part because of the risk of infective complications of the extra-dural space. We were, therefore, presented with an ideal opportunity both to test the new distraction device and to examine the effect of distraction osteogenesis on the cranial vault. A sheep animal model was used in a pilot study to test the plausibility and examine the physiology of cranial-vault distraction osteogenesis using a totally implantable hydraulic device. Two sheep had a device implanted in the temporal fossa so as to push the craniotomised cranial cap upwards. Distraction was performed for 13 days beginning 1 week after craniotomy. After 9 weeks of consolidation, 5.5 mm and 7.4 mm of new woven bone of normal cranial thickness were demonstrated.

Separation of serum proteins by high-performance gel-permeation column systems.

Combined TSK-Gel G4000SW-G3000SW and Zorbax GF450-GF250 columns and a Superose 6 column were evaluated for the analytical gel permeation chromatographic separation of serum proteins. Serum elution profiles showed four distinct peaks, which were attributed to immunoglobulin M (IgM), alpha 2-macroglobulin, IgG and albumin. A reproducible shoulder on the IgG peak could be attributed to IgA. These purified serum proteins and other commercially obtained proteins with relative molecular masses between 1000 kDa and 10 kDa were chromatographed on the combined systems and the separate columns. The frictional coefficient-based hydrodynamic radii of these proteins showed a linear relationship with the inverse error function complement of their partition coefficients. Using this relationship, theoretical summation plots of the data obtained from the separate columns of a system correspond to plots that were calculated by treating the combined systems as one column. The best resolution was obtained with the TSK colums, but all three column systems were suitable for the separation of clinically important immunoglobulins in serum. The retention of positively charged proteins by the TSK columns becomes noticeable after relatively short usage and is a first sign of progressive loss of resolution.

Detection of serum proteins by high-pressure gel-permeation chromatography with low-angle laser light scattering, compared with analytical ultracentrifugation.

Human sera were subjected to analytical ultracentrifugation and "high-pressure" gel-permeation chromatography on a system of combined TSK Gel G5000 PW and G3000 SW columns. The chromatographic method produced remarkably superior resolution of the proteins, especially those exceeding 100 000 Da. We calculated the molecular masses of eluted fractions on the basis of their detection by low-angle laser light scattering and their differential refractive index. We discuss the results in relation to the clinical data.