Indication for and surgical outcomes of the distraction method in various types of craniosynostosis

Background Various surgical techniques for the treatment of craniosynostosis using distraction devices have been described over the last few years and we have applied these procedures in seven patients with varying types of craniosynostosis. The aim of this report is to clarify the advantages and disadvantages of these surgical methods and to discuss current concepts for the surgical strategy in the treatment of craniosynostosis.Material and methods From January 2001 to March 2003, 25 patients with craniosynostosis were examined. Among them, 7 patients, 5 with Apert syndrome, 1 with Crouzon disease, and 1 with multiple-synostosis, underwent surgical treatment using the distraction method with internal distraction devices, according to our treatment strategy for craniosynostosis. All patients underwent preoperative and postoperative evaluations, which included the patients neurological state, developmental quotient (DQ), and three-dimensional CT (3D-CT).Results The timing of the procedures undertaken was between the ages of 1 year 5 months and 12 years 6 months (mean age 4 years 11 months). Five patients had received previous treatment and this procedure was used as a secondary operation. Postoperative distraction distances varied from 7 to 20.5 mm (mean distraction distance: 14 mm). Satisfactory cranial volume expansion and aesthetically pleasing morphological states were achieved in all cases. Regarding complications, one patient required re-operation because of dislocation of the device and skin erosion caused by infection around the penetrated wound. Finally, in a second patient a distortion of the device occurred, but no re-operation was needed.Conclusion The advantage of the distraction method is its applicability for Toddler or Elder Children Calvarial Reconstruction to correct cosmetic and functional problems. One disadvantage is the difficulty in using it for Infantile Calvarial Normalization because of thin calvarial bones and the necessity for re-operation to remove the device, which may result in it becoming a fixation procedure, essentially contraindicated for the fast-developing brain and calvarias. However, the efficacy of this procedure is that the many advantages outweigh the disadvantages as sufficient calvarial expansion and good results using the distraction method, especially in toddler and elder children age groups, can be achieved.     

“Hydrocephalus-parkinsonism complex”: progressive hydrocephalus as a factor affecting extrapyramidal tract disorder—an experimental study

Objective To experimentally clarify the symptomatological and pathophysiological aspects of a newly proposed clinical entity, we produced an experimental rat hydrocephalus-parkinsonism complex model.Methods A total of 60 male Sprague-Dawley rats were used. Twenty rats were treated with only kaolin as controls. Hemiparkinsonism was induced in the other 40 rats by stereotactic injection of 6-hydroxydopamine (6-OHDA) directly into the right substantia nigra. Twenty of these 40 rats were then treated with an injection of kaolin into the cisterna magna 3 days after the induction of parkinsonism. Neurological features were assessed weekly for 1 to 6 weeks after 6-OHDA injection by an apomorphine-induced turning test.Results The mortality rate was 25% in the rats injected with kaolin and 10% in those with the 6-OHDA injection. The frequency of the induction of parkinsonism-like signs was minimal in the first 2 weeks and reached a maximum point 4 weeks after the 6-OHDA injection. In contrast, the rats injected with both 6-OHDA and kaolin developed hemiparkinsonism-like signs in the early stage of the progression of hydrocephalus, and the peak incidence was reached in the 2nd week after induction (p<0.05 compared with the 6-OHDA group). The severity of the neurological disturbance was also significantly more prominent in the latter group.Conclusion These results suggest that the hydrodynamic effect in the early period of ventriculomegaly is the mechanism for signs of parkinsonism in 6-OHDA-treated rats. It could be concluded that the hydrocephalic state aggravates parkinsonism, if any as the associated condition, and it may be a new clinical entity of hydrocephalus symptomatology with a specific pathophysiology.An erratum to this article can be found at     

Schwannoma of the spinal accessory nerve--case report

A 60-year-old woman presented with a rare schwannoma arising from a spinal accessory nerve at the C1-2 levels manifesting as cervico-occipital pain. The tumor was removed by surgery with the involved segment of the nerve. She had no postoperative neurological deficit. Histological examination confirmed the diagnosis of schwannoma. Surgical removal is recommended for such cases.     

Relationship between development of nephrotoxicity and blood concentration of cyclosporine A in bone-marrow transplanted recipients who received the continuous intravenous infusion

This study was undertaken to investigate the relationship between blood concentration of cyclosporine A (CsA), administered intravenously by a 24-h continuous infusion, and drug-induced nephrotoxicity or hepatotoxicity. It was investigated retrospectively in 8 patients who had received an allogeneic bone marrow transplant (BMT). The correlation between daily doses and blood concentration of CsA was not significant. Then, the data of blood concentration of CsA and renal or liver function test result were divided into 5-d periods from the date of transplantation, and the mean value for each period was calculated. The maximum values of blood urea nitrogen (BUN) and serum creatinine (SCr) were consistently observed only after the period when the 5-d mean CsA concentration reached the peak level: the maximum BUN and SCr values were witnessed at Periods 2 to 10 and at Periods 1 to 9, respectively. On the other hand, no consistent correlation was found between the 5-d mean CsA concentrations and liver function test result. We also investigated the relationship between renal function and the cumulative dose or AUC of CsA. The parameters of renal function tests reached peak levels at the cumulative dose of 4000 to 10000 mg and at the cumulative AUC of 280000 to 660000 ng/ml.h. These results suggest that: 1) a deterioration of renal function occurs usually after the peak blood concentration of CsA is attained, and 2) the monitoring of the blood concentration of CsA is useful in predicting renal dysfunction in post-BMT patients.     

Recovery from lactacidosis-induced glial cell swelling with the aid of exogenous anion channels

Hypotonic challenge induces transient swelling in glial cells, which is typically followed by a regulatory volume decrease (RVD). In contrast, lactic acidosis (lactacidosis) induces persistent cell swelling in astrocytes without an accompanying RVD. In the present study, we studied the mechanisms by which lactacidosis interferes with normal volume regulation in rat astrocytic glioma C6 cells. Following exposure of C6 cells to a hypotonic challenge, a current was detected that exhibited properties consistent with those of volume-sensitive outwardly rectifying (VSOR) anion channels. When exposed to in vitro conditions designed to simulate lactacidosis, C6 cells failed to respond to hypotonic stress with an RVD, and VSOR anion currents were not activated. When added to C6 cells, an anion channel-forming protein purified from Helicobacter pylori, VacA, was found to form anion-selective channels in the plasma membrane, and the activity of the VacA channel was not affected by lactacidosis (pH 6.2). Cells preincubated with VacA and then exposed to lactacidotic conditions underwent transient swelling followed by RVD. In contrast, application of a cation ionophore, gramicidin, failed to inhibit lactacidosis-induced persistent cell swelling. From these results, we conclude that inhibition of a volume-sensitive anion channel contributes to persistent swelling induced by lactacidosis in glial cells. Introduction of anion channel activity into glial cells might provide a novel approach for treating cerebral edema, which is associated with lactacidosis in cerebral ischemia or head injury.     

Correlation between MRI findings and second-Look operation in cholesteatoma surgery

Two-staged intact canal wall tympanoplasty is a common operation for treatment of middle ear cholesteatoma. MRI provides better tissue differentiation of the middle ear and/or mastoid, which often become occupied with soft density tissue after the first operation. If MRI was able to detect the presence of a recurrent or residual cholesteatoma with sufficient sensitivity and specificity, this may facilitate a decrease in the number of second-look procedures. This study compared MRI findings to surgical findings at second-look surgery and calculated the correlation rates between the two sets of findings. Thirty ears having undergone intact canal wall tympanoplasty for cholesteatoma at the initial operation were examined by MRI prior to the second look. Otoscopic findings of the tympanic membrane were nonsuspect in all cases. The true positive rate was 11/30 (37%) and the true negative rate was 10/30 (33%), leading to a radiosurgical correlation of 70%, whereas the false positive rate was 6/30 (20%) and the false negative rate was 3/30 (10%). This indicates that 30% of the MRI findings were incorrect. Therefore, at the present time, MRI does not appear as a likely replacement for second-look surgery in cases of intact canal wall tympanoplasty.     

Glomerular lesions associated with the Crow-Fukase syndrome

Summary Three cases of the Crow-Fukase syndrome without radiographic changes of multiple myeloma are reported, with special reference to the glomerular changes seen. Proteinuria was detected in one case, although decreased renal function was observed in all (GFR: 41.0, 62.0, 74.1 ml/min respectively) at the time of renal biopsy. Glomerular changes were similar in all three cases. The main characteristic changes were mesangial proliferation and thickening of the glomerular capillary walls. Pictures by light microscopy were therefore similar to that of MPGN. On electron microscopy, the thickened capillary walls showed circumferential mesangial interposition and the subendothelial zone was electron-lucent and contained small dense granules or flocculent deposits. By immunofluorescent microscopy, no immunoglobulins, complement components or light chain were detected in the glomeruli except in one case.