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Background: The timing and extent of surgery continue to be a matter of discussion in current craniosynostosis management., Patients and Methods: We retrospectively reviewed the first 159 children seen from 1996 to 2000 of a total of 243 patients presenting with suspected craniosynostosis. We did not establish a surgical indication in 74 children (47%), either because their malformations were of different origin without associated craniosynostosis (11/7%) or they presented with only mild clinical manifestations of sutural synostosis (63/40%). In 85 cases (53%), we established a surgical indication. Parents did not follow our recommendation in two cases (1%). In 83 cases, we performed diagnosis-related remodeling at the age of 4-12 months. Demographic data, clinical follow-up findings, and regular photo documentation were analyzed., Results: After a mean follow-up of 3 years and 8 months, the results were satisfactory in 68 cases (82%). Of 15 cases (18%) with only limited improvement, two children were submitted to a second intervention. Another two children had to be reoperated on immediately for insufficient hemostasis. In the largest subgroup of children (47) with sagittal synostosis, there was no difference in outcome between surgery performed before and after 6 months of age., Discussion: Surgical indication and assessment of results are based on subjective criteria which limit the comparison with the literature. Our results correspond to those of similar published series. Most of the unfavorable results could be attributed either to inadequate adaptation of the surgical technique to the individual form of the malformation or to a significant secondary loss of correction in multi-sutural synostosis., Conclusions: The overall results of surgery within the first year of life were satisfactory in 82%. Standard procedures need to be adapted carefully to the individual form of craniosynostosis to avoid unfavorable results. We therefore propose a new surgical technique based on a statistically averaged modular system of skull models to replace freehand remodeling.

The hydrostatic dual-switch valve (DSV) was implanted in 56 patients suffering from hydrocephalus of different causes. Evaluation of the clinical status 3 and 6 months after the operation revealed excellent and good neurological recoveries in the vast majority of cases. Only 7 patients demonstrated an unsatisfactory result according to the grading of Stein and Langfitt. The CT follow-up, evaluated by the reduction of the Evans index, was characterized by only minimal or even no reduction of the ventricular size in more than half of the patients. Only 2 patients of our series developed overdrainage-related problems. 5 cases are presented to illucidate the danger of overdrainage resulting from the implantation of conventional differential-pressure valves, and the possible solution of this problem by hydrostatic devices like the DSV. Our series gives strong evidence, that reestablishing physiological pressure-ranges after shunting is paralleled by a good clinical outcome independent of the ventricular size after shunting.

Continuous monitoring of cerebral metabolism would be desirable for early detection of vasospasm in SAH patients. Bedside-microdialysis, a new technique for on-line monitoring of cerebral metabolism, may reflect changes seen in cerebral vasospasm diagnosed by transcranial Doppler sonography (TCD). This report represents the first case of combined TCD monitoring and on-line microdialysis from the brain extracellular fluid in a SAH patient. A 48-year-old woman suffered subarachnoid hemorrhage grade IV according to Hunt and Hess. Angiography revealed an aneurysm of the left carotid artery. The aneurysm was clipped 45 hours after bleeding. The microdialysis catheter was inserted after aneurysm clipping into the white matter of the left temporal lobe. Sampling of microdialysates started immediately, analyzing time for glucose, lactate, pyruvate and glutamate was four minutes. Postoperatively, the patient was doing well and microdialysis and TCD parameters remained within normal range. On the third postoperative day a shift to anaerob metabolism (decrease of glucose, increase of lactate and the lactate-pyruvate ratio up to pathological levels) and an increase in glutamate was observed suggesting insufficient cerebral perfusion. The patient progressively deteriorated clinically. Vasospasm was diagnosed by TCD monitoring 36 hours after onset of ischemic changes monitored by microdialysis. After elevation of mean arterial blood pressure, TCD values and metabolic parameters normalized. Interestingly, the pathological changes in on-line microdialysis preceded the typical increase in blood flow velocity by TCD and the clinical deterioration. Our case suggests, that bedside-microdialysis may be useful for early detection of vasospasm and continuous surveillance of treatment and may be a new guide to treat ischemic neurological deficits following SAH.

Since the beginning of 1995 the new hydrostatic dual-switch valve (DSV) was implanted in 35 adult patients with hydrocephalus of different etiology. 26 patients suffered from normotensive hydrocephalus (10 idiopathic and 16 symptomatic), and 9 patients from hypertensive hydrocephalus of various origin. The first 21 cases of this cohort were compared in a randomized study with a comparable group of 21 hydrocephalic patients who received a conventional differential-pressure (DP-) valve. The clinical status and CT were assessed prior to shunting, 14 days and 3 and 6 months after the operation. The reduction of ventricular size was evaluated by the measurement of the Evans Index. The CT follow-up in the DSV group was characterized by an only minimal (14) or only slight (16) reduction of ventricular size in the vast majority of cases. A comparison of 21 patients with a DSV and the patients with DP valves, evaluated by measuring the reduction of the Evans Index, revealed a distinctly higher percentage of significant regressions in the DP valve collective, without doubt due to chronic overdrainage. The overall clinical result of our 35 patients with a DSV was excellent and good in 31 patients, but the outcome seems to be more dependent on the preshunt damage of the brain than on hydrocephalic aspects. A neglegible incidence of subdural effusions in the DSV group compared to 11 cases in the DP valve collective reflects the ability of the DSV to prevent overdrainage. The capability of the DSV to maintain the IVP within physiological limits after shunting, especially in the upright position, is documented by a comparison with possible unphysiological IVP variations in other valve constructions, which depend on the level of implantation, subcutaneous pressure or CSF flow through the valve.

The Currarino triad is a hereditary transmitted syndrome, originally defined by Currarino as ASP-association, consisting of an anorectal malformation, a sacral bony defect and a presacral mass. In most cases autosomal dominant transmission is suggested. In family members one or two features of the syndrome may be missing, indicating an incomplete form of this complex. We describe two unrelated girls at the age of 8 and 9 months respectively with ASP-association. Family screening in both patients showed 8 additional cases with a complete or incomplete Currarino triad, four of them being asymptomatic. A review of the literature up to 1991 revealed 48 patients with ASP-association. In more than 80% of cases, this complex is diagnosed in the first decade, whereas incomplete Currarino syndrome is diagnosed predominantly in adults. Most frequently the presacral mass in ASP-association was reported to be an anterior meningocele (47%) and a benign teratoma (40%). The number of patients with Currarino syndrome has been underestimated so far. We recommend anorectal examination, pelvic ultrasound and pelvic x-rays in all patients with a history of chronic constipation since early childhood. Positive findings should lead to further investigations such as barium enema, MRI, myelography and family screening. Close cooperation between pediatric surgeons and neurosurgeons is required to ensure adequate surgical treatment, considering both the risk of malignant degeneration as well as the risk of intraoperative nerve damage. Thus, radical excisional surgery is not obligatory in every case of Currarino syndrome.

In 20 patients with known or suspected supratentorial arteriovenous malformations, an attempt was made to see how far CT angiography with 3-dimensional reconstructions is able to make a diagnosis and to differentiate the various components of the angioma. Spiral CT was performed following an intravenous bolus injection of 60-80 ml of iodine containing contrast medium. In all patients the diagnosis was confirmed by intra-arterial DSA of the vertebral vessels. In 13 patients, av malformations could be diagnosed following multiplanar 3-D reconstructions which agreed with the findings on DSA. The large supplying vessels, the nidus and the large draining veins could be defined with certainty. In 6 patients follow-up examination after embolisation was performed. The results could be demonstrated in three dimensions and the success of treatment could be documented unequivocally. CT angiography with 3-D reconstruction is able to supply important information in the majority of intracranial av malformations, both during initial investigation and following treatment.

In this study, the possibility of non-invasive, three-dimensional demonstration of aneurysms of the basal cerebral arteries by means of spiral CT was investigated. The first step was to obtain exact definition of optimal examination parameters. Angio CTs at appropriate levels were performed on 10 subjects and time/density curves of the arterial and venous phases obtained in order to optimise the beginning of the arterial spiral CT series. The second step in this investigation was to examine 7 patients; in 6 of these basal aneurysms had been demonstrated by DSA. By means of multiplanar three-dimensional reconstruction from the data of the spiral CT it was possible to demonstrate 7 aneurysms with a diameter between 5 and 18 mm. Their position and relationship to the bony skull was also shown.

Aim of this study was to assess the value of Gd-DTPA administration in MRI of cervical disk herniations. In 34 patients, showing 40 cervical disk herniations, plain proton density- and T1-weighted as well as contrast-enhanced T1-weighted 2D-FLASH images were generated. Contrast between the herniated material and the CSF already was sufficient without administration of Gd-DTPA in all cases. Sufficient contrast between the herniations and the intraforaminal structures was obtained in 13/40 cases and 40/40 cases on plain and contrast-enhanced images, respectively. Definition of the herniated disks was judged to be sufficient in 30/40 cases on plain images and 40/40 cases on contrast-enhanced images. The diagnostic value of the images was improved in 3/12 lateral and 7/8 intraforaminal herniations by contrast administration, whereas there was no significant increase in diagnostic value in posterior or posterolateral herniations. As a result, Gd-DTPA administration is recommended if definition of lateral and intraforaminal disk herniations in MRI is poor.

In a group of 25 patients the coronal views of plain and Gd-DTPA-enhanced MRI studies were correlated with CT and operative findings. All series included sagittal, transverse and coronal FLASH sequences (GE 500/6, flip angle 70 degrees), sagittal proton density-(SE 2500/15) and T2-(SE 2500/70) weighted images. MR diagnosis proved to be correct in all cases. Compared to CT and axial MRI herniated disk material in the lateral intravertebral space and its relationship to nerve roots was demonstrated more clearly on coronal views.

In this study, 50 patients were examined by a Siemens' "Somatom Plus"; continuous 2 mm sections between the third lumbar and first sacral vertebra were obtained. The accuracy of the 3-D reconstruction software programme was checked by measuring a polyethylene phantom of a second lumbar vertebra. Reconstruction was carried out with a threshold definition of 150 Hu. In each case, four standard projections and medio-sagittal, medio-lateral and coronary sections were reconstructed, photographed and compared with axial CT sections and conventional radiographs. All these imaging procedures were suitable for the diagnosis of osteochondrosis and chondrosis. Spondylosis was diagnosed more frequently on 3-D CT. Spondylarthrosis, with narrowing of the intervertebral foramina and root canals is shown particularly well by 3-D CT, since the entire extent of these structures can be seen. 3-D surface reconstruction of the lumbar spine is useful in the diagnosis of lumbar spondylarthrosis with narrowing of the root canals and of the spinal canal. This method of axial Ct is superior to conventional radiography of the lumbar spine in the usual two planes.

A study has been carried out to evaluate contrast and image quality of cervical structures using multislice 2D-flash sequences with long repetition times (TR = 400 ms.) and short echo delay times (TE = 5.8 ms.). The examinations were carried out using ten normals with an MRI of 1.5 Tesla and flip angles of 10, 20, 30, 50, 70 and 90 degrees. The best contrast between intervertebral disc and surrounding tissue was obtained between 50 and 70 degrees, best contrast between compact bone and CSF with 10 degrees. In order to demonstrate degenerative changes of the cervical spine, it appears sensible to use a combination of these angles. The described sequences produce good images of the cervical structures with little image degradation. Compared to T1-weighted spin-echo sequences, the method has a number of significant advantages, such as variations in image contrast, higher maximal number of slices, contiguous imaging and less imaging time.