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Surgery in children with epilepsy is a new, evolving field. The important practical issues have been to define strategies for choosing the most suitable candidates, as well as the type and the optimum timing of epilepsy surgery. This study was undertaken to illustrate these points. To identify the factors that correlated with outcome, the authors analyzed a series of 200 children (aged 1–15 years, mean 8.7 years) who underwent surgery at the Hôpital Necker-Enfants Malades between 1981 and 1996. In 171 cases (85.5%) the epilepsy was medically refractory and was associated with focal cortical lesions. Surgery consisted of resection of the lesion without specifically attempting to identify and remove the "epileptogenic area." In the group of children whose seizures were medically refractory, the mean follow-up period was 5.8 years. According to Engel's classification, 71.3% of these children became seizure free (Ia), whereas 82% were in Class I. A multivariate statistical analysis revealed that among all the factors studied, the success of surgery in a patient in whom there was a good clinical-electroencephalogram-imaging correlation, depended on the patients' having undergone a minimally traumatic operation, a complete resection of the lesion, and a short preoperative seizure duration. After the surgical control of epilepsy, behavior disorders were more improved (31% of all patients) than cognitive function (25%). The patient age at onset, duration and frequency of seizures, intractability of the disease to therapy, and seizure characteristics, were correlated with cognitive, behavioral, and academic performance pre- and postoperatively. Multivariate statistical analysis revealed that cognitive dysfunction correlated highly with the duration of epilepsy prior to surgery, whereas behavioral disorders correlated more with seizure frequency. These data must be taken into account when selecting patients for surgical treatment and when deciding the timing of surgery. Early surgical intervention allows for optimum brain development.

This 3-month-old boy was first assessed by a pediatrician for progressive macrocrania in October 1986. A computerized tomography scan revealed a Dandy‐Walker malformation (DWM) with mild ventricular dilation. The patient was asymptomatic and therefore followed clinically and radiologically. At the age of 2 years, progressive hydrocephalus required cystoperitoneal shunt placement. Postoperative magnetic resonance (MR) imaging revealed progressive herniation of the lower pole of the cyst in the upper cervical canal (Fig. 1A) and the development of a syringomyelic cavity (Fig. 1B) that continued to increase in spite of three shunt revisions (Fig. 1C). The patient developed normally but the onset of gait disturbances prompted us to perform a suboccipital decompression, C-1 laminectomy, and duraplasty at the age of 9 years. An MR image obtained 3 months later (Fig. 1D) showed a significant decrease in the size of the syrinx. The association between DWM and syringomyelia has rarely been described, either as isolated case reports 1,5 or as an occasional finding in large autopsy or clinical series. 2‐4 Milhorat, et al., 4 recently reported three cases of DWM with syringomyelia in a series of 105 autopsy specimens obtained in syringomyelia patients. The authors included these observations in the group that they called “communicating central canal syrinxes.” They stated that the occurrence of syringomyelia in DWM would be the expression of a participation of the central canal “in the hydrocephalus process like a fifth ventricle.” This case seems to show that the cause of syringomyelia in DWM could be multifactorial. The crowding of the occipital foramen induced by the herniation of the lower pole of the cyst could be responsible for the progression of the syringomyelia, acting with a Chiari-like mechanism in spite of the presence of a working cerebrospinal fluid shunt. In such cases, suboccipital decompression and duraplasty are effective treatments.

✓ Cases of craniosynostosis usually fall into well-demarcated categories: those related to a syndrome or those identified by a combination of suture involvement and morphological appearance. Between 1976 and 1995, 53 (3.6%) of 1474 cases in the craniofacial databank were assessed and designated as nonsyndromic but unclassifiable. The records and radiological studies obtained in these patients were retrospectively analyzed and comparisons were made with patients classified in the databank as having simple craniosynostoses. It proved possible to divide the formerly unclassifiable cases into two groups: those with “two-suture disease” (Group A) and a “complex” group (Group B) in which more than two sutures were affected. Group A consisted of 36 cases (68%) of patients presenting with clear evidence of simultaneous involvement of two sutures but with no progression over time to suggest a more diffuse pansynostosis. Suture involvement was as follows: 17 of 36 sagittal plus one coronal; seven of 36 sagittal and metopic; six of 36 sagittal plus one lambdoid; and six of 36 metopic plus one coronal. The only significant difference between the Group A cases and the cases of simple craniosynostoses was in the percentage requiring a second operation (24% vs. 5%, p < 0.0001). Group B consisted of 17 cases in which the patients presented at a slightly earlier age (mean 1 year) with severe morphological changes and multiple suture involvement. At the time of surgery, six of 17 patients showed large areas of lacunae within the cranial vault, making craniectomy the only option. In Group B, 10 of 17 patients displayed bilateral lambdoid plus sagittal suture involvement resulting in marked occipital recession posteriorly, whereas anteriorly in six of these 10 patients there was a massive frontal bone associated with posteriorly located coronal sutures. In contrast, there were also four patients in Group B with bilateral coronal plus metopic involvement resulting in a small frontal bone. There was a trend toward a lower intelligence quotient and a worse morphological outcome in the patients in Group B, but again the only result attaining statistical significance when compared to the databank was the rate of second operation (37.5 vs. 5%, p < 0.0001). “Two-suture synostosis” is a relatively straightforward condition and is treatable with standard craniosynostosis techniques. However, possibly as a result of surgical compromise when two sutures are involved, the rate of reoperation is far higher than in simple suture cases. In contrast, patients in the “complex” group presenting with severe multisuture involvement require a more tailor-made approach to their management that often entails a second procedure.

This study reports a selected group of 45 patients with symptomatic low-grade stenoses of the carotid bifurcations, detected by real-time B-mode echotomography, and correlates the results of this technique with those of the Doppler flow study and carotid angiography. The Doppler study with spectral analysis was negative in all cases, whereas a correlation between the echotomographic and angiographie results was observed in 20 among 27 patients explored by angiography (74%). The causes of failure of angiography in some cases are briefly discussed. The almost absolute sensitivity of high resolution B-mode echotomography in detecting these early, sometimes symptomatic atherosclerotic changes of the carotid artery wall is emphasized.

Two rare cases of cerebral cavernous angiomas in two infants. 9 and 6 months old, respectively, are reported and the other 11 cases in the literature concerning patients in the first year of life are reviewed. Cavernous angiomas of the brain occur rarely in the first year of life and present with seizures and head enlargement. On computed tomographic scan they typically appear as large, hyperdense, unenhanced masses, with large cysts and must be differentiated from tumors more common in infants, such as teratomas, ependymomas, and mixed tumors. Total removal is often possible, even with large lesions, because of the small amount of bleeding and the well-defined limits of the mass.

Neuroendoscopic surgery is being used as an alternative to traditional shunt surgery and craniotomy in the management of hydrocephalus and intracranial fluid-filled cavities. In this study, the authors evaluated the incidence and type of complications occurring after neuroendoscopic procedures that were performed in a consecutive series of pediatric patients at a single institution to determine the effectiveness of neuroendoscopy in such patients.Four hundred ninety-five neuroendoscopic procedures were consecutively performed in 450 pediatric patients at one institution over a 10-year period. Charts were retrospectively reviewed. A complication was defined as follows: 1) any postoperative neurological deficit that was not observed before surgery; 2) any event occurring during surgery that resulted in the procedure being aborted; or 3) any adverse event occurring within 7 days postsurgery that resulted in a modification of the normal postoperative care. However, headache, vomiting, and fever without cerebrospinal fluid (CSF) pleocytosis were not considered complications. Complications were observed in 40 (8.1%) of 495 procedures. Two patients had two complications. One patient died of diffuse brain edema following endoscopic biopsy sampling of a basal ganglia tumor (mortality rate 0.2%). Other complications observed were abandonment of the procedure in eight cases, CSF leakage in 11 (with associated wound infection in one), intraventricular hemorrhage in six (with external drainage needed in four), intraparenchymal hemorrhage in three, subdural collection in eight (with subdural-peritoneal shunt placement needed in seven), transient oculomotor palsy in two, and transient hemiparesis in one.Many complications can be avoided by determining the correct diagnosis and using suitable techniques and instruments. Most complications can be managed conservatively and do not produce long-term morbidity. Complex procedures in most patients and simple procedures in patients with preoperative risk factors carry the highest hazard. Every attempt should be made to optimize the surgical technique. The most serious and potentially the most lethal complication remains arterial bleeding from injury to the basilar artery complex.