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The case of a child with Apert syndrome is presented in which the development and rupture of an intracranial mycotic aneurysm occurred secondary to multiple infectious complications following craniofacial surgery. An endovascular procedure was utilized in an attempt to embolize the aneurysm and parent vessel. The patient recovered from her infections, but retained a residual right hemiparesis and left cranial nerve III palsy at the time of discharge. To our knowledge, this is the first report of a mycotic aneurysm developing after a craniofacial procedure. Risk factors leading to aneurysm formation in this case are presented, as well as a literature review of neurological complications following craniofacial surgery., (Copyright 2001 S. Karger AG, Basel)

Neural tube defects (NTD) are one of the most common birth defects and are caused by both environmental and genetic factors. The approach to identifying the genes predisposing to NTD, through linkage analysis and candidate gene analysis, is reviewed along with characteristics of a large, nationally ascertained cohort of families. Results from specific assessments of p53, PAX3 and MTHFR failed to suggest that these genes play a major role in NTD development in these families. Advances in genetic laboratory and statistical techniques have made this a prime opportunity for investigation into the causes of complex disorders, such as NTD. However, traditional approaches may prove to be challenging due to the difficulty of ascertaining samplable multiplex families., (Copyright 2000 S. Karger AG, Basel)

Endoscopic third ventriculostomy (ETV) has gained widespread acceptance as an effective way to manage hydrocephalus in selected patients. To determine which patient groups have the highest chance of successful ETV, a retrospective case review was performed. From June 1992 to December 1996, 97 patients underwent a total of 98 ETVs at our institution. There were 59 males and 38 females with a mean age of 8.1 years (range 1 day to 29.5 years). Twenty-six of 98 procedures (26%) were abandoned due to either unfavorable anatomy, inability to perform a cisternostomy, or hemorrhage. Follow-up data was available in 92 patients for a mean of 24.2 months. The rate of successful ETV in 71 patients, with either complete shunt avoidance or removal, varied widely by diagnosis and patient age. The highest success rates were achieved in patients with aqueductal stenosis, tectal plate tumor, myelomeningocele and posterior fossa tumor. Complications included one transient herniation syndrome, one basilar artery perforation, 2 cases of ventriculitis, one transient decrease in level of consciousness, and one transient hemiparesis. We feel these results support the continued use of ETV in only carefully selected patients with hydrocephalus.

The morbidity and mortality associated with third ventriculostomy has decreased significantly over the past 75 years since its introduction by Walter Dandy. Now more commonly performed using an endoscopic method, the significant morbidity of third ventriculostomy has dropped to approximately 5%; essentially that associated with ventriculoscopy in general. However, the possible complication of massive subarachnoid hemorrhage resulting from perforation of the basilar artery or its branches in the course of fenestration of the floor of the third ventricle has only recently been reported. In our case, subsequent to a vascular injury, a pseudoaneurysm developed at the site of vascular perforation, which was then appropriately controlled. The patient has since made a full recovery. Our goal is to remind the endoscopist of this unusual complication and to discuss our management strategies.

Two female infants with callosal agenesis, infantile spasms, chorioretinal lacunae, optic disc colobomas and cortical heterotopias were diagnosed with Aicardi syndrome. A choroid plexus papilloma was found in one patient, and choroid plexus cysts were found in the other. Choroid plexus lesions are common findings in the Aicardi syndrome and are discussed in this paper.

On the basis of experimental studies on mouse embryos in which spina bifida development of the brain ventricular system. Mouse embryos were exposed and surgically treated at gestation day 12 to produce spina bifida aperta, and operated embryos were cultured exo utero in the peritoneal cavity of the pregnant dam. Embryos were harvested at gestation day 14. Open neural tube defects resulted in 7 out of 10 embryos surviving surgery. Histological examination revealed that the sizes of ventricles in both the supra- and infratentorial compartments were greatly reduced in embryos with spina bifida aperta compared with control embryos. This result supports the hypothesis that leakage of cerebrospinal fluid through the opening of the caudal spine may be a major cause of brain abnormalities in patients with myelomeningocele.

The sequential appearance of two different brain tumors in the same patient without intervening radiation or chemotherapy is a rare event, most often seen in hereditary cancer syndromes. We present one such case of sequential tumors, along with their molecular analysis. A 17-year-old male presented with a pilocytic astrocytoma arising in the fourth ventricle at the pontomedullary junction. Six and one half years later, a pineoblastoma was discovered in the fourth ventricle, rostral to the first tumor site. Both tumors were treated by gross-total surgical resection. Following resection of the pineoblastoma, the patient underwent craniospinal irradiation and systemic chemotherapy. Single-strand conformation polymorphism analysis showed that the patient had neither a germ-line mutation nor a somatic tumor mutation in the p53 tumor suppressor gene. Coupled with the lack of a family history of cancer, these data suggest that these were not manifestations of Li-Fraumeni syndrome, but rather two sporadic tumors which arose via a p53-independent mechanism.

Knowledge of long-term outcome and complications of selective dorsal rhizotomy is limited due to the relatively recent introduction of the procedure. We describe 2 patients with cerebral palsy who developed lumbar spinal stenosis several years after selective dorsal rhizotomy. These patients also had substantial lateral trunk sway during gait and walked for several years with limited assistive devices. This abnormal gait pattern in combination with the changes from the selective dorsal rhizotomy may lead to the development of spinal stenosis.

This case report describes a 5-year-old child who sustained fatal craniocervical injuries as the result of an inflating automobile air bag. Although air bags have in general been shown to reduce the severity of injury to occupants of vehicles involved in front-end collisions, a growing number of incidents suggests that in some instances the air bag system itself may cause injuries, some of which can be serious or even fatal. Small children and infants in infant seats travelling in the front passenger seat appear to be particularly at risk. The increasing number of reports of air bag mediated injuries highlights the need for changes in both system design and possibly the threshold speed required for air bag deployment.

With the availability of better endoscopes, improved lighting and increased instrumentation, the use of ventriculoscopy and ventriculostomy in the management of hydrocephalus is becoming increasingly more common. Neurosurgeons recognized the potential for endoscopic surgery early in this century, but were frustrated in many of their attempts at treatment due to the poor quality of the instruments available. Nevertheless, much progress has been made, and the stage was set for better results with modern instrument design. This paper reviews the history of endoscopes in neurosurgery and ponders the direction these instruments will take us in the near future.

The method used in the intraoperative portion of the selective dorsal rhizotomy procedure for the selection of abnormal circuits within the central nervous system of children with spasticity due to cerebral palsy is described. The decision to transect dorsal rootlets is made on the basis of electrophysiological, behavioral and medical criteria. In 100 cases the average number of rootlets transected was 64%. Of the total stimulated, 23.3% exhibited normal electrophysiological and behavioral responses. Decision making regarding the preservation or transection of the remaining 12.7% of the segments is described. This information is presented in an effort to increase understanding of this particular portion of the procedure and to encourage standardization of the procedure between centers in the future.