Ruptured intracranial mycotic aneurysm: an unusual infectious complication following craniofacial surgery.

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.

Anatomical suitability of C1-2 transarticular screw placement in pediatric patients.

OBJECT: Craniovertebral instability is a challenging problem in pediatric spinal surgery. Recently, C1-2 transarticular screw fixation has been used to assist in craniovertebral joint stabilization in pediatric patients. Currently there are no available data that define the anatomical suitability of this technique in the pediatric population. The authors report their experience in treating 31 pediatric patients with craniovertebral instability by using C 1-2 transarticular screws. METHODS: From March 1992 to October 1998, 31 patients who were 16 years of age or younger with atlantooccipital or atlantoaxial instability, or both, were evaluated at our institution. There were 21 boys and 10 girls. Their ages ranged from 4 to 16 years (mean age 10.2 years). The most common causes of instability were os odontoideum (12 patients) and ligamentous laxity (eight patients). Six patients had undergone a total of nine previous attempts at posterior fusion while at outside institutions. All patients underwent extensive preoperative radiological evaluation including fine-slice (1-mm) computerized tomography scanning with multiplanar reconstruction to evaluate the anatomy of the C1-2 joint space. Preoperatively, of the 62 possible C1-2 joint spaces in 31 patients, 55 sides (89%) were considered suitable for transarticular screw placement. In three patients the anatomy was considered unsuitable for bilateral screw placement. In three patients the anatomy was considered inadequate on one side. Fifty-five C1-2 transarticular screws were subsequently placed, and there were no neurological or vascular complications. CONCLUSIONS: The authors conclude that C1-2 transarticular screw fixation is technically possible in a large proportion of pediatric patients with craniovertebral instability.

Down syndrome and craniovertebral instability. Topic review and treatment recommendations.

The diagnosis and management of occipital-atlantal and atlantoaxial instability in Down syndrome patients is a challenging problem in pediatric spine surgery. To date, no systematic review of this topic has been presented on this confusing and sometimes contentious issue. This topic review will focus on the biomechanical and radiographic foundations for which treatment recommendations in Down syndrome patients are made. In addition, otolaryngologic and anesthetic considerations in Down syndrome are also discussed, as well as advances in surgery that have made the operative fusion of these patients easier and safer.

A new occipitocervical fusion construct in pediatric patients with occipitocervical instability. Technical note.

Posterior occipitocervical stabilization procedures were successfully performed in 10 patients (nine boys and one girl) 16 years of age or younger by using C1-2 transarticular screws coupled with a rigid occipitocervical construct. The average length of follow-up evaluation was 18.8 months (range 5-37 months). No implant failed and all fusions were successful without the use of an external orthotic halo device.

Endoscopic third ventriculostomy: an outcome analysis.

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.

Choroid plexus papilloma and cysts in the Aicardi syndrome: case reports.

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.

Astrocytoma and pineoblastoma arising sequentially in the fourth ventricle of the same patient. Case report and molecular analysis.

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.

Meningitis due to Ochrobactrum anthropi: an emerging nosocomial pathogen. A report of 3 cases.

We describe 3 cases of Ochrobactrum anthropi meningitis following the implantation of pericardial allograft tissue to cover dural defects following craniotomy. Following an extensive epidemiologic investigation, the tissue allograft was found to have been contaminated with this unusual organism during the harvesting and processing of the tissue in the tissue bank. This organism was only susceptible to imipenem, tetracycline, gentamicin, and ciprofloxacin. The clinical presentation of these patients was subacute. Two of the patients developed osteomyelitis of the bone flap; while another developed a relapse of infection along a former ventriculoperitoneal shunt track 6 months after the initial infection. Appropriate clinical outcome was only observed after removal of tissue allograft implants, debridement of devitalized tissue and bone, removal of shunt devices, and prolonged courses of antibiotics. No deaths were observed.

Selective effects of ketamine on amino acid-mediated pathways in neonatal rat spinal cord.

Many ion channels have been proposed as target sites for anaesthetic action; for some agents multiple receptors-ion channels may be implicated. In addition to acting as a non-competitive antagonist at glutamate NMDA receptors, ketamine also affects other ion channels. The present study was undertaken to determine if the effects of ketamine in an integrated portion of the central nervous system involve multiple actions at glutamate non-NMDA, glutamate NMDA, and GABAA receptor. The effects of ketamine 1-50 mumol litre-1 were examined on three pharmacologically distinct responses in isolated superfused neonatal rat spinal cord: the monosynaptic reflex (glutamate non-NMDA); a slow ventral root potential (VRP) with a large NMDA-mediated component; and the dorsal root potential (DRP) (GABAA). Ketamine, at concentrations relevant to anaesthesia (1-50 mumol litre-1), reversibly depressed the area under the curve of the slow VRP in a concentration-dependent fashion. The effects of ketamine were selective for the early (0-1 s) component of the slow VRP. The monosynaptic reflex was unaffected at these concentrations. The actions of ketamine resembled those of the NMDA antagonist APV. Dorsal root potentials evoked by dorsal root stimulation or by muscimol were either unaffected or reversibly depressed by ketamine 1-20 mumol litre-1. The concentrations tested include the anaesthetic range for both rats and humans. The effects of ketamine on neurotransmission in this preparation can be accounted for entirely by its action at NMDA receptors. Glutamate non-NMDA receptors were unaffected and GABAA transmission was not enhanced.(ABSTRACT TRUNCATED AT 250 WORDS)

Pediatric cervical spine instrumentation using screw fixation.

From July 1986 to August 1993 we performed 24 pediatric cervical spine screw fixation procedures on 23 patients 16 years of age or less. The types of cervical instrumentation procedures performed were as follows: anterior cervical plates 12, posterior C1-2 screw fixations 8, posterior lateral mass plates 2, odontoid screw fixations 2. The mean age of all patients was 14.2 years (range 6-16). Indications for operation included traumatic instability in 20 cases, congenital instability in 1 case, 2 cases of postoperative swan neck deformity, and one reoperation for early graft and hardware failure. Six of the 23 patients had persistent instability following previous failed fusions (3 with 1 prior surgery, 2 with 2 prior surgeries, and 1 with 3 prior surgeries). Eight patients had improvement of their neurological status following operation and 15 remained at their preoperative level of neurological function. No patient was worse neurologically after their procedure. There were no long-term instrumentation, graft or fusion failures. Two complications occurred. One was the aforementioned graft and hardware failure requiring reoperation, the other was a superficial wound infection treated successfully with antibiotics. We feel that cervical spine fixation techniques have increased our ability to stabilize the pediatric cervical spine and have proven to be safe and effective.

p53 mutation and loss of heterozygosity on chromosomes 17 and 10 during human astrocytoma progression.

The human brain tumor, astrocytoma, typically progresses through three histopathologically defined stages with the passage of time: one premalignant stage, low-grade astrocytoma; and two malignant stages, anaplastic astrocytoma and glioblastoma multiforme. We correlated the results of a sequence analysis of the tumor suppressor gene, p53, and a restriction fragment length polymorphism analysis of chromosomes 17 and 10 in 45 patients with cerebral astrocytomas at different stages. To detect p53 mutations in tumor DNA, we analyzed polymerase chain reaction products corresponding to every p53-coding exon for single-strand conformation polymorphisms and confirmed the mutations by sequencing. Loss of heterozygosity (LOH) was determined by Southern transfer analysis of somatic and tumor DNA from these same patients using polymorphic markers for various loci on chromosomes 10 and 17. p53 mutations were found in 7 of 25 glioblastomas (28%), in 5 of 14 anaplastic astrocytomas (36%) but in 0 of 6 low-grade astrocytomas. p53 mutations were found in 62% of patients with LOH on chromosome 17p. These results indicated that p53 inactivation is a common genetic event in astrocytoma progression that may signal the transition from benign to malignant tumor stages. LOH on chromosome 10 was found in 61% of glioblastomas, in 23% of anaplastic astrocytomas, but in 0% of low-grade astrocytomas. LOH on chromosome 10 and p53 mutation were found together only in patients with glioblastoma multiforme (22%), suggesting that these genetic changes may accumulate during astrocytoma progression.

Neurenteric cysts of the posterior fossa: recognition, management, and embryogenesis.

Neurenteric cysts are endothelium-lined structures most commonly encountered in the lower cervical or upper thoracic spinal cord. The occurrence of neurenteric cysts within the cranial vault is unusual. We present three patients with neurenteric cysts located within the posterior fossa: one near the jugular foramen deforming the 4th ventricle, a second in the cerebellopontine angle, and a third in the prepontine cistern. Several different theories have been advanced to explain the embryogenesis of neurenteric cysts. We review these theories and conclude that cranial neurenteric cysts may arise from a disturbance of early gastrulation, shortly after the onset of primitive streak regression.

Cerebral granular cell tumor occurring with glioblastoma multiforme: case report.

A 65-year-old man presented with 4 weeks of partial right visual field loss. A left occipital granular cell tumor was diagnosed via open biopsy. No specific tumoricidal therapy was given, and the patient returned 2 weeks later with fluent dysphasia and mild right hemiparesis, and formed visual hallucinations. A large left parietotemporal mass, separate from the granular cell tumor, was diagnosed as a glioblastoma multiforme by stereotactic biopsy. The histogenesis of cerebral granular cell tumors is controversial; this case supports recent speculation of their possible glial origin.

Management of posthemorrhagic hydrocephalus in the low-birth-weight preterm neonate.

Over a period of 34 months from 1987 to 1990 we inserted ventricular catheter reservoirs (VCR) into 20 premature low-birth-weight infants who had developed progressive, symptomatic posthemorrhagic hydrocephalus following grade III or IV intraventricular hemorrhages. The mean estimated gestational age was 27.7 +/- 5.3 weeks and mean birth weight was 1,041 +/- 699 g. The ventricular catheter reservoirs were placed on day of life 30.7 +/- 29.7 and tapped for a total of 3-34 days at varying frequencies and for varying volumes. Of the 20 patients, 4 died on days of life 25, 76, 88, and 187. There were two reservoir infections, both occurring in infants who eventually died. The 16 survivors have been followed from 2 to 24 months (adjusted age). Four (25%) remain shunt-free and 3 have undergone VCR removal. There have been two shunt infections in the 12 shunted patients; ten shunt revisions have been performed overall. At the time of last follow-up, 14 patients were old enough to undergo neurodevelopmental evaluation. Five patients (36%) were 'normal' on gross neurological screening examination, 5 (36%) had 'mild developmental delay' and 4 (28%) had 'significant developmental delay'. We feel these data support the continued use of ventricular catheter reservoirs in the management of posthemorrhagic hydrocephalus and offer hope that some of these patients might remain shunt-free and most will have a normal or mildly delayed neurodevelopmental outcome.