A new technique for the repair and reconstruction of frontoethmoidal encephalomeningoceles by medial orbital composite-unit translocation.

A frontoethmoidal encephalomeningocele is a herniation of brain and meninges through a congenital bone defect in the skull at the junction of the frontal and ethmoidal bones. Between 1992 and 1999, we treated 145 cases of frontoethmoidal encephalomeningocele. Before 1993, the operation was performed in two stages. An intracranial repair by neurosurgeons preceded the external extirpation of the mass. In 70 cases that were operated on after 1993, a one-stage closure of the skull defect with a medial orbital composite-unit translocation technique was used. The medial orbital rim on each side, with intact periosteum, medial canthal ligament and lacrimal apparatus, was translocated as a unit to the midline. The advantages of this technique are that it allows convenient access to resect the herniation mass and close the defect, it restores normal interorbital and intercanthal distances and it eliminates the need for a transnasal medial canthopexy. Augmentation rhinoplasty can be avoided in most cases by tilting the composite unit with its preserved blood supply.

Primary diffuse leptomeningeal gliomatosis simulating tuberculous meningitis.

Three patients are reported on who presented with communicating hydrocephalus due to presumed tuberculous meningitis. Subsequent clinical deterioration despite antituberculous chemotherapy prompted reassessment with FDG-PET scanning and meningeal biopsy in one case and repeat CSF cytology with special staining in the second. The third patient died and postmortem confirmed a diagnosis of primary diffuse leptomeningeal gliomatosis. In the first two patients, MRI of the entire neuraxis showed no evidence of a primary intraparenchymal tumour. These cases emphasise the need for repeated reassessment in patients with culture negative lymphocytic meningitis. In addition, this is the first report of FDG-PET scanning in leptomeningeal gliomatosis.

Skull base chordomas: correlation of tumour doubling time with age, mitosis and Ki67 proliferation index.

The aim of the study was to assess the relationship between the rate of clinical tumour growth and various histological features, including Ki67 labelling index, in skull base chordoma. Cases of skull base chordoma from 19 patients (six female, 13 male; age range 8-63 years) were reviewed and the diagnosis confirmed based on histological and immunohistochemical features. In each biopsy cellularity, pleomorphism, mitotic activity, apoptotic bodies, necrosis and inflammatory cell infiltrate were graded and Ki67 labelling index (LI) calculated as a measure of proliferation. Tumour doubling time was assessed by quantitative analysis of tumour volumes in post-operative magnetic resonance images and correlated with age, sex, histological parameters and Ki67 LI. It was shown that increasing patient age, the presence of mitotic figures or a Ki67 LI in excess of 6% were associated with faster growing tumours.

Morphologic study of 120 skull base defects in frontoethmoidal encephalomeningoceles.

Frontoethmoidal encephalomeningocele is a herniation of brain and meninges through a congenital bone defect in the skull at the junction of the frontal and ethmoidal bones. From 1992 to 1996, 120 cases of frontoethmoidal encephalomeningocele were seen in our institutes, and the morphology of the skull defects was studied. The patients underwent thorough physical examinations and radiographic investigations including spiral three-dimensional computed tomography scan. Together with intraoperative findings, we found more types of the defects than previously reported. Our findings were categorized into the following types: type I, a single external opening between frontal, nasal, ethmoidal, and orbital bones; type IA, opening is limited between two bones of the area; type IB, opening is extended transversely or cephalad to involve adjacent structures; type II, multiple external openings in the region; type IIA, all of the openings are limited types; type IIB, one or more of the openings is/are extended type(s) that involve adjacent structures. There are 14 subtypes in these two types: 3 in type IA, 6 in type IB, 3 in type IIA, and 2 in type IIB. This classification is helpful in understanding the herniation pathway and in keeping informative records.

Non-isotopic molecular cytogenetics in neuro-oncology.

The molecular genetic analysis of brain tumours has been the focus of considerable interest for a number of years. However, these studies have been largely directed towards understanding the fundamental biological processes involved in tumorigenesis and the techniques which have been used require considerable molecular biological skills. Unfortunately, there has not been the impetus to correlate basic biological studies with clinical or neuropathological features. The development of non-isotopic molecular cytogenetic in situ hybridization (ISH) techniques which can be applied to archival tumour material provides an opportunity to address a wide range of neuropathological questions at a genetic level. Identification of specific chromosomes has been made possible by the isolation of probes which recognize the highly repeated sequences present in the centromeric regions of individual chromosomes. Libraries of human chromosome-specific painting probes are also available. A range of probes which bind to the whole or part of specific single copy genes are becoming available. These can be detected with either fluorochromes with different emission colours or with enzymatic detection systems in either interphase nuclei derived from fresh, fixed and embedded tumour samples, touch preparations or smears (so-called 'interphase cytogenetics') as well as conventional metaphase spreads. Comparative genomic hybridization can be used to scan the entire genome for deletions or amplifications without any pre-existing information about the likely locations of these abnormalities or the availability of any specific DNA probes. These techniques can be used to identify aneuploidy or structural alterations in individual chromosomes and are likely to yield important information about the location of genes important in the pathogenesis of brain tumours and may also provide the basis for the refinement of diagnostic or prognostic criteria of these neoplasms.