Hindbrain decompression for Chiari-syringomyelia complex: an outcome analysis comparing surgical techniques.

The natural history of Chiari malformation and our ability to alter it is poorly understood, and reported results of hindbrain decompression show high recurrence rates. We report 11 years of experience of hindbrain decompression, to evaluate long-term outcome related to surgical technique. The results of patients who underwent hindbrain decompression between 1994 and 2005 were retrospectively analysed. We identified 96 patients from operative records and reviewed all clinical records for presenting symptomatology and examination findings. Decompression technique includes traditional decompression, duraplasty and bone--only decompression. Symptoms at the last available follow-up visit were defined by the assessing clinician as resolved, improved, unchanged or worse. There were 35 males and 61 females with a mean age of 33 years (range 6 - 62 years). The mean length of follow-up is 3.6 years with a range of 6 months to 9 years. Postoperative resolution or improvement in symptoms was seen in 75 patients (78%). Drop attacks and headaches were the most likely to respond to hindbrain decompression, showing improvement or resolution in 100 and 92% of cases. Dysaesthetic arm pain and weakness carried the worse prognosis with only 20% having symptom resolution. Sixteen patients had only bony decompression leaving the dura intact. In eight patients (66%) the headaches resolved following bony decompression alone. However the headaches were unchanged in 25% of cases. Dysaesthetic pain and weakness was unchanged in 60%. Restoration of CSF flow dynamics at the foramen magnum by surgical decompression does not consistently result in resolution of symptoms in all patients. Identification of predictors of successful outcome following decompression, coupled with early intervention and appropriate choice of procedure may result in improved outcomes. Although this is a retrospective study it suggests that bone only decompression should be reserved for patients with isolated headache.

Outcome predictors and complications in the management of intradural spinal tumours.

The results of the management of 115 patients with intradural spinal tumours are presented. Data was collected retrospectively from the case notes. Tumours were categorized as intramedullary or extramedullary for statistical analysis. Meningioma, schwannoma and ependymoma accounted for 70% of tumours. Complete macroscopic excision was achieved in 84% of extramedullary and 54% of intramedullary tumours. There were two post-operative deaths, one of which was secondary to methacillin-resistant staphylococcus aureus (MRSA) meningitis. Cerebrospinal fluid leak (10%) and meningitis (7%) were the commonest complications. Ninety-six percent of patients with extramedullary tumours improved or remained unchanged on the Frankel scale. In the intramedullary group, 82% remained unchanged or improved after treatment. Pre-operative functional status was a predictor of good post-operative function for intra- and extramedullary tumours and for intramedullary tumours a good post-operative Frankel score predicted long-term survival.

En bloc removal of the lower lumbar vertebral body for chordoma. Report of two cases.

En bloc removal of the lower lumbar vertebral bodies (VBs) is a major surgical challenge. The authors describe the surgical technique used in two patients who presented with chordoma confined to the L-5 and L-4 VB, respectively. These tumors were diagnosed using magnetic resonance (MR) imaging during investigation for back pain. Both patients underwent a combined (two-stage) anterior-posterior approach. In the first case the posterior stage of the procedure was followed by an anterior retroperitoneal approach, and in the second case a lateral retroperitoneal approach was used. Complete en bloc excision of the tumor was achieved in each case, even though in the second case the VB fractured when it was mobilized. The correlation between the MR imaging findings and surgical specimens was remarkable. The authors conclude that en bloc resection is feasible in these cases. Because mobilization of the VB is more difficult in the lateral approach, the authors favor the anterior retroperitoneal approach. The authors anticipate the need for such procedures to increase with the widespread use of MR imaging, which demonstrates the extent of these tumors with remarkable accuracy.

Prehospital deaths in the Yorkshire Health Region.

OBJECTIVES: To estimate the proportion of prehospital deaths in a British population of trauma victims which may be preventable, and to investigate the effect of death at the scene and death in transit on potential survivorship. METHODS: Blinded review, by four specialists with an interest in trauma, of necropsy results and details of age, sex, and mechanism of injury for prehospital trauma deaths in the Yorkshire Health Region in a 12 month period. RESULTS: Complete records were traced on 305 of 337 trauma deaths, 190 being recorded as dead on arrival of emergency services and 115 dead on arrival at hospital. In the group declared dead at the scene, three of the four assessors considered 93% of deaths to have been inevitable and only 2% as potential survivors (25% of this group sustaining inevitably fatal injuries such as brain avulsion or decapitation). In the group dead on arrival 81% were felt to be inevitable deaths and 5% potential survivors. CONCLUSIONS: There seems to be less scope for salvage of victims of trauma death in a British population than has been recorded in America, possibly due to a higher proportion of blunt trauma deaths here. Those who die in transit consist of a less severely injured group with a higher potential for survival.

Undiagnosed epidermoid cyst presenting as trigeminal neuralgia: a need for MRI.

A case of an epidermoid cyst in the cerebello-pontine angle presenting with facial pain is reported. The tumour was discovered during neurovascular decompression of the fifth nerve. A pre-operative CT scan was normal. The role of neuroradiological investigation in young patients with trigeminal neuralgia is discussed.

Expression of epidermal growth factor receptor in human glioblastoma multiforme.

The expression of epidermal growth factor receptor was determined in a series of 88 patients with glioblastoma multiforme using a monoclonal antibody to the receptor. Positive staining was seen in 17 patients but this did not have prognostic significance when compared with known clinical prognostic factors using a computer model. The significance of receptor expression in these tumours is discussed.

Statistical modelling in analysis of prognosis in glioblastoma multiforme: a study of clinical variables and Ki-67 index.

Statistical modelling was used to analyse clinical features and the Ki-67 proliferation index in a study of 77 cases of glioblastoma multiforme. Relative youth, frontal tumour site and treatment with external beam radiation had an important positive influence on survival. The pre-operative Karnovsky score and the presence of necrosis were not related to outcome. Despite the attractive hypothesis that rapidly proliferating tumours might be associated with a worse prognosis compared with slowly proliferating lesions, the Ki-67 index in this study offered no prognostic information even when individual sites were considered separately. The use of this form of computer modelling and its role in analysis of prognostic data is discussed.

Idiopathic common peroneal nerve palsy--a review of thirteen cases.

Thirteen cases of idiopathic common peroneal nerve palsy were reviewed and the clinical presentation and course evaluated. Incomplete nerve palsy of unknown aetiology has a good prognosis and all the patients in this group made a full recovery without surgical intervention. Those presenting with a clinically and electrophysiologically complete lesion made no recovery, despite surgical intervention in two cases. It is suggested that these lesions can safely be treated conservatively as long as there is no identifiable cause.

Paraganglioma of the cauda equina. Report of three cases.

The authors report the clinical, radiological, and pathological findings in three cases of paraganglioma of the cauda equina. In one case, magnetic resonance imaging and neurochemical study results are described. No specific identifying features were encountered either clinically or radiologically that were helpful in making a distinction between this and other more common tumors at this site such as ependymoma or neurofibroma. At surgery, these neoplasms were well-circumscribed red fleshy tumors. Histological examination of one paraganglioma showed a superficial resemblance to ependymoma, and this may be particularly true on initial assessment by frozen section or smear. The use of electron microscopy and immunohistochemical demonstration of synaptophysin in these tumors allowed a confident diagnosis to be made. Neurochemical assessment in one case showed very high levels of serotonin and a turnover of dopamine similar to that of human cerebral cortex. Paraganglioma of the cauda equina is an uncommon tumor with just over 50 cases reported in the world literature. The clinical course of these tumors is benign and they should be completely removed at surgery to prevent later recurrence.

The clinical, radiological and histopathological features of cerebral primitive neuroectodermal tumours.

The clinical, radiological and pathological features of eight cases of supratentorial primitive neuroectodermal tumour are reviewed. These are tumours of children and young adults presenting with symptoms and signs of raised intracranial pressure. Radiologically they are characterized by a large enhancing mass lesion exciting little or modest surrounding oedema, with a propensity to develop in the frontal lobes. One tumour exhibited the pathological features of a primitive neuroectodermal tumour (PNET) with ependymal differentiation (ependymoblastoma). The rest showed no light microscopy patterns to indicate differentiation. Immunohistochemistry was helpful as it excluded other causes of a 'small blue cell' tumour but did not help in assessing differentiation. Ultrastructural examination of this group of apparently undifferentiated tumours showed focal markers of neuronal differentiation. Although features of neuronal differentiation can be found ultrastructurally in these tumours this is only evident after prolonged searching, often of several blocks, making assessment very prone to sampling errors. The term PNET thus remains appropriate and serves to group such tumours together to facilitate rational clinical management.

Carotico-cavernous fistula after trans-sphenoidal hypophysectomy.

A case is presented of a 48-year-old woman with an iatrogenic carotico-cavernous fistula consequent upon a transphenoidal hypophysectomy. Successful closure of the fistula was achieved by interventional radiology. The causes and management of vascular injuries during trans-sphenoidal pituitary surgery are reviewed and discussed.

Systemic metastasis from primary intracranial germinoma: a case report and literature review.

A case of systemic haematogenous metastasis occurring post-operatively from a primary suprasellar intracranial germinoma is reported. While local invasion and direct spread associated with surgical incision and shunt procedures are well known, haematogenous metastasis of these tumours is extremely rare. Problems associated with establishing this diagnosis are discussed.

Financial burden of childhood cancer.

Fifty-nine of 73 families of children referred for treatment of cancer during 1980 co-operated in a study of the financial consequences of the illness. Except for two social class I families who declined to take part, the sample was representative of the childhood cancer population and families were of similar socioeconomic status to the general population. During the first, inpatient, week week of treatment the sum of income lost plus additional expenditure exceeded 50% of total income in over 45% of families. During a subsequent week of outpatient treatment, loss of income plus additional expenditure amounted to more than 20% of income in over half the families. These problems affected all the groups studied and were not confined to lower paid or those living furthest from the centre. Financial help was available from charitable sources and the DHSS towards travel, extra nourishment, and heating costs but could not be obtained to compensate for loss of earnings. The families of children who died had difficulty in meeting the cost of funerals. Families of children with cancer need more help than is at present available, especially to offset loss of income and the cost of funerals.