What matters for people with brain cancer? Selecting clinical quality indicators for an Australian Brain Cancer Registry.

BACKGROUND: The goal of a clinical quality registry is to deliver immediate gains in survival and quality of life by delivering timely feedback to practitioners, thereby ensuring every patient receives the best existing treatment. We are developing an Australian Brain Cancer Registry (ABCR) to identify, describe, and measure the impact of the variation and gaps in brain cancer care from the time of diagnosis to the end of life. METHODS: To determine a set of clinical quality indicators (CQIs) for the ABCR, a database and internet search were used to identify relevant guidelines, which were then assessed for quality using the AGREE II Global Rating Scale. Potential indicators were extracted from 21 clinical guidelines, ranked using a modified Delphi process completed in 2 rounds by a panel of experts and other stakeholders, and refined by a multidisciplinary Working Group. RESULTS: Nineteen key quality reporting domains were chosen, specified by 57 CQIs detailing the specific inclusion and outcome characteristics to be reported. CONCLUSION: The selected CQIs will form the basis for the ABCR, provide a framework for achievable data collection, and specify best practices for patients and health care providers, with a view to improving care for brain cancer patients. To our knowledge, the systematic and comprehensive approach we have taken is a world first in selecting the reporting specifications for a brain cancer clinical registry.

Diagnosis and localisation of insulinoma: the value of modern magnetic resonance imaging in conjunction with calcium stimulation catheterisation.

CONTEXT: Preoperative localisation of insulinoma improves cure rate and reduces complications, but may be challenging. OBJECTIVE: To review diagnostic features and localisation accuracy for insulinomas. DESIGN: Cross-sectional, retrospective analysis. SETTING: A single tertiary referral centre. PATIENTS: Patients with insulinoma in the years 1990-2009, including sporadic tumours and those in patients with multiple endocrine neoplasia syndromes. INTERVENTIONS: Patients were identified from a database, and case notes and investigation results were reviewed. Tumour localisation by computed tomography (CT), magnetic resonance imaging (MRI), octreotide scanning, endoscopic ultrasound (EUS) and calcium stimulation was evaluated. MAIN OUTCOME MEASURE(S): Insulinoma localisation was compared to histologically confirmed location following surgical excision. RESULTS: Thirty-seven instances of biochemically and/or histologically proven insulinoma were identified in 36 patients, of which seven were managed medically. Of the 30 treated surgically, 25 had CT (83.3%) and 28 had MRI (90.3%), with successful localisation in 16 (64%) by CT and 21 (75%) by MRI respectively. Considered together, such imaging correctly localised 80% of lesions. Radiolabelled octreotide scanning was positive in 10 out of 20 cases (50%); EUS correctly identified 17 lesions in 26 patients (65.4%). Twenty-seven patients had calcium stimulation testing, of which 6 (22%) did not localise, 17 (63%) were correctly localised, and 4 (15%) gave discordant or confusing results. CONCLUSIONS: Preoperative localisation of insulinomas remains challenging. A pragmatic combination of CT and especially MRI predicts tumour localisation with high accuracy. Radionuclide imaging and EUS were less helpful but may be valuable in selected cases. Calcium stimulation currently remains useful in providing an additional functional perspective.

Clinical features, management and recurrence of symptomatic Rathke's cleft cyst.

Rathke's cleft cyst (RCC) is a rare lesion of the pituitary gland that is often asymptomatic. The objective of this study was to further characterise the presenting symptoms, endocrinopathy and potential for recurrence of this lesion in an Australian population. The files of 12 patients with pathologically confirmed RCCs were reviewed retrospectively. Common presentations included headache, endocrinopathy, and visual disturbance. Uncommon presentations included 1 patient with pituitary apoplexy. The cysts varied in size from 6 mm to 20 mm; 8 had a suprasellar component. 1 patient had evidence of a RCC with xanthogranulomatous change. RCC recurrence was noted on follow-up MRI in 5 patients. In conclusion, RCCs are rare lesions that can present with an array of clinical symptoms. The relatively high rate of recurrence may indicate a link between this pathology and craniopharyngioma, although many patients with recurrent RCC do not require repeat resection and remain asymptomatic.

Anterior clinoid mucocele causing acute monocular blindness.

We report a patient who presented with acute painful monocular visual loss reminiscent of idiopathic optic neuritis. After treatment with corticosteroids was commenced, an anterior clinoid mucocele causing compressive optic neuropathy was demonstrated on imaging studies. The diagnosis was proven histopathologically following excision of the lesion via craniotomy. Although very rare as a cause of optic neuropathy, anterior clinoid mucocele should be excluded by adequately imaging the optic canal, as visual prognosis is poor if surgical decompression is not performed in a timely fashion.

The diagnosis and differential diagnosis of endogenous Cushing's syndrome.

Cushing's syndrome (CS) is a physically and psychologically disabling disease associated with high morbidity resulting from inappropriate elevation of circulating free cortisol levels. The main features of CS are disturbance of the normal circadian rhythm of cortisol secretion, impairment of the normal feedback of the hypothalamo-pituitary-adrenal (HPA)-axis, and excessive integrated 24 hours cortisol secretion. All biochemical tests used for the diagnosis of CS rely upon the ascertainment of a disturbance of these features. However, the diagnosis of CS (endogenous hypercortisolism) still remains a challenge, although the evolution of several diagnostic tests has allowed diagnosis at an earlier stage. In the initial investigation of CS, tests of high sensitivity are required to identify patients at risk, which are followed by tests of high specificity to confirm the diagnosis and establish the precise aetiology. This review will discuss the various causes of endogenous CS and focus on established and evolving diagnostic procedures used for its diagnosis, as several studies with large number of patients have recently appeared in the literature validating current practice and proposing improved diagnostic algorithms.

Arthroscopic subacromial decompression performed under local anesthesia.

Shoulder arthroscopy is usually performed under general anesthesia or interscalene block. General anesthesia may be contraindicated and interscalene block sometimes fails. We had 8 patients who were at high-risk and, therefore, shoulder arthroscopy and decompression were performed under local anesthesia in the beach-chair position. We used 50 mL of 1% lidocaine; 30 mL were infiltrated into the skin and underlying tissues and into the glenohumeral joint, and the rest was infiltrated into the subacromial joint. Before the infiltration, all patients were given 5 mg midazolam and 0.1 mg fentanyl intravenously. All 8 patients were satisfied with the analgesia provided by the anesthesia. We conclude that arthroscopy and subacromial decompression can be readily performed under local anesthesia in combination with efficient sedation.

[The treatment of meniscal tear with bio-absorbable arrow--3 years follow-up].

BACKGROUND: The introduction of bio-absorbable materials has improved the ability to offer a simple fast and friendly solution to meniscal tears. The technique of meniscal suture with bio-absorbable arrows is associated with lower morbidity and complication rates. AIM: The clinical evaluation of treatment of meniscal tear by suture with bio-absorbable arrows. METHODS: During the period 1997-2004, 14 patients underwent arthroscopic all-inside meniscus repair. The "Biofix" bio-absorbable arrows fixation technique was used. Fourteen patients were followed--11 males and 3 females. Their mean age was 23 years (range 16-34 years). Follow-up period was an average of 34 months (range 6-72 months). Sport injuries were the main cause of meniscal tear (60%). Other causes included: work accidents (20%) and military accidents (20%). All the tears were located in an area suitable for suture in the "red-red" or "red-white" zone. RESULTS: One patient had a tear of the lateral meniscus. Four patients had anterior cruciate ligament (ACL) tear. The mean length of the tear was 9.6 mm. Tears were fixed with 2.5 arrows in average. The clinical evaluation was by the Lysholm Score. Excellent results were reported in 10 patients, good in one patient, fair in 2 patients and poor in 1 patient. One patient suffered from a transient tender point on the medial joint line due to protruded arrow tip, one patient needed a menisectomy due to unresolved symptoms and one patient underwent ACL reconstruction. CONCLUSION: Meniscal suture with bio-absorbable arrows is an easy and reliable suture technique compared to the classical methods and it seems to have a lower complication rate. Further studies are needed to establish the quality of the repair and the associated morbidity.

A rapid method for analyzing serum pro-insulin-like growth factor-II in patients with non-islet cell tumor hypoglycemia.

CONTEXT: Non-islet cell tumor hypoglycemia (NICTH) results from the hypersecretion of pro-IGF-II by a large, usually mesenchymal tumor. Detection of pro-IGF-II in serum is a potential tumor marker in these patients. OBJECTIVE: The aim of this study was to validate a rapid and reliable method for determining serum pro-IGF-II. PATIENTS: Serum samples from 16 patients with NICTH were studied. MAIN OUTCOME MEASURE: The main outcome measure was serum concentration of pro-IGF-II determined by immunoblot analysis of pro-IGF-II and mature IGF-II after 16.5% tricine-SDS-PAGE, which was compared with pro-IGF-II measured by standard RIA after size-exclusion acid chromatography. RESULTS: The analyses of patients' sera by size-exclusion acid chromatography showed that 68 +/- 19% of IGF-II were present in the pro-IGF-II form, whereas only 18 +/- 4% corresponded to pro-IGF-II in controls. Scanning densitometry of immunoblots showed 67 +/- 16% in the bands corresponding to pro-IGF-II in patients' sera, compared with 27 +/- 9% in controls. The detection sensitivity of tricine-SDS-PAGE method was the same as for size-exclusion chromatography, but the tricine-SDS-PAGE method is quicker and requires smaller amounts of serum. CONCLUSION: Tricine-SDS-PAGE followed by IGF-II immunoblot analysis provides a rapid, reproducible, and sensitive method for the separation of serum pro-IGF-II from mature IGF-II and is a useful laboratory evaluation of patients with a clinical diagnosis of NICTH.

Cerebellar liponeurocytoma.

A case of cerebellar liponeurocytoma in a 34-year-old man is reported. There are only 19 other cases reporting this entity in the medical literature. The diagnostic, radiological and clinical features associated with this tumour are reviewed and discussed in relation to our case. The differences in behaviour and prognosis between medulloblastoma and cerebellar liponeurocytoma are presented with the corresponding implications for management.

Outcome scoring in neurosurgery: the cognitive outcome score, an alternative to the Glasgow outcome scale.

The qualitative method, the Glasgow outcome scale, remains the convention for patient outcome assessment in the neurosurgical literature. This is despite the fact that sampling methods subject to bias confer low confidence in the conclusions. An alternative, quantitative method, the cognitive outcome score (COS), decreases bias, is cost effective and delivered in less than 10 min. Utilisation of the COS in statistical correlations against common clinical parameters is discussed in theory and practice.

Extradural hematoma causing venous sinus obstruction and pseudotumor cerebri syndrome.

CASE REPORT: The case of a small right occipital extradural hematoma in a 10-year-old girl is presented. The patient had bifrontal occipital headache out of proportion to the size of the hematoma, as well as diplopia. MR venography revealed compression and occlusion of the dominant right transverse sinus resulting in a pseudotumor cerebri syndrome. DISCUSSION: The relationship between venous sinus obstruction and the pseudotumor syndrome is discussed. Clinicians should be aware of the possible effects of an extra-axial collection on the cerebral venous sinuses.

Metabolites from cerebrospinal fluid in aneurysmal subarachnoid haemorrhage correlate with vasospasm and clinical outcome: a pattern-recognition 1H NMR study.

Following subarachnoid haemorrhage the most significant complication is sustained cerebral vascular contraction (vasospasm), which may result in terminal brain damage from cerebral infarction. Despite this, the biochemical cause of vasospasm remains poorly understood. In this study, the global high-concentration metabolite composition of CSF has been correlated with patient outcome after subarachnoid haemorrhage using multivariate statistics and 1H NMR spectroscopy. In total, 16 patients with aneurysmal subarachnoid haemorrhage (aSAH) were compared with 16 control patients who required a procedure where CSF was obtained but did not have aSAH. Multivariate statistics readily distinguished the aSAH group from the heterogeneous control group, even when only those controls with blood contamination in the CSF were used. Using principal components analysis and orthogonal signal correction, vasospasm was correlated to the concentrations of lactate, glucose and glutamine. These pattern recognition models of the NMR data also predicted Glasgow Coma Score (54% within +/- 1 of the actual score on a scale of 1-15 for the whole patient group), Hunt and Hess SAH severity score (88% within +/- 1 of the actual score on a scale of 1-5 for the aSAH group) and cognitive outcome scores (78% within +/- 3 of the actual score on a 100% scale for the whole patient group). Thus, the approach allowed the prediction of outcome as well as confirming the presence of aSAH.

Traumatic unilateral deep cerebral venous infarction.

A case of unilateral deep cerebral venous infarction in a young man associated with mild head injury is presented. The diagnosis is made on the basis of the clinical, radiological, operative and histopathological findings. We propose that a thrombosis or obstruction of the anterior segment of the basal vein of Rosenthal (BVR) was the primary pathology. This is discussed in respect to current knowledge of deep cerebral venous anatomy and embryology.

Pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement.

OBJECT: Pseudotumor cerebri, or benign intracranial hypertension, is a condition of raised intracranial pressure in the absence of a mass lesion or cerebral edema. It is characterized by headache and visual deterioration that may culminate in blindness. Pseudotumor cerebri is caused by venous sinus obstruction in an unknown percentage of cases. The purpose of this study was to investigate the role of cerebral venous sinus disease in pseudotumor cerebri and the potential of endoluminal venous sinus stent placement as a new treatment. METHODS: Nine consecutive patients in whom diagnoses of pseudotumor cerebri had been made underwent examination with direct retrograde cerebral venography (DRCV) and manometry to characterize the morphological features and venous pressures in their cerebral venous sinuses. The cerebrospinal fluid (CSF) pressure was measured simultaneously in two patients. If patients had an amenable lesion they were treated using an endoluminal venous sinus stent. Five patients demonstrated morphological obstruction of the venous transverse sinuses (TSs). All lesions were associated with a distinct pressure gradient and raised proximal venous sinus pressures. Four patients underwent stent insertion in the venous sinuses and reported that their headaches improved immediately after the procedure and remained so at 6 months. Vision was improved in three patients, whereas it remained poor in one despite normalized CSF pressures. CONCLUSIONS: Patients with pseudotumor cerebri should be evaluated with DRCV and manometry because venous TS obstruction is probably more common than is currently appreciated. In patients with a lesion of the venous sinuses, treatment with an endoluminal venous sinus stent is a viable alternative for amenable lesions.

Neuroimaging findings in a suprasellar granular cell tumor.

A 27-year-old woman presented with a history of amenorrhea, visual disturbance, and diabetes insipidus. Magnetic resonance imaging showed a large enhancing suprasellar mass with associated edema involving the left striatum. The lesion was hypometabolic on fluorodeoxyglucose-positron emission tomography. At surgery, a subtotal resection of a vascular tumor that appeared to arise from the posterior pituitary and hypothalamus was carried out. Pathologic examination revealed a granular cell tumor. We report the preoperative neuroimaging findings in this rare posterior pituitary stalk tumor.

Surgical management of cerebral metastases from melanoma: outcome in 147 patients treated at a single institution over two decades.

OBJECT: The aim of this study was to review the outcome of patients who underwent surgery for treatment of cerebral metastatic melanoma. METHODS: A retrospective analysis was performed in 147 patients with cerebral metastases from melanoma who were treated surgically at a single institution between 1979 and 1999. Almost all patients underwent postoperative wholebrain radiation therapy. The mean patient age was 53 years (range 17-76 years); 69% of patients were male. A single cerebral metastasis was identified in 84% of patients, although 56% had synchronous extracranial metastases. The 30-day postoperative mortality rate was 2% and neurological symptoms resolved or improved in 78% of patients. Recurrence of intracerebral disease was seen in 55% of patients and 26% died of intracerebral metastases. Twenty-four patients underwent reoperation for recurrent cerebral disease. The median survival duration from the time of surgery for all patients was 8.5 months; the 3- and 5-year survival rates were 9% and 5%, respectively. Factors that significantly influenced survival on univariate analysis were the number of cerebral metastases (p = 0.015), a macroscopically complete excision (p < 0.05), and reoperation for recurrence (p = 0.02). The presence of extracranial metastases did not significantly influence survival. On multivariate analysis only the number of cerebral metastases significantly affected survival (p = 0.04). CONCLUSIONS: For the majority of patients with cerebral metastases from melanoma, surgery with adjuvant radiation therapy is a treatment option that improves neurological symptoms and produces minimal morbidity. Long-term survival (> 3 years) most likely occurs in patients with a single cerebral metastasis and no demonstrable extracranial disease. Reoperation for recurrent cerebral disease may be appropriate in selected cases.