Documenting the process of developing the Victorian voluntary assisted dying legislation.

Many countries across the world have legislated for their constituents to have control over their death. Commonalities and differences can be found in the regulations surrounding the shape and practices of voluntary assisted dying (VAD) and euthanasia, including an individual's eligibility and access, role of health professions and the reporting. In Australia there have been perennial debates across the country to attempt legislative change in assisting a terminally ill person to control the ending of their life. In 2017, Victoria became the first state to successfully legislate for VAD. In describing the Victorian process that led to the passage of legislation for VAD, this paper examines the social change process. The particular focus of the paper is on the vital role played by a multidisciplinary ministerial advisory panel to develop recommendations for the successful legislation, and is written from their perspective.

Mobile pediatric neurosurgery: rapid response neurosurgery for remote or urgent pediatric patients.

OBJECT: Time-critical neurosurgical conditions require urgent operative treatment to prevent death or neurological deficits. In New South Wales/Australian Capital Territory patients' distance from neurosurgical care is often great, presenting a challenge in achieving timely care for patients with acute neurosurgical conditions. METHODS: A protocol was developed to facilitate consultant neurosurgery locally. Children with acute, time-critical neurosurgical emergencies underwent operations in hospitals that do not normally offer neurosurgery. The authors describe the developed protocol, the outcome of its use, and the lessons learned in the 9 initial cases where the protocol has been used. Three cases are discussed in detail. RESULTS: Nine children were treated by a neurosurgeon at 5 rural hospitals, and 2 children were treated at a smaller metropolitan hospital. Road ambulance, fixed wing aircraft, and medical helicopters were used to transport the Newborn and Paediatric Emergency Transport Service (NETS) team, neurosurgeon, and patients. In each case, the time to definitive neurosurgical intervention was significantly reduced. The median interval from triage at the initial hospital to surgical start time was 3:55 hours, (interquartile range [IQR] 03:29-05:20 hours). The median distance traveled to reach a patient was 232 km (range 23-637 km). The median interval from the initial NETS call requesting patient retrieval to surgical start time was 3:15 hours (IQR 00:47-03:37 hours). The estimated median "time saved" was approximately 3:00 hours (IQR 1:44-3:15 hours) compared with the travel time to retrieve the child to the tertiary center: 8:31 hours (IQR 6:56-10:08 hours). CONCLUSIONS: Remote urgent neurosurgical interventions can be performed safely and effectively. This practice is relevant to countries where distance limits urgent access for patients to tertiary pediatric care. This practice is lifesaving for some children with head injuries and other acute neurosurgical conditions.

Influence of comorbidities in idiopathic normal pressure hydrocephalus - research and clinical care. A report of the ISHCSF task force on comorbidities in INPH.

Idiopathic normal pressure hydrocephalus (INPH) is a syndrome of ventriculomegaly, gait impairment, cognitive decline and incontinence that occurs in an elderly population prone to many types of comorbidities. Identification of the comorbidities is thus an important part of the clinical management of INPH patients. In 2011, a task force was appointed by the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders (ISHCSF) with the objective to compile an evidence-based expert analysis of what we know and what we need to know regarding comorbidities in INPH. This article is the final report of the task force. The expert panel conducted a comprehensive review of the literature. After weighing the evidence, the various proposals were discussed and the final document was approved by all the task force members and represents a consensus of expert opinions. Recommendations regarding the following topics are given: I. Musculoskeletal conditions; II. Urinary problems; III. Vascular disease including risk factors, Binswanger disease, and white matter hyperintensities; IV. Mild cognitive impairment and Alzheimer disease including biopsies; V. Other dementias (frontotemporal dementia, Lewy body, Parkinson); VI. Psychiatric and behavioral disorders; VII. Brain imaging; VIII. How to investigate and quantify. The task force concluded that comorbidity can be an important predictor of prognosis and post-operative outcome in INPH. Reported differences in outcomes among various INPH cohorts may be partly explained by variation in the rate and types of comorbidities at different hydrocephalus centers. Identification of comorbidities should thus be a central part of the clinical management of INPH where a detailed history, physical examination, and targeted investigations are the basis for diagnosis and grading. Future INPH research should focus on the contribution of comorbidity to overall morbidity, mortality and long-term outcomes.

Treating pediatric hydrocephalus in Australia: a 3-year hospital-based cost analysis and comparison with other studies.

OBJECT: The aim of this study was to quantify the financial costs of surgical intervention in patients with newly diagnosed hydrocephalus and patients with treatment failure or complications of previously treated hydrocephalus between 2007 and 2009 at the Children's Hospital at Westmead in Sydney, Australia. METHODS: This was a retrospective study of patients who underwent shunt insertion, shunt revision, treatment of an infected shunt, and endoscopic third ventriculostomy (ETV) between 2007 and 2009. Actual hospital costs associated with each inpatient stay were obtained from the accounting office of Children's Hospital at Westmead. Patients with hydrocephalus secondary to trauma, malignancy, or other complex conditions (except myelomeningocele) were excluded. RESULTS: Hydrocephalus-related procedures comprised approximately one-third of neurosurgical procedures performed each year. From 2007 to 2009, there were 192 admissions during which 300 procedures were performed for 162 patients. The total cost was $4.78 million (Australian) with an average cost of $1.59 million per year. The cost per admission for shunt insertion and ETV were similar ($13,905 vs $14,128, respectively). The average cost per admission for shunt revision was $9,753. However, shunt infection was associated with 40% of total costs, averaging $83,649 per admission. Management of patients with myelomeningocele undergoing insertion of shunt procedures in the same admission accounted for an average cost of $50,186. CONCLUSIONS: Hydrocephalus is a chronic condition that imposes a significant and growing economic burden upon the Australian hospital system. Seventy-five percent of hydrocephalus-related hospital expenditure is used to surgically treat patients for complications or failure of previously treated hydrocephalus. Further research into the economic impact of pediatric hydrocephalus on the Australian health care system and concerted research efforts in the area of effective long-term surgical treatment and complication minimization are essential.

Paediatric germ cell tumours of the central nervous system: Results and experience from a tertiary-referral paediatric institution in Australia.

A retrospective analysis was conducted on consecutive patients with intracranial germ cell tumours diagnosed and treated from 1 January 1997 to 31 December 2007 to assess and determine demographic factors and treatment outcomes of children with these tumours treated in a major paediatric referral hospital in Australia. In this study, intracranial germ cell tumours represented 4.8% of paediatric brain tumours seen. Of the 21 patients identified, 15 (71.4%) were diagnosed with pure germinoma and six (28.6%) with non-germinomatous germ cell tumours (NGGCT) or mixed tumours. One patient received chemotherapy alone, two patients were treated with radiation alone and the remaining 18 received a combination of chemotherapy and radiotherapy. A total of 33 neurosurgical operations were performed with 15 biopsies via open, endoscopic or transphenoidal means; nine open resections; and nine procedures for hydrocephalus comprising seven third ventriculostomies and two ventriculoperitoneal shunts. For patients with pure germinomas, the 5-year disease-free rate (DFS) was 93.3%, and overall survival (OS) rate was 100% compared to NGGCT or mixed tumours (DFS 50%; OS 50%) (DFS p=0.019, OS p=0.004). The data presented show that pure germinomas carry a favourable prognosis. The data also support that treatment with induction chemotherapy followed by dose-attenuated radiotherapy is an effective alternative with results comparable to historical controls treated with craniospinal irradiation. Although chemoradiotherapy has become the mainstay of treatment in intracranial germ cell tumours, surgery remains integral to the management of this condition. Surgery remains important in establishing the histological diagnosis, as well as in the treatment of hydrocephalus. Furthermore, debulking procedures may be advocated in NGGCT as they are often resistant to chemotherapy.

The role of venous sinus outflow obstruction in pediatric idiopathic intracranial hypertension.

OBJECT: The authors examined the role of venous sinus obstruction in the etiology of idiopathic intracranial hypertension (IIH) by reviewing more than 200 MR venograms performed in suspected cases of IIH. METHODS: Individual MR venograms performed in cases of suspected IIH at the Children's Hospital at Westmead in Sydney, Australia, were reviewed. The authors excluded cases in which an intervention was performed before the scan or a structural cause for venous obstruction was identified. Cases with confirmed hydrocephalus were also excluded. For each of the 145 remaining scans, the authors completed a detailed review on a slice-by-slice basis of the 2D source images used to compile the rendered 3D MR venogram. The anatomical configuration of the dural venous sinuses and any areas of decreased flow in circulation were then noted. Where possible, they correlated their radiological findings with evidence of raised intracranial pressure based on LP opening pressures. They also reviewed a control group of 50 MR venograms. RESULTS: Seventy-six (52%) of 145 scans showed evidence of venous obstruction in the dominant-side circulation. Substantial nonphysiological collateral circulation was seen in 68% of cases with dominant-sided obstruction, suggesting a process of recanalization. In contrast, in the absence of dominant-sided obstruction, collateral circulation was uncommon. In 27 cases, CSF opening pressure measurements were available. In 20 cases the opening pressures were in excess of 20 cm H(2)O. Of those, 17 demonstrated evidence of dominant-sided venous outflow obstruction. Among those cases, the median opening pressure was 34 cm H(2)O. Dominant-sided venous outflow obstruction was seen in only 2 of 50 MR venograms in the control group. Furthermore, evidence of collateral circulation was also uncommon in the control group. There was a highly statistically significant difference between rates of dominant-sided venous obstruction in the suspected IIH and control groups (p ≤ 0.001). CONCLUSIONS: A majority of patients presenting for investigation of suspected IIH demonstrated evidence of dominant-sided venous obstruction on MR venogram. In addition there was a high correlation between elevated CSF opening pressures and dominant-sided venous sinus obstruction. This correlation was further supported by evidence of collateral recanalization in patients with elevated CSF pressures and dominant-sided venous obstruction. A control group of 50 MR venograms indicated that dominant-sided venous outflow obstruction is an unlikely incidental finding, and a highly statistically significant difference was found between rates of obstruction in the suspected IIH and control groups.

Expression of AQP1 and AQP4 in paediatric brain tumours.

Aquaporins (AQP) are water-channel proteins with roles in tumour cell migration, angiogenesis, cerebral oedema and cell-cell adhesion. We aimed to determine the expression of AQP1 and AQP4 in paediatric brain tumours. Twenty tumour bank specimens were subject to Western blot analysis and quantitative polymerase chain reaction (PCR) to determine the expression of AQP1 and 4. Immunohistochemical staining was used to determine the distribution of AQP1 and 4 expression. Medulloblastomas, primitive neuroectodermal tumour, germinomas and higher grade gliomas did not express AQP1 and 4. Of the ependymomas, those in the posterior fossa all demonstrated markedly increased expression of AQP1 and 4. A supratentorial ependymoma demonstrated a moderate increase in AQP1 but not AQP4. Pilocytic astrocytomas demonstrated high levels of AQP1 and 4 but had a more variable pattern of staining. AQP1 and 4 have relevance to paediatric brain tumours and are worthy of further investigation in developing potential therapeutic strategies.

Metachronous multifocal desmoid-type fibromatoses along the neuraxis with adenomatous polyposis syndrome.

Desmoid-type fibromatosis, aggressive fibromatosis, or desmoid tumor is an uncommon benign but locally aggressive fibroblastic lesion. Although intraabdominal desmoid-type fibromatoses are well described in association with adenomatous polyposis syndrome, their occurrence along the neuraxis is extremely rare. The authors report the case of a 14-year-old boy with metachronous intracranial and spinal desmoid-type fibromatoses with preceding medulloblastoma. He was ultimately diagnosed with adenomatous polyposis syndrome. This is the first reported case of spinal desmoid-type fibromatosis in association with adenomatous polyposis syndrome. The identification of an underlying genetic instability allows for screening to detect lesions and institute measures to avoid preventable mortality from nonneurological tumors.

Aquaporins: relevance to cerebrospinal fluid physiology and therapeutic potential in hydrocephalus.

The discovery of a family of membrane water channel proteins called aquaporins, and the finding that aquaporin 1 was located in the choroid plexus, has prompted interest in the role of aquaporins in cerebrospinal fluid (CSF) production and consequently hydrocephalus. While the role of aquaporin 1 in choroidal CSF production has been demonstrated, the relevance of aquaporin 1 to the pathophysiology of hydrocephalus remains debated. This has been further hampered by the lack of a non-toxic specific pharmacological blocking agent for aquaporin 1. In recent times aquaporin 4, the most abundant aquaporin within the brain itself, which has also been shown to have a role in brain water physiology and relevance to brain oedema in trauma and tumours, has become an alternative focus of attention for hydrocephalus research. This review summarises current knowledge and concepts in relation to aquaporins, specifically aquaporin 1 and 4, and hydrocephalus. It also examines the relevance of aquaporins as potential therapeutic targets in hydrocephalus and other CSF circulation disorders.

Endoscopic transnasal decompression for management of basilar invagination in osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is a disorder of bone development caused by a genetic dysfunction of collagen synthesis. Basilar invagination (BI) is an uncommon but serious complication of OI. Brainstem decompression in OI is undertaken in certain circumstances. Transoral-transpalatopharyngeal ventral decompression with posterior occipitocervical fusion has become the treatment of choice when required. This technical note outlines a novel endoscopic transnasal approach for ventral decompression. The literature is reviewed and a strategy for the management of BI in patients with OI is outlined.

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.

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.

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.

Changes in cerebral blood flow during cerebrospinal fluid pressure manipulation in patients with normal pressure hydrocephalus: a methodological study.

The combination of cerebral blood flow measurement using (15)O-water positron emission tomography with magnetic resonance coregistration and CSF infusion studies was used to study the global and regional changes in CBF with changes in CSF pressure in 15 patients with normal pressure hydrocephalus. With increases in CSF pressure, there was a variable increase in arterial blood pressure between individuals and global CBF was reduced, including in the cerebellum. Regionally, mean CBF decreased in the thalamus and basal ganglia, as well as in white matter regions. These reductions in CBF were significantly correlated with changes in the CSF pressure and with proximity to the ventricles. A three-dimensional finite-element analysis was used to analyze the effects on ventricular size and the distribution of stress during infusion. To study regional cerebral autoregulation in patients with possible normal pressure hydrocephalus, a sensitive CBF technique is required that provides absolute, not relative normalized, values for regional CBF and an adequate change in cerebral perfusion pressure must be provoked.

Pattern of white matter regional cerebral blood flow and autoregulation in normal pressure hydrocephalus.

The mean cerebral blood flow (CBF) has generally been demonstrated to be lower in normal pressure hydrocephalus (NPH) than in normal controls. We investigated the distribution of the regional peri- and paraventricular white matter CBF (WM CBF) in NPH at baseline and during a controlled rise in intracranial pressure (ICP). Twelve patients with idiopathic NPH (mean age 69 years) underwent a CSF infusion study. CBF was measured by H2(15)O PET at baseline and then during the steady-state plateau of raised ICP. The PET images were co-registered and resliced to 3D structural T1-weighted MRIs. Ten healthy normal volunteers served as control subjects for baseline CBF determination only. Profiles of the regional distribution of the baseline WM CBF and of the percentage change in WM CBF as a function of distance from the ventricles were plotted. The global mean baseline CBF in patients (28.4 +/- 5.2 ml/100 ml/min) was lower than in the control subjects (33 +/- 5.4 ml/100 ml/min) (P < 0.005). In patients, the profile of the regional WM CBF at baseline showed an increase with distance from the ventricles (P < 0.0001), with a maximal reduction adjacent to the ventricles and progressive normalization with distance, whereas in controls no relationship was apparent (P = 0.0748). In 10 patients, the rise in ICP during the infusion produced a fall in cerebral perfusion pressure (CPP) and a significant decrease of the global mean CBF from 27.6 +/- 3.1 to 24.5 +/- 2.9 ml/100 ml/min (P < 0.0001). The profile of the percentage changes in regional WM CBF in patients showed a U-shaped relationship with distance from the ventricles (P = 0.0007), with a maximal decrease skewed on the side of the lateral ventricles at around a mean distance of 9 mm. The WM CBF is reduced in NPH, with an abnormal gradient from the lateral ventricles towards the subcortical WM. An excessive decrease in CBF is brought about by reductions in CPP and appears to be maximal in the paraventricular watershed region. These results are discussed in the light of previous hypotheses concerning the aetiology of periventricular CBF reduction in NPH.

Normal pressure hydrocephalus and cerebral blood flow: a PET study of baseline values.

Regional cerebral blood flow (CBF) was studied with O(15)-water positron emission tomography and anatomic region-of-interest analysis on co-registered magnetic resonance in patients with idiopathic (n = 12) and secondary (n = 5) normal pressure hydrocephalus (NPH). Mean CBF was compared with values obtained from healthy volunteers (n = 12) and with clinical parameters. Mean CBF was significantly decreased in the cerebrum and cerebellum of patients with NPH. The regional analysis demonstrated that CBF was reduced in the basal ganglia and the thalamus but not in white matter regions. The results suggest that the role of the basal ganglia and thalamus in NPH may be more prominent than currently appreciated. The implications for theories regarding the pathogenesis of NPH are discussed.