Malignant vestibular schwannoma with intracerebral metastasis.

BACKGROUND: Malignant change in vestibular schwannoma is rare and intracranial metastatic deposits have not been reported. CLINICAL PRESENTATION: We report the case of a 64 year old woman with a benign vestibular schwannoma who underwent translabyrinthine excision in 1996 and Gamma Knife® radiosurgery (Elekta AB, Stockholm, Sweden) (GKRS) in 2006. She presents 10 years after GKRS with progressive neurological deterioration. Histopathologic analysis confirms a malignant peripheral nerve sheath tumour, WHO grade IV with subsequent metastatic spread to the left thalamus confirmed on biopsy. CONCLUSION: We report a rare case of a vestibular schwannoma metastasizing with histological confirmation. It also reminds us of malignant conversion of a benign vestibular schwannoma following GKRS and subsequent aggressive behaviour, with poor prognosis.

The impact of endoscopic transsphenoidal pituitary adenoma surgery on endocrine function: a single-centre study.

INTRODUCTION: The outcome for pituitary endocrine function following endoscopic transsphenoidal surgery remains unclear. This study aims to evaluate endocrine outcomes following endoscopic surgery in order to provide a benchmark to assist in the counselling of patients perioperatively. METHODS: A prospectively held pituitary database was retrospectively analysed for all adult pituitary adenoma patients undergoing endoscopic surgery from May 2011 to May 2017. All operations were performed by a single neurosurgeon at a regional centre for pituitary surgery. Functioning and non-functioning adenomas were included. Hormonal status was assessed at most recent follow-up. RESULTS: One hundred forty-five patients (69 M, 76 F) were included in the study with a median age of 52 years. Median follow-up was 52 months. Eighty-eight patients (61%) were not taking any hormone replacement medications, whilst 57 patients (39%) required hormone replacement therapy (HRT) preoperatively. Preoperatively, 29 patients (20%) had hypothalamo-pituitary-adrenal (HPA) axis dysfunction, 39 patients (27%) had thyroid axis dysfunction, 11 males (16%) and 7 females (9%) had gonadal axis dysfunction, and one patient had preoperative diabetes insipidus. Postoperatively, 26 patients (18%) had a new deficiency in pituitary function, whilst 6 patients (11%) were able to cease HRT. Nineteen patients (13%) had new HPA axis deterioration, 12 (8%) had new thyroid axis dysfunction, 8 males (11%) and 4 females (5%) had gonadal axis deterioration, and 6 patients (4%) had new diabetes insipidus (DI). CONCLUSIONS: The ability to restore pituitary function following endoscopic surgery remains limited, whilst new deficits still occur. It is essential that patients are counselled accordingly as hormonal replacement therapy can have a significant impact on quality of life. Larger longer-term collaborative studies of endocrine outcome in endoscopic pituitary surgery are needed.

CSF rhinorrhoea after endonasal intervention to the anterior skull base (CRANIAL): proposal for a prospective multicentre observational cohort study.

BACKGROUND: The endonasal transsphenoidal approach (TSA) has emerged as the preferred approach in order to treat pituitary adenoma and related sellar pathologies. The recently adopted expanded endonasal approach (EEA) has improved access to the ventral skull base whilst retaining the principles of minimally invasive surgery. Despite the advantages these approaches offer, cerebrospinal fluid (CSF) rhinorrhoea remains a common complication. There is currently a lack of comparative evidence to guide the best choice of skull base reconstruction, resulting in considerable heterogeneity of current practice. This study aims to determine: (1) the scope of the methods of skull base repair; and (2) the corresponding rates of postoperative CSF rhinorrhoea in contemporary neurosurgical practice in the UK and Ireland. METHODS: We will adopt a multicentre, prospective, observational cohort design. All neurosurgical units in the UK and Ireland performing the relevant surgeries (TSA and EEA) will be eligible to participate. Eligible cases will be prospectively recruited over 6 months with 6 months of postoperative follow-up. Data points collected will include: demographics, tumour characteristics, operative data), and postoperative outcomes. Primary outcomes include skull base repair technique and CSF rhinorrhoea (biochemically confirmed and/or requiring intervention) rates. Pooled data will be analysed using descriptive statistics. All skull base repair methods used and CSF leak rates for TSA and EEA will be compared against rates listed in the literature. ETHICS AND DISSEMINATION: Formal institutional ethical board review was not required owing to the nature of the study - this was confirmed with the Health Research Authority, UK. CONCLUSIONS: The need for this multicentre, prospective, observational study is highlighted by the relative paucity of literature and the resultant lack of consensus on the topic. It is hoped that the results will give insight into contemporary practice in the UK and Ireland and will inform future studies.

Current perspectives on recurrent pituitary adenoma: The role and timing of surgery vs adjuvant treatment.

The clinical course of pituitary adenoma can be highly variable. Aggressive pituitary tumours may require multimodal therapy with multiple operations. Even standard pituitary adenomas exhibit relatively high long-term recurrence rates and delayed intervention is often required. The indications for revision surgery in the endoscopic era are expanding for both functioning and nonfunctioning tumours, including access to the cavernous sinus and intracranial compartments. Although revision surgery can be challenging, it has been demonstrated to be both safe and effective. The question of the use of early radiotherapy in pituitary adenoma remains controversial. Our increasing understanding of pituitary tumour biology facilitates individualized treatment and surveillance protocols, with early intervention in high-risk adenoma subtypes. In this review, we discuss the treatment options for recurring pituitary tumours and focus on the role of revision surgery.

The effect of the revised WHO classification on the incidence of grade II meningioma.

Introduction: A retrospective study over a three-year period at University Hospital of Wales (UHW) of the incidence of atypical cranial grade II meningioma both pre and post 2016 revision of The World Health Organisation (WHO) classification of tumours of the central nervous system.Subjects: All available histology from January 2015 to December 2017 of patients with cranial meningiomas.Method: Institutional online reporting system Welsh Clinical Portal to identify patients and view histology reports.Results: Overall 164 patient histology results were analysed (median age 59, age range 23-82, 74% females). There were 55 patients in 2015: 69.1% grade I, 29.1% grade II and 1.8% grade III. There were 109 patients in 2016/17; 68.8% grade I, 29.4% grade II, 1.8% grade III.Discussion: There is significant variability in the reported incidence of grade II meningioma, likely due to variation in local interpretation of diagnostic criteria. Neuropathologists at our institution have reported brain invasion as grade II prior to 2016. This was due to compelling published evidence that brain invasive meningiomas have recurrence and mortality rates similar to that of grade II meningioma as defined using other criteria. The new 2016 WHO criteria now recognise this specifically. As other institutions adapt to the amended 2016 guidelines we anticipate that there will be a greater consensus in line with our incidence rates of grade II meningioma.

Spontaneous cerebrospinal fluid rhinorrhoea with meningitis secondary to ecchordosis physaliphora.

Transclival CSF fistulae are rare. We report a 40-year-old female who presented with meningitis after a short history of CSF rhinorrhoea. The defect resulted from the rare notochordal remnant, ecchordosis physaliphora (EP), and was successfully resected and repaired endoscopically.

Is 30-day readmission an accurate measure of morbidity in cranial meningioma surgery?

Introduction: 30-day readmission rate is a widely adopted marker of quality and performance of acute care, but validity is not well demonstrated. Readmission data following cranial meningioma surgery is not well described and we sought to assess risk factors for readmission. Subjects: All adult patients who underwent cranial meningioma resection from January 2015 to December 2017 in a single institution. Methods: Using Welsh Clinical Portal electronic data to identify readmission within and beyond 30 days to both the index hospital and regional hospitals. Causes of readmission were recorded. Results: 160 patients were included (76.3% female, median age 58, age range 23-78). 28 cases (17.5%) were emergency admissions, median length of initial admission 7 days. 42 patients (26.3%) had seizures at presentation. Total readmission rate was 13.8% (median age 54.5, pre-operative seizure rate 40.9%, median length of readmission 9 days). 13 (59.1%) patients presented within 30 days and 9 (40.9%) >30 days. Readmission causes were seizure, neurological deficit, thromboembolic, infection, CSF, bleeding and social. Causes after 30 days were the same except social or neurological deficit. Discussion: Readmission rates are not associated with age, tumour location, tumour grade, admission route or initial length of stay. 40.9% of readmissions presented outside of the 30-day post-operative time. 30-day readmission rates may not be the most suitable method to demonstrate neurosurgical unit performance in meningioma surgery.

Trigeminal neuropathy in vestibular schwannoma: a treatment algorithm to avoid long-term morbidity.

BACKGROUND: Trigeminal neuropathy (TGN) can occur as a presenting feature of vestibular schwannoma (VS) or as an adverse effect of radiosurgery. This study was designed to evaluate a treatment algorithm for presenting symptoms of TGN in patients with VS, and a new radiosurgery dosimetric tolerance to avoid TGN after treatment. Outcome was measured after microsurgery (MS), stereotactic radiosurgery (SRS), hypofractionated stereotactic radiotherapy (HSRT), and fractionated radiotherapy (FRT). METHODS: A prospectively held VS database was retrospectively analysed from 2011 to 2016 at a tertiary university hospital. All patients who underwent MS from 2011 and all patients who underwent radiotherapy (SRS, HSRT, FRT) from 2015 were studied. Patients on surveillance and neurofibromatosis type 2 patients were not included. Patient demographic data, tumour characteristics, presenting symptoms, and post-treatment outcomes were analysed. RESULTS: Eighty-eight patients were included in the study (43 microsurgery, 45 radiotherapy). Twenty-seven (31%) patients presented with TGN symptoms. The median age of patients included was 56.5 (range 6-72 years), with a median follow-up for MS and SRS of 38 and 20 months, respectively (range 10-80 months). All 27 patients with TGN were offered MS as per protocol. Three patients declined, or were not fit for surgery, and received FRT. Complete resolution of TGN symptoms was achieved in all 24 patients who underwent MS and 33% (1/3) of patients with FRT. Eleven patients experienced transient post-operative complications (pseudomeningocele (6), meningitis (3), venous sinus thrombosis, cerebellar haemorrhagic contusion, and posterior fossa haematoma). Of the 45 patients in the radiotherapy cohort, 36 were suitable for SRS, of which 30 patients who met the dose-volume constraints for trigeminal nerve underwent single-fraction SRS and 6 patients who did not meet the constraints received HSRT. Nine patients (20%) received FRT including three patients with pre-treatment TGN. None of the patients developed new TGN symptoms following SRS or HSRT. CONCLUSIONS: Our algorithm to select the optimal treatment modality appears to achieve comparable or better long-term outcome. Microsurgical resection in our cohort resulted in complete resolution of symptoms in all patients. None of our SRS- or HSRT-treated patients developed TGN during the follow-up period. The adherence to strict trigeminal nerve dose-volume constraints for SRS remains critical to minimise TGN post treatment. Fractionated radiotherapy is an alternative for patients who refuse surgery or those who are unfit for surgery.

Free middle turbinate mucosal graft reconstruction after primary endoscopic endonasal pituitary surgery.

The objective is to assess whether free middle turbinate (FMT) graft reconstruction, after endoscopic endonasal pituitary surgery, combines an acceptably low post-operative cerebrospinal fluid (CSF) leak rate with acceptable rhinological morbidity. This study identified 50 patients who underwent endoscopic endonasal pituitary surgery by the senior author in our teaching hospital between May 2011 and June 2012. FMT graft reconstruction was used in 32 cases. 18 patients were judged pre-operatively as not suitable for FMT reconstruction according to a novel skull base reconstructive algorithm. Outcomes examined were: length of inpatient stay; post-operative CSF leak rate; volume of gross tumour resection; and rhinological morbidity. The rhinological morbidity was measured by the completion of the 22 item sinonasal outcome test (SNOT-22) questionnaire by all 32 patients at 6 weeks and 6 months post-surgery. 32 patients were included in the study. 9 patients had functioning microadenomas and 23 macroadenomas. The median inpatient stay was 2 days. There were no post-operative CSF leaks. The rate of gross tumour resection, confirmed on post-operative MRI, was 87.5 %. The mean SNOT-22 score was 31.9 at 6 weeks and 23.4 at 6 months post-operation-a statistically significant drop. The use of the FMT graft in the reconstruction of the sella defect after endonasal endoscopic pituitary surgery provides a robust dural repair with an acceptable rhinological morbidity profile. FMT grafting as part of a defined skull base reconstructive algorithm results in a CSF leak rate of zero and allows early patient discharge without the need for nasal packing or lumbar drains.

Contemporary management of low--grade glioma: a paradigm shift in neuro-oncology.

Supratentorial diffuse intrinsic low-grade gliomas represent a distinct but heterogenous group of tumours, with the propensity to grow and to differentiate into malignant tumours. They have been historically viewed in the 'benign' spectrum of intrinsic brain tumours, so a watch-and-wait policy was often adopted. With recent advances in our understanding of the natural history of these tumours, combined with advances in surgical technique, an aggressive approach is now recommended. Increasing quality evidence of the impact of tumour resection and multicentre trials of adjuvant radiotherapy and chemotherapy have led to a new algorithm for low-grade glioma management. This review aims to outline the emerging evidence that has shifted neuro-oncology practice.

Pineal parenchymal tumours of intermediate differentiation - An evidence-based review of a new pathological entity.

Pineal region lesions are uncommon, and pineal parenchymal tumours (PPT) account for 20-30% of tumours in this area of which pineocytomas (PCs) and pineoblastomas (PBs) are more prevalent. In 2007, the World Health Organisation (WHO) reclassified PPT from two subgroups (PC and PB) into four, including pineal parenchymal tumours of intermediate differentiation (PPTID). PPTID have been further divided into low- and high-grade lesions (WHO II and III), but due to their rarity have proven difficult lesions to diagnose and a paucity of literature means their optimal treatment options are a challenge to define. This article is a review of the literature of PPTID highlighting diagnostic criteria, a discussion on the role of surgery and radiotherapy, including treatment paradigms and reported outcomes for these problematic neoplasms.

Use of day 1 early morning cortisol to predict the need for glucocorticoid replacement after pituitary surgery.

BACKGROUND: Assessment of adrenal reserve in patients who have undergone pituitary surgery is crucial. However, there is no clear consensus with regards to the type and timing of the test that should be used in the immediate post-operative period. Recently, there has been increased interest in measuring post-operative cortisol levels. We present our data utilising day 1 post-operative early morning cortisol as a tool to assess adrenal reserve in steroid-naive patients. METHODS: A retrospective analysis of endoscopic pituitary surgery undertaken over a 2-year period. 82 patients underwent 84 surgeries in total. Patients who were already on glucocorticoids pre-operatively and patients with Cushing's disease, pituitary apoplexy and those without follow-up data were excluded, leaving a study group of 44 patients with 45 operations. A 9am day 1 post-operative cortisol value of > 400 nmol/L was taken as an indicator of adequate adrenal reserve. All the patients were reassessed at 6 weeks with a standard short synacthen test (SST) using 250 micrograms of intravenous synacthen. RESULTS: 22 out of 45 patients had a cortisol value of > 400 nmol/L on day 1 post-operatively and were discharged without glucocorticoid supplementation. Of these, only 2 patients subsequently failed the SST when reassessed at 6-8 weeks. The remaining 23 patients had a cortisol value of < 400 nmol/L on day 1 post-operatively and were discharged on hydrocortisone 10 mg twice daily. At 6-8 weeks, nine continued to show suboptimal stimulated cortisol levels whereas the remaining fourteen patients showed adequate adrenal reserve. The 9 am cortisol value had high specificity (81.8%) and positive predictive value (90.9%) for integrity of the HPA axis. Sensitivity was 58.8% and negative predictive value was 39.1%. CONCLUSION: A day 1 post-operative early morning cortisol is a useful tool to predict adrenal reserve post-pituitary surgery, enabling clinicians to avoid unnecessary blanket glucocorticoid replacement.

Cerebellar pilocytic astrocytoma in adults: a management paradigm for a rare tumour.

BACKGROUND: Pilocytic astrocytoma is one of the commonest subtypes of glioma to affect children. However, they are rarely diagnosed in patients over the age of 18 years. In adults, these tumours appear more frequently supra-tentorially than in the cerebellum and some reports suggest a different clinical course in adults. We reviewed ten patients aged 18 or over who had been operated on for cerebellar pilocytic astrocytoma to assess the impact of tumour biology and extent of resection on outcome in adults. METHOD: Patients were identified from a neuropathology database and a retrospective chart review of ten patients was performed. Recorded data included patient demographics, tumour location, presenting features, radiological appearance, extent of surgical resection, tumour recurrence and Ki-67 proliferation index. RESULTS: Nine patients were men and one patient was a woman. Median follow up is 41.5 months (range 15-334 months). Complete surgical resection was achieved in nine of the patients operated in our institution. One patient had prior subtotal resection elsewhere. Tumour recurrence was seen only in the two patients with subtotal resection, at 7 and 25 years. Ki-67 ranged from <1 to 10% and appears to have no correlation to recurrence. No patients in this series had adjuvant treatment. CONCLUSIONS: Cerebellar pilocytic astrocytomas in adults should be treated with macroscopic complete surgical resection whenever possible. If this is achieved, long-term survival rates are excellent, whereas subtotal resection carries a high risk of tumour recurrence. Ki-67 is less important prognostically than the extent of initial resection.

Clinical features of silent corticotroph adenomas.

BACKGROUND: Silent corticotrph adenomas represent a distinct pathological subtype of non-functioning pituitary adenomas that are traditionally believed to carry a more aggressive biological behavior and higher potential for recurrence. METHODS: We conducted a retrospective review of all silent corticotroph adenomas treated and followed at our institution over the last 10 years. We reviewed clinical, radiological and pathological features. The series was compared to a matched cohort of ACTH-negative, non-functioning adenomas to compare clinical, radiological and pathological features. Our results were compared to the literature. RESULTS: Twenty patients met our inclusion criteria. Fifty-six percent of the patients were females. Mean age was 51 years (range 24-78 years). Visual dysfunction was the most common clinical presentation (38 %). Thirteen percent of the cases presented with acromegaly secondary to double adenoma (silent corticotroph adenoma and growth hormone adenoma) and 13 % presented with pituitary tumor apoplexy. All the tumors were macroadenomas. Frank cavernous sinus invasion occurred in 31 % of the cases. The patients who presented with acromegaly did not achieve remission postoperatively. In the remaining patients, recurrence occurred in 14 % of the cases over a mean follow-up period of 41 months. Compared to non-functioning adenomas, silent corticotroph adenomas were more likely to bleed (p value 0.014) and have double adenoma (p value 0.047). There was no difference in recurrence rates between silent corticotroph adenomas and non-functioning adenomas (p value 0.647). CONCLUSION: These results suggest that silent corticotroph adenomas have some unique features compared to non-functioning adenomas. Within the limits of our follow-up duration and sample size and our review of the literature, we would recommend that the traditional view to manage all silent corticotroph adenomas with adjuvant radiation should be reconsidered. We suggest adopting an initially more conservative follow-up surveillance and delay of upfront radiation until there is clear evidence of tumor recurrence.

Long term neuropsychological outcome and management of 'decompensated' longstanding overt ventriculomegaly in adults.

OBJECTIVE: We aimed to study a group of patients presenting with symptomatic longstanding overt ventriculomegaly in adults (LOVA) requiring treatment (endoscopic third ventriculostomy), and to study their long term neuropsychological status and functionality. METHODS: Twenty patients were treated with endoscopic third ventriculostomy on presentation and were studied with neuropsychological assessments including the repeatable battery for the assessment of neuropsychological status and the hospital anxiety and depression scores. Pre- and post-operative MRI studies were used to assess the patency of the endoscopic third ventriculostomy (ETV) along with clinical outcome scores. RESULTS: A variety of presenting symptoms were experienced including headache (90%), worsening imbalance, attacks of loss of consciousness and cognitive decline. Mean age of patients was 52 years (range, 17-78) with mean head circumference of 60.9 cm. ETV was successful in controlling symptoms in 89% of patients with three patients requiring shunts (gravitational valves). There were no subdurals after surgery. All patients did universally badly in all aspects of the neuropsychological studies without showing any undue anxiety or depression. CONCLUSION: LOVA patients can decompensate at any time in their adult life with acute symptoms, which are generally well controlled with ETV and with few complications. Sadly, the long-term effects of carrying 'asymptomatic' or compensated hydrocephalus since childhood are of a significant cognitive cost to these patients.

Olfactory Outcomes after Resection of Tuberculum Sella and Planum Sphenoidale Meningiomas via a Transcranial Approach.

Objectives Controversy exists surrounding the optimal approaches to tuberculum sella meningioma (TSM) and planum meningioma (PM). Olfaction is infrequently considered within this context but is nonetheless an important quality of life measure. The evolution of olfactory outcomes following contemporary transcranial surgery remains unclear. This study reviews olfactory outcomes after supraorbital craniotomy for TSM or PM and defines temporal trends in its recovery. Study Design A prospective study of a patients who underwent a minimally invasive supraorbital craniotomy for TSM or PM was conducted at a single neurosurgical center. Participants & Main Outcome Measures All patients were questioned about olfaction at presentation 3 months postoperatively, 12 months postoperatively, and annually thereafter (median follow-up = 37 months). The olfactory status of patients was categorized as normosmia, anosmia, hyposmia, parosmia, (altered perception of odours), or phantosmia, (olfactory hallucinations). Results Twenty-two patients were included in the study analysis, (range = 27-76). Precisely, 3 months after surgery, seven patients had normal olfaction (32%). Six patients were anosmic, (27%) four hyposmic, (18%), three parosmic, (14%), and two were phantosmic (9%). At 1-year follow-up, almost half of patients (10; 48%) were normosmic, while two patients (9.5%) were anosmic. There were no further improvements in olfaction between 1 year and long-term follow-up. Conclusion Subfrontal transcranial approaches for TSM or PM appear to be associated with changes in olfaction that can improve with time; these improvements occur within the first year after surgery. Impacts upon olfaction should be considered when selecting a surgical approach and patients counseled appropriately.

Low grade glioma: a qualitative study of the wait and see approach.

BACKGROUND: There is no consensus on the best management of adults with presumed low grade glioma (LGG). Studies have suggested uncertainty and anxiety associated with a wait and see approach contribute to reduced quality of life. This study aims to explore the impact of a diagnosis of LGG, to address concerns regarding the uncertainty of the diagnosis and the role of wait and see from the patient's perspective. METHODS: Qualitative research methodology was used. A semi-structured interview was conducted with 24 patients with imaging evidence of LGG but no prior intervention. All patients had been followed for at least one year prior to interview. Verbatim transcripts were subjected to thematic analysis. RESULTS: The median age of participants was 47 (range 21-82) and the median duration of follow-up 37 months (range 12-156 months). Fifty percent presented with seizures. Five overarching themes emerged from the data; 1) patients experience initial devastation followed by acceptance and low anxiety; 2) absence of symptoms mitigates anxiety concerning the possibility of progression; 3) patients would prefer to defer surgery until there is progression or a change in their quality of life; 4) anxiety is reduced by trust in the physician; 5) quality of life is not affected by the diagnosis, as fear of morbidity from intervention is greater than the fear of uncertainty. CONCLUSIONS: The wait and see approach does not contribute to anxiety or reduction in quality of life in patients with LGG.

Antibiotic-impregnated ventriculoperitoneal shunts--a multi-centre British paediatric neurosurgery group (BPNG) study using historical controls.

BACKGROUND: Ventriculoperitoneal shunt infection remains a significant problem. The introduction of antibiotic-impregnated shunt (AIS) systems in the prevention of shunt infection may represent a potential advance; however, there are no randomized controlled trials to establish a robust evidence-based practice. Previously published single-institution cohort studies have provided varying results on the efficacy of AIS systems in the prevention of shunt infection. In this study, we evaluate combined outcomes from three paediatric neurosurgical units in the use of AIS systems for paediatric patients with hydrocephalus. METHODS: The three units established independent databases with data collected from varying time frames. All procedures, where a complete AIS system or part was implanted into patients from 0-16 years in age, were included. The primary outcome measure was shunt infection rate. Shunt procedures were classified as de novo (DNS) and clean revision (CRS). An infant (<1 year) de novo insertion subgroup was also analyzed. AIS shunts were compared to a historical control of non-AIS shunts and results were analysed by centre using an odds ratio with a 95% confidence interval and combined across centres by meta-analysis. RESULTS: A total of 581 AIS implantation procedures were performed in all three units. The comparative non-AIS historical cohort comprised of 1,963 procedures. The pooled effect estimate indicated a clinical advantage for AIS shunts compared to non-AIS shunts, odds ratio (OR), 0.60 (95% CI 0.38, 0.93). The de novo infant group comprised 153 AIS systems, and 465 de novo shunts in the historical non-AIS cohort. Again the pooled effect estimate indicated a clinical advantage for AIS shunts compared to non-AIS shunts, OR 0.38 (95% CI, 0.17; 0.85); however, there was a large overlap of confidence intervals in the results from the different sites indicating the uncertainty in the treatment effect estimates. Over 80% of organisms were gram positive in the infected AIS cohort with a median time to infection of 19 days. Two rifampicin-resistant organisms and three MRSA organisms were detected. CONCLUSION: Data from this exclusively paediatric multi-centre historical control study suggest that AIS may significantly reduce infection rates in de novo and clean revision shunt implants. Although the possibility of bias cannot be excluded due to study design, this is the largest study on an exclusively paediatric cohort comparing standard shunts to AIS implants. Future double-blinded RCTs are needed to confirm AIS efficacy.

Cognitive and functional outcome in spina bifida-Chiari II malformation.

PURPOSE: The long-term outcome in spina bifida-Chiari II-hydrocephalus complex is poorly understood. Traditional neurosurgical outcome measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health, disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth. METHODS: Adult patients under routine follow-up were assessed in a joint neurosurgery/neuropsychology clinic. Patients completed lifestyle questionnaires, the hydrocephalus outcome questionnaire (HOQ) and underwent cognitive testing. Clinical variables including number of shunt revisions, shunt infection and surgical decompression of foramen magnum, which may influence outcome, were investigated. RESULTS: Twenty-one adults with a median age of 35 years were investigated. All had treated hydrocephalus, and eight had foramen magnum decompression for headache or progressive brainstem symptoms with stabilisation of symptoms in seven and improvement in one. Only eight patients were living independently, five were in paid employment and five work voluntarily. HOQ scores for cognitive function were lower (0.56 ± 0.20; mean ± standard deviation (SD)) than those for physical (0.64 ± 0.15) and social-emotional (0.65 ± 0.17) health. Cognitive function varied across the cohort with attention most severely affected (73.9 ± 17.0; mean ± SD). Repeated episodes of shunt malfunction or foramen magnum decompression were not associated with a worse cognitive function. CONCLUSIONS: Despite intervention in childhood and adequate cerebrospinal fluid diversion the prognosis for independent living into adulthood remains poor. All patients have elements of cognitive impairment. Structural brain abnormalities may be more important determinants of cognitive outcome than shunt malfunction.