Screening for cerebrovascular disorder on the basis of family history in asymptomatic children.

Background: Cerebrovascular disorders represent a group of uncommon, heterogeneous, and complex conditions in children. We reviewed the screening practice for the detection of cerebrovascular disorder in asymptomatic children referred to our neurovascular service on the basis of a positive family history and parental and/or treating physician concern.Methods: Retrospective case-note review of referrals to our neurovascular service (July 2008-April 2018). Patients were included if the referral was made for screening, on the basis of a positive family history of cerebrovascular disorder. Symptomatic children, those with previous cranial imaging, or children under the care of a clinical geneticist (i.e. due to the child or their relative having HHT or mutations in KRIT1) were not eligible for inclusion.Results: Forty-one children were reviewed, 22 males (Median age 10.7 years, range 0.6-15.6 years). This represented 22% of the total number of referrals over a 10-year period. Twenty-nine children had an MRI/MRA brain. Twenty-eight children were referred due to a family history of intracranial aneurysm and/or subarachnoid haemorrhage, but only two had two first-degree relatives affected. Ten children were referred due to a family history of arteriovenous malformation. Three children were referred due to a family history of stroke. No cerebrovascular disease was detected during the study period (n = 29).Conclusions: Parental and/or physician concern generated a substantial number of referrals but no pathology was detected after screening. Whilst general screening guidance exists for the detection of intracranial aneurysms, consensus guidelines for the screening of children with a positive family history do not, but are required both to guide clinical practice and to assuage parental and/or physician concerns.

Management of paediatric hydrocephalous with Miethke fixed pressure gravitational valves. The Alder Hey Children's Hospital experience.

OBJECTIVE: The management of paediatric hydrocephalous remains challenging with the complication and revision rates being consistent in the literature. We hypothesise that the use of a fixed pressure gravitational valve for all de novo shunt insertions decreases the rate of functional revisions and that by implementing the routine use of gravitational valves in children, we would see a reduction in over-drainage and slit ventricle syndrome. METHODS: Retrospective data collection in a single centre, between February 2010 and August 2018. All patients undergoing fixed pressure gravitational Miethke valve insertion were included. We collected data on patients' demographics, reason for shunt insertion, type of valve and time to and reason for first revision. Data analysis was done with SPSS. RESULTS: A total of 235 patients were included in our study (124 males, 111 females), aged from 0 to 18.6 years (median 0.28). A total of 99 shunt revisions were documented, 30 of which secondary to ventricular catheter malfunction and 28 secondary to infection. The overall mechanical valve survival rates were 88.5%, 86.4% and 85.5% at 1, 2 and 5 years, respectively. Shunt revision due to over-drainage was documented in only 3 cases (1.3%). CONCLUSION: Our results are in agreement with existing literature regarding shunt failures secondary to all extrinsic factors to the valve (infection and mechanical failure). We have shown that the use of a Miethke fixed pressure valve for all de novo shunt insertions in paediatric hydrocephalus decreases the need for functional revisions with valve survival rates being superior to the ones described for other types.

Patients Attitude to Technology : A Way to Improve Hydrocephalus Management and Follow up Using Smartphone Intelligent Application.

Smartphone applications ("apps") have become ubiquitous with the advent of smartphones and tablets in recent years. Increasingly the utility of these apps is being explored in healthcare delivery. Hydrocephalus is a condition that is usually followed by a neurosurgeon for the patient's life. We explore patient acceptability of a mobile app as an adjunct to outpatient follow-up of patients with hydrocephalus. A questionnaire was circulated amongst patients with hydrocephalus (adults and children). Patients were asked questions about their hydrocephalus; expectations for outpatient follow up, whether they have smartphone/tablet/internet access and whether they would be interested in a mobile app for their long term hydrocephalus follow up. 191 patients completed questionnaires, 98 respondents were adults (mean age 46.1) and 93 were children less than 18 years old (mean age 8). Overall 36.1% of patients did not know the cause of their hydrocephalus. 96.7% have a shunt. 76.5% of adults and 80.6% of children had 1-4 shunt surgeries, 14.3% of adults and 11.8% of children had 5-9 shunt surgeries, 3.1% of adults and 5.4% of children had 10-14 shunt surgeries. 71.7% of patients expect to be followed-up routinely in clinic for life. All children had smartphones or tablets, compared to 86.7% of adults. Children were more interested in a hydrocephalus app, 84.9% saying yes, compared to 71.4% of adults. Adults who were not interested in the app did not have a smartphone or tablet. Hydrocephalus management is a lifelong task and innovations in technology for engaging patients in its management are vital. The majority of patients are interested in mobile apps for outpatient management of hydrocephalus. We will follow this up with a feasibility study of a custom designed hydrocephalus app.

Correction to: Open resection of hypothalamic hamartomas for intractable epilepsy revisited, using intraoperative MRI.

The authors apologize to have sent a final manuscript draft omitting "Athanasius Chawira" from the list of authors. The correct list of authors is given in this article.The original article has been corrected.

Open resection of hypothalamic hamartomas for intractable epilepsy revisited, using intraoperative MRI.

INTRODUCTION: Hypothalamic hamartomas (HHs) are rare non-neoplastic lesions which cause drug-resistant epilepsy with associated behavioural, psychiatric and endocrine issues. With the development of new minimally invasive techniques for the treatment of HH, there is a need to reappraise the effectiveness and safety of each approach. We review the outcomes of HH patients treated surgically, utilizing intraoperative magnetic resonance imaging (IOMRI), by a team of Alder Hey NHS Foundation Trust tumour and epilepsy neurosurgeons since 2011. METHODS: Patient records of all HH cases operated on since 2011 were reviewed to confirm history of presentation and clinical outcomes. RESULTS: Ten patients have undergone surgery for HH under the dual care of Alder Hey tumour and epilepsy neurosurgeons during this period. Eight cases had a midline transcallosal, interforniceal approach with the remaining 2 having a transcallosal, transforaminal approach. All patients had an IOMRI scan, with 40% needing further tumour resection post-IOMRI. Forty percent had a total resection, 3 patients had near-total resection and 3 patients had subtotal resection (~ 30% tumour residual on post-operative MRI). No new neurological complications developed post-operatively. Hypothalamic axis derangements were seen in 3 cases, including 1 diabetes insipidus with hypocortisolaemia, 1 hypodipsia and 1 transient hyperphagia. Eighty percent are seizure free; the remaining two patients have had significant improvements in seizure frequency. CONCLUSIONS: IOMR was used to tailor the ideal tumour resection volume safely based on anatomy of the lesion, which combined with the open transcallosal, interforniceal route performed by surgeons experienced in the approach resulted in excellent, safe and effective seizure control.