Raine's syndrome: rare disease from neurosurgical perspective.

Raine's syndrome (RS) is a rare genetic disorder. Only 25 cases are in literature. Occurs due to genetic mutation resulting in deranged bone metabolism. Few cases are reported discussing the neurosurgical ramifications of the disease. We report a child diagnosed with RS. He was presented with multisutural synostosis requiring craniofacial intervention with two vault expansions. Additionally, required VP shunt due to hydrocephalus. We consider our case unique among reports of RS, as our patient has survived for 10. He died due to valve obstruction of the VP shunt. We also present a review of relevant medical literature.

Part II: Blood Transfusion and Donor Exposure in the Surgical Management of Trigonocephaly Patients: A Protocol From Alder Hey Craniofacial Unit.

Trigonocephaly is a craniofacial malformation caused by premature fusion of the metopic suture. Surgical correction frequently results in the need for blood transfusion. Transfusion complications include transfusion-transmitted infections (TTIs), immune-mediated reactions, and volume overload. Donor exposure (DE) describes the number of blood products from unique donors with increasing DE equating to an increased risk of TTI. We evaluate data on 204 trigonocephaly patients covering 20 years of practice with respect to blood transfusions and DE. This represents the largest series from a single unit to date. A protocol based on our experiences has been devised that summarizes the key interventions we recommend to minimize blood transfusions and DE in craniofacial surgery. Patients operated on between 2000 and 2020 were included. DE and a range of values were calculated including estimated red cell loss (ERCL) and estimated red cell volume transfused (ERCVT). Groups were established by relevant interventions and compared using the Mann-Whitney U test. Mean DE fell from 1.46 at baseline to 0.85 ( P <0.05). Median allogenic transfusion volume fell from 350 mL at baseline to 250 mL ( P <0.05). Median ERCL fell from 15.05 mL/kg at baseline to 12.39 mL/kg and median ERCVT fell from 20.85 to 15.98 mL/kg. Changes in ERCL and ERCVT did not reach statistical significance. DE can be minimized with the introduction of key interventions such as a restrictive transfusion policy, preoperative iron, cell saver, tranexamic acid, and use of a matchstick burr for osteotomies.

Consensus Statement from India on the Renal Benefits of ARNi, SGLT-2i, and Bisoprolol in Chronic Kidney Disease.

Chronic kidney disease (CKD) is a major contributor to morbidity and mortality in India. CKD often coexists with heart failure (HF), diabetes, and hypertension. All these comorbidities are risk factors for renal impairment. HF and CKD are pathophysiologically intertwined, and the deterioration of one can worsen the prognosis of the other. There is a need for safe renal pharmacological therapies that target both CKD and HF and are also useful in hypertension and diabetes. Neurohormonal activation achieved through the activation of the sympathetic nervous system (SNS), the renin-angiotensin-aldosterone system (RAAS), and the natriuretic peptide system (NPS) is fundamental in the pathogenesis and progression of CKD and HF. Angiotensin receptor neprilysin inhibitor (ARNi), sodium-glucose cotransporter 2 inhibitors (SGLT-2i), and selective ß1-blocker (B1B) bisoprolol suppress this neurohormonal activation. They also have many other cardiorenal benefits across a wide range of CKD patients with or without concomitant HF, diabetes, or hypertension. This consensus statement from India explores the place of ARNi, SGLT-2i, and bisoprolol in the management of CKD patients with or without HF and other comorbidities.

Current Place of SGLT2i in the Management of Heart Failure: An Expert Opinion from India.

Heart failure (HF) is a global health concern that is prevalent in India as well. HF is reported at a younger age in Indian patients with comorbidity of type 2 diabetes (T2DM) in approximately 50% of patients. Sodium-glucose cotransporter-2 inhibitors (SGLT2i), originally approved for T2DM, are new guideline-recommended and approved treatment strategies for HF. Extensive evidence highlights that SGLT2i exhibits profound cardiovascular (CV) benefits beyond glycemic control. SGLT2i, in conjunction with other guideline-directed medical therapies (GMDT), has additive effects in improving heart function and reducing adverse HF outcomes. The benefits of SGLT2i are across a spectrum of patients, with and without diabetes, suggesting their potential place in broader HF populations irrespective of ejection fraction (EF). This consensus builds on the updated evidence of the efficacy and safety of SGLT2i in HF and recommends its place in therapy with a focus on Indian patients with HF.

The Promise of Cilnidipine in Hypertension with Comorbidities: National Consensus Statement: National Consensus Group Comprises Cardiologists, Nephrologists, and Diabetologists from India in a National Meet at New Delhi held on 22nd May 2022.

The rapidly increasing burden of hypertension is responsible for premature deaths from cardiovascular disease (CVD), renal disease, and stroke, with a tremendous public health and financial burden. Hypertension detection, treatment, and control vary worldwide; it is still low, particularly in low- and middle-income countries (LMICs). High blood pressure (BP) and CVD risk have a strong, linear, and independent association. They contribute to alarming numbers of all-cause and CVD deaths. A major culprit for increased hypertension is sympathetic activity, and further complications of hypertension are heart failure, ischemic heart disease (IHD), stroke, and renal failure. Now, antihypertensive interventions have emerged as a global public health priority to reduce BP-related morbidity and mortality. Calcium channel blockers (CCB) are highly effective vasodilators. and the most common drugs used for managing hypertension and CVD. Cilnidipine, with both L- and N-type calcium channel blocking activity, is a promising 4th generation CCB. It causes vasodilation via L-type calcium channel blockade and inhibits the sympathetic nervous system (SNS) via N-type calcium channel blockade. Cilnidipine, which acts as a dual L/N-type CCB, is linked to a reduced occurrence of pedal edema compared to amlodipine, which solely blocks L-type calcium channels. The antihypertensive properties of cilnidipine are very substantial, with low BP variability and long-acting properties. It is beneficial for hypertensive patients to deal with morning hypertension and for patients with abnormal nocturnal BP due to exaggerated sympathetic nerve activation. Besides its BP-lowering effect, it also exhibits organ protection via sympathetic nerve inhibition and renin-angiotensin-aldosterone system inhibition; it controls heart rate and proteinuria. Reno-protective, neuroprotective, and cardioprotective effects of cilnidipine have been well-documented and demonstrated.

Secure windows for child safety: a retrospective study of window falls in children, aiming to raise prevention awareness.

PURPOSE: The aim of the study is to enhance understanding, raise awareness and inform prevention programmes regarding potential factors that lead to severe paediatric injuries caused by unintentional falls from windows. METHODS: This is a retrospective review from a major Trauma Centre, covering the majority of North West England and North Wales and included children under the age of 16 that had sustained falls from windows and were hospitalised between April 2015 and June 2020. RESULTS: Overall, 825 patients' records have been reviewed, 39% of which exhibited neurosurgical injuries (322 admissions). The most common cause of injury was falls (42%), out of which 19% was identified as falls from windows which was eventually the core focus of this review (25 patients). The records showed that 72% of the falls were not witnessed by another individual, suggesting that children were being left unattended. Average GCS recorded at presentation was 11.2 and 56% of cases were identified as severe major traumas. With a mean stay of 2.2 days in ICU, 1.6 days in HDU and 6 days in the neurosurgical clinic, average treatment costs per patient were £4,493, £651 and £4,156 respectively. Finally, 52% of patients were identified to require long-term physiotherapy/occupational therapy due to permanent disabilities, 20% long-term antiepileptic treatment for seizures and 44% long-term psychological services input. CONCLUSION: This study presents our experience at a major tertiary trauma centre in the UK over a 5-year period, from a paediatric neurosurgical injuries perspective due to fall from windows. We aim to raise awareness and highlight the importance of establishing prevention programmes which would hopefully decrease the incidence of paediatric window falls.

Variation between Clinician-Recorded and Downloaded Invasive Blood Pressure in Extremely Preterm Infants.

OBJECTIVE: This study aimed to examine the variation between clinician-recorded and continuously downloaded invasive blood pressure (BP). STUDY DESIGN: Prospective study where invasive BP data were downloaded every 10 seconds for the first week of life. Hourly clinician-recorded BP was recorded. Agreement between the two methods were examined. RESULTS: A total of 1,180 BP measurements were examined from 42 preterm infants with a mean (standard deviation [SD]) gestation and birthweight of 25.7 weeks (1.4) and 802 g (177) respectively. The mean (SD) bias was -0.11 mm Hg (3.17), but the 95% limits of agreement (LOA) varied between -6.3 and +6.1 mm Hg. Inotrope usage was significantly higher for BP measurements that fell in the 5% outliers when compared with those that fell within the 95% LOA (62.7 vs. 44.6%, p = 0.006). CONCLUSION: Clinicians showed no systematic bias to over- or underrecord BP, but some of the greatest differences were found in infants receiving inotropes. KEY POINTS: · BP is a commonly recorded cardiovascular parameter in the neonatal intensive care unit.. · Invasively measured BP remains the gold standard.. · Clinician-recorded BP showed no systematic bias in over-or underrecording invasive BP..

Intracranial Empyemas in the COVID-19 Era: A New Phenomenon? A Paediatric Case Series and Review of the Literature.

INTRODUCTION: We present the largest series of paediatric intracranial empyemas occurring after COVID-19 infection to date, and discuss the potential implications of the pandemic on this neurosurgical pathology. METHODS: Patients admitted to our centre between January 2016 and December 2021 with a confirmed radiological diagnosis of intracranial empyema were retrospectively reviewed, excluding non-otorhinological source cases. Patients were grouped according to onset before or after onset of the COVID-19 pandemic and COVID-19 status. A literature review of all post-COVID-19 intracranial empyemas was performed. SPSS v27 was used for statistical analysis. RESULTS: Sixteen patients were diagnosed with intracranial empyema: n = 5 prior to 2020 and n = 11 after, resulting in an average annual incidence of 0.3% prior to onset of the pandemic and 1.2% thereafter. Of those diagnosed since the pandemic, 4 (25%) were confirmed to have COVID-19 on recent PCR test. Time from COVID-19 infection until empyema diagnosis ranged from 15 days to 8 weeks. Mean age for post-COVID-19 cases was 8.5 years (range: 7-10 years) compared to 11 years in non-COVID cases (range: 3-14 years). Streptococcus intermedius was grown in all cases of post-COVID-19 empyema, and 3 of 4 (75%) post-COVID-19 cases developed cerebral sinus thromboses, compared to 3 of 12 (25%) non-COVID-19 cases. All cases were discharged home with no residual deficit. CONCLUSION: Our post-COVID-19 intracranial empyema series demonstrates a greater proportion of cerebral sinus thromboses than non-COVID-19 cases, potentially reflecting the thrombogenic effects of COVID-19. Incidence of intracranial empyema at our centre has increased since the start of the pandemic, causes of which require further investigation and multicentre collaboration.

Health-related quality of life following cranioplasty - a systematic review.

Cranioplasty is a neurosurgical procedure that repairs a defect in the skull Coupled with the underlying pathology cranioplasty associated morbidity can have a large impact on patient quality of life, which is often poorly explored. The objective of this systematic review was to identify patient-reported outcomes evaluating health-related quality of life following cranioplasty. The review protocol was registered on PROSPERO (CRD42021251543) and a systematic review was conducted in accordance with the PRISMA statement. PubMed, Embase, CINAHL Plus, and the Cochrane databases were searched from inception to 1 May 2022. All studies reporting HRQoL following cranioplasty were included. Reporting was assessed using the ISOQOL checklist and risk of bias was assessed using the Newcastle-Ottawa Scale or the Johanna-Briggs Institute Scale, as appropriate. A total of 25 studies were included of which 20 were cross-sectional and 2 longitudinal. Most studies utilized study specific questionnaires and Likert scales to assess HRQoL. The studies found a significant improvement in physical functioning, social functioning, cosmetic outcome, and overall HRQoL following cranioplasty. Further longitudinal studies utilising validated measurement tools are required to better understand the effect of cranioplasty at a patient level.

Patient Tailored Surgery in Saethre-Chotzen Syndrome: Analysis of Reoperation for Intracranial Hypertension.

Saethre-Chotzen syndrome (SCS) is a syndromic craniosynostosis with pathogenic variants in the TWIST1 gene showing a broad phenotypic spectrum. Controversies exist in the literature regarding surgical management with single one-stage versus patient-tailored surgery and the related reoperation rate for intracranial hypertension of up to 42%. At our center, SCS patients are offered patient-tailored surgery with single-stage fronto-orbital advancement and remodeling or fronto-orbital advancement and remodeling and posterior distraction in an individually determined order. The authors' database identified 35 confirmed SCS patients between 1999 and 2022. Involved sutures in craniosynostosis were left unicoronal (22.9%), bicoronal (22.9%), sagittal (8.6%), bicoronal and sagittal (5.7%), right unicoronal (2.9%), bicoronal and metopic (2.9%), bicoronal, sagittal and metopic (2.9%), and bilateral lambdoid (2.9%). There was pansynostosis in 8.6% and no craniosynostosis in 14.3% of the patients. Twenty-six patients, 10 females, and 16 males were operated on. Mean age at the first surgery was 1.70 years, and 3.86 years at the second surgery. Eleven of 26 patients had invasive intracranial pressure monitoring. Three patients presented with papilledema before the first surgery and 4 afterward. Four of the 26 operated patients were operated initially elsewhere. The other 22 patients were initially referred to our unit and underwent patient-tailored surgery. Nine of these patients (41%) had a second surgery, and 3 (14%) of them were because of raised intracranial pressure. Seven (27%) of all operated patients had a complication. Median follow-up was 13.98 years (range, 1.85-18.08). Patient-tailored surgery in a specialized center and long-term follow-up allow for a low reoperation rate for intracranial hypertension.

The Paediatric Vault Score (PVS)-A Novel Scoring Tool for Prioritisation of Surgical Management of Craniosynostosis Patients.

Craniosynostosis is the premature fusion of the skull sutures, resulting in abnormal skull shape and volume. Timely management is a priority in avoiding raised intracranial pressure which can result in blindness and neurodevelopmental delay. Due to the COVID-19 pandemic, theater access was reduced. A risk stratification scoring system was thus devised to score patients attending surgery and aid in prioritization according to surgical need. The authors present the Paediatric Vault Score (PVS), which can also be customized to each unit's individual protocols. Ten patients on the waiting list were randomly selected and their clinical information was summarized in uniform anonymized reports. Six craniofacial consultants were selected as assessors and given 1 week to independently rank the patients from 1 to 10. Each scorer's ranking was verified against the PVS template and concordance was analyzed using the Kendall tau correlation coefficient (KT). Three cycles of the scoring process were carried out. Improvements were made to the scoring tool following cycle 1. Cycle 1 revealed 2 clinicians to be concordant with the PVS system and 4 to be discordant. Cycle 2 revealed all 6 clinicians to be concordant, with a mean KT score of 0.61. The final cycle revealed all 6 clinicians to be concordant, with a mean KT score of 0.70. Four scorers increased their concordance once the scoring sheet was introduced. Kendall's correlation of concordance calculated the interrater reliability to be 0.81. The PVS is the first known vault scoring system to aid in risk stratification and waiting list prioritization.

An Esthetic Scoring System for Scaphocephaly Assessment and Outcomes: A Pilot Study.

Scaphocephaly is the commonest form of craniosynostosis with a varied presentation consisting of many morphological components and a range of possible surgical interventions. However, with regard to esthetic assessment, there is no universally applied assessment system. The aim was to develop a simple assessment tool encompassing multiple phenotypic components of scaphocephaly. This was done by piloting a red/amber/green (RAG) scoring system to judge esthetic outcomes following scaphocephaly surgery using photographs and experienced observers. Standard photographic views of 20 patients who had undergone either passive or anterior 2/3 vault remodelling were scored by 5 experienced assessors. Using a RAG scoring system before and after scaphocephaly correction according to 6 morphological characteristics: visual impression of cephalic index, calvarial height, bitemporal pinching, frontal bossing, posterior bullet, and displacement of the vertex. All 5 assessors were asked to score the preoperative and postoperative views independently. The RAG scores were each assigned a number (1-3) and added to give a composite score (range 6-18) and these were averaged between the 5 assessors. There was a highly statistically significant difference between both preoperative and postoperative composite scores ( P <0.0001). A subgroup analysis of the postoperative composite score between the 2 surgical techniques showed no significant difference ( P =0.759). The RAG scoring system can be used to assess esthetic change following scaphocephaly correction and it provides both a visual analogue and a numerical indicator of change. This assessment method needs further validation but is a potentially reproducible way to score and compare esthetic outcomes in scaphocephaly correction.

Role of Iron Therapy in Heart Failure: A Consensus Statement from India.

Iron deficiency (ID) with or without anemia is frequently observed in patients with heart failure (HF). Uncorrected ID is associated with higher hospitalization and mortality in patients with acute HF (AHF) and chronic HF (CHF). Hence, in addition to chronic renal insufficiency, anemia, and diabetes, ID appears as a novel comorbidity and a treatment target of CHF. Intravenous (IV) ferric carboxymaltose (FCM) reduces the hospitalization risk due to HF worsening and improves functional capacity and quality of life (QOL) in HF patients. The current consensus document provides criteria, an expert opinion on the diagnosis of ID in HF, patient profiles for IV FCM, and correct administration and monitoring of such patients.

Angiotensin Receptor-Neprilysin Inhibitor Therapy and Cardiac Remodeling in Heart Failure: Consensus Statement from India.

Adverse cardiac remodeling refers to progressive structural and functional modifications in the heart because of increased wall stress in the myocardium, loss of viable myocardium, and neurohormonal stimulation. The guideline-directed medical therapy for Heart failure (HF) includes Angiotensin receptor-neprilysin inhibitor (ARNI) (sacubitril/valsartan), ß-blockers, sodium-glucose co-transporter 2 (SGLT2) inhibitors, and mineralocorticoid receptor antagonists (MRA). ARNI is under-prescribed in India despite its attractive safety and efficacy profile. Therefore, the consensus discusses objectives and topics related to ARNI in the management of cardiac remodeling, and experts shared their views on the early timely intervention of effective dosage of ARNI to improve the diagnosis and enhance mortality and morbidity benefits in cardiac reverse remodeling (CRR).

The Power and Promise of Angiotensin Receptor Neprilysin Inhibitor (ARNI) in Heart Failure Management: National Consensus Statement.

;Heart failure (HF) is a huge global public health task due to morbidity, mortality, disturbed quality of life, and major economic burden. It is an area of active research and newer treatment strategies are evolving. Recently angiotensin receptor-neprilysin inhibitor (ARNI), a class of drugs (the first agent in this class, Sacubitril-Valsartan), reduces cardiovascular mortality and morbidity in chronic HF patients with reduced left ventricular ejection fraction (LVEF). Positive therapeutic effects have led to a decrease in cardiovascular mortality and HF hospitalizations (HFH), with a favorable safety profile, and have been documented in several clinical studies with an unquestionable survival benefit with ARNI, Sacubitril-Valsartan. This consensus statement of the Indian group of experts in cardiology, nephrology, and diabetes provides a comprehensive review of the power and promise of ARNI in HF management and an evidence-based appraisal of the use of ARNI as an essential treatment strategy for HF patients in clinical practice. Consensus in this review favors an early utility of Sacubitril-Valsartan in patients with HF with reduced EF (HFrEF), regardless of the previous therapy being given. A lower rate of hospitalizations for HF with Sacubitril-Valsartan in HF patients with preserved EF who are phenotypically heterogeneous suggests possible benefits of ARNI in patients having 40-50% of LVEF, frequent subtle systolic dysfunction, and higher hospitalization risk.

Role of Bisoprolol in Heart Failure Management: A Consensus Statement from India.

In India, heart failure (HF) is an important health concern affecting younger age groups than the western population. A limited number of Indian patients receive guideline-directed medical therapy (GDMT). Selective ß-1 blockers (BB) are one of the GDMTs in HF and play an important role by decreasing the sympathetic overdrive. The BB reduces heart rate (HR) reverse the adverse cardiac (both ventricular and atrial), vascular, and renovascular remodeling seen in HF. Bisoprolol, a ß-1 blocker, has several advantages and can be used across a wide spectrum of HF presentations and in patients with HF and comorbid conditions such as coronary artery disease (CAD), atrial fibrillation (AF), post-myocardial infarction (MI), uncontrolled diabetes, uncontrolled hypertension, and renal impairment. Despite its advantages, bisoprolol is not optimally utilized for managing HF in India. This consensus builds on updated evidence on the efficacy and safety of bisoprolol in HF and recommends its place in therapy with a focus on Indian patients with HF.

Cranioplasty with hydroxyapatite or acrylic is associated with a reduced risk of all-cause and infection-associated explantation.

OBJECTIVE: Cranioplasty remains an essential procedure following craniectomy but is associated with high morbidity. We investigated factors associated with outcomes following first alloplastic cranioplasty. METHODS: A single-centre, retrospective cohort study of patients undergoing first alloplastic cranioplasty at a tertiary neuroscience centre (01 March 2010-01 September 2021). Patient demographics and craniectomy/cranioplasty details were extracted. Primary outcome was all-cause explantation. Secondary outcomes were explantation secondary to infection, surgical morbidity and mortality. Multivariable analysis was performed using Cox proportional hazards regression or binary logistic regression. RESULTS: Included were 287 patients with a mean age of 42.9 years [SD = 15.4] at time of cranioplasty. The most common indication for craniectomy was traumatic brain injury (32.1%, n = 92). Cranioplasty materials included titanium plate (23.3%, n = 67), hydroxyapatite (22.3%, n = 64), acrylic (20.6%, n = 59), titanium mesh (19.2%, n = 55), hand-moulded PMMA cement (9.1%, n = 26) and PEEK (5.6%, n = 16). Median follow-up time after cranioplasty was 86.5 months (IQR 44.6-111.3). All-cause explantation was 12.2% (n = 35). Eighty-three patients (28.9%) had surgical morbidity. In multivariable analysis, the risk of all-cause explantation and explantation due to infection was reduced with the use of both hydroxyapatite (HR 0.22 [95% CI 0.07-0.71], p = .011, HR 0.22 [95% CI 0.05-0.93], p = .040) and acrylic (HR 0.20 [95% CI 0.06-0.73], p = .015, HR 0.24 [95% CI 0.06-0.97], p = .045), respectively. In addition, risk of explantation due to infection was increased when time to cranioplasty was between three and six months (HR 6.38 [95% CI 1.35-30.19], p = .020). Mean age at cranioplasty (HR 1.47 [95% CI 1.03-2.11], p = .034), titanium mesh (HR 5.36 [95% CI 1.88-15.24], p = .002), and use of a drain (HR 3.37 [95% CI 1.51-7.51], p = .003) increased risk of mortality. CONCLUSIONS: Morbidity is high following cranioplasty, with over a tenth requiring explantation. Hydroxyapatite and acrylic were associated with reduced risk of all-cause explantation and explantation due to infection. Cranioplasty insertion at three to six months was associated with increased risk of explantation due to infection.

Common carotid artery blood flow volume in extremely preterm infants.

AIM: The aim of this study was to determine carotid blood flow volume, a surrogate for cerebral blood flow, using Doppler ultrasound in extremely preterm infants. METHODS: In infants <29 weeks, right common carotid artery flow volume (RCCAF) was calculated from vessel diameter and intensity-weighted mean velocity measured using Doppler ultrasound on days 1 and 3. In addition, left ventricular output (LVO), ductus arteriosus characteristics and invasive mean arterial blood pressure (MABP) were obtained. RESULTS: Sixty infants with mean gestation of 25.8 weeks were studied. The median RCCAF increased from 12 (IQR 9-15) mL/kg/min on day 1, to 14 (IQR 12-18) mL/kg/min on day 3 (p = 0.007). RCCAF was positively correlated with invasive MABP on days 1 and 3. RCCAF significantly correlated with LVO in infants with closing or closed ductus arteriosus on day 1. Using multiple regression analysis, RCCAF was significantly associated with invasive MABP on day 1 and to inotropic treatment on day 3. CONCLUSION: Doppler ultrasound can be used to measure RCCAF in extremely preterm infants receiving intensive care. RCCAF increased during the first three days and was positively related to invasive MABP on day 1. Values were lower than previously described in more mature infants. CLINICAL TRIAL REGISTRATION: ISRCTN 83507686.

Sudden death associated with the use of suction drains - a report of 2 cases following uneventful cranioplasty and literature review. Is the use of suction drains safe?

BACKGROUND AND IMPORTANCE: The use of drains, including suction drains in neurosurgery is individual preference-based, rather than scientific evidence-based. Furthermore, the use of suction drains has been associated with significant risks to patients, including sudden death. CLINICAL PRESENTATION: We present 2 cases of unfortunate sudden deaths following uneventful cranioplasty procedures, both of which were associated with the use of a suction drain. We also review the literature focusing on the benefits and risks in the use of suction drains, and discuss pathophysiological mechanisms underlying sudden death associated with their use. CONCLUSION: There is no substantial evidence to support the use of suction drains in neurosurgery. Furthermore, they have been associated with significant complications, including risk to life. Our experience and literature review suggest that the risk of sudden death is disproportionately higher following cranioplasty. We do not recommend the use of suction drains in cranial neurosurgery, and we strongly recommend against their use in cranioplasty procedures.

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.