Internal neurolysis: 'nerve combing' for trigeminal neuralgia without neurovascular conflict - early UK outcomes.

INTRODUCTION: Internal neurolysis (INL) is a surgical procedure where trigeminal nerve fibres are separated between the pons and porus trigeminus to relieve trigeminal neuralgia (TN). We report pain and functional outcomes to evaluate its safety and efficacy. MATERIALS AND METHODS: Prospective cohort of all patients undergoing retrosigmoid craniotomy and INL between 2015 and 2017 at University Hospital Southampton. Patients with type I (6) or type II (2) refractory TN and no clear neurovascular conflict were offered INL as an alternative to partial sensory rhizotomy. Barrow Pain Intensity Scale (BNI) and Brief Pain Inventory Facial scores (BPI-Facial) were assessed. Minimum follow-up was 2 years'. RESULTS: Eight patients (7F:1M) underwent INL. Two had MS. Pre-operatively, all had severe pain (BNI grade V) and the median BPI-Facial score was 115 (range 79-123).. There were no unexpected complications. On last follow-up, six (75%) had no pain (BNI grade I), while two (25%) had recurred (at 5 and 27 months). Median BPI-Facial score for all patients on the last follow-up was 20 (range 18-91) reflecting dramatically improved quality of life and activities. CONCLUSIONS: INL is a potentially safe and effective treatment for refractory TN. Long-term efficacy is unknown, but early results are promising.

Ruptured intra-cranial arachnoid cysts: a case series from a single UK institution.

INTRODUCTION: Intracranial arachnoid cysts are a common incidental finding; however presentation with cyst rupture is a recognised complication. Patients are advised to avoid contact sports to reduce the risk of cyst rupture but the risk of rupture is not currently known. The aim of this study is to describe a single institution's experience of managing ruptured intra-cranial arachnoid cysts. METHOD: A retrospective case note review of all patients admitted to a single institution with a ruptured intra-cranial arachnoid cyst between 2005 and 2016 (inclusive). Medical records were reviewed for demographics, history of trauma, surgical treatment and radiological evidence of cyst rupture. RESULTS: Fourteen patients were identified for inclusion with an average age of 23.4 years (range 7-57) and 10 (71%) were male. Nine patients (64%) had a documented history of head trauma. Eleven patients (78.6%) required neurosurgical intervention which included hygroma/haematoma evacuation only (n = 5), haematoma evacuation with cyst fenestration (n = 4) and cyst fenestration/shunting only (n = 2). Twelve patients (85%) experienced full recovery and the remaining two had persisting headaches or neurological symptoms. DISCUSSION: Cyst rupture remains an uncommon cause for presentation with arachnoid cysts. However the majority are associated with head trauma and thus current advice to avoid contact sports seems justified.

Prenatal diagnosis of arachnoid cysts: a case series and systematic review.

INTRODUCTION: Primary cysts are believed to arise from the splitting of the arachnoid membrane during prenatal development and can be diagnosed in utero. Prenatal diagnosis is uncommon; therefore, the evidence base for counselling expectant mothers is limited. The purpose of this article is to present a case series and review the current literature on prenatally diagnosed arachnoid cysts. METHOD: A keyword search of hospital electronic records was performed for all patients with a prenatally diagnosed arachnoid cyst at a tertiary neurosurgical centre. Case notes were reviewed for all patients diagnosed between 2005 and 2017. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to structure a systematic review of all English language articles published up to May 2018. RESULTS: A total of eight eligible patients were identified from our own records and 123 from the literature. Sixty-eight per cent of patients had a normal outcome. Sixty-three per cent of patients underwent surgical intervention which was not associated with abnormal outcome. The diagnosis of syndromic/genetic diagnosis (p < 0.001) and the presence of other intra-cranial anatomical abnormalities (p = 0.05) were significant predictors of abnormal outcome. CONCLUSION: The pathogenesis and prognosis of a prenatal arachnoid cyst diagnosis remain unclear. These results suggest favourable outcomes from simple cysts without associated abnormalities and expectant mothers should be counselled accordingly. A wider prospective review is required to better established evidence-based practice.

Posterior reversible encephalopathy syndrome: a case following reversible cerebral vasoconstriction syndrome masquerading as subarachnoid haemorrhage.

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic clinico-radiological diagnosis typically presenting with headache, encephalopathy and visual disturbance accompanied by a unique neuroradiological pattern of symmetrical parieto-occipital vasogenic oedema. Here we present the case of a 51-year-old woman who presented to hospital following a thunderclap headache, initially thought to be secondary to a subarachnoid haemorrhage (SAH). A tiny anterior choroidal artery aneurysm was demonstrated on cerebral angiogram. At surgical clipping, no evidence of haemorrhage was observed. Post-operatively, the patient developed delayed right-sided hemiparesis, managed with aggressive hypertensive treatment, and later, with onset of septicaemia, central visual loss. Computed tomography (CT) brain scans demonstrated oedematous changes within the parieto-occipital regions bilaterally and later areas of infarction. The initial diagnosis of SAH was revised to reversible cerebral vasoconstriction syndrome (RCVS), which gave rise to PRES. To our knowledge, this is the first reported case of RCVS with concomitant PRES and cerebral infarction.

Use of intrathecal urokinase in repeated shunt and external ventricular drain blockage from high CSF protein due to an optic pathway glioma.

INTRODUCTION: High cerebrospinal fluid (CSF) protein is a recognised association of optic pathway gliomas. This can occlude ventriculoperitoneal (VP) shunt catheters or external ventricular drains (EVD). CASE REPORT: We describe an 8-year-old boy with an optic pathway glioma, who had frequent episodes of a blocked VP shunt and EVD due to high CSF protein level. This responded favourably to repeated urokinase instillation into the catheter lumen. We present the course of treatment and the method of administration and review the literature behind thrombolytic therapy for occluded catheter devices. CONCLUSION: Intrathecal urokinase was effective in unblocking the EVD and lysing the protein clots within the ventricle. We found no previously published cases of intrathecal urokinase for this problem. This novel intervention seemed promising as a safe and effective means of maintaining EVD patency in cases complicated by excessive protein loads in CSF and hydrocephalus.

Degree and Duration of Functional Improvement on Long-Term Follow-Up of Spinal Dural Arteriovenous Fistulae Occluded by Endovascular and Surgical Treatment.

BACKGROUND: Long-term outcomes following occlusion of spinal dural arteriovenous fistula (SDAVF) are poorly understood and are based on small series reporting predominantly short-term outcomes. The degree and duration of improvement remain unclear. In this study, we analyzed long-term outcomes following occlusion in a cohort of patients with SDAVF. METHODS: This was a single-center cohort study of patients with SDAVF identified from a prospective database. Outcomes were assessed using a modified Aminoff and Logue Disability (ALD) Scale, the modified Rankin Scale (mRS), and patient-reported outcomes at presentation and long-term follow-up. Both angiographic recanalization and idiopathic functional recurrence rates were calculated. RESULTS: Of the total of 67 patients with SAVDF identified, 59 were eligible for inclusion in this study. Fifty-seven of these 59 (97%) underwent occlusion. Twenty-two patients underwent initial embolization; 12 were occluded. Two patients recanalized. Eleven patients underwent surgery after attempted embolization; 10 were occluded. Thirty-seven patients treated by surgery only were occluded. The median duration of follow-up was 63 months (range, 12-240 months). After occlusion, the ALD gait and urinary scores improved by a median of 1 point. Although the median mRS score was unchanged, 49% of the patients experienced improvement, most by 1 point. There was no difference between the patients occluded by embolization or surgery, but those requiring both approaches had worse gait and urinary scores (P = 0.005 and 0.03, respectively). The duration of symptoms by itself had no effect on outcomes (P = 0.61). Following occlusion, 5 patients experienced an idiopathic late functional deterioration. Of 16 patients presenting with paraplegia, 13 (81%) improved, with a median mRS improvement of 1 point. CONCLUSIONS: Following occlusion, patients with SDAVF experienced a modest improvement in symptoms, most commonly by 1 point on the ALD scale. Idiopathic late deterioration was seen in 9.1% of patients.

Endoscopic biopsy and third ventriculostomy for the management of pineal region tumors.

OBJECTIVE: To assess the histologic accuracy of endoscopic biopsy samples of the pineal region. Pineal region tumors usually present with acute hydrocephalus. Histologic diagnosis is paramount, as it greatly influences treatment. Endoscopic techniques can combine histologic diagnosis with relief of the obstructive hydrocephalus in a single operation. Because pineal region tumors can be heterogeneous, initial biopsy samples may not represent the most aggressive portion of the tumor. METHODS: This retrospective study reviews our experience of endoscopic third ventriculostomy combined with biopsy of the lesion. The histologic diagnosis as a result of the initial biopsy was compared with the final histologic diagnosis to establish the accuracy of the endoscopic biopsy sample in aiding diagnosis. RESULTS: Forty-seven patients underwent an endoscopic third ventriculostomy. All but 1 patient underwent a concurrent biopsy of the space-occupying lesion and 39 of 46 patients (85%) had a histologic diagnoses. In the remaining 7 patients (15%), the histology was negative; in 6 cases, the second attempt to obtain a histologic diagnosis was successful (2 repeat endoscopic biopsy samples, 2 resections, 2 stereotactic biopsy samples). In 1 patient a presumed low-grade tectal tumor was followed up with sequential scanning. Twenty-eight patients underwent subsequent operations (24 resections, 4 stereotactic biopsies). In 6 of 28 patients (21%), the histologic report was amended after the second procedure. CONCLUSIONS: The endoscopic biopsy sample yields an accurate histologic diagnosis for most pineal region tumors, with a positive histologic sample in about 85% of patients. However, the results must be interpreted cautiously, as the heterogeneous nature of these tumors may lead to an approximately 21% error rate in the initial tumor diagnosis.

The natural history of cranial dural arteriovenous fistulae with cortical venous reflux--the significance of venous ectasia.

BACKGROUND: The quoted risk of hemorrhage from dural arteriovenous fistulae with cortical venous reflux varies widely, and the influence of angiographic grade on clinical course has not previously been reported. OBJECTIVE: To assess the risk of hemorrhage and the influence of angiographic grade on this risk, compared with known predictors of hemorrhage such as presentation. METHODS: Seventy-five fistulae with cortical venous reflux identified in our arteriovenous malformations clinic between 1992 and 2007 were followed up clinically, and their angiograms were reviewed. RESULTS: There were 8 hemorrhages in 90 years of follow-up. The annual incidence of hemorrhage before any treatment was 13%, and 4.7% after partial treatment, giving an overall incidence of 8.9% before definitive treatment. Borden and Cognard grades were poor discriminators of risk for lesions with the exception of Cognard type IV lesions. These lesions, characterized by venous ectasia, had a 7-fold increase in the incidence of hemorrhage (3.5% no ectasia vs 27% with ectasia). Patients presenting with hemorrhage (20%) or nonhemorrhagic neurological deficit (22%) had a higher incidence of hemorrhage than those with a benign presentation (4.3%), but this may be directly linked to the presence of venous ectasia. CONCLUSION: In this series untreated dural arteriovenous fistulae with cortical venous reflux had a 13% annual incidence of hemorrhage after diagnosis. There was a significant difference between those with and without venous ectasia. This should be confirmed by further studies, but probably defines a high-risk subgroup of patients that requires rapid intervention.

The SILVER (Silver Impregnated Line Versus EVD Randomized trial): a double-blind, prospective, randomized, controlled trial of an intervention to reduce the rate of external ventricular drain infection.

BACKGROUND: Cerebrospinal fluid (CSF) infections associated with external ventricular drain (EVD) placement attract major consequences. Silver impregnation of catheters attempts to reduce infection. OBJECTIVE: To assess the efficacy of silver catheters against CSF infection. METHODS: We performed a randomized, controlled trial involving 2 neurosurgical centers (June 2005 to September 2009). A total of 356 patients requiring an EVD were assessed for eligibility; 325 patients were enrolled and randomized (167 plain, 158 silver); 278 patients were analyzed (140 plain, 138 silver). The primary outcome measure was CSF infection as defined by organisms seen on Gram stain or isolated by culture. Secondary outcome measures included ventriculoperitoneal (VP) shunting. RESULTS: There was a significant difference in infection risk between the 2 study arms: 21.4% (30/140) for plain catheters vs 12.3% (17/138) for silver catheters (P = .0427; 95% confidence interval [CI]: 1.015-3.713). Patients who had an EVD infection had more than double the risk of requiring a VP shunt compared with patients without an EVD infection (45.7% [21/46] vs 19.7% [45/229], respectively, P = .0002; 95% CI: 1.766-6.682). There was also a significant difference in VP shunt risk with infection: plain (55.2%; 16/29) vs the silver arm (29.4%; 5/17); P = .0244 (95% CI: 1.144-11.695). A multivariate analysis demonstrated that infection risk was increased by duration of EVD placement (odds ratio: 1.160), spontaneous intracranial hemorrhage (odds ratio 4.958) and decreased by silver catheters (odds ratio: 0.423). CONCLUSION: The study provides Class I evidence that silver-impregnated catheters reduce CSF infection.

Pathological laughter and crying in patients with pontine lesions.

Pathological laughter and crying (PLC) has been widely documented in the medical literature in association with various pathological processes in the brainstem, particularly infarction. However, it remains poorly understood. The authors present a case report and analyze all the cases in the literature to try to localize a putative faciorespiratory center. This 13-year-old girl developed a pontine abscess subsequent to sphenoid sinusitis. This increased in size despite antibiotic treatment, and she developed PLC. The abscess was then stereotactically aspirated, with resolution of the symptoms. A PubMed search of the term "pathological laughter and crying" was performed. From these papers all reported cases of PLC were identified. Cases without neuroimaging were excluded. The remaining cases were categorized as small lesions permitting accurate localization within the pons, or large nonlocalizing lesions. All images of localizing lesions were magnified to the same size and placed on a grid. From this an area of maximal overlap was identified. The authors identified 7 cases of small localizing lesions with adequate imaging. The area of maximal overlap was in the region of the anterior paramedian pons. All the lesions involved this region of the pons. There were 28 further reports of large lesions that either resulted in gross compressive distortion of the pons or diffusely infiltrated it, and thus, although implicating involvement of a pontine center, did not allow for localization of a specific region of the pons. The authors report a case of PLC caused by a pontine abscess. Symptoms were reversible with stereotactically assisted aspiration and antibiotic administration. Analysis of the lesions reported in the literature showed a pattern toward a regulatory center in the pons. The most consistently involved region was in the anterior paramedian pons, and this may be the site of a faciorespiratory center.

Endoscopic third ventriculostomy in the treatment of childhood hydrocephalus: validation of a success score that predicts long-term outcome.

OBJECT: The goal of this study was to externally validate the proposed Endoscopic Third Ventriculostomy Success Score (ETVSS), which predicts successful treatment for hydrocephalus on the basis of a child's individual characteristics. METHODS: The authors retrospectively identified 181 cases of consecutive endoscopic third ventriculostomy (ETV) performed in children at a single neurosurgery center in the United Kingdom. They compared actual success at both 6 and 36 months, with mean predicted probabilities for low, moderate, and high chance of success strata based on the ETVSS. Long-term success was calculated using Kaplan-Meier methods and comparisons were made by means of unpaired t-tests. RESULTS: Overall, 166 primary ETVs were performed; ETV success was 72.9% at 6 and 64.5% at 36 months. At long-term follow-up, the mean predicted probability of success was significantly higher in those with a successful ETV (99 patients) than in those with a failed ETV (67 patients) (p = 0.001). The ETVSS accurately predicted outcome at 36 months; the low, medium, and high chance of success strata had mean predicted probabilities of success of 82%, 63%, and 36%, and actual success of 76%, 66%, and 42%, respectively. The overall complication rate was 6%. CONCLUSIONS: The ETVSS closely predicted the overall long-term success rates in high-, moderate-, and low-risk groups. The results of this study suggest that the ETVSS will aid clinical decision making in predicting outcome of ETV.