Craniopharyngiomas remain tumours associated with significant morbidity. Gross total removal provides favourable results in terms of recurrences. If this cannot be achieved safely, adjuvant radiotherapy is beneficial in preventing tumour re-growth. Childhood- and adult-onset lesions generally behave similarly.

Objective-To determine the efficacy of oral nimodipine in reducing cerebral infarction and poor outcomes (death and severe disability) after subarachnoid haemorrhage.Design-Double blind, placebo controlled, randomised trial with three months of follow up and intention to treat analysis. To have an 80% chance with a significance level of 0-05 of detecting a 50% reduction in an incidence of cerebral infarction of 15% a minimum of 540 patients was required.Setting-Four regional neurosurgical units in the United Kingdom.Patients-In all 554 patients were recruited between June 1985 and September 1987 out of a population of 1115 patients admitted with subarachnoid haemorrhage proved by the results of lumbar puncture or computed tomography, or both. The main exclusion criterion was admission to the neurosurgical units more than 96 hours after subarachnoid haemorrhage. There were four breaks of code and no exclusions after entry. One patient was withdrawn and in 130 treatment was discontinued early. All patients were followed up for three months and were included in the analysis, except the patient who had been withdrawn.Interventions-Placebo or nimodipine 60 mg was given orally every four hours for 21 days to 276 and 278 patients, respectively. Treatment was started within 96 hours after subarachnoid haemorrhage.End points -Incidence of cerebral infarction and ischaemic neurological deficits and outcome three months after entry.Measurements-Demographic and clinical data, including age, sex, history of hypertension and subarachnoid haemorrhage, severity ofhaemorrhage according to an adaptation of the Glasgow coma scale, number and site of aneurysms on angiography, and initial findings on computed tomography were measured at entry. Deterioration, defined as development of a focal sign or fall of more than one point on the Glasgow coma scale for more than six hours, was investigated by using clinical criteria and by computed tomography, by lumbar puncture, or at necropsy when appropriate. All episodes of deterioration and all patients with a three month outcome other than a good recovery were assessed by a review committee.Main results-Demographic and clinical data at entry were similar in the two groups. In patients given nimodipine the incidence of cerebral infarction was 22% (61/278) compared with 33% (92/276) in those given placebo, a significant reduction of 34% (95% confidence interval 13 to 50%). Poor outcomes were also significantly reduced by 40% (95% confidence interval 20 to 55%) with nimodipine (20%

OBJECTIVE We describe the first cohort-based analysis of the impact of genetic disorders in craniosynostosis. We aimed to refine understanding of prognosis and pathogenesis, and to provide rational criteria for clinical genetic testing. METHODS We undertook targeted molecular genetic and cytogenetic testing of 326 children requiring surgery for craniosynostosis, born in the years 1993-2002, presenting to a single craniofacial unit, and followed up until the end of 2007. RESULTS Eighty-four children (and 64 relatives) had a pathological genetic alteration, comprised of 86% single gene mutations and 14% chromosome abnormalities. The FGFR3 P250R mutation was the single largest contributor to the genetic cases (24%). Genetic diagnoses accounted for 21% of all craniosynostosis and were associated with increased rates of many complications. Children with an initial clinical diagnosis of non-syndromic craniosynostosis were more likely to have a causative mutation if the synostosis was unicoronal or bicoronal (10 of 48 cases) than if it was sagittal or metopic (none of 55; P=0.0003). Repeat craniofacial surgery was required in 58% of children with single gene mutations but only 17% of those with chromosome abnormalities (P=0.01). CONCLUSIONS Clinical genetic assessment is critical for the management of children with craniosynostosis. Genetic testing of non-syndromic patients (as a minimum, for FGFR3 P250R and FGFR2 exons IIIa/c), should be targeted to those with coronal or multisuture synostosis. Single gene disorders that disrupt physiological signalling in the cranial sutures often require re-operation, whereas chromosome abnormalities follow a more indolent course suggesting a different, secondary origin of the associated craniosynostosis.

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Assessment of intracranial pressure (ICP) is essential in the management of acute intracranial catastrophe to limit or actively reduce ICP. This article provides background information and reviews the current literature on methods of measuring ICP in children. Indications for ICP measurement are described for children with traumatic brain injury, shunt insertion or malfunction, arachnoid cyst, craniosynostosis, and prematurity. Various methods of ICP monitoring are detailed: non-invasive, indirect (lumbar puncture, visualevoked potentials, fontanelle compression, and optic nerve sheath), and direct assessment (ventricular cannulation, and epidural, subdural, and intraparenchymal devices). Normal levels of ICP will depend on the age and position of the child during monitoring. This article provides clinical and research-based evidence in this area where there is currently limited guidance.One of the frequent questions about neurological critical care of children relates to indications and methods for measurement of intracranial pressure (ICP) and the appropriate therapy for raised ICP. 1 Literature on methods of measuring ICP in children is relatively rare and there is no clear guidance on when and where to measure ICP in routine clinical practice. Advances in monitoring technology and new microsensor tips of minimally invasive catheters have improved the ease, safety, and accuracy of ICP measurement, allowing more widespread use in emergency and elective situations.Interpreting results of ICP measurement should remain in the hands of experienced clinicians and must never stand for themselves when taking into account clinical history and clinical symptoms. This review is targeted to give a clinically orientated overview of daily practice of ICP monitoring in a variety of situations. 2,3 Historical background The most simple and longest-standing method of measuring ICP is to perform a lumbar puncture and to observe the opening and closing pressure. This indirect and, at the same time, imprecise procedure is still commonly used. However, as a technique it has significant disadvantages and inaccuracies.Recognition of these problems led Lundberg et al. to develop intraventricular pressure monitoring in the 1960s. 4-6 Lundberg et al. also described the various wave patterns associated with intracranial pathology. 4-6 Ventricular catheterization 2-3 remains the criterion standard for ICP measurement today, but it was recognized early on that there were problems, especially with infection, which limited the length of usage of such monitoring, and there were often technical difficulties with cannulating a compressed or deviated ventricle in situations where ICP was raised.In an attempt to overcome the difficulties associated with ventricular ICP measurement, a variety of extra-ventricular devices were developed during the next 20 years, including subdural devices based on saline-filled transducers (e.g.

Background-Saethre-Chotzen syndrome (SCS) is a syndromic craniosynostosis defined by a genetic mutation affecting the TWIST1 gene on chromosome 7p21. SCS is typically associated

Object The presence of raised intracranial pressure (ICP) in untreated nonsyndromic, isolated sagittal craniosynostosis (SC) is an important functional indication for surgery. Methods A retrospective review was performed of all 284 patients presenting with SC to the Oxford Craniofacial Unit between 1995 and 2010. Results Intraparenchymal ICP monitoring was performed in 39 children following a standard unit protocol. Monitoring of ICP was offered for all patients in whom nonoperative management was considered on the basis of minimal deformity or in cases in which parents were reluctant to agree to corrective surgery. These patients presented at an older age than the rest of the cohort (mean age 56 months), with marked scaphocephaly (16/39, 41%), mild scaphocephaly (11, 28%), or no scaphocephalic deformity (12, 31%). Raised ICP was found in 17 (44%) patients, with no significant difference in its incidence among the 3 different deformity types. Raised ICP was not predicted by the presence of symptoms of ICP or developmental delay or by ophthalmological or radiological findings. Conclusions The incidence of raised ICP in SC reported here is greater than that previously published in the literature. The lack of a reliable noninvasive method to identify individuals with elevated ICP in SC mandates consideration of intraparenchymal ICP monitoring in all patients for whom nonoperative management is contemplated.

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